Shinichi Naito

Long-Term Results After Nonshunt Operations for Esophageal Varices in Children

The clinical results of nonshunt operations for esophageal varices in 15 children were evaluated. The varices were caused by congenital extrahepatic portal obstruction (EHPO) in 10, liver cirrhosis or fibrosis (C/F) in 3, and idiopathic portal hypertension (IPH) in 2. The operative procedures were transthoracic esophageal transection with paraesophageal devascularization (TR) for 2 EHPO patients under 5 years of age, TR combined with splenectomy and paragastric devascularization (Sugiura procedure) for 11 (8 EHPO, 3 C/F), and splenectomy with devascularization (SP) or splenectomy for the 2 IPH patients. In the EHPO patient under 5 years of age, TR is associated with a likelihood of gastric or esophageal hemorrhage resulting from hypersplenism, gastric congestion, or persistent distal esophageal varices, which can be treated with partial splenic arterial embolization (PSE), endosclerotherapy, or an additional abdominal procedure. The Sugiura procedure has provided satisfactory long-term results, without rebleeding from esophageal varices, in patients with EHPO and C/F for 1 to 20 years. But EHPO patients who undergo the Sugiura procedure before age 6 can have gastric hemorrhage, because of mucosal congestion, for more than 10 years after the procedure, and selective gastric arterial embolization (GAE) might be necessary. In some EHPO patients, especially young ones who have variceal bleeding, a significant increase in hepatopetal portal flow may not develop, but hepatofugal natural shunts may progress. Therefore we recommend direct operative procedures, ie, TR for patients < or = 6 years of age and a one- or two-stage Sugiura procedure for those over 7 years old.(ABSTRACT TRUNCATED AT 250 WORDS)

Invasive cholangiocarcinoma identified in congenital biliary dilatation in a 3-year-old boy.

We herein report a 3-year-old male demonstrating invasive cholangiocarcinoma (CC) associated with congenital biliary dilatation (CBD). A 3-year-old Japanese boy was admitted to our hospital with abdominal pain and vomiting. Computed tomography and magnetic resonance imaging demonstrated a dilated extrahepatic bile duct. A diagnosis of Todanis type 1a CBD was made. Intraoperative cholangiography demonstrated the presence of pancreaticobiliary maljunction but could not reveal any tumor lesion in the bile duct. The excision of extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy were performed. On gross inspection, we could not find any tumor lesion in the resected specimen. However, the postoperative histopathologic examinations confirmed the presence of well-differentiated tubular adenocarcinoma with lymphovascular invasion. Most of the carcinoma remained within the mucosal layer, and the carcinoma was identified at both the distal and proximal surgical margins of the bile duct. We scheduled additional surgery to eradicate the residual carcinoma, but informed consent for the extra surgery could not be obtained. A close follow-up with abdominal computed tomography has been going on without either additional surgery or adjuvant chemotherapy about for 1 year. The patient has so far been clinically doing well without any obvious symptoms of recurrent disease. To our knowledge, this report is the youngest case of CC associated with CBD.

Familial occurrence of congenital bile duct dilatation

The occurrence of congenital bile duct dilatation (CBD) in both a mother and her daughter was recently experienced at Niigata University Hospital and Niigata Shimin Hospital. Bile duct dilatation with anomalous pancreaticobiliary ductal junction (AP-BDJ) was disclosed in both. Intrahepatic bile duct dilatation was only in the mother. Removal of dilated bile duct, cholecystectomy, and hepaticojejunostomy were performed in both. Fourteen cases of CBD from seven families were collected from literature and discussed.

Massive pulmonary gas embolism in a neonate with ileal atresia and meconium peritonitis

We presented the case of a neonate with portal venous gas and pulmonary gas embolism. The patient presented with severe respiratory distress and abdominal distension 12 hours after birth. An ultrasound revealed intravascular microbubbles moving into a pulmonary artery that were traveling from the portal venous system through a ductus venosus. Additional clinical observations were hypotension and a sudden decrease in end-tidal carbon dioxide with a markedly discrepant high Pco(2), indicating a massive pulmonary gas embolism. Operative findings revealed congenital ileal atresia and meconium peritonitis with abscess. Gas-forming Escherichia coli was recovered from the abscess contents. The patient had respiratory distress, shock, disseminated intravascular coagulation, and intractable diarrhea but eventually recovered and was discharged on the 131st postoperative day.

Intestinal neuronal dysplasia-like histopathology in infancy

The present patient was delivered at a gestational age of 27 weeks. She had abdominal bloating with symptoms of respiratory distress. We suspected Hirschsprung disease (HD) or small intestinal stricture, but examinations were not definitive. Exploratory laparotomy was performed at 97 days of age. Intraoperative findings showed no evidence of small intestinal stricture or changes in intestinal caliber. A transanal drainage tube was inserted, and decompression therapy and intestinal lavage were started. Rectal mucosal biopsy was performed at 184 days of age, and HE and acetylcholinesterase staining showed intestinal neuronal dysplasia (IND)‐like pathological findings. At 15 months, giant ganglia were no longer present on follow‐up rectal mucosal biopsy, and the pathological diagnosis was normoganglionosis. It should be recognized that while the enteric nervous system is developing, IND‐like pathological findings may be seen as a pathological condition during the maturation process.

Rectal membranous atresia: sutureless endoscopic repair

Rectal atresia with a normal anus is rare, and the choice of the surgical procedure employed for its correction is controversial. A male infant with supralevator-type rectal membranous atresia who had presented with perforation of the sigmoid colon at 2 days of age and subsequently had a sigmoid colostomy for decompression underwent resection of the atretic lesion with the aid of the colonofiberscope at 1 month of age. Bougienage was employed for 6 months after the operation. No functional deficits have been observed, and the infant remains continent.

Near-infrared fluorescence cholangiography with indocyanine green for biliary atresia. Real-time imaging during the Kasai procedure: a pilot study

IntroductionHepatoportoenterostomy (HPE) with the Kasai procedure is the treatment of choice for biliary atresia (BA) as the initial surgery. However, the appropriate level of dissection level of the fibrous cone (FC) of the porta hepatis (PH) is frequently unclear, and the procedure sometimes results in unsuccessful outcomes. Recently, indocyanine green near-infrared fluorescence imaging (ICG-FCG) has been developed as a form of real-time cholangiography.MethodsWe applied this technique in five patients with BA to visualize the biliary flow at the PH intraoperatively. ICG was injected intravenously the day before surgery as the liver function test, and the liver was observed with a near-infrared camera system during the operation while the patient’s feces was also observed.ResultsIn all patients, the whole liver fluoresced diffusely with ICG-containing stagnant bile, whereas no extrahepatic structures fluoresced. The findings of the ICG fluorescence pattern of the PH after dissection of the FC were classified into three types: spotty fluorescence, one patient; diffuse weak fluorescence, three patients; and diffuse strong fluorescence, one patient. In all five patients, the feces evacuated after HPE showed distinct fluorescent spots, although that obtained before surgery showed no fluorescence. One patient with diffuse strong fluorescence who did not achieve JF underwent living related liver transplantation six months after the initial HPE procedure. Four patients, including three cases involving diffuse weak fluorescence and one case involving spotty fluorescence showed weak fluorescence compared to that of the surrounding liver surface.ConclusionWe were able to detect the presence of bile excretion at the time of HPE intraoperatively and successfully evaluated the extent of bile excretion using this new technique. Furthermore, the ICG-FCG findings may provide information leading to a new classification and potentially function as an indicator predicting the clinical outcomes after HPE.