Impact of age at diagnosis on clinical features in children with anomalous arrangement of the pancreaticobiliary duct.

AIMS: In patients with an anomalous arrangement of the pancreaticobiliary duct (AAPBD), clinical presentations may differ between infants and older children. The optimal timing of surgery remains controversial, particularly in early infancy. The aim of this study was to evaluate the clinicopathological features and clinical outcomes using comparative methods between infants and older cases. MATERIALS AD METHODS: From 1983 to 2007, a total of 85 consecutive children with AAPBD were treated at our institute. They included 46 with the cystic type, 33 with the fusiform type, and 6 with the non-dilatation type. These patients were divided into 2 age groups: "infant" (n=9), <12 months old; and "older", >1 year old (n=76). A retrospective study was performed. RESULTS: Mean age was 5.2 months (range, 8 days-11 months) in the infant group and 5.2 years (range, 1.2-17.3 years) in the older group. Jaundice was significantly more frequent in the infant group ( P<0.05), whereas abdominal pain was more common in the older group ( P<0.001). Bleeding tendencies such as cranial hemorrhage or bloody stools were noted in only 3 infants. In terms of liver histology, liver cirrhosis was observed in 2 infants, one of whom was a 3-month-old girl with severe jaundice resulting in living-donor liver transplantation, despite bile drainage. A single postoperative death occurred due to an adenocarcinoma arising in a choledochal cyst in a 12-year-old girl. CONCLUSIONS: Problems characteristic of infantile AAPBD were a severe bleeding tendency and irreversible liver cirrhosis, which could develop as young as 3 months old. The surgical recommendation for infantile AAPBD is thus early surgery before the age of 3 months to prevent liver failure.

Clinical course of obstructive jaundice associated with operated meconium peritonitis in neonates.

BACKGROUND/PURPOSE: Meconium peritonitis (MP) may induce prolonged cholestasis after laparotomy. In this study, we investigated the postoperative clinical course of MP retrospectively and discuss the relationship between MP and the development of obstructive jaundice, including biliary atresia (BA). PATIENTS AND METHODS: Between 1979 and 2008, 23 infants with MP underwent laparotomy at our institution. Eleven of the 23 infants (47.8%) developed obstructive jaundice postoperatively. The medical charts of these 11 infants were reviewed. RESULTS: The causative disease underlying MP included jejunoileal atresia in 10 and cloacal anomaly in 1. Of these 11 infants, 4 had acholic stools. Nine of the 11 improved with conservative management including an expectant approach, choleretic agents, and exchange blood transfusion. To differentiate the diagnosis from BA, open cholangiography was required in 2 cases following negative HIDA scintigraphy and a small gallbladder on ultrasonography. One of these 2 cases was diagnosed as BA and underwent hepatic portoeneterostomy simultaneously, after which the infant became jaundice free. CONCLUSIONS: Postoperative cholestasis after MP was a transient condition in most cases. However, ultrasonography and HIDA scintigraphy should be performed to differentiate BA in infants with MP who show prolonged jaundice with acholic stools.

Significance of the anomalous arrangement of the pancreaticobiliary duct in the etiology of biliary atresia.

BACKGROUND/PURPOSE: The anomalous arrangement of the pancreaticobiliary duct (AAPBD) is one theory used to explain the etiology of biliary atresia. We investigated whether AAPBD could be involved and evaluated its significance for the etiology of biliary atresia. MATERIALS AND METHODS: Of 43 patients with biliary atresia, the area between the common bile duct and the duodenum could be visualized by operative cholangiogram in 5 patients with an uncorrectable type of biliary atresia. Three of the 5 showed an anomalous arrangement of the pancreaticobiliary duct. In these 3 patients, the type of anomalous arrangement of the pancreaticobiliary duct and the length of the common channel were studied by operative cholangiogram. Histological findings of the gallbladder and the common bile duct were examined in addition to the measurement of the serum amylase levels. RESULTS: All 3 patients showed AAPBD with the P-C type of pancreaticobiliary junction. The length of the common channel ranged from 7 mm to 12 mm. Two of the 3 cases did not show an elevated serum amylase level. Epithelial hyperplasia of the gallbladder was observed in one patient, while the other two showed no hyperplasia. Inflammatory changes in the mucosa of the gallbladder and the common bile duct were not remarkable in these 3 patients. CONCLUSIONS: From these results it seems that AAPBD in biliary atresia might not be an etiological factor for atresia of the extrahepatic bile duct, but might be an associated anomaly in biliary atresia. Other factors should be examined to clarify the etiological factor leading to lumenal obstruction of the extrahepatic bile duct.

The significance of steroid therapy after hepatoportoenterostomy in infants with biliary atresia.

