Comparison of long-term outcome according to involved aganglionic segments of total colonic aganglionosis.

ABSTRACT: Total colonic aganglionosis (TCA) is a rare form of Hirschsprung disease, with more severe symptoms than rectosigmoid Hirschsprung disease. We aimed to evaluate the surgical outcomes according to the involved segments of TCA.Patients with aganglionosis extending from the anus to at least the ileocecal valve were included. The medical records of 33 TCA patients from 1981 to 2014 were reviewed. Three groups were analyzed based on the involved segment (jejunum, jejunoileal junction, and distal ileum).The median age at the pull-through operation was 6.2 (3.3-114) months. The median follow-up duration was 216 (21-411) months. Transition zone in the jejunum, jejunoileal junction, and distal ileum was identified in 3, 5, and 25 patients, respectively. The most common method of operation was Duhamel pull-through. Perianal excoriation and enterocolitis were the most common postoperative complications. The complication rates were 45% to 51% and not different among the groups. The defecation frequency normalized 3 years postoperatively, and body weight started to recover after 2 years irrespective of the involved segment.Therefore, close monitoring with proper management of defecation and body weight for at least 2 to 3 years postoperatively is required.

Incidence of necrotizing enterocolitis in South Korea from 2007 to 2017.

OBJECTIVES: The aim of this study was to investigate the trends of the incidence of and mortality from necrotizing enterocolitis (NEC) in South Korea. METHODS: Claim data were extracted with diagnosis code, including all stage of NEC. Kaplan-Meier curves for cumulative mortality rates are presented by birth weight (Bwt) and gestational age (GA). RESULT: The total number of NEC cases was 5840. The ratio of males to females was 1.2:1. There were 11.9 cases per 10,000 births. There were approximately 10 cases per 1000 preterm births and 50 per 1000 very preterm births. The mortality of patients born under 37 weeks decreased from 38.7% in 2007 to 20.9% in 2017. Male sex, lower GA, and lower Bwt were risk factors for mortality. CONCLUSIONS: NEC incidence and mortality have been decreasing over time in South Korea. Male sex, lower GA, and Bwt were risk factors for mortality.

Circulating microRNA expression profiling in young obese Korean women.

BACKGROUND/OBJECTIVES: This study investigates correlations between circulating microRNAs (miRNAs) and obesity-related parameters among young women (aged 20-30 years old) in Korea. SUBJECTS/METHODS: We analyzed TaqMan low density arrays (TLDAs) of circulating miRNAs in 9 lean (body mass index [BMI] < 25 kg/m2) and 15 obese (BMI > 25 kg/m2) women. We also performed gene ontology (GO) analyses of the biological functions of predicted miRNA target genes, and clustered the results using the database for annotation, visualization and integrated discovery. RESULTS: The TLDA cards contain 754 human miRNAs; of these, the levels of 8 circulating miRNAs significantly declined (> 2-fold) in obese subjects compared with those in lean subjects, including miR-1227, miR-144-5p, miR-192, miR-320, miR-320b, miR-484, miR-324-3p, and miR-378. Among them, miR-484 and miR-378 displayed the most significant inverse correlations with BMI (miR-484, r = -0.5484, P = 0.0056; miR-378, r = -0.5538, P = 0.0050) and visceral fat content (miR-484, r = -0.6141, P = 0.0014; miR-378, r = -0.6090, P = 0.0017). GO analysis indicated that genes targeted by miR-484 and miR-378 had major roles in carbohydrate and lipid metabolism. CONCLUSION: Our result showed the differentially expressed circulating miRNAs in obese subjects compared to lean subjects. Although the mechanistic study to reveal the causal role of miRNAs remains, these miRNAs may be novel biomarkers for obesity.

Impact of hospital size on the outcomes of appendectomy in children: an analysis of a comprehensive nationwide pediatric dataset.

PURPOSES: The purpose of this study was to investigate the outcomes after appendectomy in children according to hospital size. METHODS: The records of 11,565 patients with the diagnosis-related group code for appendectomy were extracted from HIRA-Pediatric Patient Sample from 2012 to 2016. The number of hospital visits and the length of stay in hospital within 30 days after appendectomy were analyzed. RESULTS: Patients who were treated at large-sized hospitals were more likely to be younger, more likely to reside in metropolitan areas, and tended to receive laparoscopic surgery. The number of hospital visits within 30 days in patients managed by medium- and large-sized hospitals decreased in comparison to small-sized hospitals. The length of hospital stay in large-sized hospitals was decreased in comparison to small- and medium-sized hospitals. A subgroup analysis revealed that complicated appendectomy did not have a significant impact on the difference in the length of hospital stay between hospital sizes. CONCLUSION: The number of hospital visits and the length of hospital stay was higher in small-sized hospitals in comparison to large-sized hospitals. Appendectomy performed in the larger hospital showed better outcomes in pediatric patients. We recommend that pediatric surgical procedures be performed in large hospitals, and that proper incentives be given for procedures to be performed by pediatric specialists.

