Takeshi Miyano

Laparoscopic extramucosal pyloromyotomy versus open pyloromyotomy for infantile hypertrophic pyloric stenosis: Which is better?☆

BACKGROUND/PURPOSE The aim of this study was to evaluate the advantages or disadvantages of laparoscopic pyloromyotomy compared with open transumbilical fold pyloromyotomy. METHODS Thirty consecutive laparoscopic extramucosal pyloromyotomies (LP) performed from 1994 to 1997 were compared with 30 consecutive open pyloromyotomies (OP) performed during the same period with regard to age at operation, body weight, thickness of hypertrophied pyloric muscle, operating time, time of return to full feeding, frequency of postoperative emesis, surgical complications, and degree of surgical stress reflected by interleukin-6 (IL-6). LP was performed according to conventional techniques, and OP was performed using a transumbilical fold approach. RESULTS The groups were matched for age at operation, preoperative clinical and physical status, laboratory data, and size of the hypertrophied pylorus assessed by ultrasonography. There was a learning curve with LP; the average operating time required for the first 10 cases was significantly longer than the time required for OP, but later cases took just as long as OP cases. Time taken to full feeding was significantly shorter in the LP group than the OP group (LP, 38 hours v OP, 64 hours). One case was converted from LP to OP because of mucosal perforation. The incidence of postoperative emesis was significantly higher in the OP group than in the LP group (OP, 25% v LP, 3%). The mean length of hospitalization was significantly shorter in LP (P < .01). The intraoperative peak values of IL-6 in LP were significantly lower than those in the OP group (P < .01). CONCLUSIONS The advantages of LP are improved cosmesis, decreased surgical stress with earlier postoperative recovery, and shorter hospitalization. Because LP uses reusable devices, and the mean period of hospitalization is shorter, average operating costs could be reduced, representing a net saving in total hospital charges.

Complications after cyst excision with hepaticoenterostomy for choledochal cysts and their surgical management in children versus adults

The aim of this study was to review the cases 200 children and 40 adults who had cyst excision combined with hepaticoenterostomy (CEHE) for choledochal cyst, with particular emphasis on post-CEHE complications and their surgical management. Patients who had CEHE at the age of 15 years or less were defined as children, and those aged 16 years or older were defined as adults. The mean age when patients became initially symptomatic was 3 years in children and 26 years in adults. Eleven adults became symptomatic as children (< or = 15 years of age). The mean age of CEHE in children and adults was 4.2 years and 35 years, respectively. The time interval between the onset of initial symptoms and CEHE was significantly less in children than in adults (P < .0001). Of the 200 children, 176 had primary CEHE, and 24 had secondary CEHE converted from cystoenterostomy or other biliary surgery. Seventy children had intraoperative cyst endoscopy, which enabled us to examine the proximal intrahepatic bile ducts for stenosis and debris, and to wash out debris, protein plugs, and stones from the intrapancreatic ducts. Of the 40 adults, 22 had primary CEHE, 18 had secondary CEHE. The mean follow-up period was 10.9 years in children and 10.7 years in adults. The number of patients with post-CEHE complications in children and adults was 18 (9.0%) and 17 (42.5%), respectively. The post-CEHE complication rate in children was significantly lower than in adults (P < .0001). The 18 children had 25 post-CEHE complications such as cholangitis, intrahepatic bile duct stones, pancreatitis, stone formation in the intrapancreatic terminal choledochus or pancreatic duct, and bowel obstruction. Twenty-seven post-CEHE complications developed in the 17 adults including 2 cases of cholangiocarcinoma. There were no post-CEHE complications in the 70 children who had intraoperative cyst endoscopy. No stone formation was seen in the 145 children who had CEHE at the age of 5 years or less. Eight stone formations were seen in seven (12.7%) of the remaining 55 children aged over 5 years. Stones developed in seven (17.5%) adults. The incidence of post-CEHE stone formation in children aged 5 years or less was significantly lower than in other children and adults (P < .0001). Reoperation was required in 15 children: revision of hepaticoenterostomy in 4, percutaneous transhepatic cholangioscopic lithotomy (PTCSL) in 1, excision of intrapancreatic terminal choledochus in 2, endoscopic sphincterotomy of the papilla of Vater in 1, pancreaticojejunostomy in 1, and laparotomy for bowel obstruction in 6. Ten adults required reoperations: revision of hepaticoenterostomy in 2, PTCSL in 2, left hepatic lobectomy in 1, endoscopic sphincterotomy in 2, exploratory laparotomy in 2, and adhesiolysis in 1. The authors conclude that early diagnosis followed by CEHE is the treatment of choice for choledochal cyst, and intraoperative cyst endoscopy is recommended as a valuable adjunct to CEHE.

