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Self-assessment questions

A 40-year-old woman with lung cavitation

Sushil Kumar Ahlawat, Pankaj Malhotra, Venkatesh

A 40-year-old woman presented to the emergency room with intermittent fever, cough, haemop-tysis and dyspnoea for one week. She also had a history of episodic breathlessness with wheezingfor the last 5 years. She did not drink alcohol or smoke tobacco, denied intravenous drug abuseor use of steroids. On physical examination, the patient was febrile (temperature, 38.5 C), tach-ypnoeic and tachycardic. Blood pressure was 150/90 mmHg. Examination of lungs revealedbilateral coarse crackles and rhonchi. The rest of the systemic examination including cardiovas-cular system was normal. Serial chest X-rays are shown in figures 1 to 3.

Figure 1 Chest X-ray (PA view) at presentation

Figure 2 Chest X-ray (PA view), one week later

Figure 3 Chest X-ray (lateral view), one week later

Questions

1 What are the findings on serial chest X-ray ?2 What is the diVerential diagnosis ?

Postgrad Med J1999;75:619636 The Fellowship of Postgraduate Medicine, 1999

Department of

Internal Medicine,Postgraduate Instituteof Medical Education

& Research,Chandigarh 160 012,IndiaS K AhlawatP MalhotraVenkatesh

Correspondence toSushil K Ahlawat, 55, Sector24-A, Chandigarh- 160 023,India

Accepted 2 March 1999

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2/19

Answers

QUESTION 1

The first chest X-ray (figure 1), which wastaken at presentation, shows bilateral non-homogenous opacities and a thick walled cavityin the left mid-zone with surrounding consoli-dation. The second X-ray (figure 2), taken aweek later, shows bilateral, multiple lungcavitation with airfluid levels. Pulmonary

cavities in both upper and lower lobes are alsoseen on lateral view (figure 3). The chest X-rayin figure 4, taken approximately 4 weeks afterstarting treatment, shows significant resolutionof pulmonary lesions with antibiotic therapy.

QUESTION 2

The diVerential diagnosis of cavitating lesion inlung is given in the box. Inflammatory lesionsare the most common cause of lung cavities.The number of cavities may vary from one tomany. If the lesion is single, abscess fromnecrotising Gram-negative or staphylococcalpneumonia should be the first consideration,especially if patient is acutely ill with a severepneumonia that cavitates.

If multiple cavities are present, the infectionis likely due to haematogenous dissemination(septic emboli), and a source for this dissemi-nation should be sought. The source could beright-sided endocarditis or infected venousthrombi.

Tubercular cavities are usually located in theupper zone, either the posterior segment of theupper lobe or apical segment of lower lobe.Because of the high concentration of tubercu-lous bacilli in the cavity, patients usually have asmear strongly positive for acid-fast bacilli.Lung abscesses secondary to aspiration are fre-quently right-sided and most often involve thelower zone. Most patients with anaerobic lung

abscess would have risk factors for aspiration

such as poor dental hygiene, seizure disorder,alcoholic blackouts, etc. The manifestation isoften indolent, with complaints of cough, fever,and malaise lasting for weeks to months. Avariety of fungi may cause cavitary lesions.

Aspergillus, Mucor, and Candida spp rarelycause severe disease in patients without neutro-penia. The endemic fungi (histoplasma, blasto-

myces, and coccidiodes), however, may causecavitary lesions in immunocompetent hosts.These organisms should be considered in thediVerential diagnosis of a cavitary lesion iftravel to an endemic area has occurred.

Pulmonary lesions due to infected bullae/cysts are thin-walled cavities with smooth out-line. Cavitation may rarely complicate pulmo-nary embolism with infarct, which are usuallyseen in the lower zone. Thick-walled cavitiesmay result from pulmonary vasculitis, of whichWegeners granulomatosis is the prototype.Wegeners granulomatosis often results inbilateral multiple cavitary lesions, single lesionbeing less common. Neoplasia, either primary

(bronchogenic carcinoma, lymphoma) ormetastatic (from squamous cell) involving thelung, also may cavitate. These are usually thickwalled and fluid level is seen more commonlywith primary tumours than with metastasis.

Follow-up

Initial laboratory work-up revealed normocyticanaemia, leucocytosis with shift to left andtoxic granulation. Arterial blood gas analysisshowed mild hypoxaemia which improved withoxygen therapy. Blood biochemical laboratoryvalues were normal. Sputum Gram stainshowed Gram-positive cocci in clusters and

culture grew Staphylococcus aureus (methicillinsensitive) on four occasions. Sputum smearsand cultures were negative for acid-fast bacilli.Repeated blood cultures did not grow anyorganisms. Echocardiogram did not show anyabnormalities.

The patient was treated with antibiotics, ini-tially with cefotaxime/amikacin, later changedto cloxacillin. She became afebrile after 2 weeksof therapy, but antibiotics were continued for 6weeks to promote cure. In addition, chestphysiotherapy was helpful in drainage of thelung abscess.

Figure 4 Chest X-ray(PA view), 4 weeks aftertherapy

DiVerential diagnosis of cavitation onchest X-ray

x cavitating pneumonia: S aureus, Gram-negativebacilli (Klebsiella,Pseudomonas, Legionella),anaerobes, mycobacteria, fungi, pneumocystis

x septic emboli, bacterial or fungalx Wegeners granulomatosis or pulmonary

vasculitisx pulmonary infarctionx

infected bullae or cystsx neoplasia: primary or secondary

620 Self-assessment questions



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Discussion

Staphylococcal pneumonia is an uncommon,but serious infection. Despite appropriate anti-biotic treatment, mortality in staphylococcalpneumonia remains high.13 Staphylococcalpulmonary infections are typically nosocomialand usually occur in older adults (sixth decadeor older) with concomitant medical illnesses.1

Watanakunakorn3 reported the cases of 44

patients with bacteraemic S aureus pneumonia(positive sputum and blood culture). Allpatients had at least one underlying condition(intravenous drug abusers were excluded fromthe study). Virtually all patients in a study byKaye et al 1 had some underlying illness,particularly chronic pulmonary disease. Ourpatient was relatively younger, however she didhave a history of chronic pulmonary airwaydisease.

No single radiological presentation is diag-nostic ofS aureus pneumonia. However, multi-ple cavitation or pneumatoceles are character-istically seen, particularly in children andintravenous drug abusers. These cavities are

usually thin walled (24 mm) and are slightlyindistinct on their outer border. Abscessformation has been reported in 2370% ofadult patients presenting with S aureus pulmo-

nary infections.46 However, in recent studies,1 2

abscess formation was seen in 1620% ofpatients and multiple abscesses occurred infre-quently. In the study by Kaye et al1 all patientshad single abscesses. The chest X-rays in thesepatients typically show multilobar infiltrates,predominantly in the lower lobes, and oftenbilateral.1 5 6 The frequency of pleural involve-ment is variable, with estimates ranging from5% to 48%.1 5 6 Our patient had bilateralinvolvement on presentation and went on todevelop multiple abscesses, however, she didnot have pleural involvement.

Despite the availability of eVective antibiot-ics, mortality continues to be 3032%.1 2 Thismay reflect the fact that patients with S aureuspneumonia tend to be elderly and have othersignificant illnesses. Treatment is with apenicillinase-resistant penicillin such as naficil-lin, cefazolin or vancomycin (penicillin allergicor methicillin-resistant S aureus), and 46weeks of therapy is required to promote cure.

Final diagnosis

Multiple staphylococcal lung abscesses.

Keywords: pneumonia; Staphylococcus aureus

1 Kaye MG, Fox MJ, Bartlett JG, Bramn SS, Glassroth JG.The clinical spectrum of staphylococcus aureus pulmonaryinfection. Chest 1990;97:78892.

2 Woodhead MA, Radvan J, MacFarlane JT. Adult commu-nity acquired staphylococcal pneumonia in the antibioticera: a review of 61 cases. Q J Med1987;64:78390.

3 Watanakunakorn C. Bacteremic staphylococcal pneumonia.Scand J Infect Dis 1987;19:6237.

4 Hausmann W, Karlish AJ. Staphylococcal pneumonia inadults. BMJ1956;2:8457.

5 Fisher AM, Trever RW, Curtin JA, Schultze G, Miller DF.Staphylococcal pneumonia: a review of 21 cases in adults.NEngl J Med 1958;258:91928.

