EDITOR IN CHIEFEDITORASSOCIATE EDITORS

ASSISTANT EDITOREDITORIAL BOARD

VOl 1 NO 1 APRIL - JUNE 1996 QUARTERLY

JOURNAL OFSURGERYPAKISTAN

F.U.BaqaiAbdul AzizAsaduflah KhanAbdul Moead KaziJamshed AkhtarAzhar HusainIrshad WaheedMohammad ShamimMasooclJavaidSaghir AhmedS.A.JilaniRashid Ahmad ChoudhryZahida Baqal

EDITORIAL CONSULTAlns

AUSTRALIABANGLADESH

EGYPTINDIA

JORDANNEPAL

REPUBLIC OFMALDIVES

SRI LAlKAU.K.

U.A.E.PAKISTAN

Earl R.OwenAhsanullah ChowdhuryM.Kabiruddin AhmedHumayunKabirChoudhuryRaffat KamelTehemtonE.UdwadiaAhmed Abdul HaiIbrahim BaniSaniI.B. ThappaA.K.Sharma

Imteyaz MohsinA.P.R. AluwihareJohn HadfieldJ.S.P. LumleyEssa KazimAbdullah Jan JaffarAdib ul Hasan RizviChangez Hakim KhanFaiz Mohammad KhanGhulam Ali MemonIjazAqeelInamal Haq ShamiJan MohammaedMemon ..Muhammad Iqbal·Moizuddin·M.Younis Khatri .M.NaeemA. khan ':. Nazir M.KBZi .Shah Nawaz ShahSikander ShaikhSyed Azhar AhmedTariq Saeed MuftiTipu SultanZ.K.KaziZafarullah Chaudhry

Published by: Dr.AbdulAzlz forProf.F.U.Baqai, Baqai PostgraduateMedical Institute, IIIC,1/12, NazimabBd,KarachiAddress for correspondence: CoL. '(Retd.) Masudul Haque,ExecutiveSecretary, Surgical Publications,BaqaiPostgraduate Medical Institute, IIIC.1/12,Nazimabad, karachi, Tel & Fax;S887333Subscribeption rates: Per copy-inPakistan RS.100/",in SAARC countriesU.S. $20, in other countries U.S.$30,Annual- in Pakistan Rs.300!= in SARRCcountries US $ 60, in other countriesU.S.$90.Layout by: Aleemuddin Siddqiui.

EDITOR'S NOTE

1ORIGINAL ARTICLES

II

2v1------------------------------------------------------------

6

JJJCf{ A ten years study of lymphangiomatousmalformation at NIGH

Pattern of malignancy in children

Normal appendices in patients operatedfor suspected acute appendicitis

Jamshed Akhtar, Farhat Mirza,Soofia Ahmed, Asadullah Khanand Abdul Aziz

Jamshed Akhtar and Abdul Aziz

Iatrogenic gastrointestinal perforations

Shiasta Khan, Farhat Abbas andHizbullah Shaikh

Nee/am Javid, Lubna Ishaq Bhatti,Jawad Sher, Abid Qazi, Iftikhar A.Jan and Naeem-uz-Zafar Khan

Role of fine needle aspiration biopsy andcytology (FNABG) in a cold noduleof thyroid and its comparison withhistopathology

Shahida Afridi, Asadullah Khan,S.Waqar Ahmed and Irshad Waheed

Benign oro-facial tumours Nasreen Amanat

A Study of cases of large intra-abdominalIymphangiomas- A review of literature

Imtiaz Lewis Qureshi, Jaffer-uz-Zaman,Ahmed Sultan Khan, Abid Qazi IftikharJan, Naeem-uz-Zafar Khan 26

Sacrococcygeal teratoma presenting as abenign lesion in older age group

Jaffer-uz-Zaman, Imtiaz Lewis Qureshi,Ahmed Sultan Khan andNaeem-uz-Zafar Khan 30

Minimally invasive surgery

, REVIEW ARTICLE

Irshad Waheed, Kalsoom Junejo 34

Liposarcoma of clavicle Jamshed Akhtar, Abdul Aziz andFarhat Mirza 36

Abdul Sattar Memon, Jan MohammadMemon and Abdul Ghani Soomro 37

Tuberculosis of penis

Phocomelia Imtiaz Lewis Qureshi, Uzma FarheenHashim, Zia-ul-Haque and Naeem-uz-Zafar Khan. 39

9

13

18

22

Remarkable changes have taken place in the health delivery systems in the 20th Century and we expect to livemuch longer and healthier than our grand parents. Yet despite this progress, scores of health problems remainto be solved. Efforts are being made all over the world to find answers to these unsolved problems by carrying

out research for the benefit of the ailing millions. The fruits of this research and experiences are being shared byresearchers through medical journals and publications. A large number of journal are published all over the world andscores of new ones are added each year, yet no journal exists to share the wide experiences of researchers in theSAARC region, which housed almost one quarter of the world population with common medical problems and similardiseases and disease patterns.

An ocean of scientific and clinical material exists in the SAARC region and the Journal of Surgery Pakistan is ourattempt to tap this information. It has a wider scope as the SAARC Association of Surgeons (Pakistan) has beenjoined by International College of Surgeons (Pakistan Chapter) in this Venture. The purpose of this publication is tobring together all the members of theses two societies on a common platform. As this journal is to be distributed to allsurgeons and Heads of Surgery in Pakistan, internationally through International College of Surgeons (Pakistan), aswell as the SAARC countries, it will serve to bring the surgeons of these societies and region closer and willencourage exchange of knowledge and useful interaction.

Editor

This first issue which is in your hands, contains articles on topics of great importance by Pakistani surgeons and wehope to have the benefit of the experiences and studies of surgeons in SAARC countries in our future issues. We alsoplan to introduce a "Letters to the Editor" portion where surgeons will be encouraged to express views on similarstudies and give their comments and observations on published articles and reviews.

We may add that a journal of this nature cannot be published and distributed without the active support andcollaboration of the pharmaceutical and other Industry business houses. It could provide a great opportunity to thepharmaceutical Industry to advertise their firm, services and products, besides promoting the cause of surgicalprofession. We would therefore welcome advertisements for our future issues.

We are grateful to the contributors to this first issue and the firms that advertised in it.

Journal of Surgery Pakistan Vol.1 (1); April-June 1996

A 10 YEARS STUDY OF LYMPHANGIOMATOUSMALFORMAITON AT N.I.C.H.

JAMSHED AKHTAR, FARHAT MIRZA, SOOFIA AHMED, ASADULLAH KHAN, ABDUL AZIZ.

ABSTRACT

Between 1st January. 1984 and 31st December 1993. 126 cases oj the Lymphangiomatousmalformation were managed at National Institute oj Child Health. Karachi. Most oj thepatients were under 6 months oj age. Male domination was noted. Six neonates presentedin emergency with respiratory distress. Cervico-facial region was involved in 61.9% oJ thecases. Surgery was treatment oj choice. Over all results were satisfactoru. Therewere only two deaths in this series which were in non operated cases.

KEY WORDS: Cystic hygroma. Lymphangioma.

INTRODUCTIONLymphangiomatous malformation is the congenitalmaldevelopment of the lymphatic system. It may takethe shape of cysts of variable sizes when it is knownas cystic hygroma. (Fig 1) The capillary and cav-ernous types of lesions are termed as lymphan-giomas. Many modes of treatment have beendescribed in literature with variable results. We pre-sent our experience of this malformation over a peri-od of ten years.

Figure 1 Massive Cystic Hygroma of Head and Neck Region

MATERIALS AND METHODSDepartment of Pediatric Surgery at N ICH providesservices notonly to people of Karachi but also to therest of Sindh and lower Baluchistan. Few cases are

Dr. Abdul Aziz,Dept of Paedriatic Surgery,National Institute of Child Health, Karachi

referred from up country as well. 120 cases wereadmitted as elective cases and 6 cases were admit-ted in emergency due to variable degree of respi-ratory distress, Elective cases underwent routineinvestigations like complete blood count and X-raychest, where indicated. Other investigations weredone according to the site of involvement. Whenjudged fit enough they were given appointment forsurgery. No case was operated in emergency, Inmajority of the cases total excision was carried out.Conservative surgery was done in 10 cases of hugecystic hygromas of the face and neck region withinfiltration of the deeper structures. Staged proce-dures were carried out in 4 cases-2 involved tongueand 2 involved trunk. Complications noted in post-operative period included infection, leakage of thelymph and recurrence, Two deaths were recorded inour series.

RESULTS126 cases were studied during 10 years from 1984to 1993. There were 78 (61,9%) male and 48female (38.1 %) patients. Age distribution is g\venin Table-I. Most of the cases were under sixmonths of age, In the regional distribution cervico-facial region was the most common part involved.Table II. Total excision was performed in 110cases (88.9%), partial excision was done in 10cases (8,0%) and staged procedure was per-formed in 4 cases, (3,1%). Post-operative compli-cations are given in Table III.

2Journal of Surgery Pakistan Vol.1 (1); April-June 1996

DR. ABDUL AZIZ

Table I

Age Distribution

Age Distribution No. of cases Percentage

Day 1 to 6 Months 42 33.3%

6 Months to 12 Months 14 11.2%

1 Year to 3 Years. 52 41.2%

5 Years and above 18 14.3%

Table II

Regional Distribution

Regional Distribution No. of cases Percentage

Cervical Region 68 53.9%

Face 10 8.0%

Tongue 2 1.6%

Chest Wall 12 9.6%

Abdominal Wall 4 3.2%

Upper Limb (including Axilla) 16 12.5%

Lower Limb 10 8.0%

Perineum 4 3.2%

Table III

Postoperative Complications

Early No of cases Percentage

Respiratory Distress 4 3.2%

Wound Infection 4 3.2%

Chest complications 6 4.8%

Transient Facial nerve paresis 2 1.6%

LATE

Contracture 2 1.6%

Facial nerve Paralysis 2 1.6%

Recurrence 8 6.4%

3

DISCUSSIONCystic hygroma and lymphangioma are benign, softcompressible malformations of the lymphatic system.Head and neck region is the most common site ofinvolvement'. These lesions usually appear after birthbut upto 40% can be seen in new born", Cullen et alhave reported a series of 30 cases of cervical cystichygroma diagnosed in the first trimester of preg-nancy. In the modern era of antenatal diagnosis withultrasonography, it is now possible to arrange forappropriate means to save the life in case of hugeinfiltrating cystic hygroma at the time of birthv. Thisaspect of management is still lacking in under-developed countries where lack of co-ordinationamong various specialities leads to high morbidityand mortality.

The number of cases reported from under-devel-oped countries is more than from developed coun-tries. Ricciardelli et al has presented a series of 34cases in 10 years", In 10 years period we have stud-ied 126 cases and a similar number of cases havebeen presented by Ninh and Ninh", The reason forthis difference is population which is much more inunder-developed countries like ours with largedrainage area and because of paucity of health ser-vices. Another point to be emphasized is the useof diagnostic techniques like ante-natal ultrasono-graphic detection of these cases. Complications likemiscarriage in first trimester have been noted byTurnpenny et al in fetuses affected by this malfor-mation'. Longo et al has described 5/1000 incidenceof abortion with cystic hygromas. Abortions on med-ical grounds with these malformations could beanother reason for the lesser number of cases fromwestern countries". Shulman et al have empha-sized an increased risk of fetal chromosomalabnormalities especially autosomal Trisomy'°.Tanaka et al have reported more than 20% mortalityin post-natal period following deliveries in suchcases". He advised cesarean section and in uteroendo-tracheal intubation of the neonate to save life.Tricoire et al have reported 8 cases of familialcystic hygroma in 3 tamilies". The cystic hygromahas also been noted in the siblings.

Miyabara et al have mentioned in their study aboutthe presence of cystic hygroma in twins with con-joined pregnancy". Cystic hygroma has been associ-ated with certain other abnormalities. Droste et alhave pointed out its association with Turner syn-drome". Achiron et al have described its associationwith congenital heart defects's. Macleod et al haveassociated this condition with hydrops fatafis". Bulaset al have reported a case of Fryn's syndrome pre-senting with cystic hyqrorna". Gorenstein et al

Journal of Surgery Pakistan Vol.1 (1); April-June 1996

I

10 years study of lymphangiomatous malformaiton at N.I.C.H

have described the association of venousaneurysm with cystic hygroma's.

Rare sites for cystic hygroma have been mentionedin the literature and have been noted by us as well,for example mediastinal cystic hygroma, a rare lesionreported by Muraskas et a1'9. Their lesion was in theanterior mediastinum causing airway obstruction,mediastinal shift and hypoplasia of the lung. We havenoted lesion in maxillary part of the cheek with exten-sion into retro-orbital and anterior intracranialfossa. Other rare sites mentioned in literatureinclude parotid region20 and bone" etc.

The diagnosis is usually made on history and physi-cal examination. Ultrasonography, C.T. scanning,M.R.1. and lymphangiography are useful in delineat-ing the extent of the lesion. Various modes oftreatment have been described in addition tosurgery. Dickerhoff et al have mentioned the useof chemotherapy, like cyclophosphamide in non-resectable cystic hyqromas=. Bleomycin and OK-432have been used by somo=>. Recurrence is a com-mon problem especially in massive lesions. Scallyhas reported recurrence in adults". In our studyrecurrence was noted in the case of tongue and trunkand in two cases involving cervico-facial region.

In conclusion, lymphangiomatous malformations arenot infrequent. If facilities are available and a cystichygroma is suspected in the ante-natal period, evenif of small size, detailed ultrasonographic examinationand karyotyping are recommended because of itsfrequent association with chromosomal defects.

Surgery, either total excision, or staged excision isthe treatment recommended. Recurrence is known inmassive lesions.

REFERENCES1. Singh, S., Baboo, ML, Pathak IC.: Cystic lym-

phangioma in children. Report of 32 casesincluding lesions at rare sites. Surg. 1971: 69,947-951.

2. Thompson OM., Kasperbauer JL. Congenitalcystic hygroma involving larynx, presentingas an airway emergency. J Natl Med Assoc.1994: 86, 629-32.

3. Langer JC., Fitzgerald PG., Desa D., et al.Cervical cystic hygroma in the fetus: Clinicalspectrum and outcome. J Pediatr Surg. 1990:21, 58-61.

4. Cullen MT., Gabrielli S., Green JJ. Diagnosis

and significance of cystic hygroma in the firsttrimester. Prenat Diagn.1990: 10,643-51.

5. Riccardelli EJ., Richardson MA. Cervicofacialcystic hygroma: patterns of recurrence andmanagement of difficult case. Arch OtolaryngolHead neck surg. 1991: 117, 546-53.

6. Ninh TN., Ninh TX. Cystic hygroma in chil-dren. A report of 1236 cases. J PediatrSurg.:1974,:9, 191-95.

7. Turnpenny PD., Gonasegaran R., Smith CN.,et al. Recurrent miscarriage, cystichygroma and incontinentia. Br J ObstetGynecol. 1992: 99, 920-1.

8. Longo M., Pecoraro G., Zarcone R., et al.Cystic hygroma : 2 case reports and review ofliterature. Minerva Ginecol.(ltalian). 1994: 46,28593.

9. Christodoulou CN., Kotoulas IG., Agapitos EB.et al. Intrafetal prostaglandin F 2 Alpha admin-istration for mid trimester pregnancy termina-tion. A case report. Am J Obstet Gynecol. 1990:162, 1245-6.

10. Shulman LP., Raafat NA., Mace PC. et al.Significance of septation in isolated fetal cystichygroma detected in the first trimester. PrenatDiagn. 1994: 14, 223-6.

11. Tanaka M., Sato S., Naito H., et al.Anaesthesia management of a neonate withprenatally diagnosed cervical tumors andupper air way obstruction. Can J Anaesth.1994: 41, 236-40.

12. Tricoire J., Sarraman MF., Rolland M. et al.Familial cystic hygroma. Report of 8 cases in 3families. Genet Couns. 1993: 4, 265-9.

13. Miyabara S., Suzomori K., Uemura Y. et al.Fetal cystic hygroma in sibs: developmentalanalysis of co-existent cardiovascular malfor-mation relevant to pathogenesis. Bi rthDefects. 1993: 29, 303-15.

14. Droste S., Hendricks SK., Von Alfrey H., etal. Cystic hygroma colli: perinatal outcome afterpre-natal diagnosis. J Perinat Med. 1991: 19,449-51.

15. Achiron R., Rotstein Z., Lipitz S., et al. Firsttrimester diagnosis of fetal congenital heart dis-

4Journal of Surgery Pakistan Vol. 1 (1); April-June 1996

rDR. ABDUL AZIZ

ease by transvaginal ultrasonography. ObstetGynecol. 1994; 84, 69-72.

16. Macleod AM., Mchugo JM. Prenatal diagnosisof nuchal cystic hygroma. Br J Radiol. 1991, 64( 765 ); 802-7.

17. Bulas HI., Saal HM., Allen JF., et al. Cystichygroma and congenital diaphragmatic her-nia; early prenatal sonographic evaluation ofFryn's syndrome. Prenat Diagn. 1992: 12, 867-75.

18. Gorenstein A., Katz S., Rein A., et al. Giantcystic hygroma associated with venousaneurysm J Pediatr Surg. 1992: 27, 1504-6.

19. Muraskas JK., Gianopoulos JG., Husain A.,et al. Mediastinal cystic hygroma, prenataldecompression with neonatal resection andrecurrence at 19 month of age. J Perinatol.1993: 13, 381-4.

20. Goshen S., Ophir D. Cystic hygroma of the parotidgland. J Laryngol Otol. 1993: 107,855-857.

21. Law PJ., Hall CM. The clavicular overgrowthin association with cystic hygroma. SkeletalRadiol. 1991: 20, 597-9.

22. Dickerhoff R., Bode VU. Cyclophosphamide innon resectable cystic hygroma. Lancet. 1990:335, 1474-75.

23. Tanigawa N., Shimomatsuya T., Takahashin K.,et al. Treatment of cystic hygroma and lym-phangioma with the use of bleomycin fat emul-sion. Cancer. 1987: 60, 741-9.