Laparoscopic surgery in newborn infants

AbstractBackground: Thanks to various technical innovations and advances in instrumentation, laparoscopic surgical intervention is now possible for certain congenital anomalies in children. To test the applicability of laparoscopic surgery in neonates, we reviewed our personal experience of neonatal laparoscopic surgery, focusing on cardiopulmonary function, surgical procedures, problems with devices, and degree of associated surgical stress. Methods: We performed 65 laparoscopic procedures in neonates. Their ages ranged from 2 to 30 days old, and their body weights ranged from 1,980 to 4,780 g. All 65 laparoscopic procedures were carried out without mortality or serious morbidity. Results: As complications, we encountered four cases of hypothermia due to rapid insufflation of carbon dioxide (CO2). We also found that relative hypercapnea (increase in end-tidal CO2 as high as 61 mmHg) developed unless hyperventilation and a relatively high peak insufflation pressure were maintained during pneumoperitoneum. No cardiac depression developed at this insufflation pressure. Fluid and electrolyte balance during our cases of newborn laparoscopic surgery, as well as the doses and volumes of fluid and electrolytes administered, were identical to those required for open surgery. Interleukin-6 (IL-6) was measured serially to estimate the degree of associated surgical stress and was found to be significantly lower in neonates who had received laparoscopic procedures than in those who had received open procedures. Conclusion: Laparoscopic surgery can be carried out safely even in neonates.

Abnormal distribution of intestinal pacemaker (C-KIT-positive) cells in an infant with chronic idiopathic intestinal pseudoobstruction

BACKGROUND Chronic idiopathic intestinal pseudoobstruction (CIIPO) is a rare syndrome with an obscure pathogenesis. The proto-oncogene c-kit encodes a transmembrane tyrosine kinase receptor C-KIT that is critical for the development of the interstitial cells of Cajal, cells that are regarded as being the pacemaker cells of the gut. Thus, C-KIT immunopositive (C-KIT-) cells in the muscle layers of the bowel are considered to be intestinal pacemaker cells. METHODS In this study, the distribution of intestinal pacemaker cells was examined for the first time using C-KIT immunohistochemistry in an infant with CIIPO. RESULTS C-KIT+ cells were found lying on either side of the border between the two muscle layers (longitudinal and circular) of the bowel and dispersed unevenly throughout both muscle layers. Myenteric plexuses were not demarcated by C-KIT+ cells. In contrast, in controls, C-KIT+ cells were located distinctly between the two muscle layers of the small bowel and dispersed evenly throughout the muscle layers of the colon. Myenteric plexuses were clearly demarcated by C-KIT+ cells. CONCLUSIONS This case demonstrates for the first time that there is abnormal distribution of intestinal pacemaker cells in CIIPO and provides new evidence that abnormal c-kit gene expression may be responsible for autonomic gut dysmotility. C-KIT immunohistochemistry may be an indispensable tool for diagnosing CIIPO.

