Chiu-Chiang Chen

Long-term prognosis of patients with biliary atresia: a 25 year summary.

Objective: The purpose of this study was to delineate the long-term prognosis of biliary atresia (BA) in Taiwan. Study Design: From 1976 to 2000, 185 children were diagnosed with BA, 22 underwent exploratory laparotomy without Kasai operation, and 163 underwent Kasai operation, of which 141 cases had long-term follow-up and formed the basis of this study. The outcome was analyzed. Results: Among the 141 BA children studied who underwent Kasai operation, 115 (81.6%) had recoloration of stools, and 86 (61.0%) became jaundice-free (bilirubin <20 μmol/L). The resolution of jaundice and the absence of repeated cholangitis contributed to better outcome. Five and 10 year survival rates with native liver were 35% and 31%, respectively. Liver transplantation was performed in 19 patients (all but 2 with a living-related donor), and 15 (79%) survived. Five and 10 year overall survival rates for BA patients were 41.9% and 40.2%, respectively. Conclusions: The study delineated the long-term outcome of BA in an Asian country other than Japan. Survival with native liver after a Kasai operation in Taiwan was similar to that in the American and European series. Limited donors for liver transplantation in the years of the study accounted for the poor overall prognosis of BA patients in this series.

Hepatocellular carcinoma in children: Clinical review and comparison with adult cases

BACKGROUND Hepatocellular carcinoma (HCC) in children was rarely reported and usually included with hepatoblastoma in most studies of pediatric liver malignancies despite different clinical behaviors. The authors report their experience in pediatric HCC and discuss its differences from adult HCC. METHODS A retrospective review of radiographic, laboratory, pathological, and therapeutic data in 55 children with HCC was performed. The liver function was graded by modified Childs classification. Kaplan-Meier survival curves in various therapeutic and Childs groups were plotted, and log-rank test was used to detect differences among survival curves. RESULTS Although children with HCC mostly presented with advanced disease at diagnosis, disturbances of liver function were unremarkable. Sixty-eight percent of cases concurred with liver cirrhosis. The median survivals for resectable, chemotherapeutic, and untreated HCCs were 23, 3, and 2 months, respectively. Resectable HCC significantly posed a much better prognosis. However, the resectability was unsatisfactory (18.2%). Resection was limited because of anatomic unfeasibility including bilateral involvement (62.5%), portal vein thrombi (41.7%), distant metastasis (29.1%), para-aortic lymphadenopathy (18.8%), inferior vena cava thrombi (16.7%), and hilar invasion (6.3%). Distant metastasis was the most ominous for survival in children with unresectable HCC. CONCLUSIONS HCC behaved somewhat differently between children and adults. Surgical resection represented the best hope of long-term survival. The outcome in children could not keep up with that in adults because of a diagnostic delay. Hence, alpha-fetoprotein and sonography screening in carrier children should be worthwhile.

Hepatocellular carcinoma in childhood. Clinical manifestations and prognosis

From December 1964 to November 1989, 71 children from 3 to 17 years of age with the eventual diagnosis of hepatocellular carcinoma (HCC) presented at the National Taiwan University Hospital (Taipei, Taiwan, Republic of China). Forty‐three of them had pathologic proof, whereas 28 were diagnosed on a clinical and laboratory basis. A male predominance (M:F = 3.2:1) was noticed. Most patients presented in a late, advanced stage. Abdominal pain and abdominal mass were the major symptoms and signs, followed by anorexia, fever, and internal bleeding. Hydrocele, purpura, and obstructive jaundice were rare presenting symptoms. Hepatosplenomegaly, superficial venous engorgement, and ascites were the main physical signs. The prognosis for such children with HCC was very poor. Only 10% of the patients survived longer than 1 year after the onset of the initial symptom. Among 49 patients who could be followed, only two had long‐term survival of over 5 years. One patient had a small HCC with internal bleeding, whereas the other had a large HCC with abdominal distention. Both received surgical resection, and a resection was repeated for tumor recurrence in the patient with the large mass. The resectability of these 71 patients was low (9.8%). Resectability and nonicterus seemed to be the factors indicating favorable prognosis. Observation indicated that the prognosis for children with symptomatic HCC is grave but surgical resection, whenever possible, should be carried out.

The rectoanal relaxation reflex and continence in repaired anorectal malformations with and without an internal sphincter-saving procedure

From 1985 to 1994, 27 patients with high- or intermediate type imperforate anus who underwent anorectoplasty were studied for postoperative function, particularly with respect to the rectoanal relaxation reflex and continence. Fourteen of the patients had a rectourogenital fistula and were treated with posterior sagittal anorectoplasty using the fistular end as the neoanus (internal sphincter-saving). Nine patients had a blind rectal pouch and received posterior sagittal anorectoplasty using the trimmed bowel end for reconstruction of the neoanus (incomplete internal sphincter-saving). The other four had Rehbeins mucosa-stripping endorectal pull-through combined with anterior sagittal anorectoplasty (none internal sphincter-saving). A positive rectoanal relaxation reflex was found in 8 of 14 (57.1%), 7 of 9 (77.8%), and 3 of 4 (75%), respectively. It appears that the internal sphincter-saving procedure is not essential for the development of the rectoanal relaxation reflex. Compensation or adaptation most likely contributes to the presence of the rectoanal relaxation reflex, and perhaps to postoperative continence.