Toshimichi Hasegawa

Biliary Atresia Associated With Congenital Structural Anomalies

BACKGROUND/PURPOSE Although biliary atresia (BA) is rarely associated with other congenital anomalies, the presence of a distinct subgroup of patients with accompanying structural anomalies such as situs inversus, polysplenia, or portal vein anomalies has been postulated. The authors present 7 patients with this association. METHODS Of 87 patients with BA treated in the past 19 years, 7 (8.0%) have had multiple congenital structural anomalies. RESULTS These anomalies included situs inversus in 4, polysplenia in 5, preduodenal portal vein in 5, absent portal vein in 1, absent inferior vena cava in 2, malrotation of the intestine in 5, and congenital heart disease in 3 patients. In these 7 patients, hepatic portoenterostomy (HPE) was performed at the age from 63 to 158 days with an average of 92 days. The porta hepatis was abnormal in position in 1 patient. The connective tissue at the porta hepatis was diminished in 6 patients. Histologically, liver fibrosis was mild in 2 and moderate in 5 patients. Bile excretion was good initially in all patients but gradually diminished in 5 patients. Five patients had multiple episodes of cholangitis, followed by sepsis, liver failure, or cardiac failure and subsequently died at the age from 2 months to 6 years. Of the other 2 patients who underwent HPE recently, 1 is doing well and the other has had one episode of cholangitis. CONCLUSIONS BA in association with other congenital structural anomalies may have a poor prognosis. These patients have poor bile secretion after HPE mainly because of delayed operation.

Endoscopic variceal ligation in the management of gastroesophageal varices in postoperative biliary atresia

BACKGROUND/PURPOSE Gastroesophageal variceal bleeding is a serious and difficult problem in the long-term management of biliary atresia (BA). Recently, endoscopic approaches have been attempted to manage this problem. The authors have attempted endoscopic variceal ligation (EVL), a less invasive procedure than endoscopic sclerotherapy. METHODS In the past 5 years, 66 EVL procedures using standard flexible endoscope with a diameter of 9 mm (type p-30, XQ200, or XQ240; Olympus, Tokyo, Japan) were performed in 30 separate sessions on 11 postoperative BA patients. The mean age of the children was 7.8 (range, 3 to 15) years. The EVL device was a small elastic O-ring or a loop ligator. RESULTS EVL was performed for emergency hemostasis in two patients and prophylaxis for impending rupture in nine with large, blue varices, or with red spots on the variceal surface. During the initial procedure, all varices were ligated successfully, and reduction in size was noted. Of eight patients who were examined 7 to 14 days after treatment, seven (87.5%) had improved. Eight of 11 patients (72.7%) were finally cured or at least had improved after one to seven sessions of EVL. However, three patients did not show improvement after four to seven sessions because of the reappearance of the varices, development of distal lesions such as gastric varices, and acute gastric mucosal lesions. A technical complication encountered was a slippage of the O-ring in one patient. A technical difficulty was seen in ligating the giant gastric varix in one patient. There was no deterioration of liver function induced by EVL in this entire series. CONCLUSIONS EVL is an effective and feasible treatment of gastroesophageal varices in postoperative BA patients. However, reappearance or reactivation of the varices or emergence of the more distal lesions is likely to occur even after repeated EVL.

Prenatal diagnosis of congenital diaphragmatic hernia and perinatal care: assessment of lung hypoplasia

To assess the severity of lung hypoplasia, we have attempted to measure the lung thorax transverse area ratio (L/T) by using ultrasonic echography and to select immediate surgery after delivery by caesarean section. The evaluation of L/T for an index of lung hypoplasia was made by arterial blood gas data and clinical courses. Of 14 fetuses diagnosed, 13 had left sided CDH and one right sided case, from 11 to 38 weeks of pregnancy, 10 cases survived. The L/T in 14 fetuses with CDH was from 0.08 to 0.36 (mean 0.2 +/- 0.073) and was significantly lower than that of the controls. L/T was correlated best with data of arterial pH, PCO2, preductal A-aDO2 before operation and the duration of mechanical ventilation in survivors. Although L/T was also significantly low in the cases with severe grade, diaphragmatic patch closure and ECMO therapy, no significant differences were noted in L/T between survivors and non-survivors. These results indicated that L/T may predict the severity of lung hypoplasia in CDH and that the combination of perinatal management bases on prenatal diagnosis of CDH and ECMO support may improve the outcome of fatal CDH with severe lung hypoplasia.