Hepatic overexpression of MHC Class II antigens and macrophage-associated antigens (CD68) in patients with biliary atresia of poor prognosis

The pathogenesis of biliary atresia (BA) is still unknown. Progression to cirrhosis despite restoration of bile flow by successful portoenterostomy suggests that it is a progressive disease of the liver and biliary tree. Whether immunologic factors play any role in the development of this disease remains uncertain. Aberrant expression of major histocompatibility complex (MHC) Class II antigens of HLA-DR on hepatocytes and biliary epithelium is regarded as important in the progression of hepatocellular and biliary damage mediated by cytotoxic T cells. This study was undertaken to evaluate expression of MHC Class II antigen and macrophage-associated antigens (CD68) in liver of patients with biliary atresia to determine their prognostic usefulness and possible role in the pathogenesis of the disease. Liver biopsy specimens from infants with BA (n = 15), neonatal hepatitis (n = 3), and normal livers (n = 6) were studied using an indirect immunoperoxidase staining using antibodies against MHC Class II antigen and macrophage-associated antigens (CD68) as well as routine H&E and Massons trichome stain. In patients with biliary atresia, the liver biopsy specimen was obtained at the time of Kasai portoenterostomy. Expression of HLA-DR antigens and CD68 antigens was either absent or minimal in normal liver biopsy specimens. There were a few HLA-DR antigens and a few CD68-positive cells around portal tracts in all patients with neonatal hepatitis and five of the seven biliary atresia patients with successful Kasai portoenterostomy. In contrast, there was strong expression of HLA-DR antigen in bile ductules, inflammatory cells, and adjacent damaged hepatocytes and marked CD68-positive macrophage infiltrate in the portal tracts as well as hepatic lobules in two patients with good prognosis and in all eight patients with bad prognosis. Hepatic expression of MHC Class II antigen and CD68 antigens correlated well with the severity of clinical course in patients with BA and may act as a prognostic factor in these patients.

Abnormal choledocho-pancreatico ductal junction related to the etiology of infantile obstructive jaundice diseases

According to our current radiologic, histopathologic, and experimental studies, the congenital stenosis associated with anomalous choledocho-pancreatico ductal junction is considered to be the most important etiologic factor in congenital biliary dilatation. Furthermore, the possibility of pancreatic juice reflux into the biliary tract due to the abnormal choledocho-pancreatico ductal junction, which could lead to obstructive cholangiopathy, was suggested in relation to the pathogenesis of congenital biliary atresia.

Degeneration of intrahepatic bile duct with lymphocyte infiltration into biliary epithelial cells in biliary atresia.

Degenerative changes of intrahepatic bile ducts with lymphocytic infiltration into portal areas have been recognized as the characteristic histopathologic features in the liver with biliary atresia (BA). Occasionally, lymphocytic infiltration into biliary ductal epithelium was noted and has been thought to be mostly the result of nonspecific chronic inflammation. However, this histological presentation looks quite similar to that of acute graft-versus-host disease (GVHD) after bone marrow transplantation (BMT) or acute cellular rejection (ACR) after orthotopic liver transplantation (OLTx). According to this histological similarity, we presumed that the degeneration of intrahepatic bile ducts (DIBD) might have developed with lymphocytic infiltration into biliary epithelial cells (LIBE) as some part of an immunologic reaction, and focused on the DIBD with LIBE in the patients with BA. Liver specimens obtained from 31 patients with BA at the time of primary Kasai operation and 3 patients at the time of reoperation were reviewed histologically, and investigated DIBD with LIBE. Nine patients with choledochal cyst (CBD) and 5 patients with neonatal hepatitis (NH) were added to this study as a control population. All patients with BA presented with DIBD with LIBE. On the other hand, only 2 patients with CBD presented with DIBD with LIBE, and no patients with NH presented with DIBD with LIBE. The DIBD with LIBE is not limited to patients with BA, but it seems to be much more serious in this group. These findings may suggest that some sort of immunologic factors contribute to the persistent DIBD in the patients with BA.(ABSTRACT TRUNCATED AT 250 WORDS)