6 Gold JA, Davis JW. Adult staphylococcal pneumonia. Med Ann DC1959;28:3635, 4178.

The chilling tale of a patient with thrombocytopenia

Nicholas R Balcombe

An 86-year-old woman was admitted having been found collapsed at home. On examination, shewas pale, dehydrated, confused and disorientated with a rectal temperature of 28C. There wasno lymphadenopathy or bruising. Her pulse was regular at 50 beats/min and blood pressure was150/100 mmHg. Cardiorespiratory, abdominal and neurological examinations were allunremarkable. Review of this patients previous hospital notes revealed no significant illnesses inthe past and she was on no regular medication. There was no history of excess alcohol intake.Social assessment revealed that she lived alone with inadequate heating provisions. Investigationsshowed thrombocytopenia, with a platelet count of 88 109/l (reference range 150450 109/l),confirmed by analysis of two venous blood samples. Her haemoglobin was 14 g/dl, white bloodcount 8.2 109/l, with a normal diVerential count, and an erythrocyte sedimentation rate of 6 mmin the first hour. Serum urea was 11.1 mmol/l (3.58 mmol/l) with a serum creatinine of 127mol/l (50105 mol/l). Serum electrolytes, liver function tests, coagulation studies,electrocardiogram and chest X-ray were all normal, as were vitamin B12 and folate levels. Urinemicroscopy and culture revealed no evidence of urinary tract infection. Serum thyroid-stimulatinghormone was 18.93 mU/l (0.55.0 mU/l ) and free thyroxine 14 pmol/l (924 pmol/l).

Questions

1 What is the cause of thrombocytopenia?2 What complications may occur in this patient?

Self-assessment questions 621

Department of Health

Care for the Elderly,Queens Hospital,Burton Hospitals NHSTrust, Belvedere Rd,

Burton upon Trent,StaVordshire, DE130RB, UKN R Balcombe

Accepted 12 October 1998



4/19

Answers

QUESTION 1

The potential causes of thrombocytopenia arenumerous (box 1). In this patient, the cause ofthrombocytopenia was hypothermia. Follow-ing admission, she was rewarmed and rehy-drated and the following day, her resting oraltemperature had risen to 34C and although

she remained lethargic, she was no longer con-fused or disorientated. On day three her restingoral temperature was normal. On day nine, herplatelet count had r isen to normal (295 109/l).

QUESTION 2

Apart from the complications of thrombocyto-penia, which include skin purpura, mucosalhaemorrhage and prolonged bleeding aftertrauma, hypothermia may lead to other clinicalfeatures, shown in box 2.

Discussion

Thrombocytopenia occurring with low body

temperature was first described 58 years ago.1

Since then, animal studies have attempted toidentify a cause for this phenomenon.2 3 In1958, Villalobos used radioactively labeledplatelets to demonstrate hepatic and splenicsequestration during hypothermia in dogs, with

80% of the platelets returning to the circulationon rewarming.2 A later study showed the liverto be the major site of sequestration.3 Dissemi-nated intravascular coagulation and bone mar-row failure have also been postulated as causesof hypothermia induced thrombocytopenia.

Platelet sequestration as a result of hypother-mia is rare and only a few recorded casesexist.4 5 This was thought to be the cause of ourpatients thrombocytopenia. She never exhib-

ited any clinical features suggestive of dissemi-nated intravascular coagulation and clottingstudies were normal. Her initial full bloodcount did not show any evidence of bone mar-row suppression and the rapid return of theplatelet count to normal, mirroring the rise inbody temperature, would suggest a causal rela-tionship.

Although our patient experienced no prob-lems as a result of her low platelet count, thiscase illustrates that thrombocytopenia is apotentially serious complication of hypother-mia.

Final diagnosis

Hypothermia-induced thrombocytopenia.

Keywords: hypothermia; thrombocytopenia

Causes of thrombocytopenia

Reduced platelet productionx viral infections: measles, varicella, infectious

mononucleosis, congenital rubella, mumpsx drugs: thiazide diureticsx aplastic anaemiax leukaemiax myelodysplasiax myelosclerosisx marrow infiltration: carcinoma, lymphomax multiple myelomax megaloblastic anaemiax alcohol

Decreased platelet survival

Increased destructionx auto-immune idiopathic thrombocytopenic

purpurax secondary immune thrombocytopenia: SLE,

CLL, lymphomax post-transfusion purpurax drug-induced immune thrombocytopenia:

quinine, quinidine, sulphonamides, rifampicinx

heparinConsumptionx disseminated intravascular coagulationx thrombotic thrombocytopenic purpurax haemangioma (Kasabach-Merritt syndrome)

Platelet sequestrationx splenomegalyx hypothermia

Box 1

Complications of hypothermia

Haematologicalx polycythaemiax isolated thrombocytopenia or leucopeniax bone marrow suppressionx disseminated intravascular coagulation

Nervous systemx listlessnessx confusionx amnesiax loss of consciousnessx motor paralysisx sensory impairment

Cardiovascularx initial rise in cardiac output and blood pressure

and tachycardiax later fall in cardiac output and hypotension and

bradycardiax arrhythmias: atrial fibrillation, ventricular

fibrillationx ischaemic necrosis

Respiratoryx depressed respirationx tissue anoxiax respiratory acidosisx pneumonia

Metabolicx hyperglycaemiax hyperkalaemiax renal failure

Gastrointestinalx gastric erosionsx haemorrhagex pancreatitis

Box 2




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1 Tocantins LM. The mammalian blood platelet in health anddisease. Medicine 1938;17:155.

2 Villalobos TJ, Adelson E, Riley PA, Crosby WH. A cause ofthe thrombocytopenia and leukopenia that occurs in dogsduring deep hypothermia. J Clin Invest 1958;37:17.

3 Pina-Cabran JM. Hepatic and splenic platelet sequestrationduring deep hypothermia in the dog. Haemostasis 1974;2:235.

4 Vella MA,Jenner C, Betteridge DJ,Jowett NI. Hypothermiainduced thrombocytopenia.J R Soc Med1988;81:2289.

5 Chan KM, Beard K. A patient with recurrent hypothermiaassociated with thrombocytopenia. Postgrad Med J 1993;69:2279.

Hypotension and intractable vomiting in the firsttrimester of pregnancy

C Usalan, E zarslan

A 24-year-old woman, gravida 1, para 1, presented at 12 weeks gestation with abdominal pain,weakness, fatigue, nausea and vomiting of several days duration. Ten days earlier she had beenadmitted to another hospital with the same complaints; she had been diagnosed as having hyper-emesis gravidarum and hospitalised for 3 days, after which she was stabilized. Her medical historywas unremarkable and she had no family history of relevant illness. On initial evaluation, she wasin rather poor clinical condition and was pigmented to an abnormal degree, particularly aroundthe eyes and in the skin creases of the hands; buccal pigmentation was also pronounced. Bloodpressure was 100/60 mmHg, pulse 108 beats/min and body temperature was 38.7C. Physicalexamination was unremarkable except for dehydration and hypotension. The laboratory exami-nations are summarised in the table. The results of urinalysis were: specific gravity 1.016, pH 5,2+ protein, and urine sediment examination revealed a large number of erythrocytes and leuko-cytes. The urine contained Gram-negative bacteria.

Within two hours, the patient was confused, sweating and tachypnoeic. A repeat blood sugarlevel was 52 mg/dl. She was given a rapid infusion of intravenous 5% dextrose in 0.9% sodiumchloride, but did not improve. Arterial blood gases showed a profound metabolic acidosis. Heracid-base status was as follows: pH 7.12, HCO3 6 mmol/l, pO2 154 mmHg, pCO2 20 mmHg,baseexcess 22.

Questions

1 What is the diVerential diagnosis compatible with the history, clinical and laboratory findings?2 Which clues suggest an underlying endocrinologic illness?3 What could be a precipitating factor leading to the aggravation of the underlying illness?

Table Initial laboratory findings

Variables Patient Normal range

Haemoglobin (g/dl) 14.6 1218Mean corpuscular volume (fl) 84 80100White cell count (109/l) 16.6 3.610DiVerential count (%)

Neutrophils 78 4070Band forms 10 05Lymphocytes 12 2040

Platelet count (109/l) 452 150450Sedimentation rate (mm/h) 90 015Urea nitrogen (mg/dl) 28 823Creatinine (mg/dl) 1.0 0.61.2Total protein (g/dl) 6.8 67.8Albumin (g/dl) 3.8 3.24.8Uric acid (mg/dl) 6.2 2.77.8Glucose (mg/dl) 62 70110Sodium (mmol/l) 124 136147Potassium (mmol/l) 5.6 3.55.5Alanine transaminase (IU/l) 28 540Aspartate transaminase (IU/l) 32 833


Division of InternalMedicine, HacettepeUniversity, Ankara,

TurkeyC UsalanE zarslan

Correspondence toC Usalan, Sinan Cad. 49/12,Dikmen, 06450 Ankara,Turkey

Accepted 18 February 1999



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Answers

QUESTION 1

The diVerential diagnosis is septicaemia,severehyperemesis gravidarum, adrenal crisis, acutethyrotoxicosis, or hyponatraemic encephalopa-thy.

