Introduction

The Ikeda portoduodenostomy can be performed in patients with biliary atresia but insufficient jejunum to create a Roux-en-Y portoenterostomy. We report a case of a 2 year old girl who underwent this procedure for biliary atresia splenic malformation and ultrashort gut, followed by later combined liver small bowel transplantation.

 

Case report

The patient was noted to have echogenic bowel on antenatal ultrasound scans. Exploratory laparotomy on day 2 of life revealed biliary atresia splenic malformation with midgut deletion from presumed intrauterine volvulus. Only 10cm of jejunum was left intact. At the time, no therapeutic option was given but parents demanded a second opinion so parenteral nutrition was commenced.
On day 11 of life the patient was transferred to our centre. She underwent a portoduodenostomy for biliary drainage (Ikeda procedure). A jejunal stoma and a colonic mucous fistula were created. She was commenced on parenteral nutrition and slowly increasing enteral feeds. The jejunostomy effluent was refed into the distal colon. A system of intermittent clamping of the jejunal tube was introduced in an attempt to dilate the proximal small bowel for subsequent lengthening but this failed.
At 9 months of age a third surgical procedure was performed with reestablishment of intestinal continuity and creation of a gastrostomy. The duodenocolic anastomosis did not function well, and bilious fluid refluxed out of the gastrostomy. To maintain colonic health this fluid was refed into the colon via a colonic tube. A fourth operation at 10 months of age found a cirrhotic liver and it was not possible to improve the bowel anastomosis. Her liver function began to deteriorate and she was listed for transplantation.

She underwent liver small bowel transplant at 19 months of age. Induction immunosuppression was basiliximab followed by tacrolimus and prednisolone maintenance. Transplant course was uncomplicated. Enteral feeds via nasogastric tube were started one week post transplant and increased with reduction of parenteral nutrition. At discharge 28 days after transplant she was fully enterally fed with finger foods, top-up nasogastric tube feeds and a supplemental overnight feed.

Her body weight was 12 kg. The serum albumin was 42 g/L, the white cell count was 6.51 x 10⁹/L, and lymphocyte count was 2.79 x 10⁹/L. Surveillance ileoscopy at 1 month after operation showed well preserved villi and crypt architecture.

There has been one subsequent complication of abdominal wall abscess which was treated with antimicrobial therapy. At five months of follow up, she remains clinically well. She is gaining weight without nutritional support and has normal developmental milestones.

 

Conclusion

Ikeda portoduodenostomy in biliary atresia, splenic malformation with ultra-short gut can be performed for biliary drainage and does not preclude later successful liver small bowel transplantation.