24. Ogita S., Tsuto T., Deguchi E., OK 432 ther-apy for unresectable lymphangioma in chil-dren. J Pediatr Surg. 1991: 26, 263 70.

25. Scally CM., Black JH. Cystic hygroma, mas-sive recurrence in adult life. J Laryngol Otol.1990: 104,908-10.

5 Journal of Surgery Pakistan Vol.1 (1); April-June 1996

PATTERN OF MALIGNANCY IN CHILDRENJAMSHED AKHTAR & ABDUL AZIZ

ABSTRACTOver a period oj five years eighty one tumors were seen with varied presentation. No significant differ-

ence was observed as regard to sex distribution. More than 20% oj tumors were observed in patients

under one year oj age. Wilm's tumor was the most common tumor in this study. Late presentation and

high mortality were the significant Jeatures. We stress on coordinated efforts to collect relevant epi-

demiological data so thatjuture planning could be made in order to reduce the morbidity and mortality.

KEY WORDS: Tumor Registry, childhood malignancy.

INTRODUCTIONIncidence of cancer is derived from population basedstatistics collected by tumor registries. In our countryand infact in our part of the world such comprehen-sive data does not exist because of the non-existenceof such organizations. Prevalence and incidencerates are frequently used in epidemiological studiesas the basis for planning and evaluating health pro-grams. As we do not know prevalence and incidenceof tumors in our population, disease pattern can notbe evaluated and no future projection can be made. Itis for this reason we conducted a study in patients onour surgical service to look at the statistics and toevaluate our results and to make future recommenda-tions in this regard.

MATERIALS AND METHODSA prospective epidemiological study was planned andconducted between 1st. July 1989 and 30th. June1994 in one of the two units of Paediatric Surgery atNational Institute Of Child Health, Karachi to collectthe relevant details on childhood malignancies uptothe age of 12 years on a structured proforma. Thesubjects included were all those who attended oursurgical service. Patients sent to us for lymph nodebiopsies following diagnosis of lymphoma on thebasis of fine needle aspiration cytology were notincluded as they were managed in medical unit. Ourdepartment does not deal with neurosurgical or oph-thalmologic malignancies so these cases were

Dept. of Paediatric Surgery, NICH, Karachi

excluded from the study. A total of 81 cases wereseen during this period with tumors involving almostevery part of the body.

RESULTSDuring five years study period no significant differencewas seen as regards to the yearly distribution and sexinvolvement. There were 40 male and 42 femalepatients. More than 20% of tumors were seen in infants.(Table I ). Wilm's tumor was the most common tumor inthis study, (Table II). Follow up of four most commontumors is given in table.1I1.

Table-1Age Distribution

upto 1 year 19

Between 1-5 years 25

Above 5 years 37

Table IITypes of Tumours

Tumour Number

Nephroblastoma 12

Neuroblastoma 6

Ewing's sarcoma 6

Rhabdomyosarcoma 4

Sacrococcygeal Teratoma 9 (Malignant - 1)

Cervical Teratoma 4

6Journal of Surgery Pakistan Vol.1 (1); April-June 1996

'- ....•.----

Jamshed Akhtar, Abdul Aziz

Ovarian Tumor 9 (Malignant - 2)

Testicular Tumor 3

Lymphoma (Abdominal) 3

Adrenal Carcinoma 2

Squamous Cell Carcinoma 2

Ganglioneuroblastoma

Ganglioneuroma

Chondrosarcoma

Osteosarcoma

Osteoclastoma

Fibroliposarcoma

Hepatoblastoma

Adenolymphoma (parotid gland)

Others 13

Total 81

Table IIIFollowup chart

NO. On On Follow Diedchemotherapy up

Lost tofollow up

Tumor

3 5Wim's tumor 12 3

Neuroblastoma 6 4

Ewing's sarcoma 6 2 3

2 2Rhabdomyosarcoma 4

DISCUSSIONIn developed countries cancer is second only to trauma

as the leading cause of death in children 'Accordlnq tothe western studies incidence of major malignant neo-plasm is 124.5 / 1,000,000 in Caucasian children under15 years of age2

• No such statistics are available forPakistan. Sporadic reports from various centers can befound in different periodicals but coordinated studies are

lacking. Zaidi and Jafer/ collected 213 malignant tumorsover a period of six years from southern part of Pakistan.Almost 50% of tumors were lymphomas, leukemias andretinoblastoma. Renal and bone tumors comprised 38cases ( 18% ). It has been suggested that as Wilm'stumor occurs with the least geographical variation itshould be used as standard of comparison. In anotherstudy from Armed Forces Institute of Pathology,Rawalpindi, 840 malignant tumors in children underfifteen years of age were collected in twelve years fromnorthern areas of Pakistan'. More than 2/3rd of thecases were leukemias, lymphomas, C.N.S. and eyetumors. Renal tumors comprised almost 5% of the totalcases followed by soft tissue sarcomas. In a study from

Pakistan Institute Of Medical Sciences Islamabad Wilm'stumor comprised 10.3% (49) of the 475 malignant tumorscollected in 6.5 years'. In the above mentioned seriessignificant male predominance was seen which is notborne out by our study. In our study a large number oftumors were seen in infants under one year of age whichhighlights the importance of their inclusion in differentialdiagnosis of masses. The most common tumor in ourstudy was Wilm's tumor followed by bone tumors, neu-roblastoma and soft tissue sarcomas. This order is alsoseen in other studies. We came across different tumorswith varied presentations like Osteosarcoma involvingproximal phalanx of finger, Liposarcoma involving clav-icle and Chondrosarcoma in supra clavicular region.These sites are rarely involved and are rarely reported inliterature'". Cervical teratoma, a rare lesion of which only212 cases are reported in about 130 years". We man-aged four such cases in a short span of time. An impor-tant observation made in this study was late presenta-tion. Most of the tumors were either in stage III or IV.Looking at the follow up, a large number of patientseither died or lost to follow up and are presumed dead.The treatment plan involves multidisciplinary programsthat include surgery, chemotherapy, radiation and to lim-ited extent immunotherapy. Chemotherapy has dramati-cally improved the prognosis of many paediatric neo-plasma particularly Wi 1m's tumor and rhabdomyosarco-mag. These drugs are costly and treatment is also pro-longed so, drop out rate is high and so is the mortality.Help from social workers and voluntary organizations inthis regard is to be appreciated.

In conclusion, a paediatric oncology group should beestablished to compile the important statistics. The datacollected will be of help in future planning includingresearch. At present more funds should be generatedboth for diagnosiS and treatment of childhood malignan-cies.

REFERENCES

1. Morgan ER, Baum ES. Tumors. in RaffenspergerJG ( ed.) Swenson's Paediatric Surgery, 5th. edi-tion, section V, Appleton & Lange publishers.1990: 345-346.

2. Young JL, Miller RW Incidence of malignanttumors in US. children. J Pediatr. 1975: 8, 254.

3. Zaidi SHM, Jafery NA. Childhood tumors inKarachi.JPMA. 1977: 27, 346-349 ..

4. Ahmed M, Khan AH Mansoor A. The pattern ofmalignant tumors in northern Pakistan. JPMA.1991: 41, 270-273.

7 Journal of Surgery Pakistan Vol.1 (1); April-June 1996

5.

Pattern of rnalignancy in children

Islam SS, Bukhshee MJ, Islam S, et al. Presentstatus of Wilm's tumor at the children's hospital,PIMS. Pak J Pediatr Surg. 1994: 1,60-70.

6.

and joint disorders. 2nd. ed., vol. 6, chap. 91. WBSaunders Co. 1988: 3602-4011.

Price CGH, Zhuber K, Kuntschik MS, et al.Osteosarcoma in children. A study of 125 cases. JBone Joint S urg. 1975: 57-B, 341-345.

Jordan R, Gauderer M. Cervical teratoma: Ananalysis, literature review and proposed classifica-tion. J Pediatr Surg. 1988: 23, 583-591.

8.

9.7. Resnick D, Tumors and tumor like diseases in

Resnick D, Niwayama G. ( ed ). Diagnosis of bone

D'Angio GJ, Evans A, Breslow N, et at, The treat-ment of wilm's tumor: Results of second Nationalwilm's tumor study. Cancer. 1981: 47, 2302.

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Journal of Surgery Pakistan Vol.1 (1); April-June 1996 8

NORMAL APPENDICES IN PATIENTS OPERATED FORSUSPECTED ACUTE APPENDICITIS

AbstractSHAISTA KHAN, FARHAT ABBAS AND HIZBULLAH SHAIKH

The morbidity and mortality associated with delays in diagnosis dictates that appendectomybe done early on strong clinical suspicion. No single laboratory or imaging technique hasgained specificity [or acute appendicitis. In the age group 15-25 years, specially in women,other clinical diseases may mimick appendicitis to such an extent that the number oj appen-dectomies may rise to an un-acceptable level. In order to assess our "normal" appendices inappendectomies rates, we reviewed all the histology specimens oj appendices removedbetween January 1986 - January 1987. Amongst 60 appendectomies performed during thisperiod, 54 (90%) were for suspected acute appendicitis, where as 6 (10%) were incidentalappendectomies. Histologically 14 (25%) appendectomies suspected for acute appendicitiswere reported as normal. This is well within range of normal appendices removed, asreported in literature. 12 (85.7%) of these 14 patients were females, it emerges, therefore.that in young females a more intense effort to diagnose acute appendicitis preoperativelymust be made. The use oflaparoscopy could be exploitedjurther.

Key words: Appendicectomy. Normal appendix.

INTRODUCTIONAcute appendicitis can be a difficult diagnosticproblem. Frequently appendices removed on clini-cal suspicion are reported histologically as nor-rnal'". The morbidity and mortality associated withdelays in diagnosis especially, at extremes of age,dictates that appendicectomy be done early onstrong clinical suspicion. However, specially inwomen in the 15-25 years age group, other clinicalsyndromes mimic appendicitls"" to such an extentthat the incidence of unnecessary appendicectomymay rise to unacceptable levels. For these tworeasons it is necessary that every effort be madeto accurately diagnose acute appendicitis beforesurgery.

It is considered acceptable to remove 5-25%(4)of nor-mal appendices as a safeguard against underdiag-nosing a potentially lethal condition. An increaseover this range may be interpreted as a sign ofunnecessary explorations and review by individualhospitals can serve as a basis of quality assurance inhealth care.

Dr. Shaista Khan. Dept. of Surgery,Aga Khan University Hospital, Karachi

In order to assess our 'normal appendix' appendicec-tomy rates, we undertook the study reviewing allappendectomies done in a 13 months period, fromJanuary 1986 to January 1987.

PATIENTS AND METHODSThe case notes of all patients who underwentappendicectomy between January 1986 - January1987, were retrospectively reviewed. The casenotes of patients with histopathologically normalappendix and in whom appendicectomy was notincidental to another operation, were scrutinised.Age, sex, the clinical parameters used for diagno-sis (including duration of symptoms, nature andlocation of pain, abdominal tenderness and asso-ciated complaints like nausea, vomiting and pyrex-ia) complete blood count, urinalysis and the finaldiagnosis were noted.

RESULTSBetween January 1986 - January 1987, a total of1784 patients were admitted to the department ofSurgery (Table I). 1663 (93.2%) required surgicalprocedures. Of these 60, 3.3%, (30 males and 30females) procedures were for removal of appendix.

Journal of Surgery Pakistan Vol. 1 (1); April-June 19969

Normal Appendices in patients operated for suspected acute appendicitis

Six patients (10%) required incidental appendicec-tomy and were excluded from the study, leaving 54patients operated for suspected appendicitis. Theage distribution was 3.5 to 61 years with mean agebeing 25 years. Fourteen patient (26%) werefound to have histologically normal appendix.Twelve (85.7%) of fourteen patients were females.Their ages ranged from 15-25 years. There wasno appreciable difference in the symptomatologybetween groups of patients with inflamed and nor-mal appendix. Similarly, localized pain in the rightlower quadrant with elicitable rebound tendernesswas noted equally in both groups (Table II).Leucocytosis was noted to be a poor index in dif-ferentiating acute appendicitis from non inflamedappendix, similar to experience by other qroups'",86% of the latter revealing leucocytosis in excessof 10,OOO/cmm. Two of the 14 patients (14.2%)developed minor complications including onewound infection. Another patient with underlyingCrohns disease later developed sub-acute intesti-nal obstruction felt to be related to underlyingCrohns disease. Appendicectomy in this patientwas carried out with uneventful recovery with noresidual enterocutaneous fistula. There were nodeaths. Among 14 patients, eleven (8.5%)patients revealed underlying diseases adequatelyexplaining the cause of abdominal pain (Table III).The spectrum of disease in our patients was nottoo dissimilar to the experience in the West(41(Table IV).

TABLE I

$9fgfq~A~m!~9n$.1784

(January 1986 - December 1987)

No surgical proceduremanagement

+Other surgical procedures1661 (96.7%)

+Incidental Appendectomy

6 (10%)

+Acute Appendectomy40 (74%

+Normal Appendix14 (25.9%)

TABLE IICOMPARISON BETWEEN CLINICAL PRESENTATION OF

NORMAL AND ACUTE APPENDICITIS

ACUTE (40) NORMAL (14)

PAIN

Typical 26 (65%) 9 (64%)

RIF 8 (20%) 2 (14%)

~A~ty~p~ic~a~I 4~(1~0~%~) 3~(21%)

NAUSEANOMITING

Nausea 7 (17%) 2 (1~%)_

'.f0miting 27 (67% 7 (50%)

~N~i1 6~(1~5~%~) ~5(36%)

FEVER

Above 39 C 5 (13%) 3 (21%)

Below 39 C 5 (36%)

6 (43%)22 (55%)

Normal Temp. 12 (30%)

LOCAL TENDERNESS/REBOUND

Tenderness

Tenderness & Rebound

11 (28%) 2~(~1_4°_~~)__28 (70%) 12 (86%)

TABLE IIIPATHOLOGY OF 14 CASES WITH NORMAL APPENDIX AT THE

AGA KHAN UNIVERSITY HOSPITAL

CECAL DIVERTICULUM

CROHN'S DISEASE 2

INFARCTED OMENTUM

PRIMARY PERITONITIS 2

SALPINGITIS 2

MESENTERIC ADENITIS

CORPUS LUTEUM RETENTION CYST

T.B. GREATER TROCHANTER

DIAGNOSIS NOT KNOWN

14

TABLE IVDISEASES MIMICKING ACUTE APPENDICITIS'

DISEASE NO. OF PATIENTS

Diagnosis at operation 32

Surgery required

Meckel's diverticulitis

13

3

Perforated cecal diverticulitis

Perforated cecum by foreign body

Adenocarcinoma of appendix

Leiomyosarcoma of duodenum

Acute cholecystitis

Torsion of gallbladder

Torsion of omentum

Journal of Surgery Pakistan Vol. 1 (1); April-June 1996 10

Shaista Khan, Farhat Abbas and Hizbullah Shaikh

Torsion of right ovarian cyst 2

Ruptured ectop_ic~p_re~g~n_a_n_c~y _

Surgery not required

Nonspecific mesenteric lymphadenitis

19

9

~I~~_ding follicular or luteal ovarian cysts

_Pe_l_vic~fl~mma:=-t~0~ry,-:"d~is,,:e~a~s~e=2 _

5

Endometriosis

Tuberculous ileitis---------------

Yersinia pseudotuberculos_is _

Diagnosis after surgery 10

By investigation

Salmonella infection

Gallstones-----------------------------------------

Acute pancreatitis

Urinary tract_in_f_e_c_ti_o_n _

E3..e.nignovari<:~_eratoma 1

Infectious monoculeosis 1-----.-~ ---

3

3

By second laparotomy

Ischemicc-'c~o_li~ti~s _

• From Lau et al - The American Journal of Surgery, Volume 148 September

1984 (reproduced by perrnisston).

It emerges, therefore, that young women in the agegroup of 15-25 stand a higher risk of misdiagnosisand hence require more intensive investigation andobservation before exploration. In this cohort ofpatients, laparoscopy has proved helpful in visualiz-ing 25% of normal appendices.

DISCUSSIONDebate has occurred for many years on the accept-able rate of unnecessary appendicectomy resultingfrom inaccurate diagnosis in acute abdomen. Themajor concerns of a surgeon managing a patient withacute abdominal pain are the risks associated with anegative exploration versus the hazards of conserva-tive management, which may allow an appendix toperforate. Most studies report a low complication ratefollowing unnecessary appendicectomy. The compli-cations are usually minor: wound infections andhaematomas. Additional financial losses, due toabsence from work in patients with continuing symp-toms, non appendicular in origin, remains a problem.However, Lau et al in 1984(4

) reported an overall com-plication rate of 14%. On the other hand surgery fol-lowing perforation of an inflamed appendix, continuesto carry significant morbidity and rnortality'". In thiscontext the acceptable rate would vary with the hospi-tal setting. In a district hospital with limited diagnos-tic services, the practice would be towards earlyexploration to avoid perforation of the viscus. Poorpatient compliance may also influence the decision tomanage conservatively. On the contrary, a well

j~,

equipped tertlary care centre should allow a moreaccurate preoperative diagnosis and hence reducethe number of negative surgical exploration. In theformer setting an acceptable rate may exceed 30%,whereas the latter should restrict the number to 20%or less. A small percentage of this group may requirefurther investigations and hospitalization.Conversely, delayed exploration culminating in perfo-ration of the organ, increases the morbidity and costsdue to prolonged hospitalization. Charles McBurney'sclassical paper in 1899 quoted in(6

), emphasizing theneed for early appendicectomy to avoid perforationwith its ensuing complications probably resulted in aspate of appendicectomies and instigated Harta'" tostate that 'the perfect man is the man without anappendix'. A review by Hichardson'" revealed acuteinflammation in only 58% of the patients followingappendicectomy. In a survey conducted in differenthospitals in United States of Arnerica'" between theyears 1924 to 1963, of the 64,591 surgically removedappendices, 35,223 (54.5%) were normal and werelabelled by pathologist as 'prophylactically removed'or 'appendicectomy for chronic appendicitis'. In acomparative study conducted in Northern Europev'?the incidence of normal appendices was 40-50%,whereas, a similar study from Britain'!':"? reported20% rate. This wide discrepancy is probably attribut-able to different attitudes towards surgical interven-tion and perhaps also due to availability of medicalservices in different countries. Recent surveys'? havetended to give more conservative figures with a 5 to27% incidence of normal appendices.

