Increased population of immature enteric glial cells in the resected proximal ganglionic bowel of Hirschsprung's disease patients.

BACKGROUND: Enteric glial cells are essential for normal gastrointestinal function. Abnormalities in glial structure, development, or function lead to disturbances in gastrointestinal physiology. Fatty acid-binding protein 7 (FABP7) is a marker of immature enteric glial cells, whereas S100 is expressed only by mature glial cells. Patients with Hirschsprung's disease (HSCR) often suffer from dysmotility and enterocolitis despite proper surgery. We designed this study to determine the distribution and expression of glial cells in patients with HSCR compared to normal controls. METHODS: We investigated FABP7, S100, and PGP 9.5 expressions in both the ganglionic and aganglionic bowel of patients with HSCR (n = 6) versus normal control colon (n = 6). Protein distribution was assessed by using immunofluorescence and confocal microscopy. Gene and protein expressions were quantified using quantitative real-time polymerase chain reaction (qPCR), Western blot analysis, and densitometry. RESULTS: qPCR and Western blot analysis demonstrated a significantly increased FABP7 expression in ganglionic specimens compared to control specimen (P < 0.05). Confocal microscopy revealed FABP7+ glia cells lie under the colonic epithelium and in close apposition to enteric neurons in the ganglionic bowel. CONCLUSIONS: The significantly increased number of immature enteric glial cells (EGCs) in the ganglionic bowel of HSCR patients may have adverse effect on the function of enteric neurons and intestinal barrier and thus predispose these patients to intestinal motility problems and enterocolitis.

Parenteral nutrition-associated liver disease in extremely low-birthweight infants with intestinal disease.

BACKGROUND: The aim of this study was to investigate factors associated with the development of parenteral nutrition-associated liver disease (PNALD) and to examine the clinicopathological relationship of PNALD in extremely low-birthweight infants (ELBWI). METHODS: The subjects were 13 ELBWI who had received PN because of intestinal perforation or functional ileus between 2000 and 2013. We measured the serum levels of biochemical parameters, including aspartate aminotransferase, alanine aminotransferase, and direct bilirubin. Liver histopathology was examined in relation to outcome. The subjects were categorized into two groups on liver histopathology: F(+), development of hepatic fibrosis and necrosis with/without cholestasis; and F(-), no hepatic fibrosis. RESULTS: Of 13 ELBWI, five died of hepatic failure, five died of sepsis, and the other three were alive at the time of the study. Of the five infants who died of hepatic failure, two developed fulminant hepatitis without cholestasis, and the other three developed chronic cholestasis and finally hepatic failure. Postmortem histopathology in F(+) indicated not only massive hepatic necrosis, but also massive hepatic fibrosis. These histopathological findings explained the clinical presentation of portal hypertension. There were significant differences in the fasting period after intestinal disease onset between the two groups. CONCLUSION: The prolonged fasting with PN is responsible for severe hepatocellular necrosis with fibrosis and consequent lethal portal hypertension.

Efficacy and safety of strong acid electrolyzed water for peritoneal lavage to prevent surgical site infection in patients with perforated appendicitis.

PURPOSE: Our previous experimental study of perforated peritonitis in rats proved that peritoneal lavage with strong acid electrolyzed water (SAEW) has no adverse effects, reduces the bacteria count in the ascitic fluid more effectively than saline, and increases the survival rate significantly. Thus, we conducted a randomized controlled study, applying SAEW in the treatment of perforated appendicitis in children. METHODS: Forty-four patients, aged 3-14 years, were randomly divided into two groups: Group S (n = 20), in which the peritoneal cavity was lavaged with 100 ml/kg saline and the wound was washed out with 200 ml saline; and Group E (n = 24), in which the peritoneal cavity was lavaged with 100 ml/kg SAEW and the wound was washed out with 200 ml SAEW. RESULTS: No adverse effect of SAEW was observed in Group E. There was no difference in the bacterial evanescence ratio of ascitic fluid after lavage between Groups S and E (11.1 and 15.8%, respectively). A residual abscess developed in one patient from each group (5.0 and 4.2%, respectively). The incidence of surgical site infection (SSI) was significantly lower in Group E than in Group S (0 and 20%, respectively; P < 0.05). There was no difference in the duration of pyrexia, positive C-reactive protein, leukocytosis, or hospital stay between the groups. CONCLUSION: Peritoneal lavage and wound washing with SAEW have no adverse effects and are effective for preventing SSI.

Impact of cow's milk allergy on enterocolitis associated with Hirschsprung's disease.