An abnormal pancreatico‐choledocho‐ductal junction in cases of biliary tract carcinoma

A histopathologic study on how the common bile duct and main pancreatic duct open into the duodenum was performed on 72 autopsied cases of biliary tract carcinoma. Type IIIb which was considered to be an abnormal pancreatico–choledocho–ductal junction was identified in 8 of 34 cases in common bile duct carcinoma and in 4 of 24 cases in gallbladder carcinoma, while none of the control cases belonging to Type IIIb. In cases of Type IIIb, reflux of pancreatic juice may occur into the bile duct and produce the repeated inflammation on the biliary tract. Therefore, the abnormal pancreatico‐choledocho‐ductal junction was suggested to be one of the pathogenic factors which cause biliary tract carcinoma. Cancer 52:2086‐2088, 1983.

Long-term postsurgical outcome of biliary atresia

BACKGROUND/PURPOSE A successful Kasai procedure is effective in creating biliary drainage and radically altering the natural history of infants with biliary atresia (BA). Since its introduction in the 1950s, long-term follow-up would appear to show that only 30% to 50% of patients have a good long-term prognosis despite initially good surgical outcome. The authors reviewed their experience in treating BA from 1968 to 1997 to assess long-term outcome. MATERIALS AND METHODS The records of 163 patients treated surgically for BA from 1968 to 1997 were reviewed. Forty-eight (29%) were alive at the end of 1997, of whom, 14 had received liver transplants (LT). Surviving patients who had not undergone transplantation were divided into two groups according to clinical condition: group A, normal liver function without cholangitis (CG) and portal hypertension (PH) and group B, liver dysfunction with CG or PH. The study period was divided arbitrarily into three periods, 1968 to 1975 (period I, n = 34); 1976 to 1985 (period II, n = 81); 1986 to 1997 (period III, n = 48). RESULTS Thirty-four patients were alive without LT at the end of 1997. There were eight patients (mean age, 16.3+/-4.8 years) in group A, and 26 patients (mean age, 14.3+/-7.6 years) in group B. Recently, four group A patients (mean age, 19.3+/-1.9 years) shifted to group B because of sudden deterioration in condition involving severe CG with multiple bile lakes (n = 2), uncontrollable intestinal bleeding (n = 1), and liver atrophy (n = 1). Survival deteriorated with length of follow-up. There were three survivors from 34 patients treated in period I, 16 survivors from 81 patients treated in period II (three had LT), and 29 survivors from 48 patients treated in period III (11 had LT). CONCLUSIONS Although satisfactory bile drainage can be obtained with portoenterostomy, our data suggest that liver function can deteriorate progressively, with a possible turning point in late adolescence, indicating that as the length of follow-up increases, clinical assessment should be regular and comprehensive. The timing of LT in postoperative BA patients with deteriorating liver function is a vital management issue.

Lack of intestinal pacemaker (C-KIT-positive) cells in infantile hypertrophic pyloric stenosis☆

The pathogenesis of infantile hypertrophic pyloric stenosis (IHPS) is not well understood. Recent studies have shown that the protonocogene c-kit is essential for the development or maintenance of autonomic gut motility, and also show that the c-kit gene protein product (C-KIT) positive cells in the mammalian gut are responsible for intestinal pacemaker activity. This study examines cells in the pyloric muscles of 23 patients (16 with IHPS, 7 controls) for the presence of the C-KIT (C-KIT+), using immunohistochemical techniques with antihuman C-KIT sera. In the controls, many C-KIT immunoreactive (IR+) cells were observed in the muscle layers. The myenteric plexuses were demarcated by a moderate number of C-KIT-IR+ cells. However, in the IHPS patients, C-KIT-IR were either absent or significantly reduced. No C-KIT-IR+ cells were found around the myenteric plexuses. These findings suggest that a lack of c-kit expression (as an indicator of intestinal pacemaker activity) in the hypertrophic pyloric smooth muscles may be an important factor in the pathogenesis of IHPS.