Looking at the various causes of abdominal pain sug-gestive of or mimicking acute appendicitis, a review ofthe cases at the Age Khan University Hospital revealsa similarity of experience to that in the West. Thepercentage of normal appendicectomies in our ser-vice (25%) is in the higher reaches of the acceptableranqe'?', but has effected a diagnostic approach toreduce this figure. The underlying diseases encoun-tered in our experience display close similarity tothose in other series (Table IV). As reported by oth-ers, in a small number of patients in our series, thefinal diagnosis remained unclear following explo-ration.

In these patients if symptoms persists, followingsurgery, further investigations including serologicaltests, abdominal ultrasound and radiological studiesmay be indicated. If these are inconclusive, a regularfollowup for several months may uncover an underly-ing disease process. However, measures to reducethe incidence of negative appendicectomies withoutrisking an increased incidence of perforation shouldbe evaluated further. The advantages of laparoscopy

11 Journal of Surgery Pakistan Vol.1 (1); April-June 1996

Normal Appendices in patients operated for suspected acute appendicitis

in the last decade has emerged and perhaps needsto be exploited further, utilizing it, in order to arrive atan accurate diagnosis before surgery. Hospitals inPakistan should identify the rates of histologicallynormal appendices, removed on clinical suspicion ofacute appendicitis. This exercise would also bringabout an awareness and improve the clinical acumenof junior staff. the group generally involved in first linemanagement of these patients.

REFERENCES1. Chang F.C., Hogle H.H. Welling R.D.: The fate

of the negative appendix. American Journal ofSurgery. 1973 126. 752-54.

2. Perr jess, B.Bjerregaard et al. AcuteAppendicitis.: Prospective trial concerning diag-nostic accuracy and complication. AmericanJournal of Surgery. 1981 141, 232-34.

3. Silbermanv: Appendicectomy in a large metro-politan hospital. Retrospective analysis of 1013cases. American Journal of Surgery 1981 142No.8,615-18.

4. Lau WL, Fans. Wong SH.: Negative findings atappendectomy American Journal of Surgery.1984148,375-78.

5. Bomer RJ, Bell MJ, Teruberg FL.: Diagnosticvalue of the white blood count and Neutrophilpercentage in the evaluation of abdominal painin children. Surgery, Gynaecology andObstetrics 1981 152, 424-26.

6. Harte RH.: In discussion of Richardson MH.

Trans American Surgery Association. 17:221989.

7. Mcburney C.: Experience with early operativeinterference in cases of diseases of vermiformappendix. NY. Med J. Dec 21,1889,676.Richardson MH.: Appendicitis. TransAmerican Surgery Association 17:22 1899.

8.

9. Collins DC, 71,000.: Human appendix speci-mens. A final report, summarizing Forty yearsstudy. The American Journal of Proctology,1963,14 (6). 365-81.

10. Neutra R.: Indication for the Surgical treatmentof suspected acute appendicitis: a cost effec-tiveness approach. In: Bunker JP Bariness BA.Mosteller F. eds. Costs, risks and benefits ofSurgery New York - Oxford University Press1977. 277-307.

11. de Dombal FT, leaper. OJ. Stamiland JR,McCann AP, Horrocks JC: Computer aideddiagnosis of acute abdominal pain - BMJ. 1972 2,9-13.

12. Gilmore OJA, Brodrib AJM, Bronett JP et al.:Appendicitis and mimicking conditions - Lancet19752,421-4.

13. Alexander A. Dentsch, Naphtali Shami andRaphael Reiss. Are some appendicectomiesun necessary. Jou rnal of Royal College ofSurgeons. Edinburgh. 28 (1), 35-40.

14. Lucian L. Max L. Ramensofsky.: Laparoscopyfor questionable appendicitis. Anals of Surgery1980191 (4). 410-13.

Journal of Surgery Pakistan Vol.1 (1); April-June 1996 12

IATROGENIC GASTROINTESTINAL PERFORATIONS

NEELAM JAVID; LUBNA ISHAO BHATTI; JAWAD SHER, ABID OAZI . IFTIKHAR A JAN.NAEEM-UZ-ZAFAR KHAN.

ABSTRACT

Iatrogenic injuries are an unfortunate but significant aspect oj any therapeutic intervention,contributing considerably to morbidity and mortality statistics. Although the element ojhuman error may never be entirely eradicated, it can be reduced substantially by takingmeticulous precautions to ensure mistakes are never repeated. We have analysed nine casesoj unusual forms oj gastrointestinal perforations in children. The causes oj perforations.modes oj presentation, methods oj management and the subsequent outcome is discussed.Most oj the cases were managed operatively but in selected cases a conservative approachwas practiced. Only one death occurred in this series and that also due to the late presenta-tion and extremely moribund state oj the patient.

KEYWORDS: Perforation, Gastro-intestinal Perforation, Iatrogenic iryury

INTRODUCTIONThere are a host of reasons for gastrointestinal (GIT)perforations in the paediatric age group. The commonestcause nowadays may be due to the recent introductionof endoscopic instrumentation and surgical procedures.There are well known causes of spontaneous gastroin-testinal perforations due to gangrene and congenitalbowel obstruction such as midgut volvulus, intussuscep-tion, bowel atresia, Hirschsprung's disease and meconi-um ileus, where the perforation may be caused in theproximal dilated bowel.

III

!!I

In our country, there are several prevalent diseaseslike enteric fever, severe gastroenteritis and tubercu-losis which are also responsible for gastrOintestinalperforation. Recognized late and managed improper-ly, gastrOintestinal perforation leads to severe mor-bidity and mortality.

In this paper however, only those GIT perforations inchildren are reported which were produced inadver-tently and were iatrogenic in nature. A total number ofnine cases are reported, whose management strate-gies and outcome is discussed. These cases under-score the importance of awareness of the possibilitiesof unusual causes of GIT perforations.CASE SERIESCASE NO.1A two day old male, premature born .at 32 weeks ofgestation, was admitted with delay in passage ofmeconium and abdominal distension since birth. On

Prof. Naeem-uz-Zafar Khan, Head Dept. of Paedriatic SurgeryThe Children Hospital, PIMS, Islamabad

physical examination the abdomen was moderatelydistended with normally situated anal orifice. On plainX-ray there was only gaseous distension of thebowel. A lubricated thermometer was introduced tocheck the patency of the ani canal and rectum. Thisprocedure resulted in sudden deterioration of thepatient's condition with a tympanitic abdomen. PlainX-Ray abdomen showed pneumoperitoneum (fig 1). It

Fig 1: Massive pneumoperitoneum with displacement of liver towards midlineas a result of gastlic perforation from vigorous resuscitative efforts fol/owingbirth asphyxia.

Journal of Surgery Pakistan Vol.1 (1); April-June 199613

Iatrogenic Gastrointestinal perforations

was obvious that the perforation was caused inadver-tently by the thermometer. The patient was taken tothe operating room within 2 hours of the accident. Atlaparotomy, a sigmoid perforation was noted. Rest ofthe bowel was normal. After evacuation of meconium,the perforation was closed using 4.0 vicryl. Post oper-ative recovery was uneventful. On discharge patientwas tolerating oral feeds and had normal bowelhabits. We assumed that delay in passage of meconi-um was due to prematurity.

CASE NO.2A 6 month old infant was admitted with signs andsymptoms of meningitis and seizures. Managementincluded oxygen therapy through nasal tube andNasogastric feeding. Inadvertently, the nasogastrictube was connected to the oxygen cylinder. resultingin what was presumed to be gastric perforation withmassive pneumoperitoneum and surgical emphysemaof the abdomen and chest wall. Because of his gen-eral poor condition from meningitis, the patient wasinitially managed conservatively with nasogastric suc-tion, parental antibiotics and appropriate fluids. Afterinitial improvement, the patient's condition deteriorat-ed with signs of established peritonitis and thus thepatient had to be surgically explored. The whole peri-toneal cavity was full of bile and the bowel was mat-ted with fibrinous exudates. A perforation was notedon the medial side of the junction of the second andthird part of duodenum just above the opening of theampulla of Vater and adjacent to the head of the pan-creas. Because of technical difficulties, only a onelayer closure of perforation with interrupted 4.0 vicrylwas performed and a peritoneal lavage was done.The patient was treated with broad spectrum antibi-otics. nasogastric suction and parenteral fluids. Hemade an uneventful and complete recovery, bothfrom the accidental perforation and meningitis andwas discharged after a fortnight from the paediatricintensive care unit.

CASE NO.3A three day old girl, born after full term pregnancy,was admitted with a history of abdominal distensionand bilious vomiting. Baby was breastfed since birth.On examination the patient was in shock with periph-eral circulatory collapse and a tympanitic abdomen.Plain X-ray showed free subphrenic gas. A workingdiagnosis of bowel perforation due to some congeni-tal anomaly was considered. After initial resuscitation,the patient was taken to the operating room forlaparotomy. On opening the abdomen a moderateamount of seropurulent free fluid was drained fromthe peritoneal cavity. On examination of bowel. a flex-ible, wooden splinter with a sharp tip was notedwhich was 2.5 ems, long (fig 2) and was protruding

half outside and half within the lumen of the anteriorsurface of the first part of duodenum; this was thecause of perforation with peritonitis. The perforationwas closed with one stitch of 5.0 vicryl, which suf-ficed to seal the perforation. Postoperative periodwas unremarkable and the patient made a completerecovery. On further enquiry from the mother, it tran-spired that "ghutti" was given to the baby. It isassumed that this thorn was one of the componentsof the ghutti.Ghutti is a form of herbal concoction used commonly in the sub-continent by illiterate people to clean the bowel and relieve colic ofthe neonate

Fig.2: 4 years old child (Case 2) sought advice for the first time because of suddenenlargement in a sacrococcygeal teratoma present since birth

CASE N04A new born male was transferred from a local hospi-tal having had resuscitation for birth asphyxia, whichinvolved nasogastric suction, use of ambu bag, endo-tracheal intubation and correction of acidosis. Onadmission he showed signs of peripheral circulatorycollapse, tachycardia and dyspnea with a tympaniticabdomen. Plain X-ray chest and abdomen revealedfree gas under the diaphragm indicating bowel perfo-ration. After resuscitation and stabilizing the patient,alaparotomy revealed generalized peritonitis and a lin-ear tear of about one cm. In the lesser curvature ofthe stomach which was closed in two layers.Decompressing gastrostomy was also instituted. Hewas treated post-operatively with a broad spectrumantibiotic and IIV fluids. Later on, the integrity ofstomach repair was judged by a gastrograffin studybefore feeding was started. The patient was allowedhome after a fortnight from the neonatal intensivecare unit.

Case No.5A full term baby delivered by a Dai (traditional birthattendant) at home had what appeared to be a case ofdifficult labour.Vigorous abdominal massage was appliedby a Dai to assist the delivery of the child. On admis-sion, 24 hours after birth the baby was completely col-lapsed, with dusky cyanosis and abdominal distension,

Journal of Surgery Pakistan Vol.t (1); April-June 1996 14

Neelam Javid, Lubna Isaq Bhatti, Jawad Sher, Abdi Qazi, Iftikhar A Jan, Naeem-uz-Zaffar Khan

marked tachypnea with shallow breathing and profoundhypothermia. He was in shock and acidotic.A plain X Ray abdomen showed massive pneu-moperitoneum with displacement of liver and visceratowards the midline. After initial resuscitation laparo-tomy was performed and a linear tear of 1.5cms inthe lesser curvature of the stomach was noted whichhad resulted in peritonitis with pneumoperitoneum.The perforation was closed and peritoneal lavagedone. Because of the poor condition of the patient hewas put on a ventilator for assisted breathing, butdespite vigorous efforts the patient expired 12 hoursafter surgery.

The actual cause of perforation could not be estab-lished but it can be presumed that external abdominaltrauma and asphyxia was responsible for the perfora-tion and the delay in transfer of the patient led to irre-versible shock.

Case No.6A nine year old boy with a history of fresh rectalbleeding intermittently but normal bowel habits wassuspected of having rectal/colonic polyps.Sigmoidoscopy was performed under general anaes-thesia to examine and remove the polyp. A juvenilepolyp was noted at the rectosigmoid junction and itwas removed by using a cutting biopsy forcep. Thepatient was allowed home.

After four days of the procedure, the patient wasadmitted with fever, abdominal pain and distension ofthe abdomen. There was generalised tenderness,most marked in the left lower quadrant of theabdomen. A plain X-Ray showed free gas under thediaphragm and distension of loops or small intestineindicating ileus. An ultrasound was done whichshowed a small collection in the pelvis. The patientwas treated conservatively with nasogastric suction,parenteral fluids and infusion of metronidazole andgentamicin.

His general condition continued to improve and there-fore surgery was not considered. Seven days afteradmission he was passing stools normally and wastaking oral fluids .Fever had subsided. He wasallowed home on oral metronidazole and Zanitrin foranothel seven days. On follow up after a fortnight thepatient had completely recovered .In this case theperforation which was caused by the endoscopic pro-cedure was perhaps small and sealed by the adjacentviscera.

A lesson which we can draw is that conservativemanagement has a definite role in the managementof a perforated viscus especjally if it is localised and

detected late and on close monitoring the patient'scondition continues to show improvement.

Case NO.7A three year old girl presented with a history of havingingestion of floor cleaner four months earlier and hada 10 cms long irregular tight stricture of the proximalthoracic oesophagus (fig 3). On oesophagoscopy andattempted dilatation with bougies, the oesophaguswas inadvertently perforated resulting in mediastinitisand right pneumotho-rax. She was treatedconservatively in inten-sive care unit with pha-ryngeal suction, par-enteral fluids and anunder water seal chestdrain. A feeding gas-trostomy was placedafter five days.

She was allowed homeafter a fortnight stay inthe hospital. Eventuallyafter four months a retro-hilar reverse gastric tubethrough a separatelaparotomy and right tho-racotomy approach wasconstructed. She hasremained well six yearsafter her oesophagealreplacement.

Fig 3: Esophageal stricture causing complete obstruction to passage of swst-lowed gastrograffin. These strictures are prone to perforations duringattempted dilatations.

Case No 8A two year's old boy had middle oesohageal per-

foration during an attempted dilatation of anoesophageal stricture caused by caustic injury.This resulted in pneumothorax and symptoms ofmediastinitis (fig 4). He was treated conservativelywith pharyngeal suction, feeding gastrostomy tubeand parenteral fluid. Chest tube was not placedbecause of the small size of the pneumothorax.

Subsequent X-rays showed fluid in the right pleuralcavity and insertion of an underwater seal chest tubedrainage became necessary which yielded frank pus.The patient recovered without further problems andafter three weeks of admission he was allowed home.Eventually an oesophageal replacement was carriedout using right colonic conduit.

He is well for the last seven years.

15 Journal of Surgery Pakistan Vol.1 (1); April-June 1996

Iatrogenic Gastrointestinal perforations

Case No.9A two year old patient was admitted with a stricture ofthe lower third of the oesophagus secondary tountreated gastro-oesophageal reflux. An attemptedoesophageal dilatation resulted in a perforation of thelower abdominal oesophagus. This was recognisedbecause the patient was developing signs of peritoni-tis and an X-ray showed free gas under thediaphragm. Laparotomy was performed and a lineartear of 0.5 cm was noted on the anterior surface ofthe abdominal oesophagus at its junction with thestomach.

This was closed and reinforced with adjacent cardiaof the stomach. A feeding gastrostomy was placed.The patient made an uneventful recovery and wasallowed home on gastrostomy feeding after ten days.Eventually, a colonic conduit was placed as anoesophageal replacement.

Fig. 4: Post.operative apparance of the patient in Fig. 2. Note the restoration of glutealfolds and reconstition of anorectal sling

DISCUSSIONOne of the most common causes of gastrointestinalperforations today may be those due to invasive sur-gical procedures especially endoscopic instrumenta-tion and the incidence of iatrogenic perforations hasincreased with the tremendous growth in the numberof these procedures. Perforations of the oesophagusare no exception. but other etiologies do exist suchas accidental caustic ingestion, spontaneous perfora-tions, perforations resulting from foreign bodies andmalignancies. In a report- by Ballesta Lopez C. et alfrom the School of Medicine, University of Barcelonaascertaining causes of iatrogenic oesophageal perfo-rations, they found that 28.6%, of perforations fol-lowed hiatal surgical procedures, 19%, occurred afterdiagnostic endoscopies, 19%, after endoscopic dilata-tions and 19% during removal of foreign bodies.

Case 8 exemplifies this inherent hazard associatedwith oesophageal dilatations. In our previous study"this was the commonest complication we encoun-

teredo From our experience it came to light that vari-ous factors prevailing in our society militate againstthe success of frequent dilatations. Poor patientscoming from remote areas with inadequate realisationof the fact that regular dilatations are an importanttherapeutic aspect of strictures, we feel that a defini-tive procedure of oesophageal replacement, althougha major undertaking, should be offered more readilythan the usual criteria laid down for this procedure.This applies especially for those patients where com-pliance is poor, and who return only when the stric-ture is so far advanced that attempts at dilatationreadily produces perforation.

Early diagnosis with expedient management is thesecret of reducing morbidity and mortality. After anysurgical or endoscopic procedure, a high degree ofsuspicion and close monitoring will allow early detec-tion of perforation. One should watch for signs ofshock, pallor, tachycardia, tachypnea, subcutaneousemphysema, chest pain and fever.ln cases of doubt,confirmation can be achieved by an oesophagogramusing a water soluble contrast medium.

