CASE REPORT R. Samujh Æ M. Srinivasa Rao Æ J. K. Mahajan A. Bhattacharjee Æ K. L. N. Rao Oesophageal atresia and biliary atresia: a rare association Accepted: 20 November 2002 / Published online: 17 April 2004 Ó Springer-Verlag 2004 Abstract Oesophageal atresia is known to be associated with cardiovascular, gastrointestinal, genitourinary and skeletal anomalies. Its association with biliary atresia is very rare and only a few cases have been reported in the literature. Although biliary atresia could be part of a multiple anomaly syndrome, its isolated occurrence is even rare. We present a case of oesophageal atresia associated with biliary atresia. Keywords Oesophageal atresia Æ Biliary atresia Introduction Oesophageal atresia is uncommonly associated with biliary atresia. The survival of babies with this rare association is extremely uncommon and according to literature, only one baby is still alive and that too after a liver transplantation. The child under report is presently 15 months of age and is anicteric. We present the importance of an early and thorough investigation for biliary atresia in any newborn with prolonged jaundice in the postoperative period. Case report A 1-day-old male baby, weighing 2.5 kg and born by caesarian delivery because of associated polyhydramni- os, was referred due to frothing and excessive oral secretions. Further clinical and radiological examina- tions suggested the presence of oesophageal atresia. A right thoracotomy was carried out and the findings of oesophageal atresia with distal tracheo-oesophageal fis- tula were confirmed. There was a gap of 2 cm and a primary oesophageal anastomosis was done after ligating the tracheo-oesophageal fistula. The postoperative peri- od was uneventful. An oesophagogram performed on the 5th postoperative day demonstrated no leak or stricture. The child was later allowed oral feeding and kept under observation. The cardiac echography was normal. This child had persistently passed clay-coloured stool postoperatively and started to develop deepening jaun- dice with a bilirubin level of 11 mg% (188.1 lmol/l) with conjugated level at 9.2 mg% (157.32 lmol/l). Initial clinical examination revealed hepatomegaly but serum tests for neonatal hepatitis were negative. The gall bladder was not visualised on sonography, intrahepatic biliary ducts were not dilated and no vascular anomalies could be detected. The inferior vena cava was normal. Subsequently a Technetium 99m Mebrofenin hepatobil- iary scan revealed failure of the tracer to reach the duodenum even after 24 h. A peroperative cholangio- gram was planned. A laparotomy was performed on the 27th postoper- ative day following oesophageal atresia repair. However on exploration the gall bladder and biliary ducts were atretic and the liver appeared grossly normal. The per- operative cholangiogram could not be done. A liver biopsy was taken. Kasai’s portoenterostomy was per- formed with roux-en-y hepaticojejunostomy. Postoper- atively, the child started to pass yellowish stools and bilirubin levels returned to 8 mg% (136.8 lmol/l). Ex- cept for a minimal wound infection no other complica- tion was noticed. This baby was discharged on the 10th day following the Kasai procedure with choleretics (ur- sodeoxycholic acid and phenobarbitone) and antibiotics. The histopathology report was diagnostic of type III biliary atresia. At 3 months follow-up, an oesophagogram revealed no stricture or gastro-oesophageal reflux. The lung fields R. Samujh (&) Æ M. Srinivasa Rao Æ J. K. Mahajan Æ K. L. N. Rao Department of Paediatric Surgery, Postgraduate Institute of Medical Education and Research, 160 012 Chandigarh, India E-mail: rsamujh@yahoo.com Fax: +91-172-2744401 A. Bhattacharjee Department of Nuclear Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India Pediatr Surg Int (2004) 20: 467–468 DOI 10.1007/s00383-004-1178-7