FTY720 reduces T-cell recruitment into murine intestinal allograft and prevents activation of graft-infiltrating cells.

Background. Effective immunosuppression is a critical determinant of graft survival in small-bowel transplantation (SBTx). The present study was designed to determine the potency of FTY720, a newly synthesized immunosuppressant, in rat SBTx and examine the phenotype of graft-infiltrating cells to evaluate its effect on intestinal allografts. Materials and Methods. A segment of intestine of Dark Agouti rats was transplanted heterotopically into Lewis rats. The recipients were treated with or without oral FTY720 at a dose of 1 mg/kg per day. Six days after surgery, peripheral blood lymphocytes and lymphocytes from the mesenteric lymph nodes, Peyer’s patches, intraepithelial site, and lamina propria of the intestinal allograft were isolated. After the number of lymphocytes in each site was counted, the lymphocyte subpopulations in the intestinal allograft were evaluated by means of a FACScan flow cytometer using several monoclonal antibodies. Results. FTY720 treatment significantly prolonged recipient survival and strongly inhibited rejection histologically in comparison with control rats. FTY720 immunosuppression resulted in a marked reduction of lymphocyte number in the graft epithelium and lamina propria and the proportion of CD8+ and CD25+ cells. FTY720 also significantly decreased T-cell receptors and increased B cells in the graft Peyer’s patches. Conclusion. FTY720 promoted long-term SBTx recipient survival and maintained the architecture of intestinal allografts. FTY720 immunosuppression may be associated with a reduction of T-cell recruitment subsequent to the redistribution of lymphocyte subpopulations to control the proliferation and activation of graft-infiltrating cells in intestinal allografts.

Does pancreatico-biliary maljunction play a role in spontaneous perforation of the bile duct in children?

Abstract Spontaneous perforation of the bile duct (SPBD) is a rare disease in children. Pancreatico-biliary maljunction (PBM) has been postulated to contribute to its etiology. We have treated three children with SPBD over 30 years, two of whom had PBM. There was one boy and two girls aged 10 months to 2 years with symptoms of abdominal distension, vomiting, abdominal pain, jaundice, and acholic stools. The diagnosis of SPBD was made by paracentesis showing biliary ascites, and primary biliary and intra-abdominal drainage was performed in all cases. The site of perforation was at the connection of the common bile duct (CBD) with the cystic duct in all cases. In two cases reflux of contrast into the pancreatic duct was noted, the common channel was long (17 and 12 mm, respectively), and the bile amylase level in the CBD was abnormally high (50,000 and 67,000 IU/l, respectively). In the third patient there was no reflux of contrast into the pancreatic duct and the bile amylase and trypsin levels in the CBD and gallbladder were not measurable. Thus, SPBD in children may not be due solely to PBM, but may involve multiple mechanisms.

Revision laparoscopy for incarcerated hernia at a 5-mm trocar site following pediatric laparoscopic surgery.

We report the case of a 6-month-old female infant who developed post-operative bowel obstruction due to an incarcerated hernia through a 5-mm laparoscopic wound. The patient underwent laparoscopic Nissen fundoplication for gastroesophageal reflux. On day 6, she showed symptoms of ileus, and the diagnosis of a trocar wound hernia was made on day 13. The herniated intestine was reduced and the defective peritoneum and fascia were closed under relaparoscopic guidance, thus avoiding full-scale laparotomy. A trocar wound hernia causing early postoperative bowel obstruction is a rare complication, especially at 5-mm trocar puncture sites. Intraoperative dislodgment and reinsertion of working trocars may create fascial defects larger than the actual size of the trocar. All laparoscopic puncture wounds, even those <10 mm in size, should be closed at the fascial level in infants. Revision laparoscopy is considered preferable to manage trocar site complications in children.