QUESTION 2

The classical features of adrenal crisis (box 1)

are present in this patient and the severe degreeof metabolic acidosis suggests an underlyingprecipitating factor.

QUESTION 3

The precipitating factor could be infection(sepsis, urinary tract infection, etc), trauma,surgery, withdrawal of therapy, or drugs (box2).

Outcome

The patient was treated with intravenousbicarbonate infusion, and intravenous ceftriax-one for suspected sepsis due to a urinary tract

infection. Because her clinical and laboratorystatus were suggestive of acute adrenal insuY-ciency, we performed a rapid adrenocorticot-eropin (ACTH) stimulation test with a 0.25mg intravenous bolus of tetracosactrin aftertaking blood samples for basal serum cortisoland ACTH. The test showed the basal plasmacortisol to be 5 g/dl, with no evidence of aresponse to the tetracosactrin after 30 minutes.While the serum cortisol concentration was

inappropriately low, the ACTH level waselevated. These results suggested acute adrenalinsuYciency. A bolus intravenous infusion of100 mg hydrocortisone followed by a continu-ous infusion of hydrocortisone at a rate of 10mg/h was administered. Within 3 hours, therewas restoration of blood pressure and bodytemperature and a general improvement wasseen. Therapy was maintained with a continu-ous infusion of dextrose in 0.9% saline, antibi-otics, and hydrocortisone. On day 2 of theadmission the patient continued to improve.Her pH was normal, serum lactic acid levelshad dropped significantly, and blood sugar lev-els were normal. Her electrolyte status also

improved. Maternal and foetal monitoringduring the pregnancy did not show anyprofound eVects, and she delivered vaginally ahealthy infant at 38 weeks gestation.

Discussion

A wide variety of metabolic and endocrine dis-orders may complicate pregnancy. Acute adre-nal insuYciency is an emergency and is causedby a sudden, marked decline in levels ofadrenocortical hormones.1 In some cases, thecondition first appears during the pregnancy,and in others acute adrenal insuYciency canoccur in the course of a chronic insuYciency.Rarely, acute adrenal insuYciency may be the

initial manifestation of new adrenal disease inpregnancy.2 Acute adrenal crisis during preg-nancy may mimic hyperemesis gravidarum.3

Previous case reports have suggested thatacute adrenal insuYciency does not becomemanifest until the postpartum period. Thismay be partly due to a delay in diagnosis due tothe similarity of symptoms common to preg-nancy with those of Addisons disease, andpartly because placental foetal steroid produc-tion protects the mother from crisis, althoughthis latter point is controversial.1 47 Alterna-tively, patients may develop acute adrenal crisisduring pregnancy in the presence of precipitat-ing factors such as severe infection, as in our

patient.Clinical symptoms, signs and laboratoryfindings in adrenalcrisis are presented in box 1.In cases of adrenal crisis, the serum cortisolconcentration is inappropriately low, and theACTH levels will be elevated if the disease isprimary and low in secondary adrenalinsuYciency.2 However, in the pregnant patientwith hypoadrenalism, cortisol levels can bewithin the normal range for non-pregnantpatients and the diagnosis will be based on thelack of a rise of plasma cortisol after ACTHstimulation.5 6 In a patient with no known his-tory of adrenal insuYciency, it can be diYcult

Classical clinical features of adrenalcrisis

x symptoms: rapid worsening of precedingsymptoms; severe weakness, fatigue, confusion,abdominal pain, nausea, vomiting, diarrhoea

x signs: fever, low blood pressure, dehydration,skin pigmentation

x laboratory findings: hyperkalaemia,hyponatraemia, high blood urea nitrogen,

hypoglycaemia, metabolic acidosis (sometimes),low serum cortisol with high ACTH level(primary adrenal insuYciency), or low serumcortisol with low ACTH level (secondary adrenalinsuYciency)

Box 1

Precipitating causes of adrenal crisis

x in diagnosed chronic adrenal insuYciency:withdrawal of therapy, infection, trauma, surgery,dehydration, drugs

x in a normal person: blockage of hormonesynthesis (eg, by drugs such asaminoglutethimide, ketoconasole, etc, or as aresult of surgery, eg, adrenalectomy), orincreased degradation of hormones due to drugssuch as rifampicin, dilantin, phenobarbital, etc

x following massive bilateral adrenal haemorrhageas may occur in severe infection (septicaemia,meningiococcaemia, etc), HIV, coagulationdisorders, intra-abdominal surgery, adrenal

metastasisx hypophyseal causes: surgery, radiotherapy,

apoplexy, thyroid hormone replacement withoutsteroids in panhypopituitarism

Box 2




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to distinguish between Addisons disease andacute adrenal insuYciency, especially duringthe first trimester of pregnancy. It is importantto remember that mild cases can go undetectedduring pregnancy and become manifest as cri-ses at parturition or in the presence of other ill-ness such as urinary tract infection, dehydra-tion, pre-eclampsia, etc.6 7

In conclusion, acute adrenal insuYciency isa rare disorder whose diagnosis can be diYcult

during pregnancy. On the other hand, it isassociated with high maternal and/or foetal

morbidity and mortality if allowed to progress.For this reason, early recognition and interven-tion are critical.

Final diagnosis

Acute adrenal insuYciency precipitated by uri-nary tract infection.

Keywords: adrenal insuYciency; pregnancy; urinary

tract infection; vomiting; hypotension

1 Albert E, Dalaker K, Jorde R. Addisons disease andpregnancy. Acta Obstet Gynecol Scand 1989;68:1857.

2 Orth DN, Kovacs WJ, Debold CR. The adrenal cortex. In:Wilson JD, Foster DD, eds. Williams Textbook of Endocrinol-ogy, 8th edn. Philadelphia: WB Saunders, 1992; pp489619.

3 Mastrogiannis DS, Whiteman VE, Mamopoulos M,Salameh W. Acute endocrinopathies during pregnancy.ClinObstet Gynecol1994;37:7892.

4 McGill IG. Addisons disease presenting as crisis in thepuerperium. BMJ1971;ii:5667.

5 Pirist G, Posadino PM, Virdis GP, Lai G. Addisons diseasein pregnancy. Clin Exp Obstet Gynecol1984;4:15860.

6 Seaward PGR, Guidozzi F, Sonnendecker EWW. Addiso-nian crisis in pregnancy. Br J Obstet Gynaecol1989;96:134850.

7 Simcock MJ. Addisons disease in pregnancy. Med J Aust1966;1:21920.

Fatal complication of coincidental operativefinding

J S McCourtney, S Karim, M Rahilly, R Dalling

A 65-year-old man with an established diagnosis of Crohns colitis presented as an emergencywith peritonitis and free subdiaphragmatic air on erect chest X-ray. At laparotomy, a grossly dis-tended colon was noted, with two sites of perforation at the rectosigmoid and splenic flexureareas. Multiple non-dilated jejunal diverticula were noted coincidentally. Subtotal colectomy withcross-stapling of the rectum and formation of an end ileostomy was performed. He made anuneventful early postoperative recovery but during the fourth postoperative week he developedintermittent, colicky abdominal pain, vomiting and reduced ileostomy output. Subacute smallbowel obstruction secondary to adhesions was diagnosed after clinical and radiological examina-tion. Conservative treatment with intravenous fluids and nasogastric decompression produced aninitial improvement but on the 32nd postoperative day the patient suddenly collapsed and diedfrom a cardiorespiratory arrest secondary to acute renal failure and septic shock. At post-mortemexamination,evidence of proximal jejunal diverticular disease with signs of acute diverticulitis andperforation were noted along with widespread peritonitis. No histological features of Crohns dis-ease were identified in the segment of perforated jejunum, or elsewhere in the bowel, and theinflammatory process was centred around the diverticulum (figure).

Questions

1 What is the incidence of small boweldiverticula?

2 What is the aetiology of small boweldiverticulosis?3 List the common pre-operative clinical mani-

festations of small bowel diverticula.