PURPOSE: To investigate the impact of cow's milk allergy (CMA) on infants with Hirschsprung's disease (HD). METHODS: Twenty-four patients, who developed gastrointestinal symptoms before the age of 60 days and underwent surgery for HD in the period between January 2003 and December 2012, were enrolled in this study. They were divided into two groups based on CMA-related findings: stimulation index of lymphocyte stimulation test >300 % and the presence of eosinophilic infiltration in the resected colon. Ten patients were determined specimen as not having CMA (Group A), because they did not satisfy any of the criteria. The remaining 14 were determined as having possible CMA (Group B), because they satisfied either or both findings. Patient background characteristics, pre- and postoperative clinical history, and laboratory data were compared between Groups A and B. RESULTS: Pre- and postoperative enterocolitis did not occur in Group A patients. Postoperative enterocolitis was more frequent in Group B than in Group A (p = 0.04). Other clinical and laboratory data did not show significant difference between the two groups. CONCLUSION: CMA is a possible risk factor for postoperative enterocolitis in patients with HD.

The clinical significance of intrahepatic cystic lesions in postoperative patients with biliary atresia.

PURPOSE: To clarify the significance of intrahepatic cystic lesions (ICLs) after Kasai procedure for biliary atresia (BA), we reviewed the BA patients with special reference to the shape of ICLs. METHODS: For the last two decades, 75 cases have been followed up at our institution. The patients were divided into two groups: ICLs (+) with ICL and ICLs (-) without ICL. Seventeen cases of ICLs (+) were divided into two categories: the round type and the multiple-bead type. A poor prognosis was defined as mortality or the need for liver transplantation (LT). RESULTS: The ratio of round to multiple-bead types was 6:11. The percentage of poor prognoses with ICLs (+) and ICLs (-) was 47 and 53 %, respectively. The percentage of poor prognoses with round and multiple-bead types was 27 and 83 %, respectively (p < 0.05). In cases of ICLs (+), an LT was required because of hepatic deterioration in all three of the round types, and intractable cholangitis in all five of the multiple-bead types (p < 0.05). CONCLUSION: The long-term prognosis of BAs after the Kasai procedure does not necessarily depend on the development of ICLs per se, but on their shape.

Use of the medical information on the internet by pregnant patients with a prenatal diagnosis of neonatal disease requiring surgery.

PURPOSE: The purpose of this study was to clarify the current status and the problems associated with using medical information on the internet during pregnancy in patients prenatally diagnosed with fetal abnormalities at a single Japanese institution. METHODS: A written, anonymous questionnaire survey was conducted in 155 pregnant patients who had been prenatally diagnosed as having neonatal surgical diseases between January 2000 and December 2009, and their families. RESULTS: Forty-three out of the 75 responding families (57.3%) had used medical information available on the internet during their pregnancy. The availability of information, assessed during 2 year-increments, has increased rapidly in the past 4 years. When the explanation of a physician was compared with the information provided by the internet, the knowledge or impression of the disease was different in 60% of cases and similar in 33% of cases. More importantly, 60% of the patients felt that the information obtained from the internet was more pessimistic than the physician's explanation. CONCLUSION: The number of pregnant patients who have used medical information on the internet has rapidly increased in the recent years. Subjects who used this information were more likely to experience a sense of anxiety and feelings regarding the seriousness of the disease.

Airway deformation in patients demonstrating pectus excavatum with an improvement after the Nuss procedure.

PURPOSE: This study analyzed the inside appearance of the trachea and the main bronchus at the time of performing the Nuss procedure to test the hypothesis that the trachea and the main bronchus might be deformed by compression from great vessels due to sternal depression. METHODS: A retrospective cohort study included 36 patients with pectus excavatum, who were treated using the Nuss procedure between July 2001 and December 2009. The primary outcome measures were the oblateness of the trachea and bilateral main bronchus. The oblateness in patients with pectus excavatum was compared with that of the control group. Their postoperative changes and the relationship between the Haller CT index were also analyzed. RESULTS: The oblateness of the trachea and the left main bronchus in patients with pectus excavatum was significantly greater than that of the control group. A negative correlation was recognized between the percent vital capacity and the oblateness of the left main bronchus. The oblateness of the bilateral main bronchus significantly improved during the 2 years of bar placement. CONCLUSION: A significant deformation of the airways was demonstrated in patients with pectus excavatum, which improved after correcting it by means of the Nuss procedure.

The prognostic factors and the outcome of primary isolated fetal ascites.

PURPOSE: The purpose of the present study was to evaluate the prognostic factors and review the outcome of primary isolated fetal ascites. METHODS: A retrospective cohort study was conducted for fetuses with primary isolated ascites with a prenatal diagnosis between 1994 and 2009. The patients were divided into the favorable group (Group I) whose ascites were resolved by medical treatment alone and an unfavorable group (Group II) who required surgical intervention after birth due to refractory ascites. RESULTS: There were seven patients in Group I and five patients in Group II. Six of seven patients who developed ascites after 30 weeks' gestation were categorized in Group I, and four of five infants who developed ascites before 30 weeks' gestation were categorized in Group II. There was a negative correlation between the gestational age at diagnosis and the severity of the fetal abdominal distention. In Group II, the ascites resolved in two cases and was reaccommodated in another two cases after surgery. An infant with trisomy 21 received continuous drainage and eventually died of infection. CONCLUSIONS: The prognosis of primary isolated fetal ascites can be predicted based on the gestational age at diagnosis and the severity of the fetal abdominal distention.