Outcome depends on the cause, location, size andthe interval between the injuries and the initiation oftreatment. The site of oesophageal perforation alsodetermines the extent of sequelae. Prognosis isworse in thoracic lesions with significant mortality iftreatment is delayed for more than 24 hrs. Selectingthe most appropriate therapy also depends on theaforementioned factors. Although thoracotomy withreinforced primary repair and mediastinal drainagehas been recommended for cases with manifestationsof rnediastinitis'". with the advent of effective antibi-otics and better intensive care facilities, conservativemanagement consisting of antibiotics, gastric decom-pression, parenteral nutrition and thoracocentesis, ifneeded, can be appropriate especially in injurie s"resulting from nasogastric tubes.

There may be several long term complications ofoesophageal perforations such as external and inter-nal fistular formation, mediastinal abscesses andempyema of thorax.' Gastroduodenal perforationsmay also be produced in the same manner as arementioned for oesophageal perforations. In addition,in neonates gastroduodenal perforations are also pro-duced by vigorous resuscitative efforts at birth, naso-gastric tube injuries and congenital distal obstructivelesions such as atresias etc.

Perforation of the medial aspect of the second andthird part of duodenum produced inadvertently by therapid introd uction of a large amount of oxygenthrough a nasogastric tube in case 2 in our series could

Journal of Surgery Pakistan Vo/'1 (1); April-June 1996 16

Neelam Javid, Lubna Isaq Bhatti, Jawad Sher, Abdi Qazi, Iftikhar A Jan, Naeem-uz-Zaffar Khan

have been avoided if a strict colour coding of oxygentubes and nasogastric tubes are followed. One shouldalways confirm the length of nasal oxygen tube beforeconnecting it to the oxygen supply. Ideally one shouldadminister oxygen with specially designed oxygen deliv-ery tubes which are fixed on the external nares.

Gastro- duodenal perforations caused by thornymaterial contained in "ghutti", such as in case 3 in ourseries is most unusual and should be taken note of asa possible complication of local concoctions given tochildren at birth as a cultural habit in our society.Perforations in the gastroduodenal area are easy todetect because of a sudden collapse of the patientand a plain X ray will reveal free gas under thediaphragm. The site of abdominal visceral perforationhowever, can only be detected at laparotomy.Management entails vigorous resuscitative measuresand early laparotomy.A delay in diagnosis and con-comitant congenital anomalies are associated with ahigh morbidity and mortality.

In neonatal and paediatric age group the most com-mon cause of colonic perforation is distal obstructionsecondary to atresias, Hirschsprung disease andanorectal agenesis. However in this series we haveconsidered only iatrogenic perforations of the colon tohighlight the importance of an avoidable injury.

Iatrogenic injuries of the colon can be due to bariumenemas, thermometer and instrumentations. In case 1where sigmoid perforation was produced by a ther-mometer and recognised early, prompt surgical inter-vention prevented any serious sequelae. Despite itsrarity, rectal/sigmoid perforation occurring during ther-mometer instillation is one hazard that one should beaware of and take measures to avoid, particularlybecause rectal temperature is a more sensitive indi-cator of an infant's temperature than an axillaly tem-perature measurement". In contrast to the immediateaction employed in case 1, in case 6 the colonic per-foration went undetected for four days. Here thebodys defence mechanism sealed the perforation andconservative management by parenteral antibioticsand intravenous infusion was sufficient. Conservativemanagement is advocated in those patients with goodbowel preparation:", for silent colonic perforation,those with localized symptoms and signs and thosewhere diagnosis is made eight hours after colono-scopic injury". If there is a large perforation, gener-alised peritonitis or failure to improve on conservativetreatment then surgical exploration is necessary.

In summary, gastrointestinal perforation can be large-ly prevented if early diagnosis of congenital anom-alies producing luminal obstruction is entertained.Similarly iatrogenic perforations which are specificallyhighlighted in this article, could also be minimised

with awareness and practice of certain precautions,Importance of early detection of visceral perforationand prompt action cannot be over emphasised. Themainstay of deciding which course of action to followis frequent observation and accurate anticipation ofdevelopment of symptoms, hence one can obviatethe need for surgery in selected patients.

REFERENCES1. Newcomer M.K; Brazer S.R; Complications of

upper gastrointestinal endoscopy and theirmanagement. Gastrointest-Endosc-Clin-N-Am.1994 Jul; 551-70.

2. Ballesta Lopez Z.;Valiet Fernandez J.; CatarciM; Bastida Vi X; Iatrogenic perforations of theesophagus. Int-Surg. 1993 Jan-March; 28-31.

3. Naeem-uz-Zafar Khan et al: Managementaspects and associated problems of establishedesophageal strictures. Pakistan's J. Med. Sci.April-June 1993, Vo1.9: No.3; 253-260.

4. Kim Deobald J; Kozarek R.A.; Eosophagealperforation: an 8 year review of a multispecialtyclinic's experience. Am.J.Gastroenterol. 1992Sep; 1112-9.

5. Weber TW. Esophageal rupture and perforation.In: Welch IJ? Randolph JG, Ravitch MM, et al,.(Eds). Pediatric Surgery (4th Ed) Year BookMedical Publishers. Inc, Chicago. pg.727

6. Rowe I.M.; O'Neill J.A.; GrosfieldJ.L.;Fonkulsrud E.W.; Coran A.G.; Essentials ofPediatric Surgery; 1995, pg 419.

7. Ohri S.K.; Liakakos T.A.; Pathi V.; TownsendE.R.; Fountain S.W.; Primaly repail of iatro-genic thoracic esophageal perforation andBoerhaave's syndrome. Ann. Thorac. Surg.1993; pp 603-6.

8. Williams S.M.: Harned F.LK.; Recognition andprevention of barium enema complications.Curr. Probl. Diagn. Radiol.; 1991; pp 123-151

9. Morley C.J.; Hewson P.H.; Thornton A.J.; ColeT.J.; Aillary and rectal temperature measlll-el11ents in infants. Arch.Dis.Child.; 1992; pp 122-5.

10. Hall-C.; Dorricott N.J.; Donovan LA.;Neoptolemos J.P.; Colon perforation duringcolonoscopy; surgical versus conselvative man-agement. Br. J.Surg. 1991 May: 542-4.

11. Kavin.H; Sinicrope.F.; Esker.A.H; Managementof perforation of the colon at colonoscopy.Am.J.Gastroenterol. 1992 Feb; 161-7.

17 Journal of Surgery Pakistan Vol. 1 (1); April-June 1996

ROLE OF FINE NEEDLE ASPIRATION BIOPSY ANDCYTOLOGY (FNABC) IN A COLD NODULE OF THYROID

AND ITS COMPARISON WITH HISTOPATHOLOGYSHAHIDA AFRIDI, ASADULLAH KHAN, S. WAQAR AHMED, IRSHAD WAHEED

ABSTRACTThis study evaluates the diagnostic importance of Fine Needle Aspiration Biopsy and Cytology (FNAC)in apalpable cold nodule of thyroid. Conducted at Jinnah Postgraduate Medical Centre, Karachi over a period oftwo years from January 1992 to December, 1993, this study included 62 patients; 57 were reported asbenign and two as malignant on both FNABC and Histopathology. Two cases reported as "abnormal cellsseen" and "follicular cells suggestive of malignancy" turned out to be malignant on Histopathology. Onecase was a Cyst which disappeared on aspiration and did not reoccur/refill on 6 months folloui up. Theaccuracy of our result is 95% and 3.2% cases suggesting abnormality, proved to be malignant.

FNABC has an excellent patient compliance, is simple, economical and quick to perform in an out patientdepartment, to differentiate between benign and malignant conditions.

KEY WORDS:Thyroid cold nodule, Fine needle aspiration biopsy, cytology, Biopsy, FNAC, Thyroid, Cytology,Aspiration Cytology.

INTRODUCTIONFine needle aspiration biopsy and cytology of thyroidnodules is a sensitive and specific tool for detection ofthyroid cancers, thus preventing unnecessary operations.

This technique has been in use since long. It was firstintroduced for thyroid tumors in America in 1926'. LaterMartin and Ellis in the 1930'S2 studied the role of FNABCon thyroid, Iymphnode and breast. Most of the thyroidnodules are benign and these can be differentiated from

malignant by FNABC3• A solitary nodule is a common

presentation of thyroid disease and may be cold, warm orhot. Solitary cold and solid nodules have greater chances

3of being maligant (5-10%) .

FNABC of a cold nodule of thyroid has been used in

Sweden since the begining of 19504

and 8000 FNABCwere carried out during the last twenty years. Lowhagen

et al., (1979)5 also prefered this method in the diagnosis

of thyroid disease. Boey et al. (1979/ recommendedFNABC in every thyroid nodule, regardless of its clinicalnature, whether neoplastic or not. Silverman (1986) and

DEPARTMENT OF SURGERYJINNAH POSTGRADUATE MEDICAL CENTRE KARACHI

Frable (1976) recommended pre-operative use of

FNABC in a cold nodule of thyroid'. FNABC helps in theoperative planning for a definitive procedure based ontissue diagnosis.

PATIENTS AND METHODSPatients selected for this study were from Surgical Unit Iand surgical Outpatient Department of JPMC. Historywas taken and general physical and local examinationswere carried out. Thyroid scan was helpful in determin-ing the functional status of the gland. Cases of cold nod-ule on thyroid scan were selected for this study.

Material required for FNABC for specimen collection:1. Spirit swab2. 22 guage needle with syringe3. Glass slides

FNABC was performed with the patient in supine posi-tion with hyperextended neck. After examination andcleaning, negative pressure was created in the syringe,

sto aspirate the cells, with 0.2-0.3 cc air approximately.The patients were asked not to swallow or talk duringthe procedure. The swelling was fixed with one handand the other hand was used to introduce the needleinto the swelling. The needle was then moved to and fro

Journal of Surgery Pakistan Vol.1 (1); April-June 1996 18

Shahid Afridi, Asadullah Khan, S. Waqar Ahmed, Irshad Waheed

to get the tissue detached. The needle was then with-drawn and separated from the syringe; air 0.2-0.3 ccwas sucked and the needle was reattached and thematerial was expressed on slides. Examination of slideswas carried out in the Histopathology department, after

special staining [with Hematoxylin', Eosin, Orange G-6,E-A-50, or Eosin Azure-50].

RESULTS

TABLE ISEX DISTRIBUTION

Total No. of patients 62 Percentage

Female-----Male

52 83.810 16.2

M:F (Ratio) 1:5.2

TABLE IISEX DISTRIBUTION ACCORDING TO AGE

Age (Years) Male Female Number Percentage

0-1011-20 2 3 5 8.021-30 2 15 17 27.531-40 3 25 28 45.241-50 1 8 9 14.551-60 2 1 3 4.8

TABLE IIIDISTRIBUTION OF SWELLING ACCORDING TO SITE

Site No. of patients Percentage

Right Lobe 15 24.2Left Lobe 11 17.2Both Lobes 26 41.9Isthmus 5 8.1Right Lobe with isthmus 5 8.1

TABLE I VDISTRIBUTION ACCORDING TO SIZE

~Size (cm) No. of patients Percentage

1-3 5 8.13-6 28 45.27-9 21 33.910-12 6 9.613-15 2 3.2-~

19

TABLE VRESULTS OF FNABC

No. FNABC Results No. Percentage

1. No malignant cell seen 57 922. Malignant cell seen 2 3.23. Abnormal cell seen 1 1.64. Follicular cell seen 1.6

Suggestive of growth5. Cyst - Fluid 1.6

No abnormal cells seen

TABLE VICOMPARISON BETWEEN FNABC AND HISTOPATHOLOGY

62 FNABC Result 61 Histopathology Results

57 Benign Multinodular goiter31 ]

Solitary Nodule 21 57Follicular Adenoma 5

01 Abnormal Cells Papillary Carcinoma

01 Follicular Cells Follicular Carcinoma

Suggestive of malignancy

02 Malignant Papillary Carcinoma 201 Cyst Biopsy not done

Because of complete regression and

Cytology of fluid showed no abnormal cells.

DISCUSSIONThyroid diseases are quite common in Pakistan and pre-sent as a swelling in front of the neck, which may be dueto goitre, inflammation, cyst or malignancy etcH.Thereare no early clinical signs, symptoms or investigationswhich can differentiate between benign and malignantconditions preoperatively except FNABC which is theonly reliable diagnostic test and which is helpful in the

subsequent rnanaoernent" of these patients.

Single Non-functioning cold and solid nodule has a 5-10% chance of being malignant". It is recommended byLowhagen et al. that FNABC should be performed on

• 4.20,22every thyroid cold nodule, whether neoplastic or not .

FNABC is safe, Simple, economical and time saving tech-nique, requiring no anaesthesia and has no danger ofdissemination. FNABC has high rate of accuracy in expe-rienced hands. FNABC accuracy in different series ofstudies is shown in Table VII.

Thyroid pathology which can be diagnosed on FNABCalongwith clinical background includes goitre, inflamma-

Journal of Surgery Pakistan Vol. 1 (1); April-June 1996 ~

Role of fine needle aspiration biopsy and cytology (FNABC) in a cold nodule of thyroid and its comparison with hisopathology

tions, papillary carcinoma, medullary and carcinoma andanaplastic carcinoma".

TABLE VIICOMPARISON WITH OTHER STUDIES

Refferences Year No. of Cases Accuracy

Collachio et al· 1980 300 95%Niazi

lO1981 50 82.6%

Ackerman et at" 1985 420 98%Silverman et al" 1986 60 95%Hawkins et er" 1987 415 95%Cusick et al" 1990 307 58%Larosa et ar" 1991 827 97.7%Alam Saeed" 1991 56 94.4%

Present study 1992-93 62 95%

FNABC' positive for malignancy! cases have priority foradmission and treatment with better treatment plan andsingle operation.

Its limitations are that it cannot differentiate between fol-licular adenoma and follicular carcinoma because thisdistinction is made on histological criteria, including cap-sular and vascular invasion 22.

Cells obtained by FNABC can be subjected to cultureand the effects of radiotherapy and chemotherapy can bedetected. It is also useful for follow up in patients with

carcinoma treated with radiotherapy and chemotherapy".

Sometimes it is both diagnostic and therapeutic in cysticswellings. Aspiration of the fluid is the only treatment inmany cysts, owing to the fact that some thyroid cysts aredeprived of epithelial lining and emptying of their content

often results in regression of the cyst, risk of missingintracystic carcinoma is negligible (0.5%) (Gasper and

Barnesst25• Usefulness of FNA can be increased if used

in combination with electron microscopy.

Other Uses of FNABCDNA flow cytometry on the material obtained by FNABCgives rise to aneuploid peaks in malignancy but not in

. . 27benign thyroid pathology .

28

Enzyme StudyIt can be carried out like for acid phosphates in matastat-ic carcinoma of prostate. Medullay carcinoma o(4thyroidcan be diagnosed by immunoperoxidase stainingoior cal-citonin on the aspiration material.

CONCLUSION

FNABC has an excellent patient compliance, is very sim-ple, ecnomical and quick to perform in an Out Patientdepartment and leaves no scar.

It is the only pre-operative diagnostic test that can differ-entiate between benign and malignant conditions and ithas very high accuracy in experienced hands.

REFERENCES

Scanlan P, Dowling M, Dervan P, Carrigan T,Hetternan S, Firth R In. J. Med. sci., 1993,5:177-9.

2. Martin HE and Ellis EB, Biopsy by needle punctureand aspiration. Ann. Surg., 1930, 92:578-89.

3. Schwartz, shires Spencer, Principles of Surgery

4. Bailey and Lovers Short Practice of Surgery 22ndEdition.

5. Lowhagent T, William JS, Lundell g et al. ,Aspiration Biopsy and Cytology in the diagnosis ofthyroid cancer world. J. surg., 1981, 5:61.

6. Boey et aI., FNAC versus drill needle biopsy of thy-roid nodules. A controlled clinical trail surgery, 91.

7. Frable WJ Thin needle aspiration biopsy. A person-el experience with 409 cases. AM. J. cnn.Path 01. , 1979,65:168-182.

8. Handbook of Histopathological and histochemicalTechniques, Culling third edition, pp, 216-17.

9. Collachio and Oertel, 1988, Cancer.

10. Niazi M, FNABC of a cold nodule of thyroid gland.M. Phil thesis, University of Karachi, 1988.

11. Ackerman: Surgical Pathology.

12. Silverberg E: Cancer Statistics, 1977, 27:26.

.13. Hawkins H, Bellidod Bernal C et aI., Fine needleaspiration biopsy cytology in the diagnosis of thy-roid cancer and thyroid disease. Cancer, 1987,59:1206-9.

14. Cusick, Crawny N.P., Bernal C. et al. Fine needleaspiration biopsy cytology in the diagnosis of thy-roid cancer and thyroid disease. Cancer; 1990,26:807-811.

15. LaRosa, Belfiore A, Padove G et aI., The frequencyof cold thyroid nodules and thyroid malignancies in

20Journal of Surgery Pakistan Vo/'1 (1); April-June 1996

,• Shahid Afridi, Asadullah Khan, S. Waqar Ahmed, Irshad Waheed

patients from iodine deficient area cancer, 1991,pp,3096-2.

16. Alam Saeed, FNAC in cold nodule of thyroid. M.Phil thesis University of Karachi, 1991.

17. Caraway NP, Sneige N, Sam man NA: Diagncytopathology, 1993, 3:345-50.

18. Cuschieri, Giles, Moosa: Essential surgical prac-tice.

19. Davis Cristopher: Text book of surgery.

20. Burkitt, Quick, Gatt, Deakin Essential surgery.

21. Beg MHA and Mirza RA, Endemic goitre and therealities (editorial) Specialist, 1991, 7:2.

22. Current Surgical diagnosis and treatment.

23. Einhorn J Sand Franzenfs: Thin needle biopsy in

the diagnosis of thyroid disease. Acta Radiological,58, pp 321-26.

24. Khan SHand Khalig T: Evaluation of solitary coldnodule of thyroid. FNABC as a major diagnostictool. J Pak Inst Med Sci, 1990, 1:11-3.