Figure Perforated jejunal diverticulum lined byinflamed granulation tissue. Some residual diverticularmucosa is visible on the right (arrow). The lumen ofthe perforation is indicated by N


Stobhill NHS Trust,Balornock Road,Glasgow G21 3UW, UKDivision of Surgery

J S McCourtney

R DallingDepartment ofPathologyS KarimM Rahilly

Correspondence toMr JS McCourtney, StMarks Hospital, NorthwickPark & St Marks NHSTrust, Watford Road,Harrow, Middlesex HA13UJ, UK




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Answers

QUESTION 1

Small bowel diverticula are uncommon, occur-ring in 0.52.3% of small bowel contraststudies1 and 0.061.3% of post-mortemexaminations.2 They manifest most frequentlyin the sixth and seventh decades of life andwere thought to occur more commonly inmales,3 although one study has shown a

preponderance for females.1

QUESTION 2

Non-Meckelian small bowel diverticula arethought to be acquired pulsion defects causedby possible underlying abnormalities in peri-stalsis which in turn produce segmentally highintraluminal pressures.4 Jejunal diverticulosishas been described in association with systemicsclerosis and visceral neuropathy or myopathy.5

The diverticula of mucosa and submucosaemerge at natural weak sites where mesentericvessels penetrate the intestinal wall explainingthe observation that they are most commonlylocated at the mesenteric side of the bowel.6

Small bowel diverticula occur more frequentlyin the proximal jejunum and distal ileum wherethe vasa recti of greatest diameter are found. 6

QUESTION 3

Common pre-operative complications associ-ated with jejuno-ileal diverticula include haem-orrhage, obstruction, diverticulitis, perforation,malabsorption, anaemia and chronic vagueabdominal pain (box 1).6 The most commonclinical signs include hyperactive bowelsounds, hyper-resonance with epigastric per-cussion and vague epigastric discomfort onpalpation.1 Complications of small boweldiverticula may manifest in diVerent ways withvarying frequencies according to their anatomi-

cal location (box 2).

Discussion

There are no reliable diagnostic tests to confirmthe presence of small bowel diverticula. Erectabdominal X-rays may show airfluid levelsthroughout the small bowel. The classic triad offeatures associated with jejuno-ileal diverticulardisease consists of vague abdominal pain, anae-mia and dilated loops of small bowel onabdominal X-ray.7 If the presence of small boweldiverticula is suspected then a small bowelbarium follow-through study or enteroclysis canbe performed. While the former easily demon-

strates large diverticula, smaller ones may not beidentified because of inadequate filling withcontrast or due to extrinsic abdominalcompression.8 Thus, a negative barium follow-through study does not exclude small boweldiverticular disease. Workers have shown thatenteroclysis has a greater sensitivity at detectingdiverticulae.8 This investigation should be bornein mind for the patient with persisting abdomi-nal symptoms who has undergone negativeendoscopic and standard contrast studies ofboth upper and lower gastrointestinal tracts.1

Small bowel diverticulitis should also be in-cluded in the diVerential diagnosis of a small

bowel inflammatory mass demonstrated bycomputed axial tomography.9 Upper gastro-intestinal endoscopy and laparotomy are the twoother main methods for diagnosing the presenceof small bowel diverticula.5

In jejunal diverticular disease, obvious perfo-ration, bleeding, or mechanical complicationsrequire resection with primary anastomosis.Failure of medical therapy in cases of blind

loop syndrome or nonmechanical obstructionfrom severe jejunal dysmotility in the presenceof diverticula may also benefit from resection ofthe aVected segement. A dilated, hypertro-phied segment of jejunum with large diver-ticula, suggestive of a progressive form ofdiverticular disease, which is found coinciden-tally at laparotomy should also be resected.6

The majority of ileal diverticulae on the otherhand do not require operative intervention,except in cases of perforation, bleeding, orobstruction which occur less frequently than injejunal disease. Excision of a coincidental, soli-tary, non-Meckelian ileal diverticulum in anadult is not warranted.1 6

Jejunal diverticula are probably more com-

mon than reported.7 They may be easilyoverlooked at operation as they are frequentlypresent between the leaves of mesentery.4 Inasymptomatic jejuno-ileal diverticular disease,noted coincidentally at operation or duringradiological investigations, the risk of complica-tions ensuing was 18% at a mean follow-up of4.8 years in one series.10 The above caseillustrates that serious complications of jejunaldiverticular disease can arise in the earlypostoperative period. To date, there have beenno reports in the literature of jejunal diverticu-lar disease causing early postoperative compli-cations. The coincidental finding of previouslyasymptomatic jejunal diverticula at laparotomy,

whilst uncommon, should always be consideredin the diVerential diagnosis of subsequent earlypostoperative symptoms suggestive of subacutesmall bowel obstruction. Failure to do so, withattention and management focussed on morelikely causes for such symptoms may mask thetrue diagnosis with fatal consequences.

Final diagnosis

Fatal perforated acute jejunal diverticulitiscomplicating postoperative recovery followingemergency subtotal colectomy for perforatedfulminant Crohns colitis.

Clinicalmanifestationsof small bowel

diverticulosis

v asymptomaticv bleedv obstructionv perforation +/-

abscess formationv chronic abdominal

painv volvulusv enterolith formationv anaemiav malabsorption

Box 1

Learning points

v the majority of small bowel diverticula areasymptomatic

v jejuno-ileal diverticula are more likely to havecomplications than duodenal diverticula

v duodenal diverticula are more likely to bleedv jejuno-ileal diverticula are more likely to

perforate/form abscessesv asymptomatic small bowel diverticula can cause

postoperative complications

Box 2




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Keywords: jejunoileal diverticulitis; diverticulitis; small bowel perforation; Crohns disease; enteroclysis

1 Wilcox RD, Shatney CH. Surgical implications of jejunaldiverticula. South Med J1988;81:138691.

2 Chow DC, Babaian M, Taubin HL. Jejunoileal diverticula.Gastroenterologist1997;5:7884.

3 BensonRE, Dixon CF, Waugh JM.NonMeckeliandiverticulaof the jejunum and ileum.Ann Surg1943;118:37793.

4 Akhrass R, YaVe MB, Fischer C, Ponsky J, Shuck JM.Small-bowel diverticulosis: perceptions and reality. J AmColl Surg1997;184:3838.

5 Krishnamurthy S, Kelly MM, Rohrmann CA, SchuZerMD. Jejunal diverticulosis: a heterogeneous disorder causedby a variety of abnormalities of smooth muscle or myentericplexus. Gastroenterology 1983;85:53847.

6 Longo WE, Vernava AM. Clinical implications of jejunoilealdiverticular disease. Dis Colon Rectum 1992;35:3818.

7 Nobles E. Jejunal diverticula. Arch Surg1971;102:1724.8 Maglinte D, Chernish S, DeWeese R. Acquired jejunoileal

diverticular disease: subject review. Radiology 1986;158:57780.

9 Greenstein S, Jones B, Fishman EK, Cameron JL,

Siegelman SS. Small bowel diverticulitis: CT findings. AJR1986;147:2714.10 Tsiotos GG, Farnell MB, Ilstrup DM. Nonmeckelian

jejunal or ileal diverticulosis: an analysis of 112 cases.Surgery 1994;116:72632.

Multinodular goitre, dysphagia and nodularshadows in the lung

A Bhansali, S Bhadada, R Kochhar, R Muralidharan, R J Dash

A 41-year-old woman presented in December 1997 with dry cough with streaky haemoptysis of15 days duration. She had had a goitre for the last 25 years and had been diagnosed as havingthyrotoxicosis on clinical and biochemical grounds (triiodothyronine (T3) 2.8 ng/ml and thyrox-ine (T4) 220 ng/ml) in September 1995. She had received carbimazole for 6 months and remainedasymptomatic thereafter.

On examination, her body mass index (BMI) was 24.1 kg/m2. She had no pallor, lymphaden-opathy, clubbing, oedema or hyperkeratosis. Her pulse was 96 beats/min regular, blood pressure110/70 mmHg. She denied a history of fever, weight loss, loss of appetite, or recent increase ingoitre size, but had been dysphagic to solids for 23 days. She was a non-smoker and never con-sumed alcohol. She had a firm, grade III (WHO) non-tender multinodular goitre with retroster-nal extension. Systemic examinations were normal except for diVuse bilateral rhonchi. Herhaemogram and serum biochemistry, including thyroid hormone profile (T3 0.86 ng/ml, T4 78ng/ml, thyroid-stimulating hormone 0.3 U/ml) were normal. Ultrasonography of thyroid

confirmed the multinodular goitre with retrosternal extension with some areas of necrosis andspecks of calcification. Ultrasound-guided fine needle aspiration cytology (FNAC) revealed fol-licular cells with nuclear grooving and psammoma bodies. Her radiological profile is shown infigure 1. Bronchoscopy could not be completed because of laryngeal spasm. Computed tomog-raphy (CT)-guided FNAC from pulmonary lesions was inconclusive.

