Abstract: CHARGE association and DiGeorge syndrome (DGS) rarely occur together and only eight cases have been reported in the English literature. Two were associated with esophageal atresia (EA) and severe congenital heart anomalies. We report a third case of EA with tracheoesophageal fistula (EA-TEF) associated with coarctation of the aorta (CoA), CHARGE association, and DGS. The challenge for management in this complicated case is the background DGS which influences surgical outcome because of Ca++ imbalance and immune deficiency that can be life-threatening and require bone marrow transplantation.

Abstract: We report a case of a choledochal cyst (CC) associated with accessory hepatic duct (AHD) and review the literature, focusing on biliary reconstruction. CC is only rarely associated with AHD. Intra-operative endoscopy is invaluable for confirming anatomical relations and highly recommended for routine use. Reconstructive surgery is feasible for AHD in CC cases.

Abstract: Hepatic portoduodenostomy with interposition of the ileocecoappendix (HPI) can be used for the surgical treatment of biliary atresia (BA). The purpose of this study was to evaluate the long-term outcome of patients with HPI. The records of nine patients who had HPI performed for BA were reviewed. Mean age at portoenterostomy was 68.1 days (range 26-113 days). At the end of 2006, seven of the nine subjects were alive, although two required liver transplantation (LT). The two deaths occurred 94 days and 2 years after HPI due to varicella infection and variceal bleeding, respectively. Length of follow-up for the seven survivors ranged from 17 to 19 years (mean 18 years). Three subjects achieved normal liver function after HPI and have remained jaundice-free to date. Another three who were initially jaundice-free required Roux-en-Y jejunostomy (RYJ) to the ileocecum for severe obstructive cholestasis 6 months, 3 years, and 19 years after HPI secondary to stones in the cecum. RYJ was successful in these three cases, and liver function returned to normal within a few months. Two of these three have continued to have almost normal liver function, but one required LT 5 years after RYJ. The remaining case had LT because of liver dysfunction 14 months after HPI. There is a high risk for stone formation and obstruction with the HPI procedure because bile can stagnate in the ileocecum.

Abstract: Complete covered cloacal exstrophy (CCCE) is extremely rare. The anatomy of CCCE is complex and often unique, and each case must be treated individually. We present the case of a 5-year-old girl with CCCE whom we treated successfully with great improvement in her quality of life.

Abstract: AIM: We showed previously that adult small bowel could be transplanted successfully in rats without vascular reconstruction by removing the graft serosa. In this study, we assessed if granulocyte colony-stimulating factor (G-CSF) or basic fibroblast growth factor (bFGF) could improve graft survival in the same rat model. METHOD: A 10-mm-long adult small bowel graft from an adult 12-week-old Lewis rat was transplanted into a pouch created in the omentum of a 5-week-old Lewis rat (syngeneic bowel transplantation [SBTx], n = 49). Graft serosa was removed just before SBTx in the serosectomy group (n = 29) and left intact in the nonserosectomy group (n = 20). Each group was divided into 3 subgroups (sG): sG-1 had no G-CSF or bFGF; sG-2 had daily subcutaneous injections of G-CSF; and sG-3 had continuous infusion of bFGF around the graft in the omentum. All grafts were harvested 14 days after SBTx and studied histologically. A mucosal surface expansion score (MSES) was used where 0 = no mucosa on the graft, 1 = mucosa on one fourth of the graft, 2 = mucosa on one half of the graft, 3 = mucosa on three fourths of the graft, and 4 = mucosa on the whole graft. The density of CD34-positive capillaries per 1000 nuclei was also measured. RESULTS: Serosectomy group MSES were significantly higher than nonserosectomy group MSES indicating that grafts survived (P < .0001). CD34-positive capillaries in serosectomy group subgroups for mucosa were 103.9 +/- 34.2, 130.2 +/- 52.0, and 132.3 +/- 37.7, respectively; for muscle, 74.4 +/- 38.0, 86.2 +/- 32.9, and 82.4 +/- 30.3, respectively; and for omentum, 73.8 +/- 30.1, 151.3 +/- 60.3, and 140.0 +/- 49.0, respectively. Mucosal surface expansion score and overall CD34-positive capillaries for sG-2 and sG-3 were significantly higher than for sG-1 (both, P < .05). CONCLUSION: Our results suggest that G-CSF and bFGF enhance angiogenesis and mucosal surface expansion.

Abstract: PURPOSE: The aim of this study was to assess whether adult small bowel grafts (ASBGs) can survive transplantation without vascular reconstruction if graft serosectomy (SS) is performed. METHODS: Syngeneic ASBG transplants were performed in 85 Lewis rats. The entire serosa was removed just before transplantation in the SS group (n = 50) and left intact in the nonserosectomy group (n = 35). Transplanted ASBG was harvested 1, 3, 5, 7, 14, 21, or 28 days after transplantation and studied using staining with hematoxylin-eosin, immunohistochemistry for protein gene product 9.5, S-100, CD34 and vascular endothelial growth factor (VEGF), and quantification of VEGF messenger RNA (mRNA). Adult small bowel graft viability was assessed blindly using a mucosal surface expansion score (0, no mucosa; 1, mucosa on one fourth of graft; 2, mucosa on one half of graft; 3, mucosa on three fourths of graft; and 4, circumferential mucosa on graft). RESULTS: No rejection was identified in any ASBG. Average mucosal surface expansion score and VEGF mRNA expression were significantly higher in the SS group (both P < .01). Vascular endothelial growth factor protein was detected in enterocytes from day 3 posttransplant in the SS group. Distribution of protein gene product 9.5 and S-100 was normal in SS-group ASBG. CONCLUSIONS: Our results suggest that SS allows VEGF mRNA and, subsequently, VEGF protein in ASBG to be induced very soon after transplantation, which may contribute to the survival of ASBG transplanted without vascular reconstruction.