PURPOSE: Despite improvements in the surgical management of biliary atresia (BA), it is still difficult to maintain good bile flow. In the present study, we examined steroid therapy and determined the appropriate dose to achieve freedom from jaundice after hepatoportoenterostomy (HPE) in the uncorrectable type of BA. METHODS: A retrospective clinical analysis was done in 23 of 29 (79 %) cases who had become jaundice-free after undergoing HPE with steroid therapy between 1988 and 2004. A correlation between the total or mean steroid dose and the postoperative jaundice period (serum total bilirubin > 1.0 mg/dl) was evaluated using linear regression analysis. The regimen was as follows: prednisolone was given intravenously, starting with 3 to 5 mg/kg/day, and then gradually tapered with repetition until freedom from jaundice was achieved. RESULTS: Age at HPE was 72 +/- 20 days (mean +/- SD), and the postoperative jaundice period was 108 +/- 68 days. Total and mean steroid doses were 118 +/- 73 mg/kg and 1.31 +/- 0.8 mg/kg/day, respectively. There was no correlation between the total steroid dose and the period of jaundice. However, there was a significant correlation between the mean steroid dose and the period of jaundice (p = 0.021). CONCLUSION: A high mean dose of steroids could shorten the jaundice period after HPE in the uncorrectable type of BA.

Bleeding tendency as a first symptom in children with congenital biliary dilatation.

AIM OF THE STUDY: Although a bleeding tendency as a first symptom is a critical condition in congenital biliary dilatation (CBD), the clinical details of this symptom remain unclear. We assessed this condition in children with CBD in this paper. MATERIALS AND METHODS: Sixty-five children with CBD were treated at our institute between 1983 and 2004. The children, initially presenting with bleeding manifestations such as intracranial hemorrhage and bloody stools, were defined as the bleeding group, and the remaining children with digestive symptoms such as abdominal pain and vomiting were defined as the digestive group. The clinical features were compared between these two groups. RESULTS: In 6 of the 65 cases, bleeding manifestations were noted (9.2 %). All six had cystic-type choledochal dilatation. The mean age of the bleeding group was significantly younger than that of the digestive group, and bleeding was more frequent, especially in infants less than 12 months of age. In a laboratory study, the bleeding group showed a more prolonged blood coagulation time than the digestive group did. Serum amylase and lipase levels in the bleeding group were almost normal, while those in the digestive group were significantly higher. The direct bilirubin level in the bleeding group was significantly higher than that in the digestive group. CONCLUSIONS: Disturbed blood coagulation due to vitamin K deficiency related to cholestasis results in a bleeding tendency in children with CBD. Therefore, pediatric surgeons should be aware of this rare but critical condition which can be prevented by rapid and precise treatment with vitamin K supplementation.

Techniques available for the management of massive sacrococcygeal teratomas.

An infant born in the 34th week of gestation weighing 5,355 g with a massive sacrococcygeal (SC) tumor was delivered by elective cesarean section. An ultrasonographic examination showed solid and cystic components in the tumor. Resection was successfully undertaken with insertion of a Nélaton catheter into the rectum to avoid unnecessary impairment of the viscera. The tumor weighed 2,380 g, measured 25 x 14 x 11 cm, and was clinicopathologically diagnosed to be a SC teratoma. This experience and other publications show that several considerations including control of hemorrhage and coagulopathies, visceral protection, and avoidance of wound infection are necessary to facilitate the surgical management of massive SC tumors. Several suggestions are made concerning the pre- and intraoperative management of this rare tumor.

Manual reduction with the index finger for infantile intussusception. A modification of Hutchinson's maneuver.

Manual reduction with the index finger (modified Hutchinson's maneuver) enabling simple and safe manual reduction of infantile intussusception is described. The procedure was used in four patients, two boys and two girls, ranging in age from 2 to 15 months, as greater than normal compression was necessary and/or serosal splitting occurred during conventional reduction. The technique is as follows: in addition to the proximal bowel compression with Hutchinson's maneuver, the surgeon inserts his index finger into the intussusceptum via its neck. The finger enables the surgeon to sound a wider space between the intussusceptum and intussuscipiens, which is most likely to be responsive to bowel compression. Although the intussusceptions in the four patients were categorized as ileo-ileo-colic (three cases) and ileo-ileal (one case) types, this manual reduction technique was successful in all cases, no bowel rupture occurred, and hospitalization was no longer than with the conventional procedure. We conclude that this modified Hutchinson's maneuver may contribute to successful surgical reduction of infantile intussusception.

The application of radio-opaque markers prior to ileostomy in an infant with chronic intestinal pseudo-obstruction: report of a case.

We report the case of a 6-month-old boy who developed chronic intestinal pseudo-obstruction soon after birth. A rectal biopsy demonstrated immaturity of the neuronal cells in the enteral ganglion. His clinical course was stressful, with remission and exacerbation despite conservative treatment with daily bowel irrigation, prokinetic agents, and parenteral nutrition. Since the infant developed serious enterocolitis associated with the increased severity of his bowel obstruction, and no substantial gain in body weight was observed, a loop-ileostomy was performed based on X-ray findings with radio-opaque markers, which were employed to evaluate the whole gut transit time. The radio-opaque markers proved extremely useful for determining which loop of the ileum should be utilized for the ileostomy.