Questions

1 What is shown in figure 1?2 What is your diagnosis?3 What would be the approach to confirm the

diagnosis?

Figure 1 Clinical and radiological profile of thepatient


Postgraduate Institute

of Medical Educationand Research,Chandigarh 160012,India

Department ofEndocrinologyA Bhansali

S BhadadaR MuralidharanR J DashDepartment ofGastroenterologyR Kochhar

Correspondence to ProfRJ Dash

Accepted 2 September 1998



10/19

Answers

QUESTION 1

Figure 1 shows bilateral multiple nodular opaci-ties in lung fields on chest X-ray, furthersubstantiated on high-resolution CT. CervicalX-ray shows a large goitre with retrosternalextension and calcification.

QUESTION 2

With the available clinical and radiologicalprofile, a diagnosis of euthyroid multinodulargoitre with retrosternal extension and meta-static lesions in the lung can be made. Furtherwork-up to determine the primary source,whether from thyroid or elsewhere, is required.

In the presence of a long-standing euthyroidgoitre, the possibility of thyroid carcinoma withpulmonary metastases is high. However, multi-nodularity and absence of cervical lymphaden-opathy in the face of pulmonary metastasesdecreases this probability.1 The presence ofnuclear grooving and psammoma bodies canalso be seen in long-standing multinodulargoitre.2 3

QUESTION 3

The clue to the primary site of malignancycame from the widening of the prevertebralspace in the cricopharyngeal region and thepresence of malignant keratinizing squamouscells in the sputum. Barium swallow showed anarrowed segment with irregular mucosa in theupper third of the oesophagus (figure 2).Fibre-optic endoscopy revealed a growth in theupper cervical oesophagus. Brush cytologysmears from the growth confirmed squamouscell carcinoma. Her disease was classified asstage IV (any T, any N & M1) according to theTNM staging system. She received cisplatinand etoposide cyclically (3 days treatment at

4-week intervals) and palliative radiationtherapy.

Discussion

Patients with oesophageal carcinoma with meta-static disease usually present with dysphagia,odynophagia, weight loss and marked emacia-tion.4 Our patient had maintained reasonablygood health (BMI 24.1 kg/m2, haemoglobin10.6 g/dl) at presentation.She experienced tran-

sient dysphagia to solids, attributable to com-pression from the multinodular goitre.Dysphagia, however, is not a presenting mani-festation of upper cervical oesophageal carci-noma as this segment of the oesophagus iscapacious.

Squamous cell carcinoma of the oesophagus,oropharyngeal leucoplakia and palmo-plantartylosis are genetically linked. However, exceptfor a positive family history of dysphagia in twoof her first degree relatives, there were no indi-cations for this syndrome in our patient.5 Therelative frequencies of squamous cell carci-noma in the upper, middle and lower portionsof oesophagus are 17%, 55% and 33%, respec-

tively. Based on gross appearance, the fungat-ing variety accounts for 60%, ulcerative type25%, and infiltrative type 15%.5

Chronic cough in squamous cell carcinomaof the oesophagus occurs due to aspiration,tracheo-oesophageal fistula, or pulmonary me-tastases. In view of ready access to bloodvessels, haematogenous metastases to lungs,liver, kidneys and bone are common.5

Chemotherapy combined with palliative ra-diation yields better results than radiationtherapy alone in patients with localised carci-noma of the oesophagus.5 Only about 20% oflesions are resectable but surgical interventiondoes not lead to increased survival except when

the lymph nodes are not involved. The overallprognosis is poor, with 5-year survival rate lessthan 5%.5

Final diagnosis

Euthyroid multinodular goitre and squamouscell carcinoma of oesophagus with pulmonarymetastases.

Keywords: multinodular goitre; squamous cell carci-noma; oesophageal carcinoma; pulmonary metastases

Figure 2 Barium studyshowing a narrowedsegment with irregularmucosa in the upperthird of the oesophagus

Learning points

x when metastatic pulmonary lesions are seen in apatient with long-standing euthyroid nodulargoitre without enlarged lymph nodes in the neck,a non-thyroidal primary source needs to beconsidered

x nuclear grooving and psammoma bodies, whichare considered a cytological hallmark of papillarycarcinoma, can also be present in long-standingmultinodular goitre

x upper oesophageal carcinoma with lungmetastases may present with subtlesymptomatology




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1 De Groot LJ,Kaplan EL,McCormick M, Straus FH. Natu-ral history, treatment and course of papillary thyroidcarcinoma. J Clin Endocrinol Metab 1990;1:41424.

2 Gould E, Watzak L, Chamizo W, Saavedra JA. Nucleargrooves in cytologic preparations: a study of the utility ofthis feature in the diagnosis of papillary carcinoma. ActaCytol 1989;33:1620.

3 Klinck GH, Winship T. Psammoma bodies and thyroid car-cinoma. Cancer1959;12:6569.

4 Krevsky B. Tumors of the esophagus. In: Haubrich WS,SchaVner F, Berk JE, eds, Bockus Gastroenterology.Philadelphia: WB Saunders, 1995; pp 53457.

5 Gore RM. Esophageal cancer: clinical and pathologicfeatures. Radiol Clin North Am 1997;35:24363.

An unusual cause of dysphagia

Israel Gotsman, Paul Mogle, Michael Y Shapira

A 74-year-old woman was referred for evaluation of dysphagia and weight loss. She had a historyof dysphagia for solid foods, which had become worse over the past year. She had lost 5 kg inweight but her appetite was good. She suVered from mild heartburn without pain and her bowelmovements were normal. Gastroscopy showed mild gastritis with a positive culture forHelicobacter pylori. A short course of triple antibiotic therapy and omeprazole was prescribed.Follow-up endoscopy appeared normal. Her heartburn improved but the dysphagia persisted.

She had had a myocardial infarction 4 years earlier, complicated by acute mitral regurgitationdue to papillary muscle rupture. This was repaired by urgent surgery. A post-operative echocar-

diogram showed slight left ventricular enlargement with decreased global function and moderatemitral regurgitation. She was treated with frusemide and digoxin and had minimal complaints ofheart failure. She also suVered from type II diabetes and hypertension controlled by atenolol.

Physical examination revealed a thin woman with normal vital signs. The thyroid and lymphnodes were not enlarged. Heart sounds were normal with a 3/6 blowing systolic murmur at theapex radiating to the axilla. Breath sounds were reduced at the base of the right lung. Theabdomen was mildly distended but not tender. The liver and spleen were not enlarged, peristalsiswas normal. There was mild oedema of both legs. Peripheral pulses were present. Laboratorystudies showed mild elevation of alkaline phosphatase and -glutamyl transpeptidase. Albuminand cholesterol levels were normal. Haemoglobin was 14.5 g/dl. Electrocardiogram showed sinusrhythm with a non-specific intraventricular block. Chest X-ray (figure 1) demonstrated anenlarged cardiac silhouette with a small right pleural eVusion.

Questions

1 What is evident on the lateral chest X-raythat may explain the dysphagia?

2 What further examinations should be madein order to make a diagnosis?

Figure 1 Lateral chest X-ray


Hadassah UniversityHospital, HebrewUniversityHadassah

Medical School, POBox 12000, Jerusalem,Israel 91120Division of MedicineI GotsmanM Y ShapiraDepartment ofRadiologyP Mogle

Accepted 17 March 1999



12/19

Answers

QUESTION 1

The lateral X-ray (figure 1) shows an enlargedleft atrium, consistent with mitral valve disease.An enlarged left atrium can cause dysphagia tosolids due to external compression of theoesophagus. This rare cause of dysphagia isknown as cardiovascular dysphagia.1

QUESTION 2

The patients predominant complaint was dys-phagia to solids. This usually implies mechanicalobstruction of the oesophagus. Intrinsic lesionsthat obstruct the oesophagus include pepticstricture, lower oesophageal (Schatzkis) ring, oroesophageal carcinoma. Extrinsic lesions in-clude vascular abnormalities,mediastinal abnor-malities or cervical osteoarthritis. The first stepin the diagnosis is to demonstrate the anatomy ofthe oesophagus by barium swallow. Videobarium swallow showed normal movement ofthe oropharyngeal muscles and normal peristal-sis of the oesophagus. A pulsatile bulge wasnoted in the distal posterior oesophagus consist-

ent with external pressure from an enlarged leftatrium (figure 2). No reflux was apparent. Acomputed tomography (CT) scan of the chestrevealed an enlarged left atrium compressing theoesophagus. A space-occupying lesion was notevident. A manometric study of the oesophagus,performed to complete the diagnosis andexclude oesophageal dysmotility due todiabetes, revealed normal peristaltic pressures inthe oesophagus. A transthoracic echocardio-gram showed an enlarged left ventricle withmoderate global dysfunction and severe mitralregurgitation. The left atrium was enlarged.

