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.     

Outcomes of emergency surgery for acute abdomen in dialysis patients: experience of a single community hospital

Purpose

Long-term dialysis often causes physiological and metabolic problems that may affect the outcomes of surgery. The aim of this study was to elucidate whether emergency surgery for acute abdomen yields similar outcomes in dialysis patients versus non-dialysis patients.

Methods

The subjects were 126 patients who underwent emergency surgery for acute abdomen between January, 2007 and November, 2011 in our hospital. They were divided into a dialysis group (HD group; n = 9) and a non-dialysis group (non-HD group; n = 117) and their postoperative morbidity and mortality were compared.

Results

Postoperative morbidity and mortality were significantly worse in the HD group. All 9 of these patients succumbed to postoperative complications versus only 5 of the 117 patients in the non-HD group.

Conclusion

The outcomes of emergency surgery for acute abdomen were significantly worse for dialysis patients than for non-dialysis patients. Prompt diagnosis, initiation of the most suitable surgical procedure, and meticulous postoperative cares are imperative to improving the surgical outcomes of dialysis patients.     

Development of intrahepatic biliary stones after excision of choledochal cysts

Background: The incidence of intrahepatic cholelithiasis and cholangitis has not yet been well studied postoperatively in patients with choledochal cysts. Methods: One hundred three patients with choledochal cysts had operative cholangiography, underwent standard excision of a choledochal cyst with Roux-en-Y hepatico-jejunal anastomosis, and were at a mean follow-up of 12[frac12] years. The incidence of intrahepatic bile duct stones was analyzed according to the 3 morphologic types of intrahepatic bile duct observed at initial operative cholangiography: type 1, no dilatation of the intrahepatic bile ducts; type 2, dilatation of the intrahepatic bile ducts but without any downstream stenosis; and type 3, dilatation of the intrahepatic bile ducts associated with downstream stenosis. Initially, there was no evidence of intrahepatic bile duct stones in any of the 103 patients. Results: Among 50 type 1 patients, intrahepatic cholelithiasis developed in only 1 patient (2%). Among 43 type 2 patients, 1 patient (2%) had intrahepatic cholelithiasis, and 2 (5%) had postoperative cholangitis. Among 10 type 3 patients, 4 (40%) had intrahepatic cholelithiasis (P [lt ] .01), and 3 (30%) had postoperative cholangitis. Time intervals between the initial surgery and the first identification of intrahepatic stones ranged from 3 to 22 years. Conclusions: One of the major causes of formation of intrahepatic cholelithiasis has been clarified; patients with intrahepatic biliary dilatation with downstream stenosis can get intrahepatic bile duct stones long after excision of a choledochal cyst.     

Clonality analysis of childhood neuroblastoma by polymerase chain reaction for the human androgen receptor gene

Neuroblastoma is a unique tumor that occurs during childhood. Tumors in infants less than one year of age often regress spontaneously and usually have an excellent prognosis, whereas those in older patients are aggressive, leading to a fatal outcome. To shed light on these unique aspects of this tumor, we investigated the clonality of neuroblastomas by analyzing the inactivation patterns through methylation of the human androgen receptor gene on the X chromosomes in female patients. Neuroblastoma tissue samples were obtained from 12 patients, including 10 patients less than one year of age with tumors at stage 1, 2 or 4S and 2 older patients with stage 4 tumors. Except for 3 uninformative samples of infants, all the informative samples unexpectedly exhibited random methylation pattern, and tumor tissues purified with microdissection technique exhibited the same results. These data indicate that neuroblastomas examined are polyclonal in origin, an unusual finding for a neoplasm, which might be relevant to the unique aspects of neuroblastoma.     

Monitoring neutrophil engraftment in allogeneic stem cell transplantation by flow cytometric analysis of neutrophil-specific antigens NA1 and NA2

Neutrophil-specific antigen (NA) expression on neutrophils was analysed in 18 Japanese children before and after allogeneic stem cell transplantation (allo-SCT) with myeloablative regimen. Donor-recipient NA-incompatibility was present in one of eight NA1/NA2 heterozygous patients and eight of 10 NA1/NA1 or NA2/NA2 homozygous patients. After allo-SCTs from NA-incompatible donors, a neutrophil recipient-to-donor conversion was confirmed in all cases. Conversion to donor NA type was complete before the absolute neutrophil count reached 0.1 x 10(9)/l. These observations indicate that flow cytometric analysis of NA antigens is a simple and useful method for monitoring neutrophil engraftment in NA-incompatible allo-SCT.     

Living donor liver transplantation with hyperreduced left lateral segments

Liver transplantation is now an established technique to treat children with end-stage liver disease. Implantation of left lateral segment grafts (Couidaud's segments II and III) can be a problem in small infants because of a large-for-size graft. Reduced left lateral segmental liver transplantation has been recently introduced for small infants to mitigate the problem of large-for-size graft. Further reduction of the left lateral segment graft increases the possibility of supplying an adequate hyperreduced left lateral segment graft as an alternative surgical technique. We report 3 cases of our experience of transplantation using hyperreduced left lateral segment grafts from living donors.     

Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation

This report describes a case of Abernethy malformation associated with hepatopulmonary syndrome, which was resolved after shunt ligation. The clinical course indicated that hepatopulmonary syndrome can develop in Abernethy malformation in which liver function and portal pressure is normal, and liver transplantation is not the exclusive therapy for hepatopulmonary syndrome. The levels of endotoxin and endothelin 1 in the shunt blood were high, whereas those of tumor necrosis factor α and interleukin-1β were within reference range. Although pathogenesis of hepatopulmonary syndrome remains unknown, the findings in this case suggest that bacterial translocation as well as elevated endothelin 1 may play a causal role in development of hepatopulmonary syndrome.