lymphangioma of mesentery in a child

3
7ndlan) r#dle,. 46 : ~19, 11~9 LYMPHANGIOMA OF MESENTERY IN A CHILD* A Case Report HI~zuR ]~AIIMAIq, VJJAY KRISlDqA AOAI~WAL, SURgSH CHANDRA GUPTA AI4D ASHOK KUMAR SRIVASrAVA ,4ltahabad Lymphangiomas of the mesentery are exceedingly rare. The Florentine ana- tomist Benevieni is generally credited to having described the first case in 1507. Parson (1936) found 500 cases of mesenteric cyzts in the literature, of which only 10 were possibly of lymphatic origin. Beahrs c~ al. ,(1950) found only 9 cases of lymphangioma of the mesentery at Mayo Clinic from 191l to 1942. Collins and Berdes (1934) did not find e,aen a single case of chylous cyst of the mesentery in 15000 necropsies at the University of Minnesota. Due to the infrequent occurrence of these cysts and the importat,ce of their recognition and management it seemed desirable to report this case of lymphan- gioma of the mesentery. Report of a Case A.,a 3-year-old male child, was admitted to the S.N. Children's Hospital, Altahabad, on4.11.77 with the history of pain a[Jd swelling in the abdomen for ten days. The pain was severe in intensity, arising in the umbilical area, spreading all over the abdomen and coming in bouts. 1he swell- ing noticed in the lett side oi the abdomen *From the S.N. Children's Hospital, the Departments of Paediatrict and Pathology, MLN. bledical College, (lhurch Lane, Allahabad, 211002. Reeeived on June 27, 1978. gradually increased in size over a period of ten days. He had few bouts of vomiting during hospitalisatlon. Bowel movements were normal. Physical examination revealed a mode- rately well nourished child, without any significant lymphadenopathy, cyanosls, jaun- dice or oedema.. Abdomlna] examination revealed a, smooth, nontender, firm, intra- abdominal lump occupying the left lower hypochondrium, lumbar and left side of the umbilical regions, not moving with respira- tion but slightly mobile from side to side. Its limits were welt defined. The renal angles were free. No fluid was present in the peritoneal cavity. Investigations: Haematological investiga- tions except showing mild anaemia were within normal limits. A plain X-ray of the abdomen showed a soft tissue shadow occupying the left side of the abdomen. Barium meal studies showed displacement of one loop of small bowel to the extreme left side and of the rest of the bowel loops to tl;e right. I.V.P. showed both kidneys functioning normally. Clinical diagnosis of a mesenteric lump was made. Exploratory laparotomy revealed a large multicystic swelling arising from the jejunal mesentery extending upto its root. The jejunal loop was stretched over the cyst and its mesenteTic border merged with the

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7ndlan) r#dle,. 46 : ~19, 11~9

L Y M P H A N G I O M A O F M E S E N T E R Y IN A C H I L D *

A C a s e R e p o r t

HI~zuR ]~AIIMAIq, VJJAY KRISlDqA AOAI~WAL, SURgSH CHANDRA GUPTA AI4D

ASHOK KUMAR SRIVASrAVA

,4ltahabad

Lymphang iomas of the mesentery are

exceedingly rare. The Florentine ana- tomist Benevieni is general ly credited to having described the first case in 1507.

Parson (1936) found 500 cases of mesenteric

cyzts in the literature, of which only 10 were

possibly o f lymphat ic origin. Beahrs c~ al.

,(1950) found only 9 cases of lymphangioma of the mesentery at Mayo Clinic from 191l

to 1942. Collins and Berdes (1934) did not find e,aen a single case of chylous cyst o f

the mesentery in 15000 necropsies at the

University of Minnesota.

Due to the infrequent occurrence of these cysts and the importat ,ce of their

recognition and managemen t it seemed

desirable to report this case of lymphan-

gioma of the mesentery.