25. Gasper MR: Aspiration of the breast cyst. Clif Med,1961, 95:227-31.

26. Barness MC: Aspiration biopsy of rumors inobscure or difficult localities under roentgenoscopicguidance. AM J

27. RM Rodriguez P Parilla J Sola et al: Comparisonbetween preoperative cytology and intraoperativefrozen section biopsy in the diagnosis of thyroidnodules. Br. Med. J., 1994, 81:1151-54.

28. Palmer JO, MC Divitt RW Stone KR et al flowcytometeric analysis of breast needle aspirate.Cancer, 1988, 62:2387-91.

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21 Joumal of Surgery Pakistan Vol.t (1): April-June 1996

BENIGN ORO-FACIAL TUMOURSNASREEN AMANAT

ABSTRACT

Among the various developmental and acquired causes oj faciai disharmony, odontogeniccysts and tumors, dental hamartomas and reactive bone disorders rate the most common.Most oj these lesions are discovered fortuiioustu. These patients either receive no treatmentor undergo extensive resections. Detailed are Jour cases oj oral pathology i.e. odontogenictumor (ameloblastoma), odontgenic cysts (odontogenic keratocysts), dental hamartoma(compound odontome) and a reactive-bone disorder (central giant cell granuloma), as causesojJacial disharmony and their surgical management.

Key words: Central giant cell qranuloma, ameloblastoma, ameloblastic carcinoma,odontogenic keratocysts, primordial cyst.

INTRODUCTIONA patient suffering from benign oral tumor or cyst islikely to undergo different types of surgical treat-ments because of lack of consensus as to their man-agement. We present our experience of four suchcases.

CASE NO.1A seven years old female presented with a painlessswelling on the left side of face, which was noticed 8months earlier (Fig. I). A gradual increase in the sizeof this swelling led to facial asymmetry. Clinicalexamination revealed expansion of buccal and lingualplates of the left body of the mandible from the firstmandibular molar to the central-incisor region withbowing of the lower border. The swelling was hard,well circumscribed and non-tender. Panoramic viewof the mandible revealed a multilocular radiolucencyinvolving the left body of the mandible .Hematological and biochemical indices were withinnormal limits. Aspiration biopsy of the lesion wasinconclusive. Under general anesthesia, the lesionwas enucleated through an intra-oral, subperiostealapproach. The lesion was non-encapsulated, darkred in colour, friable and bled easily. The patientrequired transfusion of one unit of blood per-opera-tively. Primary dentition and tooth buds of the perma-nent dentition were preserved. The lesion was identi-fied as an osteoblastoma on histological examination.Since the clinical picture was not consistent with the

ASSISTANT PROFESSOR ORAL SURGERYA.K.U.H. KARACHI

histopathological diagnosis, a second opinion wassought and the lesion was reported to be a centralgiant cell granuloma. Radiological examination atone year follow up shows normal bone trabeculae.

Fig. I Facial assymetry with bowing of left border of mandible

CASE NO. IIA married 22 year old female, presented with aswelling on the left side of the face. There was a dis-charging sinus on the left angle of the mandible. Theswelling had first been noticed 14 months earlier witha gradual increase in size. There was a history ofpain and antibiotics were used on more than oneoccasion . Clinical examination revealed a swellingconsistent with expansion of the buccal plate of theleft mandible from the angle to the pre-molar areawith bowing of the lower border of the mandible .Intra-orally the left lower first, second and thirdmolars were missing. Panoramic view revealed acompound composite odontome occupying the left

22Journal of Surgery Pakistan Vol.1 (1); April-June 1996

Nasreen Amana!

body of the mandible (Fig. II). There was a separateunilocular radiolucency present distal to the odon-tome in the mandibular ramus and a green stickpathological fracture at the angle. Under generalanaesthesia, both lesions were enucleated, via thesubperiosteal approach, after removing the buccalplate of the left mandible. The odontome consistedof numerous discrete denticles . The fracture at theleft angle of the mandible was reduced and immobi-lized with eyelets and intermaxillary fixation.Histopathologically the proximal lesion was identi-fied as a compound odontome with dentigerouschanges in the follicle and the distal lesion was aKeratocyst. There was complete resolution of theasymmetry over an eight months' period and therehas been no recurrence of the keratocyst over a threeyears follow up.

Fig. " Panorax showing missing teeth, compound odontome and aradiolucency in the left ramus

CASE IIIA 25 year old male presented with a grossly enlargedmandible of unknown duration. An aspiration biopsyhad been attempted with inconclusive result. Clinicalexamination revealed hard, irregular surface of theentire mandible. Intra-orally the dentition was intactwith a sinus in the right premolar area. The panoraxrevealed a large multilocular radiolucency from angleto angle (Fig. III). Both canines were lying along thelower border of the mandible with both the mandibu-lar third molars in horizontal impaction. The diagno-sis of a keratocyst was made. Since the patient didnot wish to have a dental clearance, the lesion wasenucleated intra-orally via a subperiosteal incision.Mandibular canines were removed and apicectomyand retrograde fillings were done on exposed dentalroots. Immediate post-operative recovery wasuneventful but for a wound breakdown in the area ofthe sinus. Pus from the sinus was noticed abouttwelve weeks after the surgery. Samples weretaken for the culture and sensitivity and appropriateantibiotic therapy was instituted. The infection couldnot be controlled with antibiotics five months after thefirst procedure a recurrence was observed; this was

again enucleated and curettage was performed. Atthis time the right lower third molar was also removedand the post-operative recovery was uneventful.Orthograde root fillings were carried out on allmandibular teeth after the second operation. Therehas been a gradual reduction of the radiolucencyobserved on the panoramic views. Three years afterthe second operation the patient was lost to follow up.

Fig. 11/Panorax showing radiolucency from (R) coronoid to the leftcoronoid. Note all 4 impacted 3rd molars and canines

CASE -IVA fifty-year old female presented with a massivegrowth in the mandibular symphysis, extending fromthe right premolar to the left premolar region due towhich the patient was unable to close her mouth(Fig. IV). The growth itself was well demarcated,non-lobulated and hard in consistency with no fluctu-ation, bruit or tenderness to palpation.

Fig IV Grossly enlarged mandible. Patient is unable to close her mouth

Panorax revealed a well circumscribed radiolucencywith a fair amount of interspersed calcification(Fig. V). A diagnosis of a calcifying odontogenictumour or an ameloblastoma was made. Dentalclearance was performed and the lesion wasapproached intra-orally, which was found to be cal-cific, hemorrhagic and well circumscribed.Segmental osteotomy was performed leaving anintact lower border of the mandible folloWed by thor-ough curettage. Profuse bleeding was encountered

23 Journal of Surgery Pakistan Vol.1 (1); April-June 1996

Benign oro-facial tumours

from the soft tissues as well as from the diseasedbone requiring transfusion of 8 units of whole blood.At the time of recovery from anesthesia, collapse ofthe right upper lobe of lung was observed.Bronchoscopy was performed to remove the bloodclots from the tracheo-branchial tree. Subsequentrecovery was uneventful. Histopathology report con-firmed an ameloblastoma. There was a wound break-down one week post-operatively which was allowedto granulate. Eighteen months after operation therewas radiological and histological evidence of recur-rence but the patient had declined further surgery.

Fig V Panorax showing radiolucency interspersed with radio-Opacities

DISCUSSIONThe oro-facial lesions discussed above are all essen-tially benign and are usually discovered on routinedental examination or when facial asymmetrybecomes prominent. Pain is rare, unless there issecondary infection and under the circumstancesa draining sinus may also be present. On radio-logical examination these lesions appear mostlyas radiolucencies with variable amount of inter-spersed radio opacities. Some pathological entitiesthat commonly present as radiolucency in the jaw areodontogenic keratocyst, ameloblastoma, centralgiant cell granuloma, cherubisum, odontogenicmyxoma, aneurysmal bone cyst, central hemangiomaand other vascular tesions '. Since there is muchvariation in the behavior of the multilocular lesions,their management differs accordingly. During evalua-tion of radiolucent lesions of the jaw the possibility ofa vascular lesion must always be kept in mind due toits potentially serious implications. All of the lesionsdiscussed in this report are locally aggressive butdo not metastasize, in contrast to the malignanttumours of the head and neck, which are not onlylocally invasive but spread via lymphatics or theblood stream. For these malignant tumours exten-sive surgical resection coupled with pre and postoperative radiotherapy is recommended. The role ofchemotherapy still remains palliative in head andneck malignancies. For benign oral tumors, a moreconservative approach is preferable The entire

mandible or maxilla can be exposed through an ade-quately designed intra-oral subperiosteal flap. Thisapproach not only gives good access for enucleation,curettage and segmental resection but also allowsretrograde root fillings to be done at the same time.

Central giant cell granulomas may occur at any age,but the majority present between 10-25 years andoccur in the tooth bearing areas of the jaw - usuallythe maridible e. The lesion usually presents asswelling of the bone and facial as ymme tryv.Radiologically it appears as non-corticated radiolu-cency causing displacement of involved teeth andtheir root resorption-. Growth is usually rapid andwhen the cortical plate is perforated, the lesion maypresent as a peripheral giant cell granuloma.Enucleation and curettage is the treatment of choice.Recurrence does not occur even if some tissue is leftbehind.

The term odontome originally included all odonto-genic tumors and cysts. Currently it is used to des-ignate a non-neoplastic, developmental anomalythat contains fully formed enamel and dentine.Odontome can, therefore, be considered a dentalhamartoma containing calcified dental tissues5.Complex and compound odontomes are closely relat-ed malformations. Complex odontomes consist of amass of enamel, dentine and cementum which ishaphazardly arranged, whereas compound odon-tomes consist of a collection of numerous small dis-crete tooth-like structures". All gradations betweencompound and complex odontomes exist, Mostodontomes arise in association with a missing tooth.Radiographically, the compound odontomes show asa radiolucency with numerous small radio opaquedenticles. These lesions are asymptomatic unlessthere is a secondary infection when pain becomesa feature of their presentations. Surgical enucleationis the treatment of choice. The condition does notrecur and a radical approach is contraindicated.

Ameloblastoma is a benign, locally invasive tumourderived from the odontogenic epithelium. It accountsfor about 1 percent of all oral tumours. About 8% ofthese tumors occur in the mandible, of which about70% arise in the molar region and the ascendingramus. Ameloblastoma is a slowly growing tumorand is usually discovered in the fourth to fifth decadeof life. Extension of the tumor in the soft tissues is alate feature. Radiologically ameloblastoma appearsas a unilocular or a multilocular radiolucency.Although most ameloblastomas arise intra-osseously,extra oral or peripheral ameloblastomas have alsobeen reported7,8. Metastases have beenreported9,10,11,12,13 but this is still an area of contro-

Journal of Surgery Pakistan Va/,1 (1); April-June 1996 24

Nasreen Amana!

Organization, Geneva.versy. Recurrence is a common feature in themultilocular type of ameloblastoma.

CONCLUSIONCentral giant cell granuloma must be differentiatedfrom brown tumours of hyper-parathyroid ism byradio-immune assay of parathormone. It must notbe confused with the giant cell tumour of the limbswhich is an extensively destructive lesion. Centralgiant cell granuloma and compound, complex odon-tomes are benign, non recurrent entities, which areadequately treated by enucleation and curettage ..Ameloblastoma and dental keratocysts are both local-ly aggressive and exhibit a variable potential forrecurrence. Segmental resection with preservationof the lower border of the mandible, wherever possi-ble, minimizes the risk of recurrence. However, sinceloss of teeth may not be acceptable in youngerpatient is enucleation and curettage may be per-formed, despite the greater chances of recurrence.Use of cryosurgery after enucleation is recommendedto minimize recurrence. Patients who have under-gone removal of ameloblastoma and keratocyst mustbe monitored post operatively for several years.Where dental extractions, segmental or partial resec-tion of the mandible have been carried out, occlusalharmony should be restored with bone grafting andosseus integrated implants. Minor facial asymmetrywill resolve under muscle stress. Where occlusion isintact, orthognathic surgery may be considered as asecond step to resolve remaining facial asymmetry.The advantages of an intra-oral sub mucoperiostealflap is that it does not leave a facial scar and the mor-bidity is low. It also allows apecectomy as well asenucleation, curettage and/or segmental resectionthrough the same approach.

REFERENCES1. Katz JO, Underhill TE.Multilocular radiolucen-

cies.Dent Clin North Am. 1994 38 (1) 63-81

2. Soames JV; Southam JC. Oral Pathology.Oxford Medical Publication, 1985. 237.

3. Potters BJ. Tiner BD. Central giant ceUgranulo-ma. Report of a case. Oral Surg Oral Med OralPathol. 1993 75 (3): 286 -9.

4. Horner K. Central giant cell granuloma. ClinRadio. 1989 40 (6): 622-6.

5. Pindborg JJ; Kramer IRH. Histopathologicaltyping of odontogenic tumors, jaw cysts andallied lesions. 1971 Int HistologicalClassification of Tumors. No. 5 World Health

6. Budnic SO. Compound and complex odontome.Oral Surg. Oral Med. Oral Pathol. 1976), 42,501 - 6

7. Stevenson AR, Austin BW. A case ofameloblastoma presenting as an exophytic gin-gival lesion. J Peridontol. 1990 61 (6): 378-81.

8. Bucci E., Lo-Muzio L, Mignogna MD, de-Rossa-G. Peripheral ameloblastoma: Casereport. Acta-stomatal-Belgi. 1992 89 (4); 267 -9.

9. Laughlin EH. Matastasizing ameloblastoma.Cancer. 1989 1; 64 (3): 776-80.

10. Ramdas K, Jose-CC, Subhashini J. Chandi-SM.Viswanathan FA. Pulmonary matastasis of themandible treated with cisplatinum, adriamycin, 9cyclophosphamide. Cancer. 1990 61:66 (7): 1475-9.

11. Philips SO, Corio RL, Brem H, Mattox D.Ameloblastoma of the mandible with theintracranial matastasis. A case study. ArchOtolaryngol Head Neck Surg. 1992 118 (8);861-3.

12. Ueda M: Kosaki-K, Kaneda T; Imaizumi M, Abe-T. Doubling time of ameloblastoma metastasiz-ing to the lungs. Report of two cases.J Craniomaxillofac-Surg. 1992 20 (7): 320-2.

13. Nagai N, Takeshita N, Nagasuka H, InnoueM, Nishijima K; eta I . Ameloblastic carcinoma:Case report and review. J Oral Pathol Med.1991 20 (9): 460-3.

14.. Albuquerque K, Mehta S, A, Sarkar S Mehta-AR. Recurrent Amelo blastoma of the mandibleand maxilla. Indian J Cancer. 1993 30 (2) 77-81.

15. Muller S, DeRose PB, Cohen C. DNA ploidy ofameloblastoma and ameloblastic carcinoma ofthe jaws. Analysis by Image and flowCytometry. Arch Pathol Lab. Med. 1993 117(11): 1126-31.

16. Thompson 10, Ferreire R, Van-Wyk CWoRecurrent unicystic ameloblastoma 07 the max-illa. Br J Oral Maxillofac Surg. 1993 31 (3) 3:180-2.

25 Joumal of Surgery Pakistan Vol.1 (1); April-June 1996

A STUDY OF CASES OF LARGE INTRA-ABDOMINALLYMPHANGIOMAS- A REVIEW OF LITERATURE

IMTIAZ LEWIS QURESHI, JAFFER-UZ-ZAMAN, AHMED SULTAN KHAN,ABID QAZI, IFTIKHAR JAN, NAEEM-UZ-ZAFAR KHAN.,

ABSTRACT:Intra-abdominal lymphangiomas are rare lesions. In literature different diagnostic modalitiesare used in detection oj early lesions. But our experience at the Pol.M.S.with intra-abdominallymphangiomas has been totally different. All the 6 lesions in the last 8 years presentedwith enormous abdominal distention and a provisional diagnosis oj ascites. Some oj thesewere even tapped for asciticfluid analysis. In most cases the final diagnosis was made onlyat laparotomy. Interesting features oj age, sex, site oj origin, type oj lesion, and managementare discussed in this article.

KEY WORDS:Abdominal neoplasms, lymphangioma, Diagnostic errors, ascites.

INTRODUCTIONLymphangioma is a benign growth of lymphatic originmostly noted at birth''". Whether these are congenitalmalformations, hamartomas or true neoplasms is stillcontroverslal"!". 95%are seen in the neck, 2-3% inchest and the rest occur in other parts of the body'".Intra-abdominal lymphangiomas are rare. The inci-dence of these has been estimated to range from I in27,400 to 1 in 100,000 of all hospital admissicns'"'.This article dlscusses our experience with the man-agement of this rare entity and the diagnostic confu-sion which we encountered during their management.

MATERIALS AND METHODSThe medical records of all the patients with provendiagnoses of intra-abdominal lymphangiomas, pre-senting to the Children's Hospital, P.I.M.S, Islamabadfrom January 1987 to December 1994 were reviewedretrospectively. All the cases were analyzed from thestandpoint of age, sex, presenting symptoms, physi-cal examination, investigations, site of lesion, man-agement and outcome.

RESULTSDuring 8 years (Jan. 87 - Dec. 94) we saw 6 cases ofintra-abdominal lymphangiomas. The average age atthe time of presentation was 5.3 years ranging from 3to 9 years. 66.6% (n=4) were females. Two patients

Prof. M. Naeem·uz·Zafar KhanThe ChHdren's HospitalP[MS, lslarnabad

had omental lymphangiomas, 2 had mesenteric lym-phangiomas (Figure 1), One had a retroperitoneal origin(Figure 2) while another arose from the greater curvatureof the stomach (Figure 3) but was not attached to theomentum. All the patients presented with chronicabdominal distention, which in one case increased sud-denly in size (case#I). In one patient it was also associat-ed with colicky abdominal pain and occasional vomiting(Case#4). Every patient had good general health at thetime of presentation. Each patient had a positive fluidthrill. The provisional diagnosis in 66.6% (n=4) patientswas ascites based on the characteristic history andphysical findings were inadvertently tapped in 2 casesand showed straw-colored fluid.