In vitro construction of scaffold-free cylindrical cartilage using cell sheet-based tissue engineering.

PURPOSE: Tissue-engineered cartilage may offer a solution for the treatment of serious airway disease. This study developed a novel procedure to fabricate a scaffold-free cylindrical cartilage under in vitro conditions, while also evaluating the effect of a dynamic culture on the engineered construct. METHODS: Auricular chondrocytes were harvested from New Zealand white rabbits and cultivated under high-density conditions to form a chondrocyte sheet. The sheet was looped around a silicon tube and cultivated for 6 weeks in dynamic or static conditions. The engineered cylindrical cartilages were evaluated macroscopically and histologically. The expression of collagen, glycosaminoglycan content and mechanical properties were determined. RESULTS: The cylindrical cartilage was sufficiently elastic and stiff to maintain the structure without disruption. Histologically, the construct contained a Safranin-O positive cartilaginous matrix accompanied by the expression of type II collagen. The glycosaminoglycan content increased and reached 72% of the native tracheal cartilage after 6 weeks of cultivation. CONCLUSION: A novel procedure was developed for fabricating engineered cartilage, which maintained the shape and a proper level of rigidity and flexibility, under in vitro conditions using sheet-based tissue engineering techniques. This procedure may allow for the development of a tailor-made autograft and a functionally engineered trachea.

Safety and efficacy of laparoscopic percutaneous extraperitoneal closure for inguinal hernia in infants younger than 6 months: A comparison with conventional open repair.

INTRODUCTION: This study aimed to compare the safety and efficacy of laparoscopic percutaneous extraperitoneal closure (LPEC) and conventional open repair (OR) for inguinal hernia in infants younger than 6 months. METHODS: The medical records of 202 patients who had undergone LPEC or OR at an age younger than 6 months between 2010 and 2016 were reviewed. Patient characteristics and surgical outcomes were compared between LPEC and OR. RESULTS: In total, there were 120 LPEC (76 males, 44 females) and 83 OR (76 males, 7 females) cases. There were no differences in median age, median bodyweight, and presence of incarceration between the two groups (LPEC group: 3 months (range, 0-5 months), 5.7 kg (range, 2.3-9.6 kg), and 13.3%; OR group: 3 months (range, 1-5 months), 5.5 kg (range, 2.1-9.0 kg), and 12.0%). LPEC was completed in all cases without any intraoperative complications or open conversions. In the LPEC group, 60.0% of unilateral hernia patients had a contralateral patent processus vaginalis and underwent prophylactic closure. The incidence of metachronous contralateral hernia was significantly lower in the LPEC group than in the OR group (0% vs 10.0%, P < 0.01). Operative time was significantly shorter during LPEC than during OR (P < 0.01) for male patients. The recurrence rate was lower in the LPEC group (0.83%) than in the OR group (2.4%), but the difference was not significant. CONCLUSION: The present data indicate that LPEC is comparable to OR in terms of safety and efficacy in infants younger than 6 months. Moreover, among the LPEC group, there were fewer incidences of metachronous contralateral hernia, and the procedure had a shorter operative time.

Total laparoscopic excision of retroperitoneal ganglioneuroma using the hanging method and a vessel-sealing device.

An 11-year-old girl presented with a homogeneous right retroperitoneal tumor 5 cm in diameter. Nonfunctioning benign neurogenic tumor was suspected, and a laparoscopic resection was performed. The inferior vena cava ran just behind the tumor, and there were small vessels between the tumor and inferior vena cava. To create a space behind the tumor, we suspended the tumor by using a traction suture, and to obtain adequate hemostasis, we used a vessel-sealing device. The tumor was safely removed and the pathological diagnosis was benign ganglioneuroma. The postoperative recovery was uneventful, and the patient left her bed and started a regular diet on the postoperative day 1. Laparoscopic surgery is a safe, feasible method for the diagnosis and treatment of benign retroperitoneal tumors, such as ganglioneuroma.

The most reliable early predictors of outcome in patients with biliary atresia after Kasai's operation.

