Geoffrey J. Lane

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.

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.

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.

Pyloromyotomy Versus Atropine Sulfate for Infantile Hypertrophic Pyloric Stenosis

PURPOSE Atropine sulfate (atropine) and pyloromyotomy were compared for managing infantile hypertrophic pyloric stenosis (IHPS). METHODS From 1996 to 1998, cases of IHPS treated surgically (pyloromyotomy; n = 20) or medically (atropine; n = 14) at separate institutions were compared retrospectively with regard to status on presentation, physical symptoms and signs, progress, and costs. Atropine was given orally, then intravenously if ineffective. Refractory cases were referred for pyloromyotomy. RESULTS All subjects were matched for clinical and physiological status on admission. Oral atropine alone was effective in 11 cases, was converted to intravenous atropine in 2 cases, and was terminated in 1 case because of hematemesis. Two cases were referred for pyloromyotomy. All pyloromyotomies were successful. Atropine took on average, 2.6 days to take effect. The difference in time taken for normalization of pyloric muscle thickness between the 2 groups was not significant. Average time to return to full feeding was longer in the atropine group (P<.01). Costs were lower in the atropine group (P<.01). There were 2 wound infections in the pyloromyotomy group, but no adverse effects of atropine. There were no recurrences in either group. CONCLUSION This study provides reasonable evidence to support a trial of atropine in IHPS.

Does probiotics administration decrease serum endotoxin levels in infants

PURPOSE The aim of this study was to examine whether administration of probiotics to infants can change the ratio of intestinal flora and thereby decrease serum endotoxin produced by potentially pathogenic microorganisms. METHODS Nine infants including five with of biliary atresia, two with omphalocele, one each with Hirschsprungs disease and imperforate anus were studied. All patients were stable, and no antibiotics were given during this study. A probiotic mixture consisting of Streptococcus faecalis, Clostridium butyricum and Bacillus mesentericus was administered orally to each infant at 2 g/day for 2 weeks. Fecal aerobic and anaerobic bacterial cultures, serum endotoxin level, and other biochemical parameters were examined. RESULTS In fecal cultures, anaerobic bacteria including Bifidobacterium increased significantly whereas Escherichia coli, Streptococcus, and Klebsiella tended to decrease. The ratio of anaerobic to aerobic bacteria increased five times as a result of administration of probiotics, and serum endotoxin levels decreased. CONCLUSIONS Probiotics affect intestinal bacterial flora by increasing anaerobic bacteria and decreasing the population of potentially pathogenic microorganisms. A decrease in luminal endotoxin may result in less endotoxin translocation or bacterial translocation.

Initial experience with non-breath-hold magnetic resonance cholangiopancreatography: A new noninvasive technique for the diagnosis of choledochal cyst in children

BACKGROUND/PURPOSE Magnetic resonance cholangiopancreatography (MRCP) is an emerging tool for the noninvasive evaluation of the pancreaticobiliary tree. METHODS Non-breath-hold MRCP was used in three children to evaluate choledochal cyst; a first for this new modality of diagnostic imaging. In all cases, the intrahepatic and extrahepatic bile ducts, and the pancreatic duct were clearly visualized. RESULTS Two cases were found to have a fusiform choledochal cyst, and non-breath-hold MRCP demonstrated pancreaticobiliary malunion and a long common channel. In the remaining case, the size and location of the huge cyst prevented visualization of any pancreaticobiliary malunion. Endoscopic retrograde cholangiopancreatography (ERCP) in this patient failed to provide any additional information. All patients underwent cyst excision with hepaticoenterostomy, and made an uneventful recovery. CONCLUSIONS Our initial experience suggests that non-breath-hold MRCP is a reliable method for the diagnosis of choledochal cyst in children and could replace ERCP.