Fig. 1: Partly cystic partly solid multilobulaled lymphangioma arising from the rootof the mesenlery extending from the fourth part of the duodenum 10 proximaljejunum (Case #4)

I26Journal of Surgery Pakistan Vo/'1 (1); April-June 1996

+r az Lewis Qureshi, Jaffer-uz-Zaman, Ahmed Sultan Khan, Abid oazt, Iftikhar Jan, Nameem-uz-Zafar Khan'

Fig 2: Multicystic lymphangioma densely incorporating the left kidney. The speci-men also shows the kidney which had to be sacrificed.

Routine hematologic tests and LFTs in all 6 caseswere within normal range. Ultrasonography revealedlarge intra-abdominal cystic masses in 4 cases whichdisplaced the viscera peripherally but origin of thesecysts could not be ascertained. In one patient the pro-visional diagnosis of multicystic kidney was enter-tained due to the presence of multicystic mass in therenal region on ultrasonography (Case #5). Definitivediagnosis in these cases could be made intraopera-tively which also revealed their site of origin. Thepatient who presented with a sudden increase ofabdominal girth had a lymphangioma filled with darkblack colored fluid - an indication of hemorrhage intothe lesion (Figure #1).

Fig 3: Intra-abdominal lymphangioma from case #1. This large, muttnobutateo,unilocular thin walled lesion arose from the greater curvature of stomach. II con-tained darkblack fluid- an indication of hemorrhage.

In one patient the abdominal cavity was full of turbidfluid with lymphangiomatous lesion on the omentumand peripheral surface of peritoneum in a largeumbilical hernia (Case#6). These were the remnantsof lymphangioma which actually were ruptured byneedle punctures. These lymphangioma were

excised after careful dissection from the surroundingtissues except the one which was retroperitoneal inorigin (Case #5). In this case kidney had to be sacri-ficed because of the involvement of the vascularpedicle(Figures 2 & 4). The results are summarizedin Table #1.

Fig. 4: Mullicystic lymphangioma densely incorporating the left kidney. The spec-imen aslo shows the kidney which had to be sacrificed.

Table 1

No IAge Sex Symptoms Provisional Site rocedureDiagnosis

1 5 Yrs. M Chronic Abdominal Ascites Greater I Excisiondistention, wilh Omentumsudden increase

I2 3 Yrs. F Chronic Abdominal Ascites Greater I Excision

distention (tapped) curvaturestomach ,

3 7 Yrs. F Chronic Abdominal Ascites

IMesentery Excision

distention I

4 4 Yrs. M Chronic Abdominal Mesenteric Mesentery Excision

I distention cyst

5 )4 Mons I F Loin Mass Multicystic Retroperttoneal ~ ExcisionKidney & Nephr-

ectomy

i

,

II

6 9 Yrs.] F Chronic Abdominal Ascites Omentum ] Excisioni distention (tapped)

Table 1: Results of management of intra-abdomianllymangiomas

DISCUSSIONAmong the intra-abdominal lymphangiomas it has

,. been estimated that 60% are mesenteric, 25% areomental and 10% are mesocolic in origin, otherabdominal sites are rare [1",41.In children, it is report-ed to be the most frequent tumor of omentum!".Majority appear in the first year of life. 90% are evi-dent by the second year of life[31. Reports are alsoavailable in which it was detected at the age of 78years". In American population there is a 2: 1 femaleto male ratio while in Japanese population the ratio isreported to be opposite'". Apart from these reviews no

27 Journal of Surgery Pakistan Vol.1 (1); April-June 1996

sex predilection has been observed'».

A study of cases of large intra-abdominal lymphangiomas - review of literature

Numerous theories have been proposed concerningthe origin of lymphangiomas. The important postu-lates are: disturbance of endothelial secretory func-tion of the vessels, defects in embryonal organizationof the lymphatic system, obstruction of existing lym-phatic channels by an inflammatory process or a fail-ure of lymphatic spaces in embryo to join the venoussysteml3,4,58,111.Other less plausible etiologies includefailure of the leaves of the mesentery to fuse, local-ized degeneration of lymph nodes or intra-abdominaltrauma'!".

Lymphangiomas are classified primarily on a histolog-ical basis. The original classification established byWegner in 1877 is still considered to be the most reli-able. It divides lymphangiomas into simple, cav-ernous and cystic types!':".

These overgrowths are endothelium lined, round,smooth, thin walled and have clear serous or milkyfluid within them. They may vary from small growth toa very large sized lesion. Sudden enlargement can bedue to hemorrhage, vascular obstruction or a super-imposed infection. These lymphangiomas may be unior multilocular with communicating cysts":",Lymphangiomas are histologically benign but twopatients with changes of Iymphangio-endotheliomashave been reported':".

Lymphangioma may vary in size from a few centime-ters to a very large lesion containing several liters offluid occupying the entire abdominal cavity and pro-ducing marked distention. In older children the lym-phangiomas are generally of large size. This may beowing to the fact that they may have been growing fora number of years. They may be intra-mucosal, intra-mural or intraluminal and sessile and broad-based orpedunculated lesions. Generally there may be anabrupt transition between the lesion and adjacentnormally displaced tissues. Generally Iymphaniomasare solitary, but they may be multiple. When multiplelymphangiomas are present the term Iymphan-giomatosis is applied. Histological examination isnecessary for diagnosis because they must be differ-entiated from anqtornas!". In contrast to heman-giomas they usually do not have blood within thespaces and do not demonstrate immunoreactivity tofactor VIII related antigenl41.

It is reported that the diagnosis is mostly incidentalwith the help of lymphangiography, during electiveabdominal surgery, in an acute surgical abdomen orat post-mortem examination, but some are syrnpto-matic':". The symptoms are nonspecific and depend

on site and size of the lesion. The most frequentsymptoms being pain, digestive problems, palpablesoft cystic mass or even externally visible tumorssuch as pseudo-ascites. Most remain occult untilpressure symptoms occur. It can lead to obstructionof tubular structures like intestine, ureter and bile-duct. Acute abdomen may occur due to inflammationof the cyst wall, bleeding, torsion or rupture of thecyst". Galifer has reported that acute abdomencaused by lymphangiomatous cyst mimicked acuteappendicitis!'", Occasionally they may lead to intus-susception. In rectal lymphangiomas the most com-mon complaint is pain or bleedinglBI.

Although it is rare for lymphangiomas to be huge insize but all our cases were noted in this category. Atthis stage imaging techniques help differentiate lym-phangioma from ascites'v'", On a plain radiograph it

, may appear as rounded sharply outlined soft tissuemass displacing the adjacent bowel. This sign may bebetter appreciated in a gastrointestinal barium series.Calcification of the cyst wall may be occasionallynoted. On ultrasound it appears as well outlined,sonolucent transonic abdominal mass. CT scandemonstrates a non-enhancing near water densitymass !S,161.They clinically and radiologically are difficultto distinguish from other intramural masses such aslipomas, leiomyomas or early epithelial polyp.Lymphangiomas are generally pliable and changeshape in response to compression. This can beappreciable both endoscopically and radiologically.CT scan is reported to provide the exacttopoqraphy'?'. MRI is reported to be superior to CT orU/S in diagnosing a large hemorrhagic abdominallymphangioma which is reported to clinically mimic arecurrent hemoperitoneum'!". Protein losing enteropa-thy has been reported to be associated with the lesionwhich can be cured by resection of the lesion !5,9,lOI.

Effective treatment is surgical removal. Regression isunlikely'", They invade tissue planes and appear toinfiltrate normal tissues but are benipn'". Aspiration oflarge cysts lessens the difficulty with exposure duringoperation. If the cyst has invaded an abdominal organlike small bowel, spleen or tail of pancreas, total exci-sion of the involved organ is indicated'!", Prognosis isgood and it seldom recurs!15,16!.Internal and externalmarsupialization have become obsolete!"!

REFERENCES1- James Lister, Irvene M. Irving: Neonatal surgery

3rd Ed, 1990, Buterworth, p.59.

2- Juan Rosai: Ackerman's Surgical Pathology,Vol.2 7th Ed, 1990 Mosby p.549.

Joumal of Surgery Pakistan Vol. 1 (1); April-June 1996 28

\1. _

Imtiaz Lewis Qureshi. Jaffer-uz-Zaman. Ahmed Sultan Khan. Abid Oazi, Iftikhar Jan. Nameem-uz-Zafar Khan

3- Sabiston: Textbook of Surgery, 11th Ed. 1989Saunders.

4- John H Davis: Clinical surgery, 1987 C.V.Mosby. Company p. 3077-78,1485.

5- Ronald. L. Eisenberg: Diagnostic Imaging insurgery, 1987, Mc-Graw Hill. p. 466,507.

6- Louis. P. Dehner: Pediatric surgical pathology,2nd Ed, 1987 Williams & Wilkins. p. 77, 883-885.

7. Anderson's Pathology, Vol.1 8th Ed, 1985,Mosby. p. 724-726.

8. Frank. A. Oski: Principles and practice of paedi-atrics 1990, J.B. Lippincott. p. 388.

9. Fenoglio, Preiser, Lartz, Listrom, Davis,. Rilke:Gastrointestinal pathology - An atlas and text,1989 Raven press p. 578-579.

10. Colin L Berry: Paedriatic pathology, 2nd Ed.1989 Springer Verlag. p. 237.

11. I. Roisman, J. Manny, S. Fields, E. Shiloni:Intraabdominal lymphangioma. Br. J. Surg.1989, Vol. 76. May, 485-9.

12. G. Silecchia, A. Materia, D. Surgo, A. Capua:

Accidental successful treatment of a rare case ofdiffuse peritoneal Iymphangiomatosis: Fr. J.Surg. 1994, 81, 1286-1289.

13. Walker AR, Putnam TC.: Omental, mesenteric,and retroperitoneal cysts: a clinical study of 33new cases. Ann. Surg. 1973: 178: 13-19.

14. Henzel JH., Pories WJ, Burget DE, Smith JL.:Intraabdominal lymphangioma. Arch. Surg. 1966;93: 304-8.

15. Morger R., Bollinger B., Muller M.: Abdominallymphangioma in childhood. Paediatr. Pathol.1991; 26(1): 31-4.

16. Schimidt M.: Intra-abdominal lymphangioma.Kans. Med. 1992 May; 93(5): 149-ISO.

17. Liessi G., Sandini F., Spaliviero B.: CysticIymhangiomas of the abdomen. CT and US find-ings. Radiol. Med. Torino. 1989 Sep; 78 (3):204-9.

18. Lugo Olivieri CH., Taylor GA.: CT differentiationof large abdominal lymphangioma from ascites.Pediatr. Radiol. 1993; 23(2): 129-130.

19. Stoupis C., Ros PR., Williams JL.: Hemorrhagiclymphangioma mimicking hemoperitoneum: MRimaging diagnosis. J. Magn. Reson. Imaging.1993 May-June; 3(3): 541-2.

,v.noM':--;:£\b..L•C', '~\~~\.

29 Joumal of Surgery Pakistan Vol.1 (1); April-June 1996

Sacrococcygeal Teratoma Presenting as a Benign Lesionin Older Age Group

JAFFER-UZ-ZAMAN, IMTIAZ LEWIS QURESHI, AHMAD SULTAN KHAN, NAEEM-UZ-ZAFAR KHAN

ABSTRACT

Although it is a knoum fact that high percentage of sacrococcygeal teratoma left untreated. will undergo an early malignant transformation. But recently we have had an interestingexperience with 3 cases of sacrococcygeal teratoma 2 of which were entirely untreated uptothe ages of 4 and 6 years and third one was inappropriately managed, but did not under-go a malignant transformation and presented with obstructive bowel and urinary symp-toms. Details of presenting symptoms, investigative findings and management is described.A review of literature is also presented.

KEYWORDS: sacrococcygeal teratoma, persistent benign nature, teratoma

INTRODUCTIONSacrococcygeal teratoma is the most common con-genital neoplasm of neonates.lt is most commonlyobserved at birth, only a few cases have beenobserved in older children and adults" 3.71• The inci-dence varies from 1 in 10,000 to 1 in 40,000 livebirths[l2. 3.4.211.There is a female preponderance with aratio of 4: 1 [131. It is reported to be autosomally domi-nant with a strong familial tendency [1.41.

CASESCase 1A girl who had a previous surgery at the age of 8 daysfor acute urinary retention caused by intra pelvic cysticlesion, in another hospital, was admitted in October,1994 at the age of three and a half years, once againwith urinary retention. On examination she had a largecystic lower abdominal swelling which was presumed tobe distended urinary bladder. She also had bilateral dif-fuse deep cystic swelling causing the buttock to bulgeout. Ultrasound showed remarkably distended bladderoccupying lower half of the abdomen. There was anoth-er cystic swelling of 10 cm. circumference deep in thepelvis which presumably had caused bladder neckobstruction resulting in urinary retention. She also hadbilateral gross hydronephrosis. Her BUN was 55. IVUfailed to secrete the dye. X-Ray chest and CT scan didnot show any distant metastasis. Alpha feto protein wasnormal.·Prof. 1\4.Naeem Khan, Chairman and Prof. of Paediatric SurgeryThe Children Hospital,PIMS, Islamabad

On exploration through lower transverse abdominal inci-sion, a large cystic mass in the pelvis was noted (Figure1), It had displaced the bladder up into the abdomen.This was excised and it contained whitish smell-less fluidand culture revealed no growth. The cyst had endotheliallining and was reported as mature cystic teratoma.Subsequently, this relieved her symptoms and when shewas readmitted on 22 november 1994 her kidney andbladder had returned to normal size. Her BUN was 24.She was once again explored from behind after placingher in jack-knife position and rest of the multi-cystic ter-atoma along with the coccyx was removed. In addition tocyst there were solid areas containing cheesy materialand loose hair. Once again histology revealed no evi-dence of malignancy.

Fig. 1: Specimen of Case #1 who had recurrence of inadequately removed sacrococ·cygeal teratoma and on each occasion presented with acute urinary retention. Artery tor-ceps is pointing to removed coccyx which is an essential step in preventing recurrence

Journal of Surgery Pakistan voi.t (1); April-June 1996 30

Jaf/er-uz-Zaman, Imtiaz Lewis Qureshi, Ahmad Sultan Khan, Naeem-uz-Zafar Khan

Case 24 years old female consulted us for the first time withmassive swelling of the size of a football arising from thesacrococcygeal area (Figure 2 & 3). The swelling under-went repeated ulcerations and resulted in physical disabili-ty as she was unable to sit properly.On examinationthere was a broad mass of 25 cm. circumference withloss of gluteal folds and anterior displacement of analcanal. The overlying skin was very thick with multipleareas of ulceration and necrosis secondary to repeatedtrauma. The tumor was rubbery in consistency with areassuggestive of cystic lesions. Plain X-ray revealed patchycalcification. On barium enema there was evidence of intrapelvic presacral extension of tumor, but this extension wasnot palpable perabdominally. IVU was normal. X-raY' chestand CT scan revealed no evidence of metastasis. Alphafeto protein was within normal limits indicating a benignlesion.At operation a firm large multicystic tumor withIntense fibrosis fat and gluteal muscle was noted. Thisresulted in loss of tissue plains. There were scattered

patchy calcifications ofthe cyst walls. Withsome difficulty it wasexcised in its entirety.Histopathologyrevealed tissues ofectodermal, endoder-mal and mesodermalorigin. The tissue hadcystic cavitations whichwere lined by chondroidendothelium and neuraltissue was also pre-sent. No evidence ofmalignancy was foundin multiple sections. Inview of histopathology,prognosis may be con-sidered good. Postoperative progress wassatisfactory and shewas sent home.(Figure 4)

. )\"~.' i'-~.,,\\~,. •.••-\.0" ~.. . •I "'"i

Fig 4: Patient managed conservatively following an esophageal perforation from attempted bougi-nage for dilatation. Note intercostal chest tube. mediastinal widening is significant due to edema fluid.

31

Fig. 3: Cut surface 01 the removed specimen teratoma in Fig. 2.The increase in size was due to hemorrhage in the cystic part 01mature teratoma

Fig 2: A splinter which hadperforated through the firstpart of duodenum was lyinghalf outside and half withinthe lumen. This was thecontent of ghutti given to tthe neonate by grandmoth- 1er. It had caused peritonitis.

Case 3A six years old boyconsulted us for thefirst time with com-plaints of constipa-tion and urinaryretention for threemonths. He carrieda barium enemawith him whichshowed massivedilatation of colonand impacted fecalmatter. His intra-venous pyelogram showed normally functioning kid-ney with no distal urinary tract obstruction orhydronephrosis. With the provisional diagnosis ofHirschsprung disease he was taken to the operationroom for rectal biopsy. Examination under anesthe-sia revealed a solid mass displacing the rectum ante-

Fig.S: Lateral vrew- of pelvis of barium enema shows marked anterior displacementof the rectum which is occupied by a presacral intrapelvic solid mature sacracoc-cygeal teratoma with predominantly neural tissue in a 6 years old child (Case 3)

riorly. The upper margin of the mass could not bereached with the finger tip. Examination alsorevealed bilaterally palpable masses in the glutei withgluteal asymmetry. Lateral view of the pelvis in bari-um enema showed a pre-sacral mass displacing therectum anteriorly (Figure 5) while ultrasound showeda 10cms X 8cms solid mass in the pelvis. At opera-tion a large solid mass arising from the coccyx andthe last segment of sacrum was found plastered tothe rectum. It was, with some difficulty, removedand coccygectomy was done. Histopathologyrevealed a benign sacrococcygeal teratoma contain-ing neural tissue.

