Usefulness of a recanalized umbilical vein for vascular reconstruction in pediatric hepatic surgery.

PURPOSE: Pediatric surgeons currently engage in various abdominal vascular surgeries, which sometimes require vascular conduits or grafts. Herein, we report our experience with patients undergoing vascular reconstruction using a recanalized umbilical vein (rUV) and their long-term outcome. METHOD: Five patients with extrahepatic portal vein obstruction (EHPVO) underwent mesenterico-/porto-left portal vein (PV) bypass surgery using a short rUV conduit with an interposition vein graft. A sixth neonate with a huge hepatic tumor underwent PV reconstruction with anastomosis of rUV to the proximal PV stump following right hepatectomy with partial PV resection. A seventh patient underwent living donor liver transplantation for recurrent hepatoblastoma. The hepatic inferior vena cava (IVC) was resected because of tumor involvement and reconstructed by transposition of the infrahepatic IVC and interposition of rUV obtained from the donor liver graft. RESULTS: Sufficient flow through rUV was achieved and maintained in all patients without any complications during follow-up (0.7-6.9 years). Esophageal varices, splenomegaly, and other laboratory test abnormalities because of portal hypertension disappeared after surgery in patients with EHPVO. CONCLUSION: Our experience confirmed the usefulness and long-term patency of rUV as an entry to the intrahepatic PV and as a free vascular graft to reconstruct PV or IVC.

Recanalized umbilical vein as a conduit for mesenterico/porto-Rex bypass for patients with extrahepatic portal vein obstruction.

PURPOSE: Mesenterico-left portal vein (meso-Rex) bypass is as an effective modality for restoring intrahepatic portal perfusion in patients with extrahepatic portal vein obstruction. Achieving sufficient patency is difficult with end-to-side anastomosis of a bypass graft to a small or hypoplastic left portal vein in the Rex recessus. Here, we describe the use of a recanalized umbilical vein in the round ligament as a conduit for bypass construction in two patients. METHODS: Case 1 was an 11-year-old boy diagnosed with rupture of the esophageal varices and hypersplenism due to congenital extrahepatic portal hypertension. Because of persistent hypersplenism and thrombocytopenia, he underwent meso-Rex bypassing with a left iliac vein graft interposed between the umbilical vein and the superior mesenteric vein. Case 2 was a neonate with a large hepatic tumor (mesenchymal hamartoma) that developed abdominal compartment syndrome at birth. The tumor was removed by right hepatectomy with excision of the portal vein bifurcation at 3 days of age. Porto-Rex bypassing was accomplished by end-to-end anastomosis between the portal vein trunk and the umbilical vein. RESULTS: Sufficient hepatopetal portal flow through the umbilical vein was achieved in both patients and maintained for over 16 and 13 months, respectively. Although hypersplenism remained in Case 1, intrahepatic portal vein branches gradually widened and the cavernoma in the hepatic hilum disappeared within 2 months. Neither patient had symptoms or signs of portal hypertension at the most recent follow-up. CONCLUSION: Using the umbilical vein as a vein conduit may facilitate construction of a meso/porto-Rex bypass and restore intrahepatic portal vein perfusion in patients with extrahepatic portal vein obstruction.

Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children's hospital.

OBJECTIVE: Sequential strategies combining the Kasai operation as a first-line treatment and liver transplantation as a second-line option, if necessary, have been accepted for patients with biliary atresia (BA). To understand the role of the Kasai operation in the treatment of BA, it is necessary to analyze the long-term outcome of the operation alone and to evaluate the present status of survivors retaining their native livers. MATERIALS AND METHODS: A retrospective chart review was carried out for a group of 80 patients who had undergone the Kasai operation between 1970 and 1986 at the Kanagawa Children's Medical Center. RESULTS: The 5-, 10-, and 20-year survival rates of patients with their native livers were 63%, 54%, and 44%, respectively. The survival rates varied significantly depending on the type of BA, age at initial Kasai operation, era of surgery, and surgical method. By age 20, nearly half of the adult survivors had already developed liver cirrhosis and its sequelae. Episodes of cholangitis and gastrointestinal bleeding occurred after 20 years of age in 37% and 17% of the adult patients, respectively, and 20% of the adult patients died of liver failure or underwent living-related partial liver transplantation in their 20s. Five female patients gave birth to a total of 9 children, and 1 male patient fathered a child. CONCLUSIONS: Although increasing numbers of patients with BA survive 20 years or more after the Kasai operation, meticulous lifelong postoperative care should be continued for the survivors because of the possibility of hepatic deterioration.