The patient was treated for mitral regurgita-tion with after-load reduction and diuretics.She improved, the dysphagia gradually sub-sided, and she gained weight. The final diag-nosis was cardiovascular dysphagia.

Discussion

Cardiovascular dysphagia is an uncommonclinical entity that is often unrecognised (box1).14 The left atrium is a mid-line posteriorchamber of the heart, in front of the oesoph-agus. Left atrial enlargement causes dysphagiaby external compression of the oesophagus. Amanometric study of left atrial enlargement5

showed that mechanical compression causes alocalised high pressure zone in the oesophagusat the level of atrium with pressure oscillationscorresponding to the electrocardiogram.

Other suggested mechanisms for cardiovas-cular dysphagia include deranged peristalsisdue to local ischaemia of the oesophagealmucosa and nerve plexus caused by theelevated external pressure (which was not

evident in our case). Prolonged exposure of thedistal oesophagus to a high external pressuremay cause proximal oesophageal muscle fa-tigue and dysphagia.

This patient presented with dysphagia due tomechanical compression of the oesophagus byan enlarged left atrium from mitral incompe-tence. Reduction of mitral incompetence byafterload reduction ameliorated the dysphagia.Cardiovascular dysphagia is uncommon, butshould be suspected in patients with anenlarged left atrium.

Figure 2 Barium swallow of the oesophagus showingexternal compression of the distal posterioroesophagus from an enlarged, left atrium (arrow). Therepaired annulus of the mitral valve can be seen due toa ring placed there

Causes of cardiovascular dysphagia

x left atrial enlargementx aberrant left or right subclavian arteryx tortuous atherosclerotic aortax thoracic aortic aneurysm

Box 1

Cardiovascular dysphagia (left atrialenlargement)

x occurs in rheumatic heart patients with mitralvalve disease and enlarged left atrium

x is caused by luminal obstruction due tooesophageal compression by an enlarged leftatrium

Diagnosis:x barium swallow: posterior displaced oesophagus,

a smoothly compressed oesophageal lumen,delayed passage of barium

x endoscopy: pulsatile bulging mass in themid-oesophagus, normal mucosa

x oesophageal manometry: elevatedmid-esophageal base line pressure withsuperimposed cyclic pressure wavessimultaneous with the ECG

Box 2




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Final diagnosis

Cardiovascular dysphagia.

Keywords: cardiovascular dysphagia; mitral valvedisease; weight loss

1 Cappell MS. Manometric findings in dysphagia secondaryto left atrial dilatation. Dig Dis Sci 1991;36:6938.

2 McNally PR, Rak KM.Dysphagia lusoria caused by persist-ent right aortic arch with aberrant left subclavian artery anddiverticulum of Kommerell. Dig Dis Sci1992;37:1449.

3 Berenzweig H, Baue AE, McCallum RW. Dysphagia lusoria:report of a case and review of the diagnostic and surgicalapproach. Dig Dis Sci1980;25:6306.

4 Mittal RK, Siskind BN, Hongo M, et al. Dysphagia aortica:clinical, radiological and manometric findings. Dig Dis Sci1986;31:37984.

5 Cappell MS. Endoscopic, radiographic and manometricfindings associated with cardiovascular dysphagia. Dig DisSci 1995;40:16676.

Lessons from a case of tetanus in an elderlywoman

J Kwan, S Lim, S C Allen

A healthy 84-year-old Caucasian woman fell whilst gardening at home and lacerated her right leg.The wound was deep and heavily contaminated with soil and gravel. She attended an Accidentand Emergency (A&E) department for treatment and the wound was cleaned and dressed. Shehad never received tetanus vaccination in the past and at the A&E department she was given onedose of tetanus toxoid. She was sent home without any follow up. Over the next week she becameincreasingly immobile and complained of dysphagia, stiV neck and breathlessness. Her generalpractitioner admitted her into a nursing home with a presumed diagnosis of stroke. Her conditiondeteriorated over the next week and she was admitted into hospital with a provisional diagnosis ofsepticaemia from her original leg wound.

On admission she was dehydrated, hypoxic, hypertensive (230/125 mmHg) and tachycardic.She had generalised muscular spasms including trismus and neck stiVness as well as hyperexcit-ability to touch and noise. Spirometry showed a restrictive lung defect. Initial laboratory resultswere as follows: leucocytes 15.5 109/l, platelets 653 109/l, urea 15.4 mmol/l, creatinine 227mol/l and corrected calcium 2.48 mmol/l. A diagnosis of tetanus was made and she was trans-ferred to the Intensive Care Unit where she was managed according to recommended guidelinesfor the treatment of tetanus. Her pulse and blood pressure observations were monitored closely

(table).

Questions

1 Comment on the original A&E department management of this patient.2 What caused the abnormally unstable blood pressure and pulse?3 What are the most important aspects of the management of tetanus?4 What is the diVerential diagnosis and how may the initial diagnosis be confirmed?

Table Blood pressure and pulse observations on day 7

Time Blood pressure (mmHg) Pulse (beats/min)

01:00 122 / 50 8004:00 90 / 40 7007:00 125 / 60 8012:00 150 / 50 10020:00 160 / 70 12022:00 130 / 70 88


Royal Bournemouth &ChristchurchHospitals NHS Trust,Castle Lane East,

BournemouthBH7 7DW, Dorset, UK

J KwanS LimS C Allen

Correspondence toDr Joseph S K Kwan,Research Fellow,Christchurch Hospital,Fairmile Road, ChristchurchBH23 2JX, Dorset, UK




14/19

Answers

QUESTION 1

The patients wound was heavily contaminatedwith soil and gravel and, by definition,1 thewound was tetanus prone. The patient hadnever received vaccination against tetanus.According to the Department of Healthguidelines,1 patients who have never been vac-cinated and have a tetanus-prone wound

should receive an immediate dose of humantetanus immunoglobulin followed by a com-plete primary course (three doses) of tetanustoxoid. The patient in our case, however, hadnot been given a dose of human tetanus immu-noglobulin and was discharged without follow-up.

QUESTION 2

The labile blood pressure and tachycardia weremost likely a result of a combination of pain(from both the wound and muscular spasms),anxiety, sepsis and autonomic dysfunction.Autonomic dysfunction is common in patientswith tetanus and it may also present as pyrexia,

dry mouth, profuse sweating, urinary retentionand cardiac arrhythmia, which is associatedwith a mortality of over 50%.3 The unstableblood pressure of autonomic dysfunction isdiYcult to control, but some benefit has beendemonstrated with the use of propanolol andclonidine.2 4

QUESTION 3

The immediate management for severe tetanusshould include surgical debridement of thewound, intravenous metronidazole and humantetanus immunoglobulin, antispasmodictherapy, early mechanical ventilation andtracheostomy. Nursing the patient in a quietroom prevents muscular spasms from hyperex-

citability. Metronidazole has been shown to bemore eVective than penicillin in preventingdeath when used in the treatment of tetanus. Ithas been postulated that penicillin is a centrallyacting GABA antagonist, and may therefore actsynergistically with tetanospasmin in produc-ing muscular spasms.2 Early enteral orparenteral feeding is also recommended be-cause of the high caloric requirement second-ary to hypermetabolism in patients withtetanus. Subsequently, since the amount oftetanus toxin released is insuYcient to inducean adequate immune response, the patient willalso need immunisation with tetanus toxoid.

QUESTION 4

Tetanus is a clinical diagnosis. The combina-tion of a history of injury with a contaminatedwound, muscular spasms (with or without risussardonicus, trismus or opisthotonus) and signsof sepsis should raise the possibility of thediagnosis. Tetanus immunisation history is apoor predictor of immune status in elderly

people with a positive predictive value of 50%and a negative value of 76%.5 It is important toexclude other diVerential diagnoses such ashypocalcaemic tetany, orofacial infection, sta-tus epilepticus and drug-induced dystonia (eg,phenothiazines, metoclopramide). Strychninepoisoning can mimic tetanus but it does notcause trismus or abdominal rigidity. Culture ofClostridium tetani from the aVected wound ispositive in less than 30% of cases. Moreover, apositive culture is not diagnostic since not allpatients with wound colonisation develop teta-

nus. Serological tests may be used to detect thelevel of anti-tetanus antibody but their use indiagnosis is limited.