R e p o r t o f a C a s e

A. ,a 3-year-old male child, was admit ted

to the S.N. Children's Hospi ta l , Altahabad, on4.11.77 with the history of pain a[Jd swelling in the abdomen for ten days. The pain was severe in intensity, arising in the

umbilical area , spreading all over the

abdomen and coming in bouts. 1he swell-

ing noticed in the lett side oi the abdomen

*From the S.N. Children's Hospital, the Departments of Paediatrict and Pathology, MLN. bledical College, (lhurch Lane, Allahabad, 211002. Reeeived on June 27, 1978.

gradually increased in size over a period of

ten days. He had few bouts of vomit ing

during hospitalisatlon. Bowel movements were normal .

Physical examinat ion revealed a mode- rately well nourished child, without any

significant lymphadenopa thy , cyanosls, jaun-

dice or o e d e m a . . Abdomlna] examinat ion revealed a, smooth, nontender, firm, intra-

abdominal lump occupying the left lower hypochondrium, lumbar and left side of the umbilical regions, not moving with respira-

tion but slightly mobile from side to side. I ts limits were welt defined. The renal angles were free. No fluid was present in the peritoneal cavity.

Investigations: Haematological investiga-

tions except showing mild anaemia were within normal limits. A plain X-ray of the abdomen showed a soft tissue shadow

occupying the left side of the abdomen.

Barium meal studies showed displacement of one loop of small bowel to the extreme

left side and of the rest of the bowel loops

to tl;e right. I .V.P. showed both kidneys functioning normally.

Clinical diagnosis of a mesenteric lump

was made. Exploratory laparotomy revealed

a large multicystic swelling arising from the

jejunal mesentery extending upto its root. The jejunal loop was stretched over the cyst and its mesenteTic border merged with the

2 2 0 INDIAN JOURNAL OP PgDIATIglOS

cyst wall. The lumen of the cyst did not communicate with that of the jejunum.

The cyst along with 20 cm of a jejunal loop firmly attached with it was excised and end

to end anastomosis was performed. The

post-operative course was uneventful and the patient recovered completely,

The specimen measured 12 cm • 8.5 cm. The outer surface was nodular and the cut surface showed multiple cystic spaces. On

histopathological examination, large caver- nous spaces filled with lymph and collection of lymphoid tissue were seen.

D i s c u s s i o n

Lymphangioma of the mesentery is a rare entity. Common sites are neck and axilla but these have also been reported in arm, mediast inum, mouth region and abdo- men, other rare sites being kidney, spleen, and pancreas (Willis 1967). Most of the cervical and axillary growths are noticed at

birth but some do not appear until later.

In the report of Burnett a at, (1950) of 200 cases of mesenteric cysts of all types, 25% of the cases were children under

the age of 10 years. Gross (1953) reported

the discovery of congenital cysts of the omentum, mesentery and mesocolon in 19 children, all I0 years or younger.

Sex is nearly equally affected, females slightly predominat ing (Moore 1957, Willis 1967). Burnett a al. 1950) also reported a slight female preponderance (1.6:1).

There is considerable doubt as to whether these lesions are true angiomatous

malformations. Goetsch t938) believes that these lymphatic tumours are t rue infiltrat-

ing neoplasms and not merely an enlarging

cyst or malformation. Ewing (1940) consi- dered these as iymphangiomas arising from

VoL. 46, No. 37Z

embryonic lymphoid centres due to congenB tal or acquired obstruction of the lacteal~j In the opinion of Willis (1967) fluid accumulation, progressive formation df

collaterals and in some cases supervenin~ thrombosis and organisation suffice t a

account for growth of these tumoursr Anderson (1967) thought that probably these tumours arose from seqttestrations of lymphatic tissue. Gross (1953) regarded them to he arising from congenitally dis-, placed lymphatics which tailed to commtt~ nicate with the normal channels. Lympha.,, tic obstruction as a possible cause has also] been suggested (Handelsman and Ravitc~ 1954, Amos 1959).

These cysts are usually single. In some

cases a number of cysts may be present: They may be present in any part of the

mesentery but usually in the root (Andersor~ 1967). These cysts may attain a great size. !n the report of 4 cases by Moore (1957), all cysts were of a large size, two being

d u m b - b e l l shaped. These cysts were

unilocular.