Analysis of Associated Anomalies in Anorectal Malformation: Major and Minor Anomalies.

BACKGROUND: Fifty to sixty percent of patients with anorectal malformation (ARM) have at least one associated anomaly (AA). We determined the incidence of AA with the subtypes of ARM classified in accordance with the Krickenbeck classification and analyzed differences in the incidence rates of major and minor AAs according to organ system. METHODS: From January 1999 to May 2017, we retrospectively analyzed congenital anomalies in patients who underwent an anoplasty for ARM at our institution. The AAs were divided into nine organ systems. To analyze the difference in the incidence of AAs, we calculated odds ratios (ORs) using cases of perineal fistula as the base group. RESULTS: Of the 460 patients, 256 (55.7%) were male, 299 (65%) had at least one anomaly, and 274 (59.6%) had major AAs. According to organ system, AAs were most common in the genitourinary (28%), cardiovascular (25%), and spinal/vertebral systems (22.6%). Major AA was most common in the cardiovascular (23%) and spinal/vertebral and genitourinary systems (19.3%). According to ARM subtype, AAs were common in the order of cloaca (93.9%), rectovaginal fistula (85.7%), and recto-bladder neck fistula (85%). For the incidence of AAs, cloaca (OR, 15.7) and recto-bladder neck fistula (OR, 5.74) showed significantly higher ORs. In the analysis of major AAs, the cloaca (OR, 19.77) showed the highest OR, followed by no fistula (OR, 4.78) and recto-bladder neck fistula (OR, 3.83). CONCLUSION: A considerable number of patients with ARM had AAs. Our data are useful for predicting AAs in patients with ARM.

Risk factors of meconium-related ileus in very low birth weight infants: patients-control study.

Very low birth weight (VLBW) neonates experience various problems, including meconium-related ileus (MRI). This study investigated the risk factors of MRI and surgical MRI in VLBW infants. VLBW neonates admitted to the Neonatal Intensive Care Unit of Seoul National University Children's Hospital from October 2002 to September 2016 were included in the study. The diagnostic criteria for MRI were a decreased frequency of defecation with intolerable feeding, vomiting, and increased gastric residue (>50%); meconium-filled bowel dilatation in an imaging study; and no evidence of necrotizing enteritis or spontaneous intestinal perforation. Medical MRIs and surgical MRIs were managed through conventional treatment and surgical intervention. Of 1543 neonates, 69 and 1474 were in the patient and control groups, respectively. The risk factors for MRI include low birth weight (BW), cesarean section delivery, fetal distress, maternal diabetes, maternal hypertension, and maternal steroid use. Low BW and fetal distress were independent risk factors for MRI. Compared to the medical MRI group (n = 44), the risk factors for surgical MRI (n = 25) included males, younger gestational age, low BW, and meconium located at the small bowel. Male gender and low BW were independent risk factors for surgical MRI. Low BW and fetal distress were independent risk factors for MRI and male gender and low BW were independent risk factors for surgical MRI. In VLBW neonates, careful attention to the risk factors for MRI could minimize or avoid surgical interventions.

The Patients with Hirschsprung's Disease Who Underwent Pull-Through at Age Less than 1 Year: Longitudinal Bowel Function.

BACKGROUND: Frequent stooling immediately after pull-through (PT), fecal soiling, and constipation are chronic complications of Hirschsprung's disease (HD). This study aimed to investigate the longitudinal outcomes in terms of bowel function of patients below the age of 1 year undergoing PT. METHODS: We retrospectively evaluated 396 patients who underwent PT for HD between September 1979 and March 2014. Stool frequency was analyzed up to 10 years of age, and soiling and constipation were analyzed up to 15 years of age. RESULTS: After resection of the aganglionic segment (AS), stool frequency decreased over time. Furthermore, stool frequency among the three groups was similar 4 years after PT. Among the patients with aganglionic bowel resection, those who underwent the Soave procedure (SP) had an increase (0.56/day) in stool frequency than those who underwent the Duhamel procedure (DP). The soiling severity according to the AS was similar after 5 years of age. More severe soiling was better associated with patients who underwent the SP than those who underwent the DP. The constipation severity increased gradually until around 5 years and declined thereafter. More severe constipation was better associated with the DP than with the SP. CONCLUSION: The result of the analysis of stool frequency and soiling in patients with HD indicated that shorter ASs resulted in fewer bowel movements and less severe soiling. However, with the increase in patient age, the differences became similar. Compared to the DP, the SP was associated with an increased frequency of bowel movements and soiling severity; however, the constipation severity was lower.