Discussion

In the UK, tetanus is a disease of the elderlypopulation and elderly women are most at riskprobably due to a combination of inadequateimmunity and the increasing risk associatedwith a physically active old age. Tetanus immu-nisation began in 1938 for those who served inthe Armed Forces. In 1961, primary tetanusimmunisation of infants was introduced na-tionally. In 1970, tetanus immunisation wasrecommended as part of the routine manage-

ment of all wounds. Between 1984 and 1995,there were 145 notified cases of tetanus inEngland and Wales; 53% of the cases were inindividuals over 65 years and two-thirds ofthem were women.

Although tetanus is now rare, it is prevent-able by undertaking appropriate wound careand tetanus prophylaxis as recommended bythe Department of Health.1 Our case illustratesthat admission of any elderly person into a pri-vate sector nursing home without a properdiagnosis may delay investigations and treat-ment of the underlying illness. The mostimportant step in diagnosing tetanus is themaintenance of a high suspicion for the

disease.Final diagnosis

Autonomic dysfunction in an elderly patientwith severe tetanus.

Keywords: tetanus; elderly; autonomic dysfunction

Learning points

x tetanus is a disease of the elderly populationx tetanus is preventable with appropriate wound

care and prophylaxisx admission into a nursing home without a proper

diagnosis should be avoidedx autonomic dysfunction can cause labile blood

pressure and pulse in tetanus




15/19

1 Salisbury D, Begg NT, eds. Tetanus. In: Immunisationagainst infectious disease. London: HMSO, 1996; pp 20513.

2 Ernst ME, Klepser ME, Fouts M, Marangos MN. Tetanus:pathophysiology and management. Ann Pharmacother1997;31:150713.

3 Moughabghab AV, Lefilliatre P, Fenides A, Provot F.Management of autonomic dysfunction in severe tetanus:the use of fentanyl. Can J Anaesthesia 1995;42:955.

4 Gregorakos L, Kerezoudi E, Dimopoulos G, Thomaides T.Management of blood pressure instability in severe tetanus:the use of clonidine. Intens Care Med1997;23:8935.

5 Murphy SM, Hegarty DM, Feighery CS, Bernard Walsh J,Williams Y, Coakley DP. Tetanus immunity in elderlypeople. Age Ageing1995;24:99102.

Unusual presentations to a lipid clinic

M A Crook, A Mukherjee, K Marshall.

We describe the case-histories of two young men who both presented to a lipid clinic with mixedhyperlipidaemias.

Case 1A 33-year-old man had been referred to the lipid clinic by the dermatology unit because of pla-nar xanthoma that been found on a skin biopsy. Apart from previously seeing the Ear, Nose andThroat department for an 18-month history of snoring, there was no other relevant medical his-tory nor other symptoms. He was a non-smoker, rarely took alcohol and at the time was not tak-ing any medication. On examination he was overweight with a body mass index (BMI) of 29kg/m2. His blood pressure at presentation was 140/90 mmHg and pulse 72 beats/min. He wasclinically euthyroid and had planar xanthoma on his hands and tuberous xanthoma on his elbows.He had been commenced on a lipid-lowering diet and a number of investigations were performed.His fasting serum cholesterol was 12.6 mmol/l, triglyceride 5.3 mmol/l and high-density lipopro-tein (HDL) cholesterol 1.31 mmol/l. Plasma urea, creatinine and fasting glucose were normal, aswere his liver function tests. However, thyroid function tests showed a thyroid-stimulatinghormone of 257.7 mU/l (normal range 0.35.0) and free thyroxine of 1.1 pmol/l (10.319.4).Anti-thyroglobulin and antithyroid peroxidase antibodies were 396 and 240 IU/ml, respectively(0180 and 050). Apolipoprotein (apo) E phenotype performed by Western blotting showed himto be a E2/E2 homozygote. On a lipid-lowering diet instigated by a dietician and thyroxine 150g per day his fasting serum cholesterol improved to 5.11 mmol/l and triglyceride to 2.15 mmol/land his thyroid function tests were rendered normal.

Case 2

A 33-year-old man was referred to the lipid clinic by his general practioner because of hyperlipi-daemia and a family history of premature coronary heart disease. His mother had hypothyroid-ism and pernicious anaemia. On examination he was euthyroid and had no lipid stigmata. HisBMI was 31 kg/m2, blood pressure was 150/92 mmHg, and pulse 72 beats/min. Initial fastingserum cholesterol was 16.49 mmol/l, triglyceride 20.83 mmol/l and HDL-cholesterol 0.59mmol/l. His renal and liver function were normal, as was a fasting plasma glucose. However, hisTSH was 37.6 mU/l and free T4 8.5 pmol/l. Anti-thyroglobulin and anti-thyroid peroxidase anti-bodies were 2153 and 711 IU/ml, respectively. He was commenced on thyroxine which resultedin normalisation of his thyroid function, although his fasting serum cholesterol remained elevatedat 11.8 mmol/l with triglyceride of 16.7 mmol/l. His apoE phenotype also by Western blotting wasE2/E2. He was subsequently started on bezafibrate 400 mg (MONO) nocte with considerableimprovement in his lipids.

Questions

1 What lipid disorder do these two patients display?2 How can the diagnosis be confirmed?3 What other conditions is this lipid disorder associated with?


Department ofChemical Pathology

and Lipid Clinic,University LewishamHospital, Lewisham,London, UKCrook MAMukherjee AMarshall K

Correspondence toDr MA Crook, Departmentof Chemical Pathology, 5thfloor tower, Guys Hospital,London SE1 9RT, UK




16/19

Answers

QUESTION 1

They were both diagnosed as having a type IIIhyperlipoproteinaemia. Type III hyperlipopro-teinaemia, also called broad beta or remnantdyslipidaemia, is a rare familial hyperlipidae-mia whose recognition is important as it is usu-ally responsive to therapy. The treatment isusually dietary, in conjunction with a lipid-

lowering drug using a fibrate or possibly astatin.1 The palmar striae (palmar xanthomata)are considered pathognonomic for the disorderand occur in less than 50% of patients buttubero-eruptive xanthomata, typically on theelbows and knees, as well as xanthelasma andcorneal arci have been described in this condi-tion. Peripheral vascular disease is a typicalfeature of this hyperlipidaemic disorder as ispremature coronary artery disease. Serum lipiddetermination will frequently reveal hypercho-lesterolaemia and hypertriglyceridaemia, oftenin similar molar proportions. Serum HDLcholesterol is usually low. Serum low-densitylipoprotein (LDL) cholesterol may also be low

due to the fact that there is reduced conversionfrom intermediate-density lipoprotein parti-cles, although LDL cholesterol may also benormal or elevated.24

The underlying biochemical defect is one ofa reduced clearance of chylomicron and VLDLremnants. This is also known as broad betahyperlipidaemia because of the characteristicserum lipoprotein electrophoretic pattern oftenobserved (the broad beta band that is seenbeing predominately remnant particles).

An association with type III/broad betahyperlipidaemia and homozygousity for apoE2or apoE2 variants has been described. ApoEshows three common allelles, E2, E3, and E4,coded for on chromosome 19 and which are

important for the binding of remnant particlesto the remnant receptor.

The mechanism for the disorder seems to bethat apoE2-bearing particles have poor bindingto the apoB/E (remnant) receptor and thus arenot eVectively cleared from the circulation.

QUESTION 2

Serum lipoprotein electrophoresis can showthe classic type III picture with a broad betaband composed of remnant particles, althoughthis is not always present. An association oftype III broad beta hyperlipidaemia withhomozygousity for apolipoprotein E2 has beendescribed and thus apoE phenotyping or geno-

typing by a specialised laboratory can beuseful, although some patients with broad betahyperlipidaemia can show other apoE pheno-

types or variants. Another investigation thatcan be useful in establishing the diagnosis isultracentrifugation to separate the lipoproteinparticles. The cholesterol of the VLDL parti-cles is then quantitated and expressed as a totalof the serum triglyceride concentration. Inmolar terms, normal individuals show a ratio ofbelow 0.30 while ratios over 0.30 are morelikely in broad beta hyperlipidaemia, particu-larly if this is nearer 0.60.