Journal of Surgery Pakistan Vol.1 (1); April-June 1996

Sacrococcygeal Teratoma Presenting as a Benign Lesion in older age group

DISCUSSIONUsually sacrococcygeal teratoma presents as abulging mass over the sacrococcygeal region withvariable cystic to solid consistency [1.6,121. Histologicallyelements of all three germinal layers may be found[S,121.Most sacrococcygeal teratoma have malignant poten-tial and with increasing age tendency of malig-nant transformation Increases proportionally. Inneonates frequency of malignancy is approximately7"10whereas above the age of two years it rises to50% or more [1.1

1J. In an interesting case a 67 years oldmale presented with sacrococcygeal teratoma compli-cated by ulceration and necrosis, histopathologyshowed benign nature of tumor [12,131. Metastasis ofmalignant tumor may occur in regional lymph nodes,lungs, liver and vertebrae [141. These tumors are beingdiagnosed more frequently in utero due to the wide-spread use of obstetric sdribg'raphy. In fetal life it maycause hypertrophy of placenta, fetal hydrops, highoutput cardiac failure, pre-eclempsia and respiratoryinsufficiency [5,9,10,15,241. A baby may be born with bilat-eral hydronephrosis, abdominal distention or failureto pass meconium [41. It may also cause abortion orstill-birth [181. Associated abnormalities occur in 20% ofthe cases, the most common being musculoskeletal,renal, cardiac, gastrointestinal, central nervous sys-tem, spina bifida, undescended testis and congenitalhip dislocation [1,2,3,17,24 I. Tumor with large presacralcomponent may present with weakness or paralysisof legs, constipation, anal stenosis [251 and urinarysymptoms secondary to bladder outlet obstruction [4 I.

Sacrococcygeal teratoma in females is often associ-ated with agenesis of corpus collasum and arachnoidcyst. Males born with sacrococcygeal teratoma mayhave leydig cell dysfunction, abnormal spermatogen-esis or both [19,22,16,30J.

Routine ante-natal ultrasound examination helpsnot only in diagnosing the sacrococcygeal ter-atoma but also determines secondary uropathyand helps in planning the mode of delivery [2 I .

Calcifications, which may be diffuse or in the formof a recognizable structure may be a helpful radio-logical sign but do not help differentiate benignfrom malignant lesions. N- acetyl cholinesterase [231

has also been measured for diagnosing but themost useful marker is alpha-feto protein, which dif-ferentiates between benign and malignant tumorsand can be used as a prognostic factor as well asa sign of recurrence 11,20,251.

In all cases of this tumor, radical attempt should bemade to remove the tumor regardless of the size ofthe tumor and histological stage, as soon as possibleafter birth. Unusual delay in surgical treatment maybe associated with malignant degeneration, pressure

necrosis, infection or hemorrhage in the tumor.Coccygectomy is mandatory to ensure against recur-rence [251. Incision in this operation is very crucial. Aninverted V-shaped incision has been found .to be veryimportant in maintaining the shape of the gluteal fold(Figure 4) and reconstitution of the anorectalsling. Prognosis depends on type, extent, histologyand age at the time of surgery, but interestingly notthe size of the tumor.

Malignant transformation is most likely to occurbetween the age of 4 months and 5 years. However,a few cases have been reported in later ages withoutmalignant change.

REFERENCES

1. James Lister, Irvene M. Irving: Neonatalsurgery.3rd Edition.1990. Butterworth. p 142- 52.

2. M. Feldman, P. Byrne, M. A. Johnson, J. Fischer,and . G. Lees: Neonatal SacrococygealTeratoma: Multimoding Assessment. Journal ofPediatric Surgery, Vol.25, No.6 (June). 1990: pp675-678.

3. P.S. Malone, L. Spitz, E. M. Kiely, R. J.Brereton, P.G. Duffy, and P.G. Ransley: TheFunctional Sequelae of SacrococcygealTeratoma. Journal of Pediatric Surgery, Vo.25,No.6 (June), 1990: pp 679-680.

4. Roberta E. Son nino, Shirley Chou, and FrankM. Guttman: Hereditary SacrococcygealTeratomas. Journal of Pediatric Surgery,VoJ.24, No.10 (October), 1989: pp 1074-75.

5. Sheldon J. Bond, Michael R. Harrison, Klaus G.Schmidt, Norman H. Silverman, Alan W. Flake,R. Nathan Slotnick, Robert L. Anderson, StevenL. Warsof, and Donald C. Dyson: Death Due toHigh-Output Cardiac Failure in FetalSacrococcygeal Teratoma. Journal of PediatricSurgery, Vo1.25, No.12 (December), 1990:pp1287-1291.

6. Havranek-P, Hedlund-H, Rubenson-A, GuthD., Husberg M., Frykberg T., and Larsson L.T.:Sacrococcygeal Teratoma in Sweden between1978 and 1989: long-term functinal results. 'J.Pediatr-Surg. 1992 Jul; 27(7): 916-8.

7. Bloechle M., Bollmann R., Zienert A., Kalache K.,Korner H., Bartho S. Specht-U: Fetal teratomadiagnosis and management. Zentralbl-Gynakol.1992; 114(4): 175-80.

Journal of Surgery Pakistan Vol, 1 (1); April-June 1996 32

Jaffer-uz-Zaman, Imtiaz Lewis Qureshi, Ahmad Sultan Khan, Naeem-uz-Zafar Khan

8. Orut R. M.; Orut R., Fontana A., Grosso J.J.:Mature Presacral Sacrococcygeal Teratomaassociated with a sacral epignathus. Pediatr.Pathol. 1992 Jan-Feb: 12(1): 99-103.

9. Nakayama O.K., Killian A., Hill L.M., Miller J.P.,Hannakan C, Lloyd D.A., Rowe M.1. The newborn with hydrops and sacroccocygeal ter-atoma. J. Pediatr. Surg. 1991 (Dec; 26(12):1435-8.

10. Alter D.N., Reed K.L., Marx G.R., AndersonC.F., Shenker L.: Prenatal diagnosis of conges-tive heart failure in a fetus with a sacrococ-cygeal teratoma. Obstet. Gynecol. 1988 Jun;71 (6 Pt 2) 978-81.

11. West D.K., Touloukian R.J.: Meconium pseudo-cyst presenting as a buttock mass. J.Pediatr.Surg. Ig88 Sep; 23(9): 864-5.

12. Harbon S. and Pheline Y.: Giant sacrococcygealteratoma in adults. Ann-Chir-Plast-Esthet. 1989;34(2): 153-9.

13. Langer J.C., Harrison M.R., Schmidt K.G.,Silverman N.H., Anderson R.L., Goldberg J.D.,Filly R.A., Crombleholme T.M., Longaker M.T.,and Golbus M.S.: Fetal hydrops and death fromsacrooccygeal teratoma: rationale for fetalsurgery [see comments] Am. J. Obstet.Gynecol. 1989 May; 160(5 Pt 1): 1145-50.

14. Ouimet A., and Russo P.: Fetus in fetu or not?[see comments]. J. Pediatr. Surg. 1989 Sep;24(9): 926-7.

15. Roig J.; Aguilar X. Maestre J. Domingo C.Morera J.: Pulmonary mass and air meniscussign in a 19-year-old man. Chest. 1991 Aug;100(2): 562-3.

16. Lahdenne P; Dunkel L, Heikinheimo M,Koskimies AI, Siimes M.A.: Hypergonadotropichypogonadism and sperm abnormalities in menborn with benign sacrooccygeal teratoma. J.Androl. 1991 Jul-Aug; 12(4): 226-30.

17. Lahdenne P, Heikinnheimo M, Jaaskelainen J,

Merikanto J, Heikkila J, Siimes M.A.: Vertebralahnormalities associated with congenital sacro-cocygeal teratomas.: J. Pediatr. Orthop. 1991Sep-Oct; 115): 603-7.

18. Ikeda H., and Sasano N.: Demonstration of pitu-itary tissue with 6 cells immunoreactive topituitary hormones in a sacrococcygeal ter-atoma. Acta. Pathol. Jph. 1987 Jan; 37(1):117-22.

19. Borges A.C., Festugato R.R., de-Cerqueira J.C.,de-Souza M.R.: Sacral agenesis associatedwith a teratoma Arq. Neuropsiquiatr. 1987Mar; 45(1): 81-7.

20. Peterson C.U., Strickler J.G., Gonzalez R.Dehner L.P.: Uterus like mass of the small intes-tine. Heterotopia or monodermal teratoma? Am.J. Surg. Pathol. 1990 Apr; 14(4): 390-4.

21. EI-Qarmalawi, M.A., Sadik M. el-Abdel. Hadi F.,Muwaffi. R; Nageeb K.: Diagnosis and manage-ment of fetal sacrococcygeal teratoma. Int. J.Gynaecol. S\Obstet. 1990 Mar; 31 (3): 275-81.

22. Shinoura N., Kondo T., Muraoka I., TsukamotoY, Yoshioka M, Miyazawa H. :Sacrococcygealteratoma of new born infants; a report of twocases. NO.Shinkei. Geka. 1990 Jun; 18(6): 55761.

23. Kainer F. Winter R.; Hofmann. H.M.Karpf. E.F.: Sacrococcygeal teratoma. Prenataldiagnosis and prognosis. Zentralbl. Gynakol.1990; 112(10): 609-16.

24. Book B, Ries R, Wunsch. P.H., Feige A. :Prenatal diagnosis of a sacrococcygeal ter-atoma with hydrops fetal is and placentalhypertrophy-consequences for the furthercourse of pregnancy. Gaburtshilfe.Frauenheilkd. 1990 Aug. \50(8): 647 9.

25. Dominici C. Bosman C. De-Sanctis-S; Clerico. A.Thorel M.F. Ceccamea A. : Endodermal sinustumor. Histological changes induced bychemotherapy. Minerva. Pediatr. 1990 Jun.42(6): 249-52.

33 Journal of Surgery Pakistan Vol.1 (1); April-June 1996

REVIEW ARTICLE

MINIMALLY INVASIVE SURGERYIRSHAD WAHEED, KULSOOM JUNEJO

KEYWORDS: Minimally invasive surgery, laparoscopy

INTRODUCTIONEndoscopic surgery is rapidly becoming a popularalternative to open procedures. Its introduction inthe field of general surgery has changed the spe-ciality forever. The Laparoscope, once used bygynaecologists for evaluation of pelvic disordershas emerged as a leading therapeutic tool for var-ious surgical procedures. The most widelyprac-ticed amongst them is the laparoscopic cholecys-tectomy, considered as a "gold standard" treat-ment for uncomplicated symptomatic cholelithiasis.Others include laparoscopic appendicectomy, her-nia repair, thoracoscopic treatment of pneumotho-rax, persistent pleural effusion, oesophageal disor-ders, sympathectomy, arthroscopic examinationand joint repairs. Various urological and gynaeco-logical procedures have all become acceptedendoscopic procedures.

HISTORYLapara is a Greek word meaning fluent.Laparoscopy is endoscopic visualization of peri-toneal cavity. The earliest description of endo-scopic examination was from KOS school led byHippocrates (460-375 BC), the father of modernLaparoscopy . The one who introduced this wasJacobeus. He used a cystoscope for examinationof both peritoneal and pleural cavities in 1910 andused both air and water for distention of cavities.Later on the procedure underwent several modifi-cations as Zollikofer in 1925 introduced the con-cept of C02 for insufflation.

ADVANTAGESLaparoscopy has revolutionized general surgery.The increasing demand for minimally invasivesurgery is being driven by a combination of devel-opments in surgical instrumentation and patients'expectations of less pain, shorter hospital stay,improved cosmesis and early return to normalactivity.Prof. of SurgeryJinnah Postgraduate Medical CentreKarachi.

The advantages of Laparscopy are:Small incisions,minimal trauma, reduced post-operative pain, early moblization and virtualabsence of wound infection.Minimum handling of gut and rapid return ofG.I.T. functions, therefore less chances of devel-oping future adhesions.Smaller wound size causes less depression ofcell mediated immunity.Surgeons contact with patients' blood isdecreased therfore,the chances of HBV & HIVtransmission is correspondingly decreased.

DISADVANTAGESImage quality is inferior to normal binocularvision.Loss of depth perception.Reduced field of vision.Improper location of surgeon's eyes & hands rel-ative to visual display,causing misperception ofangular relationship.Minimal access causes organ extraction difficult.

The operating time cannot be ignored because ofless experience and setting up of instruments etc.;operating time may be prolonged. Therefore inspiteof shorter hospital stay, the total hospital cost is notreduced because theatre is in use for a longer timeand the instruments, especially the disposables, arequite expensive.

INSTRUMENTS AND PROCEDUREIt requires full video endoscopic facilities includingprovision of camera, television monitor and insuffla-tion equipment. Instruments required are needle, tro-car & cannula, sheath, specialized clamps, clip appli-cators, retractors, staplers, scissors & specializedinstruments for specific procedures.

Laparoscopy is carried out in operation theatre undergeneral anaesthesia, observing full aseptic precau-tions. A pre-operative consent for conversion to openprocedure should always be taken. Initally pneuoperi-

34Journal of Surgery Pakistan Va/,1 (1); April-June 1996

Irshad Waheed

toneum is created. Then laparoscope is insertedthrough a sub-umbilcal port and initial laparascopyperformed. The site of placement of ports is differentin various procedures. It is ideal to have enough portsto allow the surgeon to use two handed dissection toavoid clashing of instruments, ports should not be tooclose to each other.

COMPLICATIONSExperience in laparoscopic surgery is increasing.Success depends upon skill and experience.A sharplearning curve exists for first 25-50 cases and is char-acterized by longer operative time and risk of compli-cations . But there is good evidence to suggest thatwith adequate training and experience, the incidenceof operative complications will be reduced to a levelcomparable to open surgery.Complications due to introduction of instruments andpneumoperitoneum include:

Emphysema.Gas embolism.Thromo-embolic complications.Haemorrhage.Haemotoma.Intestinal perforation.Hyper-capnia leading to respiratory or circulatoryembrassment.Hernia at puncture site,recurrence of malignancyat port site.Aggravation of existing hernia.

Due to the operative procedureDiathermy injury of skin and abdominal wall.Diathermy injury of intestine.Bile duct injuries.Haemorrhage.Pelvic sepsisPeritonitis.Trauma to retroperitoneal structures

CONTRAINDICATIONCardiac or respiratory insufficiency.Extreme obesityAdvanced pregnancy or large abdominal tumors.Previous surgery (conversion rate is increased).Intestinal obstruction.Generalized pertonitis.

Most of the contraindications have become only relativeones as the experience and the confidence of the sur-geons increases. Thus, acute cholecystitis is no longeran absolute contraindication.

FUTUREThere is no doubt that laparoscopic surgery is here tostay, it has been through three evolutionary steps:1. Diagnosis2. Minor surgical techniques as biopsy.

3. Removal of large tissue volumes as nephrectomyor splenectomy. The currently developing fourthstep is the laparoscopic reconstruction.

The future of laparoscopic surgery should be directedtowards identifying limitations, eliminating source ofoperating errors, improvement in skill and experienceand improving existing technology and also to makeit economically justifiable. The emphasis should beon the evolution of proper, accredited training pro-grams in teaching hospitals.

REFERENCES1. Farquharson's Text book of operative surgery - 8th edi-

tion R.F. Rintoul Minimal access surgery: 353 - 360.

2. General Surgical Operations 3rd edition R.M.KirkMinimal access surgery: 167 -174.

3. Laparoscopy in Urology 1st edition 1994.Malcolm.J.Coptcoat and Adrain D.Joyce. History ofLaparoscopy :5 -9.

4. Shaw's text book of operative gynaecology 5th editionEndoscopy in gynaecology : 37 - 45.

5. Maingots Abdominal operation 8th edition SeymourSchawartz Laparoscopy : 241 -245.

6. W.A .Kmoit and S.D. Wexner 1995 Laparoscopy inColorectal surgery: a call for carefull appraisalBr.J.Surg 82: 25 - 26.

7. J.R.T.Monson, A.D.K. Hill and A.Darzi 1995Laparoscopic Colonic surgery. Br.J.Surg.82 : 150 -157.

8. M.S.wilson, G.T. Deans and W.A. Brough Prospectivetrail Lichtenstein with laparoscopic tension free meshrepair of inguinal hernia Br.J.Surg.82 : 274 - 277.

9. S.O.Wexner and S.M. Cohen Port site metastasesafter laproscopic colorectal surgery Br.J.Surg 82 : 295 -298.

10. A.J.Mc Mohan, G.Fuliarton, J.N.Baxter and P.J.O.Dwyer Bile duct injury and bile leakage in laparoscopicBr.J.Surg . 82: 307 - 313.

11. J.P.Griffith, N.J.Everitt, F.Lancaster, A. Boylston, S.J.Richards, C.S.Scotl, E.A. Benson, H.M. Sue-lingConventional cholecystectomy upon cell mediatedimmunity Br.J.Surg. 82: 677 - 680.

12. G. Crosthwaite, T. Chung, P.Dunkley, S.Shimi and A.Cuschiexi Comparison of direct vision and electronictwo and three dimensional display system on surgicaltask efficiency in endoscopic surgery Br.J. Surg. 82 :849 - 851.

13. A.S. Cole, M.N.Vipond and H.J.Espiver. Technique forclosure of port site under laparscopic visual controlBr.J.Surg. 82: 1134 -1135.

35 Journal of Surgery Pakistan Vol.1 (1); April-June 1996

.'

LIPOSARCOMA OF CLAVICLEA Case Report

JAMSHED AKHTAR, FARHAT MIRZA, ABDUL AZIZ

ABSTRACTLiposarcoma rarely arises in bone but when it does it almost invariably occurs in long bonesoj extremities.A case oj Liposarcoma arising in clavicle is reported. To our knowledge liposar-coma oj clavicle is never reported in literature.

KEY WORDS: Liposarcoma, Bone tumors.

INTRODUCTIONLiposarcoma is one of the most common malignantsoft tissue tumors in adults but liposarcoma arising inbone is extremely uncommon'. Although some pathol-ogists have doubted the very existance of primaryintraosseous liposarcoma, a number of acceptablecases have been reported".

CASE REPORTA nine year old female child presented with a swellingover left clavicular region which was noticed threemonths earlier. History of dull ache at the same sitewas also given. On physical examination a hard bonyswelling in the line of clavicle was found. Skin overclavicle was normal. Radiologically, a well definedexpanding osteolytic lesion with thinned out cortex inthe lateral half of the left clavicle was found (Fig. I)Soft tissue invasion was absent. No swelling was pre-sent in other parts of the body. Lesion was resected.There was no local infiltration. Grossly, the tumor wasgreyish yellow in colour and soft in consistency.

