Percutaneous cholangiography for the treatment of parenteral nutrition-associated cholestasis in surgical neonates: preliminary experience.

OBJECTIVE: Parenteral nutrition-associated cholestasis (PNAC) occurs in up to 60% of surgical neonates with intestinal failure, and 10% will develop end-stage liver failure. Our aim was to evaluate the effectiveness of percutaneous transhepatic transcholecystic cholangiography (PTTC) in the treatment of PNAC in surgical neonates. METHODS: A retrospective double cohort study of surgical neonates with PNAC was conducted. Patients with PNAC who received PTTC were compared to controls matched by gestational age, birth weight, sex, and parenteral nutrition duration. Percutaneous transhepatic transcholecystic cholangiography was performed under general anesthesia with ultrasound guidance. Analysis was performed using paired Student's t test and McNemar chi2 test. RESULTS: Nine PTTC patients and 9 controls were similar in mean age (35.5 +/- 3.1 vs 35.6 +/- 4.2 weeks, P = .85), birth weight (2531 +/- 879 vs 2692 +/- 1052 g, P = .28), sex (all males), and parenteral nutrition duration (51.2 +/- 29.8 vs 53.3 +/- 33.3 days, P = .74). Percutaneous transhepatic transcholecystic cholangiography was performed in 9 patients at mean corrected age of 5.5 +/- 3.4 weeks and weight of 3621 +/- 546 g. All control patients and 8 (88.9%) of 9 PTTC patients had eventual resolution of hyperbilirubinemia. Percutaneous transhepatic transcholecystic cholangiography patients experienced a more rapid rate of resolution of their cholestasis, and the mean time to resolution of conjugated bilirubin was less in the PTTC group (8.5 +/- 3.2 vs 18.5 +/- 7.6 weeks, P = .02). CONCLUSION: Therapeutic PTTC results in a 50% reduction in the time to PNAC resolution. Percutaneous transhepatic transcholecystic cholangiography may have a role as active therapy to slow progression of PNAC in surgical neonates with intestinal failure.

The versatility of the umbilical incision in the management of Hirschsprung's disease.

BACKGROUND/PURPOSE: The aim of this report is to describe how the umbilical incision provides a quick and safe alternative to laparoscopy or other abdominal incisions in the management of Hirschsprung's disease (HD). METHODS: An analysis of 24 patients with HD, who were treated using an umbilical incision as part of their operative management between 1999 and 2003, was performed. RESULTS: There were 18 boys and 6 girls (mean age at diagnosis: 16.5 +/- 20.9 days). Twenty-one had rectosigmoid HD, and 3 had total colonic HD. Eighteen patients received a 1-stage transanal pull-through with transumbilical colonic biopsies at a mean age of 33.9 +/- 25.3 days, and a mean weight of 3.8 +/- 1.0 kg. Three patients with rectosigmoid disease had more complicated HD: 2 had a colostomy (1 enterocolitis, 1 extensive colonic dilatation), and 1 had an ileostomy (for perforated cecum). All subsequently underwent transanal pull-through. Three patients with total colonic HD had an ileostomy. All stomas were situated at the umbilicus. One of these patients subsequently underwent a Duhamel procedure via the umbilicus; 1 is still waiting; and 1 died of sepsis. Complications in this cohort included a seromuscular tear of the distal sigmoid at the level of the umbilical incision (1), infection at the umbilical incision requiring antibiotics (2), obstruction (1) and death (1 sepsis, 1 from Ondine's curse). The median time to start full feeds was 24 hours. For postoperative analgesia, 13 patients required acetaminophen only, and 9 patients received low-dose morphine. After a mean follow-up of 7.3 +/- 9.7 months, there has been an excellent functional and cosmetic result in the 22 survivors. CONCLUSIONS: The umbilical incision has all the benefits of a minimal access approach, but is less expensive and requires no specialized equipment or skill. The umbilical incision is an excellent, safe, and versatile alternative to laparoscopy for the treatment of patients with the full spectrum of HD.