QUESTION 3

It is becoming apparent that it is not just inher-iting the apoE2 genotype that is important indeveloping broad beta hyperlipidaemia. Theprevalence of the apoE2/E2 genotype is about1 in 100 in the general population, yet onlyabout 1 in 510 000 individuals manifest typeIII hyperlipidaemia. A concurrent increase inserum VLDL also seems necessary for thecondition to be expressed, such as might occurin diabetes mellitus, hypothyroidism or obesity.Some patients may show either an autosomalrecessive or dominant mode of inheritance ofthe condition.

The presentation of primary hypothyroidismin two young euthyroid males with type IIIhyperlipoproteinaemia is remarkable. It isknown that the prevalence of newly diagnosedovert hypothyroidism in patients referred to alipid clinic is approximately twice that seen inthe general population.5 In view of our reportwe would suggest that secondary causes ofhyperlipidaemia should be sought in a lipidclinic. In cases of type III hyperlipoproteinae-mia, secondary causes such as obesity, diabetesmellitus and hypothyroidism should always beconsidered.

Interestingly, the lipid profile considerablyimproved in the first patient due to treatment

of his hypothyroidism with thyroxine in con-junction with dietary measures. However, thesecond patient required the addition of afibrate lipid-lowering drug. Finally, the occur-rence of hyperlipidaemia in these youngpatients provides an unusual presentation ofprimary hypothyroidism which could haveresulted in catastrophic sequelae in the longterm if not identified.

Final diagnosis

Primary hypothyroidism in two young clini-cally euthyroid males with type III hyperlipo-proteinaemia.

Keywords: hyperlipidaemia; hypothyroidism; hyperli-poproteinaemia

1 Morganroth J, Levy RI, Fredrickson DS. The biochemical,clinical and genetic features of type III hyperlipidaemia.AnnIntern Med1975;82:158.

2 Feussner G, Ziegler R. Expression of type III hyperlipidae-mia in a subject with secondary hypothyroidism bearing theapoE2/E2 phenotype. J Intern Med1991;230:1836.

3 Davignon J, Gregg RE, Sing CF. Apolipoprotein Epolymorphism and atherosclerosis. Arteriosclerosis 1988;8.

4 Zelis R, Mason DT, Braunwald E, Levy RI. EVects of lipo-proteinaemias and their treatment on the peripheral circula-tion. J Clin Invest 1970;49:1007.

5 Diekman T, Lansberg PJ, Kastelein JJ, Wiersinga WM.Prevalence and correction of hypothyroidism in a largecohort of patients referred for dyslipidaemia. Arch Intern

Med1995;155:14905.




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Extracranial cerebrovascular disease amanagement dilemma

A G Speers, S K Das

A 71-year-old man presented to the vascular clinic with a 5-year history of amaurosis fugax in hisright eye. He described this as a blanket descending over his visual field, which would come onslowly, lasting approximately 5 minutes before rapidly resolving, and was associated with a feel-ing of dizziness. Initially, attacks had occurred once every 2 months but recently the frequencyhad increased to twice a week. The patient was known to suVer from hypertension and hypercho-lesterolaemia, both of which were controlled medically. Ten years earlier, the patient hadundergone an aorto-iliac graft for peripheral vascular disease.

Examination revealed a regular pulse of 76 beats/min and a blood pressure of 136/84 mmHg;a late systolic murmur was detected in the mitral region. The right carotid artery pulse wasabsent, with a low pitched bruit. A left carotid thrill was detected with a high-pitched murmur.An ophthalmic examination confirmed embolic events in the right eye.

Duplex scan showed a diseased innominate artery with total occlusion of the right commoncarotid, 75% stenosis of the left internal carotid and occlusion of the left subclavian artery withreversal of flow through the vertebral artery. An aortic arch angiogram (figures 1 and 2) was per-formed.

Questions

1 What is the cause of the visual symptoms?2 What does the arteriogram show?3 What is subclavian steal and how would you treat it?4 How can the patients symptoms be explained?

Figure 1 Aortic arch angiogram Figure 2 Aortic arch angiogram


Department of

Vascular Surgery,

Frimley Park Hospital,Camberley, SurreyGU16 5UJ, UKA G SpeersS K Das

Correspondence toMr SK Das, ConsultantVascular Surgeon, TheHillingdon Hospital, PieldHeath Road, Uxbridge,Middlesex UB8 3NN, UK

Accepted 16 April 1999



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Answers

QUESTION 1

The patient is suVering from amaurosis fugaxor temporary blindness secondary to embolisa-tion within the retinal artery. Of significance inthis patient was the fact that he was exhibitingevidence of hypoperfusion (dizziness) which isonly seen in 5% of cases of carotid disease.

QUESTION 2Currently, duplex scan remains the first choiceof investigation for anatomical and functionalassessment of the arterial tree, as it isnon-invasive, reliable and can be repeated, thusit has almost replaced arteriogram in mostsituations.1 2 However, arteriogram is oftenobtained to confirm the duplex scan findings incomplex situations, such as in this case. Thearteriogram (figures 1 and 2) in this case con-firmed the findings of the duplex scan accu-rately and demonstrated that the right com-mon carotid is occluded and there is no fillingof the right internal or external carotid artery.There is 75% stenosis of the left internal

carotid artery and occlusion of the left subcla-vian artery at its origin. There is distal filling ofthe left subclavian by reverse flow from the leftvertebral artery.

QUESTION 3

Subclavian steal (figure 2) is a phenomenonwhere the distal blood supply in the subclavian(usually on the left) is derived from a reversal ofblood flow from the vertebral to the subclavianartery distal to the occlusion. This occursbecause the proximal segment of the subcla-vian artery has been occluded and therefore thearterial pressure gradient favours blood flow inthe reverse direction. It is usually only treated ifthe patient is symptomatic (clumsiness, dizzi-

ness or drop attacks), and is usually brought onby exertion. The main form of treatment isarterial reconstruction of the occluded seg-ment of the subclavian artery or angioplasty orstenting of the occluded segment.

QUESTION 4

The patients symptoms are the result ofembolisation from one of his carotid arteries.The most likely source of the emboli was felt to

be the left internal carotid with emboli travers-ing the circle of Willis to impact in the rightretinal artery. The other possibility was thatemboli were being released by the occludedright internal carotid artery, a phenomenaknown as carotid stump syndrome. Thus, thesurgical options available were to either tie oVthe occluded stump on the right, perform acarotid endarterectomy on the left, or do

both.

3 4

A carotid endarterectomy was performed onthe left, with no complications in the postop-erative period. Out-patient follow-up has re-vealed that the patients symptoms of embolisa-tion and hypoperfusion have resolved sincedischarge.

Discussion

This case illustrates the extensive and complexnature of atheromatous disease and thatmanagement of these patients often can be dif-ficult. In this category of patient the risk of notoperating was that these transient episodes of

blindness could lead to an event causingpermanent neurological damage. It has beenestimated that 20% of patients with amaurosisfugax will suVer from a stroke if untreated.5

This has to be weighed against the risk of mor-tality and stroke from performing a carotidendarterectomy. Mortality from this procedurehas been estimated at 1.3% after 30 days forthose with asymptomatic disease and 1.8% forthose with symptomatic disease with a risk offatal stroke of 0.47% and 0.91%, respectively.6

Attempts have been made to evaluate theeVectiveness of anticoagulating this cohort ofpatients. The general consensus appears that,although anticoagulation reduces the risk of a

cerebral event, it does not do this to asignificant enough degree.

Final diagnosis

Amaurosis fugax secondary to embolisationwithin the retinal artery.

Keywords: carotid artery; subclavian steal; amaurosisfugax

1 Chen JC. Can duplex ultrasonography select appropriatepatients for carotid endarterectomy? Eur J Vasc Surg1997;14:4516.

2 Taylor LM. The clinical course of carotid bifurcation steno-sis as determined by duplex scanning. J Vasc Surg

1988;8:255.3 Abu Rahama AF. Prospective randomised control trial ofcarotid endarterectomy with primary closure and patchangioplasty with saphenous vein, jugular vein and poly-tetrafluoroethylene. J Vasc Surg1998;27:22232.

4 Little NS, Meyer FB.Carotid endarterectomy - indications,technique and Mayo Clinic experiences.Neurol Med Chirurg1997;37:22735.

5 Way LW. Current surgical diagnosis and treatment, 10thedn. Los Altos, CA: Lange Medical Publications, 1994; pp

76171.6 Rothwell PM. A systematic comparison of stroke and deathdue to CEA for symptomatic and asymptomatic stenosis.Stroke 1996;27:2669.




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doi: 10.1136/pgmj.75.888.6191999 75: 619-621Postgrad Med J

Sushil Kumar Ahlawat, Pankaj Malhotra and VenkateshA 40-year-old woman with lung cavitation

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