A New Technique of Laparoscopic Obturator Hernia Repair:Report of a Case

An 84-year-old woman presented with ileus. Ultrasonography, a computed tomography scan, and small bowel contrast examination showed a Richter-type hernia in her left obturator orifice. Under general anesthesia, laparoscopic surgery with low-pressure (4mmHg) pneumoperitoneum was carried out using a peritoneal needle retractor, and a reduction of the strangulated intestinal loop was thus achieved. Because the hernial opening measured 5mm in diameter, it could be closed with four pieces of End-Universal stapler without polypropylene mesh. The ischemic ileum was resected, and the bowel was anastomosed extracorporeally with a minimal skin incision. She was ambulant on the first postoperative day, and her postoperative course was good. Obturator hernias are rare, but when a definitive diagnosis is made in such elderly patients, laparoscopic repair using the peritoneal needle retractor is recommended for minimally invasive surgery. We recommend doing the repair with an End-Universal stapler, since this procedure is more simple and useful for preventing infection than using polypropylene mesh in such a strangulated case.

A case of tracheal agenesis surviving without mechanical ventilation after external esophageal stenting.

Tracheal agenesis is a rare and usually lethal congenital malformation of the forgut. Although some infants can be resuscitated with an intra-esophageal intubation temporarily, long-term airway management is difficult because of the collapsing airway. We report a long-term survivor with tracheal agenesis in whom a Gortex external esophageal stent using radial traction sutures was applied to prevent the esophagus from collapsing. The patient was discharged from our hospital without mechanical ventilation or oxygen inhalation at 10 months of age. Our procedure has a potential to establish a long-term steady airway in patients with tracheal agenesis. The detail of the procedure is presented and the related literature is reviewed.

Successful percutaneous transluminal angioplasty for hepatic artery stenosis in an infant undergoing living-related liver transplantation

Abstract: A 1‐yr‐old girl underwent a living‐related liver transplant, with reconstruction of hepatic artery of 2 mm in diameter under microscopy. She developed intestinal perforation requiring closure on day 4 post‐transplant and suffered from hepatic artery stenosis (HAS) on post‐transplant day 9. Conservative therapies, such as intravenous or transluminal administration of anti‐coagulants, vasodilators or fluids, were unsuccessful and caused remarkable general edema and multiple arrhythmias as a result of increased preload. On day 15 post‐transplant, because flow velocity was remarkably reduced (as shown by Doppler ultrasound) the patient underwent percutaneous transluminal angioplasty (PTA) using a kit for coronary angioplasty. The balloon catheter was inflated [first: 1.5 mm diameter, 4 atmospheric pressure (a.p.) for 30 seconds (s); second: 2.0 mm diameter, 4 a.p. for 30 s; third: 2.5 mm diameter, 10 a.p. for 30 s]. The stenosis was successfully dilated without any complication. The patient has been doing well with normal liver functions for 4 months after PTA. From this experience, PTA can be performed for HAS after liver transplantation, even in an infantile case, with a careful technique and a special device.

Living-related liver transplantation for biliary atresia associated with polysplenia syndrome

Abstract: This report describes a 1‐yr‐old boy with biliary atresia (BA) and polysplenia syndrome (PS) who underwent successful living‐related liver transplantation (LTx). At the time of initial hepatic portoenterostomy, he was noticed to have a preduodenal portal vein (PV), non‐rotation of the intestine, and polysplenia. Because he did not achieve good bile excretion, he underwent a living‐related LTx (using a left lateral segment from his mother) at the age of 14 months. Evaluation of the vascular anatomy was made by angiography, magnetic resonance imaging (MRI), computerized tomography (CT), and Doppler ultrasound. The PV was stenotic from the confluence of the superior mesenteric vein (SMV) and splenic vein (SpV) to the hepatic hilum. The retrohepatic inferior vena cava (IVC) was deficient cranially to the renal vein and was connected to the azygous vein. The supra‐hepatic IVC was detected below the diaphragm and was connected to three hepatic veins. The common hepatic artery (HA) originated from the superior mesenteric artery. At LTx, the PV was dissected to the level of confluence of the SMV and the SpV, from which the venous graft was interposed using the donors ovarian vein. Three hepatic veins were plastied into one orifice, which was anastomosed to the grafts hepatic vein under the diaphragm. The graft vascularity and function has been good for 1 yr after LTx. In the present case, sufficient pre‐LTx evaluation of vascular anomalies seemed to help performance of the successful LTx.