Anorectal malformation associated with a perineal protrusion of the rectal mucosa: case presentation.

We present a rare case of a female neonate with an imperforate anus associated with a perineal mass which may correspond to an extrophied rectal duplication. Associated anomalies were thoracic hemivertebrae and a multicystic dysplastic kidney. Excision of the perineal lesion followed by anal transplantation and perineal reconstruction corrected the anomaly.

Papillary carcinoma with extensive squamous metaplasia arising from thyroglossal duct cyst in an 11-year-old girl: significance of differentiation from squamous cell carcinoma: a case report.

We report a case of papillary carcinoma (PC) with extensive squamous metaplasia arising from a thyroglossal duct cyst (TDC) that required differential diagnosis from squamous cell carcinoma (SCC). An 11-year-old Japanese girl presented with a 9-month history of an anterior-midline neck mass that was clinically diagnosed as TDC. Open neck biopsy revealed nested proliferation of atypical squamous cells within the cystic structures, and SCC arising from TDC was initially suspected. Further examination, however, including immunohistochemistry, revealed the tumor to be of thyroid cell origin. The patient underwent wide local resection of the thyroglossal duct carcinoma by Sistrunk procedure and cervical lymph node dissection. Microscopically, the diagnosis was of PC with extensive squamous metaplasia and metastasis to the medial submandibular lymph node. Distinction of squamous metaplasia in PC from SCC is sometimes difficult, but has a significant effect on postoperative management.

Multiple intestinal atresia and congenital bilateral perisylvian syndrome in a surviving monochorionic twin with intrauterine death of the co-twin.

This report presents a case of a surviving monochorionic twin with multiple intestinal atresia and congenital bilateral perisylvian syndrome, which developed after the intrauterine death of the cotwin. The pathology of the placenta demonstrated vein-to-vein communication between the twins and multiple intravascular thrombi in the dead cotwin.

Severe gastric damage caused by button battery ingestion in a 3-month-old infant.

Ingestion of a button battery has been considered a serious problem, causing necrosis and perforation, when impacted in the esophagus. However, such batteries in the stomach rarely cause any harm to the gastric wall, which is regarded as evidence supporting the use of conservative treatment. We present the rare case of a 3-month-old infant with severe gastric wall injury caused by a button battery lodged in the stomach. The present case suggests that button batteries located in the stomach should be removed as soon as possible, especially in infants.

Aplasia of the dorsal pancreas and choledochal cyst.

The authors describe a rare case of choledochal cyst and aplasia of the dorsal pancreas complicated with chronic pancreatitis. A 9-year-old boy presented with obstructive jaundice. After biliary drainage using pericutaneous transhepatic gallbladder drainage (PTGBD) technique, the patient underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy. The association of choledochal cyst and aplasia of the dorsal pancreas with chronic pancreatitis has never been reported previously.

Evaluation of the PELD risk score as a severity index of biliary atresia.

PURPOSE: The authors evaluated the validity of the Pediatric End-Stage Liver Disease (PELD) Risk Scoring System as a severity index for patients with biliary atresia. METHODS: Individual hospital records of 104 patients with biliary atresia were reviewed at our institution and divided into 3 groups: nontransplant survivors (n = 61), nontransplant deaths (n = 17), and transplant patients (n = 26). PELD risk scores were calculated according to Wiesner et al, multiplied by 10, and rounded to the nearest integer, as is done in determining model of end-stage liver disease (MELD) scores. RESULTS: The PELD scores showed a significant difference between nontransplant survivors (range, -21 to 15) and dying nontransplant patients during their last few months of life (range, 2 to 40). No survivors except those below the age of one year recorded scores above 10. Transplant patients had higher scores (range, -5 to 37) before transplantation than nontransplant survivors. However, the scores were not elevated in elderly patients with intractable cholangitis, fulminant variceal rupture, and hepatopulmonary syndrome. CONCLUSIONS: PELD profiling is a useful scoring system for selecting patients with the most severe liver dysfunction caused by biliary atresia. However, we advise caution in using this system for patients under the age of 1 year and for older patients with long-term complications.

Improved neutrophil function in a glycogen storage disease type 1b patient after liver transplantation.

