Shinpachi Ishizone

Liver regeneration in recipients and donors after transplantation

Reduced-size liver grafts from related donors may not be of an optimal size for adequate function in the recipient. Therefore, liver-graft regeneration is clinically important. We evaluated liver regeneration by liver-volume determinations with serial computed tomography scans in four recipients (aged 9 months to 12 years) and their donors (all fathers of the recipients) after living-related liver transplantation. Standard liver volume was calculated from the recipients body-surface area. In each recipient, the size of the transplanted liver tended to converge to the standard liver volume with time, regardless of whether initial liver-graft volume was smaller or larger than standard liver volume. In addition, transplanted liver in the recipient regenerated much faster than remnant liver in the donor, even though both consisted of the same hepatocytes, which suggests that regeneration is regulated mainly by factors other than the hepatocytes themselves.

Successful left trisegmentectomy for ruptured hepatoblastoma using intraoperative transarterial embolization

Although surgical treatment with resection for spontaneous rupture of hepatoblastoma into the free abdominal cavity is difficult in small children, it may be the only treatment available. The authors describe a 16-month-old girl who showed a progressive decrease in hematocrit and no response to blood transfusion, after spontaneous rupture of a large hepatoblastoma that extended to the pubic bone. Percutaneous transcatheter arterial embolization could not be performed because selective catheterization was impossible. Therefore, emergency surgery was conducted. After intraoperative transcatheter arterial embolization (IOTAE) to control hemorrhage, left trisegmentectomy was performed. The patient then underwent chemotherapy, followed by autologous bone marrow transplantation. The hemorrhage from the ruptured tumor was completely arrested by IOTAE, and the postoperative course was uneventful. Hepatic resection after IOTAE, followed by chemotherapy and bone marrow transplantation, represents a promising treatment for ruptured hepatoblastoma.

Duodenal atresia in dizygotic twins

The authors report duodenal atresia occurring in both members of dizygotic twins who showed no signs of Downs syndrome, and both had normal chromosomal constitutions. They both had the two unlinked end type of atresia in the second portion of the duodenum, and direct end-to-end duodenoduodenostomy was carried out. Their postoperative courses were smooth, and both babies were discharged 32 days after birth. Their mother underwent pituitary adenoma removal and partial parathyroidectomy for multiple endocrine neoplasia (MEN-type I). She had been given hormonal supplement therapy after surgery and became pregnant after exogenous gonadotropin therapy. Environmental factors may be responsible for some cases of duodenal atresia.

Completely covered cloacal exstrophy: recognition of a new clinical sub-entity

A case showing many of the typical visceral features of cloacal exstrophy is reported. The patient had fn imperforate anus, a cecal-cloacal fistula, dehiscence of the pubiic symphysis, and lumbosacral spina bifida with synsingomyelia, but the lower abdominal wall was intact without any visceral extroversion. The pertinent literature was reviewed, and it was found that this case corresponded to t typical case of “completely covered cloacal exstrophy.” Only six cases, including the present one, have so far been reported in the literature. From a clinical viewpoint, it apparently occupies an intermediate position in the wide spectrum of cloacal anomalies between “classical cloacal exstrophy” and “imperforate anus with recto-cloacal fistula,” but anatomatically and embryologically it is definitely a variant of cloacal exstrophy. In other words, it looks like an imperforate anus with recto-cloacal exstrophy, but should be treated as a variant of loacal exstrophy. The anatomy, classification, embryology, diagnosis, and management of this peculiar surgical condition are discussed, and recognition of this entity is urged.

Living-related liver transplantation: Report of experiences at Shinshu University Hospital*

In view of the relative scarcity of pediatric cadaveric donors, living-related liver transplantation has recently been accepted as an alternative approach. It is also the only method of liver transplantation available in countries where cadaveric organ procurement is prohibited. Here we describe our experience of living-related liver transplantation in 17 patients at Shinshu University Hospital. The safety of the donor operation is of paramount importance in this type of liver transplantation. In Japan, retransplantation is very difficult in the event of the liver graft becoming nonfunctional. We have therefore placed emphasis on the donor hepatectomy technique as well as on surgical procedures and postoperative care to prevent graft loss in the recipient. Fifteen of the 17 patients who received liver transplants are currently alive; and 1 died of cytomegalovirus infection, and 1 of pulmonary complications. The actuarial 1-year survival rate for our series, determined by Kaplan-Meier analysis, was 89.5%. Although living-related liver transplantation requires a complicated surgical procedure, it has achieved reasonable results for both donors and recipients. We consider that living-related liver transplantation is a useful and reasonable option for patients requiring liver transplantation.