Short notes and case reports

Congenital bronchobiliary fistula associated with biliary atresia

Y.T. Chan, W.D. Ng, W.P. Mak, M.L. Kwong and C.B. Chow Princess Margaret Hospital, Hong Kong

Correspondence to: Dr Y.T. Chan, Surgical ‘B’ Unit, Princess Margaret Hospital, Lai Chi Kwok, Hong Kong

Congenital bronchobiliary fistula is a rare developmental anomaly. So far only seven cases have been reported in the English literature’. In spite of the small number of cases, the incidence of co-existing biliary tree malformation is remarkably high. The case reported by Enjoji was associated with stenosis of the common bile duct’. Dyon reported in French a case of congenital tracheal-biliary fistula coincident with choledochal hypoplasia. Anomalous biliary drainage was suspected in Wagget’s case). The following case is the first one found to have absent the common bile duct.

Case report

A 4-day-old Chinese girl was admitted because of increasing dyspnoea and drooling of an excessive amount of greenish-yellow saliva. Chest X-ray showed bilateral patchy pneumonia. Her condition deteriorated rapidly, necessitating endotracheal intuba- tion and mechanical ventilation. A suction catheter was passed down the endotracheal tube to beyond 20 cm. Fluoroscopic examination, following injection of metrozamide, showed a bronchobiliary fistula arising from the right main bronchus. The distended biliary system terminated abruptly (Figure I ) . At thora-

Figure 2 Post-mortem examination. Note the absence of common bile duct, distension of hepatic ducts and gallbladder with greenish bile

cotomy, the thoracic part of the fistula was easily identified and excised. Histologically, the bronchial end contained cartilage. She failed to improve and expired on day 28. Post-mortem examination revealed a 3 mm hole in the right bronchus, adjacent to an intact ligature. This was believed to be the cause of a pneumothorax. The fistulous tract was traced to the junction of the hepatic ducts. The distended gallbladder joined the same junction. No trace of the common bile duct was identifiable (Figure 2).

Discussion In a patient with bronchobiliary fistula, there are certain features which should alert the clinician to the possibility of an associated biliary anomaly. In the presence of either biliary atresia or hypoplasia, the fistula represents the only or the main outlet ofbile. The presentation will obviously be early, pneumonitis intractable and discharge of bile-stained secretion particularly profuse, as so eloquently shown in this case. In contrast, diagnosis was delayed up to 14 months in Stigol’s case, and 2 years and 9 months in the one .reported by Wei t~man~,~ .

Pre-operatively, bronchobiliary fistula can only be conclu- sively diagnosed by either bronchography or bronchoscopy. The latter has been found to be very useful by most authors2-4. If biliary malformation is suspected on clinical grounds, delineation of the biliary tree can be afforded by injecting contrast down a catheter passed into the fistula via a bronchoscope* In the absence Of such technique, Operative cholangiogram is recommended after threading a catheter to the caudal part ofthe tract.

Figure 1 Radio-opaque catheter, passed through the endotracheal tube, was seen arrested at the biliary tree. Note the bulbous lower end and absence of contrast in duodenum

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Short notes and case reports

The embryology of bronchobiliary fistula remains conjec- t ~ r a l ~ , ~ . We incline to favour Bremar’s proposal that the fistula results from the joining of an accessory bronchus, passing down the mediastinum, with an aberrant hepatic duct, rather than the oesophageal duplication theory, for the following reasons: (1) The presence of cartilage in the bronchial end of the fistula support Bremar’s mechanism. (2) The frequent coexistence of biliary anomalies, as evidenced by its occurrence in 4 out of 9 reported cases, lends further support*y3. (3) ‘Correctable’ biliary atresia, as in this case, is widely believed to be developmental in origin. It may well be accompanied by another fault in embryogenesis, i.e. an aberrant hepatic duct. Alternatively, the unique association may be explained by a further postulation that

the hepatic diverticulum arose aberrantly from the lung buds instead of from the junction of the foregut and midgut.

References 1. Kalayoglu M, Okay I. Congenital bronchobiliary fistula

associated with esophageal atresia and tracheo-esophageal fistula. J Paediatr Surg 1976; 11: 4 6 3 4 .

2. Sane SM, Sieber WK, Girdany BR. Congenital bronchobiliary fistula. Surgery 197 I ; 69: 599408.

3. Waggett J, Stool S, Bishop HC e ta / . Congenital bronchobiliary fistula. J Pediatr Surg 1910; 5 : 566-9.

4. Weitzman JJ, Cohen SR, Wood LO et al. Congenital broncho- biliary fistula. J Pediatr Surg 1968; 73: 329-34.

Paper accepted 25 August 1983

Aneurysm of the superior mesenteric artery - a case of Maff ucci’s syndrome A. Simpson and S.R. Singh Sandwell District General Hospital, West Bromwich, West Midlands, UK

Maffucci in 188 11 reported an interesting combination of pathologies in a 41 -year-old woman, presenting with hard bony masses in the forearms together with overlying haemangiomata. The presence and co-existence of haeman- giomata and chondromata is now regarded as a definite entity, Maffucci’s syndrome, and appears to be due to mesenchymal dysplasia not under genetic regulation and may be associated with multiple defects in a single gene2.

The defect appears to be an inborne mesodermal abnor- mality with dyschondroplasia and haemangiomata

occurring together. It is unusual for any defect or abnor- mality to be noted at birth. The characteristic features usually become apparent about adolescence. The patients are usually women and of short stature.

Typically the lumps predominate in the hands and feet but there is a possibility of lumps occurring elsewhere, though the viscera are usually spared3.Occasionally medium-sized arteries are affected by fibromuscular dysplasia and mural aneurysms may develop. Involvement of cranial and renal vessels has been noted.

Case report A 41-year-old woman complained at the age of 14 years of swelling of the fingers. At first these swellings were red and tender and were thought to be ‘chiIblains’. Over the ensuing months the swellings were replaced by hard nodules and deformity of the finger joints. The joints primarily affected were the proximal interphalangeal of the middle fingers and terminal interphalangeal of the right index finger. There was no relevant family history.

Figure 1 Haemangioma offorearm Figure 2 X-ray ofleji hand

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