Fig. f Liposarcoma of Clavicle

-_._ .._-----------Dr. Jamshad AktharPaediatric surgeonDept. of Paediatric Surgery, NICH

Microscopically, most of the cells had vacuoles intheir cytoplasm. Areas of myxoid degeneration werealso present. No futher treatment was given. Fifteenmonths postoperatively patient is well with no recur-rence.

DISCUSSIONStewart, one of the first to report liposarcoma ofbone, discribed three cases in 1931". The tumor isslightly more common in males and may be seen inpatients of all ages. Most common site of involvementis long tubular bones of the extremities. Femur andtibia are commonly involved". Schwartz collectedfourteen cases of proven liposarcoma from literature".To establish a firm disgnosis of an intraosseousliposarcoma two criteria must be met, the tumor musthave the histologic features of liposarcoma and a softtissue origin or metastatic neoplasm must be exclud-ed4• Our case fulfills both the criteria. It is also uniqueas to its site, the clavicle, which is never reportedpreviously.

REFERENCES

Carnesale PGH Malignant tumors of bone in CrenshawAH. Campbell's operative orthopedic. 7th ed. Vol. II,chap. 33,C.V. Mosby company. 1987; 777-805

2 Ross CF, Chertsey GH. Primary osteo-liposarcoma ofbone (Malignant mesenchymoma). Report of a case.J.Bone joint Surg. 1968; 50 B, 639-643

3 Schwartz A., Shuster M., Becker SM. et al.Liposarcoma of bone. Report of a case and review ofthe literature. J. Bone joint Surg. 1970; 52-A, 171-177

4 Resnick D. tumors and tumor like diseases in ResnickD. Niwayama G. (ed) Diagnosis aof bone and joint dis-orders. 2nd. ed. vol. 6, chap. 91. W.B. Saunder Co.1988: 3602-4011.

36Journal of Surgery Pakistan Vol. 1 (1); April-June 1996

TUBERCULOSIS OF PENISA Case Report

ABDUL SATTAR MEMON, JAN MOHAMMAD MEMON, ABDUL GHANI SOOMRO

ABSTRACTA rare case of tuberculosis of penis in a man oj 50 years is presented, followed by review ofliterature.

KEY WORDS: Penis, tuberculosis, genito-urinary tuberculosis, tuberculosis oj the penis.

INTRODUCTIONT8berculosis of penis is a very rare manifestation ofthe disease; upto 1971 only 139 cases had beenreported in the literature'. Quite a long ago it wasseen as a complication of ritual circumcision, whenthe operator who had open pulmonary tuberculosisused to suck the circumcised penis". Presently tuber-culosis of the penis is essentially a disease of adults,primary or secondary".

CASE REPORTA 50 years old male from rural area of Sindh present-ed with ulceration of glans penis, burning micturationand pain with low grade fever for 8 months.Examination revealed pale underweight male of aver-age build; no other positive finding was present ongeneral examination. Systemic examination did notreveal any positive finding. Local examination ofexternal genitalia revealed ulceration of glans (cir-cumcised) penis, resembling carcinoma penis. (pho-tograph I).

Urine DR was clear, Hb 9 g% ESR 105/hr, X-raychest revealed bilateral foci of pulmonary tuberculosisin both lungs and sputum was positive for AFB.Incisional biopsy under general anaesthesia revealedit as a typical tuberculous ulcer and the patient wasput on antituberculous drugs. He responded well totreatment and the ulcer healed ( photograph II). Atpresent the patient is completely cured.

DISCUSSIONTuberculosis is a common disease of the developingcountries, however tuberculosis of penis is anextremely rare manifestation of the disease. Presentlytuberculosis of penis is a disease of adults either pri-mary from coital contact from disease already present

Dr. Abdul Sattar MemonAssoc. Professor SurgeryLiaquat Medical College, Jamshoro

PllO/ograplJ I

Photograph"

in the genital tract or due to contamination frominfected clothlnq':", Rarely penile lesion may becaused by re-inoculation from male partner throughan infected ejaculate", Secondary penile tuberculosisoccurs as a secondary manifestation from active pul-monary tuberculosis.

37 Journal of Surgery Pakistan Vol. 1 (1); April-June 1996

Tuberculosis of penis A case Report

The lesion always presents as a superficial ulcerof glans, which clinically is indistinguishable frommalignancy. The lesion can also progress to caver-nositis involving the urethra". Rarely lesion occursas a solitary nodule or as a cavernositis with ulcer-atiorr'". The diagnosis is confirmed by biopsy andresponds well to antituberculous chemotherapy.

REFERENCES1. Agarwalla, B, Mohanty, G.P., Satu, L.K. and

Rath, R.C., Tuberculosis of the penis-report oftwo cases J. Urol., 124: 927, 1980.

2. Baskin NS and MEC. Tuberculosis of penis pre-senting as subcutaneous nodule. J. Urol, 141;1430, 1989.

3. Gow, J.G., Genito-urinary tuberculosis. CamblesUrology 1992, 6th ed, Vol:1 chap:22 Philadelphia

W.B Saunders Company Scientific publications,1976.

4. Lal, M.M., Sekhon, G.S., and Dhall, J.C.,Tuberculosis of the penis, J. Ind, Med. Assoc.,65: 316, 1971.

5. Lewis, E.L. Tuberculosis of the penis. A report of5 new cases and a complete review of the litera-ture. J. Urol., 56: 737, 1946.

6. Narayana, A.S., Kelly, D.G., and Duff, F.A.,Tuberculosis of the penis. Br. J. Urol., 48(4);274,1976.

7. Ramesh V; and Vasanthi R. TuberculousCavarnositis of the penis, genito urin. MED, 55:56, 1939.

8. Veukataramaiah, N.R. Van Raalte, J.A., andDutla,S.N.; Tuberculous ulcer of the penisPostgrad, Med J., 58: 59,1982.

INTERNATIONAL SURGICAL CONGRESS' 96Karachi

ANNOUNCEMENT

The Society of surgeons of Pakistan along with the International College of Surgeons and the Society of SAARCSurgeons was organizing an International Conference on November 23, 24, 1996 in Karachi. But it is nowPOSTPONED on the request of ICS Japan and SAARC Surgeons of Sri Lanka because of time clash. New datewill be announced after SAARC meeting at Kandy in September. The theme of the Conference is "SURGERY IN

2025". The aim of the Conference is to help future surgeons in the SAARC region to develop a sense of directionin which to channel their energies during the next 30 years (ostensibly their working life span) such that the healthof their communities is improved. We have chosen for this conference, 5 spheres of influence in which surgeonsmay be interested:

• Surgical needs and resources.

• Surgical care delivery.

• Research.

• Assessment of appropriate technology

• Manpower development.

We seek participation of leading regional and international authorities in each of the areas outlined. We expectthat experts will outline and prioritize the issues or deal with a specific issue. This could be followed by discus-sions involving wider audience participation. It is hoped that concrete proposals will emerge from the presenta-tions and discussions which will be presented at the concluding session of the Conference. We would hope torecord the proceedings of the conference so they can be of use to health planners, care providers, educators andsurgeons in developing countries.

FOR DETAILS PLEASE CONTACTConference Secretary, Dr. Asadullah Khan,

College of Physicians and Surgeons, 7th Central Street, Defence Housing Authority, Karachi-75500.or Department of Surgery, Jinnah Post graduate Medical Centre, Karachi.

i~~~ ..

Journal of Surgery Pakistan Vol. 1 (1): April-June 1996 38

PHOCOMELIA

A Case report

IMTIAZ LEWIS QURESHI, UZMA FARHEEN HASHIM, ZIA-UL-HAQUE, NAEEM-UZ-ZAFAR KHAN

ABSTRACT:An interesting case of Tetraphocomelia is presented, which highlights the importanceoj regular antenatal monitoring oj expectant mothers. In addition, the judicious use ojdrugs during pregnancy, with an appropriate knowledge of teratogenic effects that they mayproduce, is advocated.

KEYWORDS:Tetraphocomelia, antenatal monitoring. teratogeniCdrugs, phocomelia.

INTRODUCTIONPhocomelia is derived from two Greek words "probe"meaning seal and "melos" meaning limb!". Thisdeformity is a severe form of transverse limb defi-ciency in which there is a proximodistal developmen-tal failure. This rare congenital anomaly can involvethe upper or lower extremities or both'«. Three typesof phocomelia are identified: complete, proximal,and distal. In complete phocomelia, the upperextremity proximal to the hand is absent, and thehand articulates with the glenoid. Patients with proxi-mal phocomelia have no upper arm, and the handand forearm articulate with the glenoid. In distal pho-comelia, the forearm is absent, and the hand articu-lates with the arm" 3J.Similar pattern of deficienciesmay be present in the lower limbs.

Fig. 1

A Child withTetraphocomelia

Prof. M. Naeem-uz·Zafar KhanHead Paediatric SurgeryChildren's Hos[otal, PIMS, Islamabad

CASEThe baby boy was a product of non-consanguineousmarriage, born to a twenty-eight years old G5 4+0healthy mother after a full term pregnancy by sponta-neous vaginal delivery with no complications. Themother did not undergo any ante-natal monitoringand there was no history of drug ingestion during theante-natal period. The baby had absence of all thefour limbs (Tetraphocomelia) except for a vestigialproximal hand bilaterally [Figure 1].The lower limbswere entirely absent, including the digits. There wasno other detectable abnormality including the cardio-vascular system. With no facilities for special limb fit-tings, the parents were "reassured" and the patientwas sent home and was lost to follow-up.

DISCUSSIONBirch-Jensen estimated that it occurred once in about75000 births'". During the late 1950s and early1960s, phocomelia became prevalent in Europe as aresult of maternal thalidomide ingestion, when itaffected 60% of the infants born to mothers takingthalidomide in first trimester''! However, cases canalso occur sporadically!".

In Great Britain, "thalidomide babies" often hadbesides phocomelia, preaxial defects that included radi-al club hand or hypoplasia of the radius, thumb, andindex finger. Patients in this group had an ulna, and thehumerus was normal, hypoplastic, or absent. Childrenwith proximal agenesis of the humerus also hadabsence of the shoulder joint. Thalidomide also affect-ed other systems and has been reported to causedepression of the nasal bridge, coloboma, microtia,deafness, hyperhidrosis, and cryptorchldism".Phocomelia may be associated with other congenital

Journal of Surgery Pakistan Vol.1 (1); April-June 199639

Phocomelia a case report

defects [13[as well as syndromes. It is a recognizedfeature of Robert-SC phocomelia, [6, 7, 8, 10,'91, HoltOrarn" 51, Schinzel Phocorneua!'" and Acrofacialdysostoses syndromes [91• Cardiac abnormalities arefrequent and may be due to the fact that cardiacstructures are formed at the same time that the limbstructures are formed in embryo 111.

Etiologically, role of teratogen like thalidornide!': 2 31,

Valproic Acid[151 and Dicyclornlnet"? has been well-established while experimental work on mice has pos-tulated retinoic acid'?', and Cadmium Chlorlder'" to beof teratogenic nature.

Antenatal routine ultrasonography can reliablydetect phocomelia prenatally!" 71. It is also veryimportant to identify associated abnormalities or syn-dromes because in cases of Robert-SC phocomeliaSyndrome early neonatal death can be expected.Other syndromes featuring phocomelia may havemuch longer survival'". Tetraphocomelia has beendetected as early as 17 weeks of intrauterine lifel71•

Surgical treatment of phocomelia is limited": 21. Mostsur ge r ie s perf 0 rm e din the s e pat ie n t s aredesigned to aid prosthetic fittings 1'1. Decisions mustbe adapted to the total disability of the child.Thus, if one arm is normal, the dysplastic limbmay be more valuable as it acts as a holder, andsteadier than any contemporary prosthesis.Unfortunately, however, phocomelia is usually bilater-al; but even then we should remember the adaptivecapacity of the legs if they are normal'?'. Digital func-tion may be improved by web space deepening or bycorrection of contracture that may facilitate the use ofprosthetic devices". Developments in prosthetics arenow so rapid that these children should be referred tospecial centers within the first 3 months. A recentdevelopment is the use of myoelectric prosthetics inthe upper limb'!".

REFERENCES1. Lovell & Winter: Paediatric Orthopedics. 2nd Ed.,

Vo1.2, 1986. J.B. Lippincott Company. p. 651-2.

2. F. William Bora, Jr.: The Paediatric UpperExtremity: Diagnosis and Management. 1986.W.B. Saunders Company. p.28.

3. Tachidjian: Pediatric Orthopedics. 2nd Ed., Vol 1,1990, W.B. Saunders Company, p.lIl.

4. Dupre. CT, Fincher. R.H.: Holt-Oram Syndrome associ-ated with hypoplastic per pheral vasculature and midsys-tolic click. South. Med. J. 1993, Apr; 86(4): 453-6.

5. Pfeiffer-R.A.; Bowing-B; Deeg-K.H.: [Variantsof radial hemimelia with and without vitium cordis(Holt-Oram Syndrome) in 2 families]. Monotsschr-Kinderheilkd. 1989 May, 135(5): 275-9.

6. Grundy-HO, Burlbaw-J, Walton-S, Dannai-C:Roberts Syndrome: Antenatal Ultrasound - ACase Report. J-Perinat-Med. 1988; 16(1): 71-5.

7. Robins-DB, LaddarRL, Thieme-GA, Boal-DK,Emanuel-BS, Zackai EH: Prenatal detection ofRoberts-SC phocomelia syndrome: Report of 2sibs with characteristic manifestations. Am-J-Med Genet. 1989 Mar;j32(3): 390-4.

8. Verloes-A, Herens-C, Van-Haldergem-L, Retz-MC, Dodi nval-P: Robe rts-SC Phoco mel iaSyndrome with exencephaly. Ann-Genet.1989; 32(3): 169-70.

9. Le-Merrer-M, Cikuli-M, Ribier-J, Briard-ML:Acrofacial dysostoses. Am-J-Med-Genet. 1989Jul; 33(3): 318-22.

10. Mid r 0 - AT, I was z k i e w i c z - Paw b w s k a - A,Puchnarewicz-A, Debeck-K: [TAR Syndrome(Congenital Thrombocytopenia and aplasia ofthe radial bones) in a 5-months-old boy]. Wiad-Lek. 1992 Jan; 45(1-2): 66-9.

11. Chitayat-D, Stalker-HJ, Vekemans-M, Delneste-D, Azouz-EM: Phocomelia, Oligodactyly, andAcrania: the Schinzel-Phocomelia SyndromeAm-J-Med-Genet. 1993 Feb 1; 45(3): 297-9.

12. Schianda-Stumpel-C, De-Die-Smulders-C,Fryns-JP, da-Costa-J, Bouckaert-P: Limb reduc-tion defects and renal dysplasia: Confirmation ofa new, apparently lethal, autosomal recessiveMCA syndrome. Am-J-Med-Genet. 1990 Sept:37(1): 133-5.

13. Schinzel-A: Phocomelia and additional anom-alies in two sisters. Hum-Genet. 1990 May;84(6): 539-41.

14. Padmanabhan-R; Hameed-MS: Characteristicsof the limb malformations induced by maternalexposure to cadmium in the mouse. Reprod-Toxical. 1990; 4(4): 291-309.

15. Verloes-A, Friklche-A, Gremillet-C, Paquay-T,Decortis-T, Pigo-J, Senterre J: Proximal pho-comelia and radial ray aplasia in fetal valproic syn-drome. Eiu-J-Pediatr. 1990 Jan; 159(4): 266-7.

16. Correy-JF, Newman-NM, Collens-JA, Burroross-EA, Burrows-RF, Curron-JI. Use of Prescriptiondrugs in the first trimester and congenital malfor-mations.Aust-N-Z-J-Obstet-Gynaecol. 1991Nov; 31 (4): 340-4.

Journal of Surgery Pakistan Vol. 1 (1); April-June 1996 40

Imtiaz Lewis Qureshi, Uzma Farheen Hashim, Zia-ul-Haque,Naeem-uz-Zafar Khan

17. Dolfe-P, Ruberte-E, Kastner-P, Petkovick-M,Stoner-CM, Gudas LJ, Chambon-P: Differentialexpression of genes denoting alpha, beta andgamma retinoic acid receptors and RABP in thedeveloping limbs of the mouse. Nature. 1989Dec. 7; 342 (6256): 702-5.

18. G.C. Llyod Roberts and J. Fixsen: Orthopedics inInfancy and Childhood. 2nd Ed., 1990.Butterworth-Heinemann: p 100-101.

19. Jones: Smith's Recognizable pattern of humanmalformation, 4th Ed., 1988. W.B. SaundersCompany. p 256-7.

PLEASE SEND US YOUR PRESENT ADDRESS AND TELEPHONE No. so THAT WE CAN POST THE COPY OF JOURNAL OF SURGERY

PAKISTAN TO YOU.

ANN 0 U NeE MEN T

A two days symposium of Society of Surgeons Pakistan (Centre) and ICS (Pakistan Chapter) is being organizedon the 2nd and 3rd January 1997 at CPSP, Karachi.

The programme of this conference will include:-

1ST DAY• Inauguration

• Hamid Memorial Lecture

• Symposium

• Lunch

• Free Papers• Business meeting and election of new office bearers of Society of Surgeons Pakistan (Centre)

• Banquet

2ND DAY• State of the Art Lectu res

• Symposium

• Lunch

• Free Papers

• Convocation of I.C.S

• Dinner and Musical Evening

All Surgeons, specially members of Society of Surgeons Pakistan and Fellows of ICS, are invited to attend andsend papers / abstracts for presentation and full papers for publication in Journal of Surgery Pakistan

NOTE

All FICS and Society of Surgeons Pakistan Fellows and Members.

41 Journal of Surgery Pakistan Vol.1 (1); April-June 1996