Clinically, the most suggestive symptorfis in many cases are repeated attackr of abdominal pain, abdominal enlargement,

vomiting and nausea (Gross 1953, Moore

1957). 80% of cases of Burnett eta l .

(1950) had abdominal pain as the most frequent symptom and 50~/o had a palpable mass, vomiting and nausea. Acute symp- toms are usually due to torsion and haemor-

rhage into the cyst. A c u t e intestinal obstruction may be produced due to stretch- ing of the gut thus obliterating the lumen of the bowel (McNab and Menzies 1949) or due to volvulus of the bowel (Rohatgi et al.

1974). Major symptoms in 13 cases

reported by Gross 0953) and 4cases by

qtAIIMAN ET AL.~LYMPHANOIOMA OF MESENTIgRy I[N' A ChiLD

]r (1957) were abdominal enlarge- .~ent, pain and nausea and vomiting. Fever, diarrhoea and constipation were aTso present in some cases. Miller (195't) reports the case of a 3-week-old infant with fever and melaena. Major symptoms ~n our case were pain, an abdominal lump and occasional vomiting.

Solitary tumours can easily be enu- ~leated without jeopardising the blood supply of the adjacent bowel whereas the tumours that ~eem to invade the adjacent tissue, need wide resection. Out of 13 cases, Gross 0953 ) had to perform resection with anastomosis in 4 cases and simple enuclea- f loe . in 7. Two cases had associated V0[~'ulus of the small bowel which was corrected. T w o cases of Moore (1957) and

4 of Handelsman and Ravitch (1954) required resection. In our case removal of the turnout with resection and end to end anastomosis was required.

S u m m a r y a n d C o n c l u s i o n

A case of lymphangioma of the mesen- tery in z 3-year-old boy is presented. Regarding the aetiopathogenesis, consensus of opinion is that these are hamartomatous malformations. Most of the cases present with pain and swelling in the abdomen, nausea and vomiting, intravenous pyelo- grams and barium meal studies may ilelp in establishing the diagnosis. Simple enuclea- tion if possible should be done. Resection with anastomosis may be required in some ~.ases,

221

References

Amos, .J.A.C. (1959). Mu|tip|e lymphatic cyst.# of the mesentery. Brit. J , Surg. 46, 588.

Anderson, W. (1967}. Boyd's Pathology for Sur- geons. 8th Ed., W.B. ,C;aunders Co.,Philadelphla, P. 147.

Beahr% O.H., Judd, E.S., Dockerty, M.B. (1950), Chylous cyst of abdomen, Surg, Clin. North Am. 30, 1081,

Benevieni. (1957). Quoted from Moore, T.G. Ann, Surg., 145,428.

Burners. W.E., Roscmond, G,P., Buhcher, R.M. (1950). Mesenterlc cyst, Arch. Surg. 60, 699.

Collins, A.N., Berdes, G.L. (1934). Chyle cysts of the mesentery. Arch. Surg. 28, $35.

gwing, S. (1940). Neoplastic Diteases.i 4th Ed.~ W.B. $aundu: Co., Philadelphia.

Goettch, E. (1938). Hygtom a colli cysticum and hygroma axillare. Arch. Surg. 36, 394.

Gross, R.E. (1953). Surgery of Infancy and Child- hood. W.B. Saunders Co., Philadelphi% P. 336.

Handelsman, J.C., Ravitch, M.M. (1954). Chylous cyst of rr~esentery in children. Ann. Surg. 140

185.

McNab, I., Menzles, T. (1959). Lymphamgioma Qf

the mesentery. Brit..7. Surg. 37, 294.

Miller, D.B. (1954). Chyle cyst of the mesentory.

Arch, Surf. Chitago, 68, 359.

Moore, T.C., (1957). Congenital cyst of mesentery. Ann. Surg. 145, 428.

Parson, E.O. (1936). True proliferating cystic iymphangioma of the mesentery. Ann. Surg. 103, 595.

Rohatgi, M., Berry, M., Des, P.K. (19"/4). Lymphangioma of mesentery, a rare tumour of infancy and childhood. Indian Pcd;a'r. 11, 231.

Willis, R.A., ~1967). Pathology of Tumours. 4th Ed. London Butterworth.