Predicting factors on the occurrence of cystic dilatation of intrahepatic biliary system in biliary atresia.

PURPOSE: Cystic dilatation of intrahepatic biliary system (CDIB) is an intractable complication of biliary atresia (BA). In this study, we investigated the predicting factors of CDIB development after jaundice resolved following hepatoportoenterostomy (HPE). METHODS: From 1988 to 2008, 28 (80.0%) of 35 uncorrectable type of BA patients became jaundice-free after HPE. Of these 28 patients, this retrospective study included comparisons of the preoperative characteristics, postoperative jaundice period, cumulative steroid dose, outcome, and liver fibrosis grade at the time of HPE between CDIB-positive and -negative groups, divided by postoperative ultrasonography findings. RESULTS: There were no differences between groups in perioperative characteristics. Liver fibrosis in the CDIB-positive group (n = 7) included grade II in two patients, grade III in four patients, and grade IV in one patient. On the contrary, the CDIB-negative group (n = 21) included 8, 11, 2, and 0 patients with grades I-IV, respectively, with a significant difference (p < 0.05) between groups. Survival rate with native liver in CDIB-positive group was significantly lower than that in CDIB-negative group (p < 0.05). CONCLUSION: Postoperative CDIB might be a poor prognostic factor, and the liver fibrosis grade at the time of HPE could be helpful to predict the development of CDIB after HPE.

Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-up.

INTRODUCTION: Choledochal cyst (CC) is closely associated with anomalous arrangement of the pancreaticobiliary duct, which is considered a high-risk factor for biliary tract malignancy. Early diagnosis and early treatment for CC could lead to a good prognosis. This study investigated late complications and long-term outcomes after surgery for CC. PATIENTS AND METHODS: Fifty-six patients with CC and over 10 years of postoperative follow-up were analyzed retrospectively. All patients had undergone total resection of the extrahepatic bile duct and hepaticojejunostomy. RESULTS: Six patients showed liver dysfunction manifested in the first 10 years after surgery, but all returned to normal thereafter. Dilatation of intrahepatic bile ducts persisted in 6 postoperatively, and in 3, this was still apparent more than 10 years after. Recurrent abdominal pain was encountered in 3, 1 had pancreas divisum with a pancreatic stone, and 1 had adhesive small bowel obstruction. Two patients developed biliary tract malignancy. A 14-year-old girl died of recurrent common bile duct cancer 2 years after the initial resection of CC with adenocarcinoma. A 26-year-old man with repeated cholangitis owing to multiple intrahepatic bile stones developed cholangiocarcinoma 26 years after the initial resection of CC. Event-free survival rate and overall survival rate were 89% (50/56) and 96% (54/56), respectively. CONCLUSIONS: Choledochal cyst generally has an excellent prognosis with early total resection and reconstruction. Long-term surveillance for the development of malignancy is still essential, especially if there is ongoing dilatation of the intrahepatic bile duct or biliary stones.

Significance of ductal plate malformation in the postoperative clinical course of biliary atresia.

PURPOSE: Ductal plate malformation (DPM) is one of the etiologic theories for the development of biliary atresia (BA). In this study, we investigated the significance of DPM in the postoperative clinical course of BA, especially as a predictive factor of jaundice clearance. METHODS: Between 1988 and 2005, 31 patients with uncorrectable BA underwent hepatoportoenterostomy and steroid therapy. Immunohistochemistry was used to characterize biliary structures using cytokeratin 19. Specimens were defined as DPM-positive if a concentric cellular arrangement was detected. This retrospective study included comparisons of preoperative characteristics, the postoperative jaundice period, and cumulative steroid doses between patients with and without DPM. RESULTS: Of the 31 patients with uncorrectable BA, 25 (80.6%) became jaundice-free. Ductal plate malformation was detected in 11 (35.5%) of the 31. Between the 2 groups, there were no differences in preoperative characteristics or in the postoperative jaundice-free rate. Among those who became anicteric, the postoperative jaundice period was 145.3 +/- 69.9 days in the DPM-positive group (n = 9) and 81.8 +/- 44.7 days in the DPM-negative group (n = 16) (P < .05). The total administered steroids were 149.7 +/- 78.2 and 95.0 +/- 60.2 mg/kg, respectively (P = .09). CONCLUSION: Ductal plate malformation may disturb bile flow and require more steroid to improve bile drainage. Therefore, the presence of DPM in the liver predicts poor bile flow after hepatoportoenterostomy in infants with BA.

The inv mouse as an experimental model of biliary atresia.

BACKGROUND/PURPOSE: The causation of biliary atresia (BA) remains unclear. However, ductal plate malformation (DPM), maldevelopment of the intrahepatic bile ducts, is 1 of the preferred theories. The inv homozygous mouse (inv mouse), created by insertional mutagenesis, shows situs inversus and jaundice. This study investigated whether the inv mouse could be an experimental model of human BA. METHODS: In the inv mice (n = 12) and wild-type littermates (n = 12), we examined the liver function and morphologic changes in the biliary tract through serum biochemical study and morphological study. RESULTS: The level of serum total and conjugated bilirubin in the inv mouse was 8.1 +/- 3.8 and 4.4 +/- 2.4 mg/dL, respectively, significantly higher than in the wild type. Macroscopically, 11 (92%) of 12 inv mice had situs inversus, and 3 (25%) of 12 mice had preduodenal portal vein. Histologically, the continuity of the extrahepatic bile duct was preserved. However, DPM, showing proliferative biliary epithelium around the intrahepatic portal vein, was found in the liver of the inv mouse. CONCLUSION: In the inv mouse, the pathologic changes in DPM were found in the intrahepatic biliary system, which were observed in some clinical cases of BA. Therefore, the intrahepatic biliary system of the inv mouse could be an experimental model of human BA with DPM.