Mast cells and gut nerve development: Implications for hirschsprung's disease and intestinal neuronal dysplasia

BACKGROUND/PURPOSE In Hirschsprungs disease (HD), the aganglionic bowel is characterized by the presence of hypertrophic nerve trunks and increased numbers of adrenergic and cholinergic nerve fibers. Intestinal neuronal dysplasia (IND), if associated with HD, occurs proximal to the aganglionic segment in HD, and is characterized by dysplasia of parasympathetic nerves, hyperganglionosis, and giant ganglia. However, the cause of such abnormalities in HD and IND is unclear. Recent reports that mast cells (MC) have been observed in direct contact with nerve fibers generally, suggest that MC are essential for nerve growth and repair. MC synthesize, store, and release nerve growth factor (NGF). NGF supports the development and functional maintenance of sympathetic and cholinergic neurons. The aim of this study was to examine the colonic distribution of MC with respect to nerves in HD and HD associated with IND. METHODS MC and NGF were examined immunohistochemically in ganglionic, transitional, and aganglionic segments of colon from 20 patients with HD (five patients associated with IND) and 15 age-matched controls. MC were counted in each of five random fields using light microscopy (x100). RESULTS Interestingly, aganglionic and IND segments had large numbers of MC in all layers compared with ganglionic segments in HD patients and controls (P< .0001). The number of MC in transitional segments was significantly less compared with ganglionic segments in HD patients and controls (P< .01). MC stained positively for NGF, and some were found in contact with abnormal hypertrophic nerve trunks in HD and giant ganglia in IND. CONCLUSIONS MC may cause hypertrophic nerve trunks and giant ganglia by releasing NGF and also may be an important factor in the excessive development of cholinergic and adrenergic nerve fibers in HD and IND.

Hyaluronic acid: A specific prognostic indicator of hepatic damage in biliary atresia

BACKGROUND/PURPOSE Hepatic fibrosis can progress in biliary atresia (BA) and is associated with capillarization of hepatic sinusoids. The significance of serum hyaluronic acid (HA) as a noninvasive indicator of histological sinusoidal endothelial cell (SEC) damage and hepatic fibrosis in BA, is investigated. METHODS A total of 28 postoperative BA patients (mean age, 11.0+/-3.7 years) and 20 normal controls (mean age, 10.5+/-2.8 years) were studied. BA patients were divided into group I, good liver function (n = 8); group II, moderate liver dysfunction (n = 10); and group III, severe liver dysfunction (n = 10). Serum HA was determined using a one-step sandwich enzyme immunoassay, and liver histological damage was confirmed immunohistochemically using an antibody against factor VIII-related antigen (FVIIIRAg), which is specific for detecting damaged SEC. RESULTS Serum HA was significantly higher (P < .0001) in group III (84.6+/-36.5 ng/mL) than in group I (15.9+/-6.9 ng/mL) or group 11 (28.7+/-10.7 ng/mL). Although immunoreactive products of FVIIIRAg were abundant in group III, they were not detected in SEC from group II. CONCLUSION Serum HA may be of value for monitoring postoperative BA patients as a noninvasive indicator of SEC damage and progressive hepatic fibrosis.

Beneficial effect of a traditional herbal medicine (inchin-ko-to) in postoperative biliary atresia patients

Abstract Inchin-ko-to (ICKT) prevents Fas-mediated liver injury. This study evaluates the effect of ICKT on conventional markers of liver function (LF) and liver fibrosis in 18 postoperative biliary atresia (BA) patients aged 3 to 23 years with elevated glutamic oxaloacetic transaminase (GOT), glutamic pyruvic transaminase (GPT), γ-glutamyl transpeptidase (γGTP) but normal serum total bilirubin (T-Bil) levels. ICKT (0.15 g/kg per day) was administered orally for 1 year. Serum GOT, GPT, γGTP, total bile acids (TBA), and T-Bil as markers of LF and hyaluronic acid (HA), prolyl hydroxylase (PH), procollagen III peptide (PIIIP), and type IV collagen as markers of liver fibrosis were measured before and after treatment in each patient and compared statistically. All patients tolerated ICKT well, and there were no side effects. The percentage of subjects who improved after ICKT was 45% for serum GOT, 72% for GPT, 72% for γGTP, 72% for TBA, 67% for HA, 40% for PH, 50% for PIIIP, and 23% for type IV collagen. Changes in the mean values of all serum markers were statistically significant (P < 0.01). It is concluded that long-term administration of ICKT in postoperative BA patients improves liver status as assessed by markers of LF and fibrosis.