Clinical features of children with Haddad syndrome: A single-center experience.

BACKGROUND/PURPOSE: Haddad syndrome (HS) is a very rare disease considered a form of neurocristopathy. It is characterized by a combination of congenital central hypoventilation syndrome (CCHS) and Hirschsprung's disease (HD). We report the clinical features and disease progression of HS to provide better care for HS patients by achieving an earlier diagnosis and optimal treatment. METHODS: Medical records of patients diagnosed with HS from 2005 to 2016 were retrospectively reviewed. Demographic data including gestational age, birth weight and height, and paired-like homeobox 2b (PHOX2B) gene mutation were collected. RESULTS: Seven males and three females were identified (mean gestational age 39.76 ±â€¯1.49 weeks, mean birth weight 3117.5 ±â€¯288.9 g). PHOX2B gene mutation was identified in all patients. Immediate ventilation care after birth was required in five patients due to poor respiration. The current median age of the children is 5.4 years (range, 1.8-10.1). Tracheostomy was performed in nine patients. Eight patients required sleep ventilation and two patients, 24-h continuous ventilation support. Six patients showed rectosigmoid aganglionosis and four patients exhibited total colonic aganglionosis, of these one had aganglionosis extended to the distal small bowel. Soiling was observed in seven patients (5 with laparoscopy-assisted transanal endorectal pull-through and 2 with Duhamel procedure) and one patient showed grade 2 constipation with Duhamel procedure. Six patients had developmental delay. All patients are alive. CONCLUSIONS: HS may require lifelong medical care. This study could be helpful to understand the clinical features of HS including associated abnormalities and disease progression. By assisting to understand the clinical features, we could provide better care for HS patients by achieving an earlier diagnosis and appropriate treatment. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level IV.

Effect of sun exposure versus oral vitamin D supplementation on serum 25-hydroxyvitamin D concentrations in young adults: A randomized clinical trial.

BACKGROUND: Vitamin D inadequacy is associated with a wide range of diseases. However, optimal strategies to improve vitamin D status, especially in Asian populations, remain unclear. We tested the hypotheses that (1) relevant sun exposure or oral vitamin D supplementation would significantly increase serum 25-hydroxyvitamin D (25OHD) concentrations compared with placebo, (2) sun exposure and supplementary vitamin D would be similar in serum 25OHD increases, and (3) the two interventions may have different effects on cardio-metabolic markers. METHODS: In this 8-week randomized placebo-controlled clinical trial including vitamin D-deficient adults in Seoul (37 °N), Korea, changes in serum 25OHD concentrations were compared between the sun exposure (daily ≥20-30 min around noon, n = 50), oral vitamin D3 (500 IU/d, n = 50), and control (placebo, n = 50) groups. RESULTS: Both sun exposure and oral vitamin D3 effectively increased serum 25OHD concentrations. Compared with placebo, the between-group least-squares mean (LSM) differences in changes were 2.2 ng/mL (95% CI: 0.2, 4.2) in the sun exposure group and 8.5 ng/mL (6.5, 10.5) in the oral vitamin D3 group. Increases in serum 25OHD were greater with oral vitamin D3 than with sun exposure (LSM difference in changes = 6.3 ng/mL, 95% CI: 4.3, 8.3). More participants in the oral vitamin D3 group (54.2%) achieved serum 25OHD concentrations ≥20 ng/mL at week 8 than those in the sun exposure (12.2%) or control (4.3%) groups. Compliance with sun exposure advice was relatively low, and only those with adequate compliance had a significant increase in serum 25OHD. Changes in the cardio-metabolic markers were mostly insignificant in all groups. CONCLUSIONS: Enhanced sun exposure and 500 IU/d of oral vitamin D3 supplementation significantly increased serum 25OHD concentrations. However, our protocol for sun exposure was not as effective as 500 IU/d of oral vitamin D3 supplementation. This trial was registered at clinicaltrials.gov as NCT03310242.

Pediatric Adenocarcinoma in Korea: A Multicenter Study.