Clinical significance of the confluence of the cystic duct in patients with anomalous arrangement of the pancreaticobiliary duct.

BACKGROUND/PURPOSE: In anomalous arrangement of the pancreaticobiliary duct (AAPBD), there is anatomical diversity of the cystic duct. In this study, we evaluated the influence of the level of insertion of the cystic duct into the extrahepatic bile duct on the pathophysiology of AAPBD. METHODS: Thirty-two children with AAPBD were examined using cholangiopancreatography. If the cystic duct entered the hepatic duct at the lower middle point of the extrahepatic bile duct, it was defined as low confluence; otherwise, it was considered as high confluence. Clinical details and radiological variables were compared between these 2 groups. RESULTS: Low confluence was noted in 8 of the 32 patients. Seven of the 8 had fusiform-type or nondilatation-type choledochus, and the cystic-type was significantly less frequent than in the high-confluence group. The diameter of the common bile duct was significantly smaller and the main pancreatic duct was significantly greater than in the high-confluence group. Pancreatitis was more common and biliopancreatic reflux on computed tomography combined with intravenous infusion cholangiography was more often seen in the low-confluence group than in the high-confluence group. CONCLUSIONS: Bile juice could regurgitate into the pancreatic duct via the low confluence of the cystic duct, resulting in severe pancreatitis in patients with AAPBD.

Social quality of life for adult patients with anorectal malformations.

BACKGROUND/PURPOSE: Pediatric surgeons who performed the initial surgery on anorectal malformations (ARM) lose contact with the patients as they become adults. In the present study, we examined 20- to 40-year-old adult patients with a history of surgery for ARM and analyzed them from the points of social quality of life. PATIENTS AND METHODS: Twenty-nine patients with ARM, aged 20 to 40, were surveyed by questionnaire or personal interview. Thirteen with high-type and 9 with intermediate-type anomalies underwent abdominoperineal rectoplasty, and 7 with low-type anomalies underwent perineoplasty between 1965 and 1985. Responses were analyzed from the perspectives of bowel, urinary, and sexual functions and social activity. RESULTS: One third of patients with high- or intermediate-type anomalies occasionally complained of fecal soiling. However, the other patients gained good bowel function and enjoyed occupational or student life without problems. Fecal soiling was the key factor disturbing occupational life, although the problem remained within a socially manageable level. All of the patients with ARM had normal urinary function. Three of the 18 male patients had sexual problems such as erectile or ejaculatory dysfunction because of associated genitourinary anomalies. Nine of the 11 female patients had regular menstruation and the other 2 had irregular menstrual periods. Five female patients were married and 4 of the 5 had children (1-5 children). Modes of delivery were normal vaginal delivery in 8 and cesarean section in 1. Only one of the 5 had a slight sacral anomaly. However, she had no apparent abnormality of bladder function and got through pregnancy and delivery without difficulty. CONCLUSION: One third of adult patients with high- or intermediate-type anomalies after abdominoperineal rectoplasty had some problems in bowel function. Fecal soiling was the key factor that disturbed their occupational life. Most of the patients had normal urinary and sexual functions if they did not have associated genitourinary anomalies and enjoyed social activities.

Spontaneous rupture of choledochal cyst with pseudocyst formation-report on 2 cases and literature review.

Spontaneous rupture and subsequent bile peritonitis are rare complications of choledochal cysts. Of these complications, the formation of a biliary pseudocyst is an unusual form, and its preoperative diagnosis is difficult. In this report, we describe 2 cases showing spontaneous rupture with biliary pseudocyst formation. Inflammatory tissue surrounded those pseudocysts, one of which was adjacent to the perforation and the other formed in the transverse mesocolon apart from the biliary tract. These pseudocysts were removed by careful dissection, and single-stage cyst excision with biliary reconstruction was successfully performed in both cases.

Outcome of infants with neuroblastoma detected by mass screening and surgically treated in Shiga Prefecture, Japan: what is the role of surgery?

To investigate retrospectively the clinical and biological features that influence the outcome of infants with neuroblastoma (NB) detected by mass screening (NBMS), and to construct surgical strategies to deal with NBMS, 20 infants diagnosed as having either NB or ganglioneuroblastoma (GNB) between 1986 and 1998 were enrolled in a study. They comprised 15 boys and 5 girls ranging in age from 7 to 14 months. The following factors were analyzed by multivariate analysis: age, stage according to the Japanese staging system at the time of diagnosis, site of the primary tumor, histologic findings, preoperative urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels, VMA/HVA ratio, lactate dehydrogenase, neuron-specific enolase, Shimada's histologic classification, amplification of the N- myc oncogene by Southern blot analysis, nuclear content, and chromosomal abnormality. The 2-year survival was 95.0% (19/20). The site of the primary tumor was the adrenal gland in 12 cases, retroperitoneum in 6, and retrothoracic space in 2. Four infants had stage I, 6 stage II, 3 stage III, 3 stage IVB, and 4 stage IV disease. Complete resection was achieved in all cases except for 1 retroperitoneal GNB. Histologic examination showed that 8 patients had NB. Of the 12 GNBs, 8 were poorly-differentiated and 1 was well-differentiated. Only 1 of the 14 examined tumors showed amplification of N- myc (20 copies). The infant with N- myc-positive NB (stage II) died 23 months after surgery in spite of aggressive postoperative chemotherapy. Multivariate analysis revealed the plasma NSE level to be a significant predictor of survival (p < 0.0143). This suggests that N- myc amplification and plasma NSE level could be closely related to the survival of infants with NBMS. The N- myc-positive NB case implies that even in locoregional NB detected by NBMS, surgical excision should play a central role in the diagnosis of its oncogenic characteristics and indicate any subsequent therapy.