BACKGROUND/PURPOSE: The purpose of this study was to determine reliable predictors of outcome of biliary atresia (BA) after Kasai's operation. PATIENTS AND METHODS: Fifty-four BA cases that underwent Kasai's operation at our institution over two decades were reviewed. The cases were divided into two groups: Group I: cases that required liver transplantation or died (n=30) and Group II: cases alive with the native liver. Serum levels of total bilirubin (TB), direct bilirubin (DB), aspartate aminotransferase (AST), alanine aminotransferase (ALT), and gamma-glutamyltransferase (GGT) were measured sequentially after surgery. For cut-off determination, receiver operating characteristic (ROC) analysis was employed. RESULTS: Serum TB, DB, AST, and ALT in Group I were significantly higher than those in Group II at 1, 2, and 3 months after surgery (p<.05). The most reliable cut-offs determined by ROC analysis were DB of 0.7 mg/dl at 2 months (sensitivity; 93%, specificity; 75%) and AST of 94 IU/L at 2 months (sensitivity; 87%, specificity; 71%). The 54 cases were re-divided into three groups according to the cut-off values: group G (good) with DB and ASTcut-offs (n=9; Group I:II=4:5), and group P (poor) with DB and AST ≥ cut-offs (n=29; Group I:II=25:4). The 15-year survival rate in groups G, M, and P was 94%, 44%, and 22%, respectively (p<.001). CONCLUSION: The combination of serum DB and AST at 2 months after Kasai's operation is a reliable predictor of long-term BA outcome.

Current status of negative treatment decision-making for fetuses with a prenatal diagnosis of neonatal surgical disease at a single Japanese institution.

BACKGROUND/PURPOSE: The termination of pregnancy because of fetal abnormalities in Japan has not been described. The aim of the present study was to analyze the current status and to evaluate the medical and ethical relevance in our institution for negative treatment decision-making for fetuses demonstrating neonatal surgical disease with a prenatal diagnosis. MATERIALS AND METHODS: The medical records of 209 fetuses with a prenatal diagnosis from 1999 to 2008 were retrospectively reviewed. The cases with a negative treatment policy were analyzed according to the potential for survival. The negative treatment policies were defined as those in which the pregnancy was not actively continued, including elective termination of pregnancy and palliative or limited treatment that are primarily provided after birth. RESULTS: The selected treatment policies were active in 162 cases and negative in 46 cases. Thirty-three cases with negative policies were in the second-half period of pregnancy. The potential for survival was high in 5 cases, moderate in 11 cases, and nonviable in 30 cases. Eight of the nonviable cases underwent either limited or palliative treatment, whereas the remaining 38 fetuses were aborted. CONCLUSIONS: The negative treatment policies in the nonviable fetuses were considered to be medically and ethically relevant. However, the number of cases with negative policies increased over the last 5 years and is therefore associated with complex ethical issues.

Three-stage reconstruction of the airway and alimentary tract in a case of tracheal agenesis.

In the few surviving cases of tracheal agenesis, infants have not been capable of oral intake because the esophagus was used as a substitute for the trachea. We performed a three-stage reconstruction of the airway and alimentary tract in an infant with tracheal agenesis. This procedure involved a double cervical esophagostomy followed by an anastomosis of the upper mid-esophagus and carinal trachea. Finally, the esophagus was reconstructed by an anastomosis of the cervical esophagus to the lower esophagus. This novel procedure may become a highly effective surgical treatment for some infants in critical condition due to tracheal agenesis.

One-trocar laparoscopy-aided gastrostomy in handicapped children.

BACKGROUND/PURPOSE: Percutaneous endoscopic gastrostomy has become a common technique to provide nutritional support to handicapped children with swallowing disorders; however, this technique is sometimes associated with serious complications. We report a novel method of 1-trocar laparoscopy-aided gastrostomy with special reference to its influence on gastroesophageal reflux (GER). METHODS: The subjects consisted of 22 profoundly handicapped children, aged from 1 to 14 years (median, 7 years). Twenty-four-hour esophageal pH monitoring was conducted preoperatively in all subjects and performed postoperatively in 12. The criteria for gastrostomy alone included no significant symptoms related to GER and distal esophageal acid exposure of less than 15%. Esophageal acid exposure was evaluated at the distal esophagus (P1) and at 10 cm proximal to P1 (P2). Stamm gastrostomy was performed by grasping the anterior gastric wall with forceps inserted through an operating channel associated with a laparoscope. Data are expressed as medians and ranges. RESULTS: There were neither perioperative mortality nor life-threatening complications except the detachment of the gastrostomy in one. Esophageal acid exposure was significantly increased postoperatively compared to preoperatively at P1 (2.1% [0.0%-4.7%] vs 4.6% [0.2%-8.7%], P = .02), but did not change much at P2 (0.2% [0.0%-1.5%] vs 0.8% [0.0%-7.6%], P = .07). No patient developed postoperative symptoms related to GER. CONCLUSIONS: One-trocar laparoscopy-aided gastrostomy is a feasible technique for handicapped children without symptomatic GER.