UNLABELLED: Patients with glycogen storage disease type 1b (GSD1b) not only show hepatomegaly, hypoglycaemia and lactic acidosis, but also neutropenia and neutrophil dysfunction. Here, we report improvement of neutropenia and neutrophil function in a 22-year-old male GSD1b patient who had undergone living-related partial liver transplantation (LT) at 18 years of age. After LT, the patient's infectious episodes decreased, gastrointestinal symptoms ameliorated, neutrophil counts increased, and neutrophil function tests normalised. CONCLUSION: Although it is not known whether this improvement was causally related to liver transplantation, this may be the first recorded case of restoration of neutrophil dysfunction in a glycogen storage disease type 1b patient.

Postoperative corticosteroid therapy for bile drainage in biliary atresia--a nationwide survey.

PURPOSE: Members of the Japanese Biliary Atresia Society were surveyed to determine their current practice regarding early use of corticosteroids after Kasai's operation. METHODS: Questions included the patient's background data, dosage, timing, complications, and outcome. Anicteric survival with the native liver was statistically compared between groups categorized by steroid dosage using Kaplan-Meier analysis. RESULTS: Among 54 institutions surveyed, a total of 222 patients with uncorrectable BA were collected from 34 responders, including 208 patients who received steroid therapy and 14 without it. Prednisolone was started during the first postoperative week in 31% and during the second week in 64%. Perforation and peritonitis occurred in 1 patient given 3 mg/kg/d of prednisolone on postoperative day 1. The survival rates of the steroid and nonsteroid groups were 58.0% and 35.7%, respectively (P = .052). The initial dose of prednisolone was or=4.0 mg/kg/d in 108 patients. The survival rates of the group receiving >or=4.0 mg/kg/d and the nonsteroid group were 58.9% and 35.7%, respectively (P = .0494). CONCLUSIONS: (1) Most surgeons use steroids. (2) Although the anicteric survival rate was higher in the steroid group, the number of patients in the nonsteroid group was too small to draw conclusions. (3) The recommended initial steroid dose is >or=4.0 mg/kg/d. (4) Complications are uncommon if administration is not started too early.

Pericardial hemangioma presenting fetal cardiac tamponade and postnatal bronchostenosis.

A case of pericardial hemangioma is described which was resected in the neonatal period due to its effect on the cardiopulmonary system. Preoperative differential diagnosis of a teratoma was difficult. Surgical extirpation resulted in massive bleeding and postoperative bronchomalacia. These complications suggest that we should choose a conservative therapy as often as possible.

Congenital absence of the portal vein: clinical and radiologic findings.

Congenital absence of the portal vein (CAPV) is a rare anomaly in which the intestinal and splenic venous drainage bypasses the liver and drains into the systemic veins through various venous shunts. In patients with CAPV, the portosystemic shunting causes disruption of the enterohepatic circulation and leads to various clinical manifestations. CAPV can be diagnosed without invasive techniques. This article illustrates the clinical and radiologic findings (including ultrasound, CT, and MRI) of CAPV.

No incidence of port-site recurrence after endosurgical procedure for pediatric malignancies.

Port-site recurrence (PSR) following laparoscopic procedures has been an unpredictable complication in adult cancer patients; however, no data exist about this phenomenon in the pediatric field. The aim of this study was to determine whether PSR, following endosurgical procedure for malignancies, is a typical complication or a rare event in the pediatric population. Eighty-one questionnaires were mailed to members of The Japanese Society of Pediatric Endosurgeons. They were asked to provide a list of their institutions that had experience with PSR after endosurgical procedures for pediatric malignancies. Among 29 institutions, a total of 129 endosurgical procedures for pediatric malignancies were reported; these included 85 laparoscopic and 44 thoracoscopic procedures, performed on 104 neuroblastomas, 8 hepatoblastomas, 7 nephroblastomas, and 10 other tumors. Of the 104 neuroblastomas, 83 were found by mass screening using high levels of urinary vanillylmandelic acid and homovanillic acid. Sixty-five of the 83 patients had their tumor excised, and 18 had their tumor biopsied by endosurgical procedures. Additionally, 47 of these patients did not require any postoperative chemotherapy. No incidence of PSR was reported in any of the patients that underwent endosurgical procedures. The PSR following endosurgical procedure is a rare phenomenon in the pediatric population. Both, laparoscopic and thoracoscopic procedures, are safe and recommended for treating pediatric malignancies, especially mass-screened neuroblastomas.