PURPOSE: Adenocarcinoma is an extremely rare malignancy in the pediatric population. Research regarding pediatric adenocarcinoma is very rare in Korea. This study aimed to investigate the clinical features of pediatric adenocarcinomas of various primary organ sites in Korea. MATERIALS AND METHODS: Pediatric patients under 18 years, diagnosed with adenocarcinoma of various sites between January 1995 and December 2016, were included. We retrospectively reviewed patient and tumor characteristics and calculated survival estimates, reported as 5-year survival rate and 95% confidence interval. RESULTS: Of 80 patients (median age, 15 years; range, 10 to 17 years), 37 (46.3%) were men, and 24 (30%) had a family history of cancer or underlying disease relevant to malignancy. The cancer locations were the colon and rectum (n=32), ovaries (n=18), stomach (n=15), lung (n=4), small bowel (n=1), and other sites (n=10). Totally, 54.8% patients (42/77) had stage 3 or 4 disease. The median follow-up period was 2.0 years (range, 0 to 20.4). The 5-year overall survival estimate for all patients, and for those with stomach, colorectal, ovarian, and other cancer sites were 57.9%±11.5%, 58.2%±25.7%, 41.5%±18.2%, 87.5%±16.2%, and 64.0%±34.4%, respectively. The 5-year survival rate differed significantly between categories of adenocarcinomas into gastrointestinal (GI) (44.7%) and non-GI adenocarcinomas (78.8%) (p=0.007). The 5-year survival rate also differed significantly according to carcinoembryonic antigen level (69.3% in < 3 ng/mL, 23.8% in > 3 ng/mL; p < 0.001). CONCLUSION: In pediatric patients, adenocarcinomas arise from various organs and are often diagnosed at advanced stages. Large, prospective studies for their accurate clinical characteristics and prognostic factors are needed.

Experiences of Rectovaginal Fistula in Anorectal Malformation.

BACKGROUND: Rectovaginal fistulas (RVFs) are very rare malformations in females with anorectal malformations (ARMs). Here, we share the clinical features of RVF and report the long-term outcomes. METHODS: RVF patients were classified using a retrospective analysis of ARM patients who underwent operations at Seoul National University Hospital between January 1999 and May 2017. The Krickenbeck continence scoring system was used to evaluate bowel function 5 and 10 years after surgery. RESULTS: Of the total 460 ARM patients, 203 were female, 7 of whom were diagnosed with RVF. The median age and weight at the time of anorectoplasty were 292 days (range, 140-617) and 8.2 kg (range, 5.5-12), respectively. Six patients had associated anomalies and three patients underwent redo-anorectoplasty. Voluntary bowel movements were observed in 6 out of 7 patients at 5 and 10 years of age. Soiling was observed in all patients at the age of five years and in 6 out of 7 patients at the age of ten years. Constipation was observed in 6 out of 7 patients at both five and ten years of age. CONCLUSIONS: An RVF is a very rare malformation, accounting for 1.5% of total ARMs and 3.4% of ARMs in females. Long-term counseling, education, and guidance are needed for effective management of patients' bowel movements. TYPE OF STUDY: Prognosis study LEVEL OF EVIDENCE: Level IV.

The effect of Absolute Neutrophil Count (ANC) on early surgical site infection in Implanted Central Venous Catheter (ICVC).

PURPOSE: The aim of this study was to evaluate surgical site infection (SSI) rates related to implanted central venous catheters (ICVC) in pediatric hematology and oncology patients with respect to absolute neutrophil count (ANC) levels. PATIENTS AND METHODS: From January 2004 to December 2015, pediatric patients with ICVC insertion were investigated retrospectively. Patients were divided into four groups according to preoperative ANC levels and Granulocyte-colony stimulating factor (G-CSF) usage. Immediate and early surgical site infections were evaluated 7 and 30 days following surgery. RESULTS: In total, 1143 patients were enrolled. Patients were placed into 4 groups: 930 patients in group 1 with an ANC≥500/µL without G-CSF, 149 in group 2 with an ANC≥500/µL after G-CSF usage, 36 in group 3 with an ANC<500/µL without G-CSF, and 28 in group 4 with an ANC<500/µL even after G-CSF administration. Rates of immediate and early SSIs were not statistically different between groups. In the two-group analysis (group 1 and 2 vs. 3 and 4), the number of immediate and early SSIs were not also different, respectively. CONCLUSION: There was no correlation between ANC levels and immediate and early SSI occurrence after ICVC placement. LEVEL OF EVIDENCE: III.

Botulinum toxin injection for internal anal sphincter achalasia after pull-through surgery in Hirschsprung disease.

Botulinum toxin (Botox) was introduced for the management of internal anal sphincter (IAS) achalasia after a pull-through procedure in Hirschsprung disease (HD). We conducted a prospective evaluation of the efficacy and safety of this Botox treatment.Our study group included 15 patients with HD (median age, 4.8 years; range, 1.7-7.4 years) who experienced persistent constipation after pull-through surgery. Rectal biopsy and colon study were performed before Botox injection to exclude agangliosis. Intersphincteric Botox injections (dose, 4 IU/kg) were performed at 3 sites, (3, 6, and 9 o'clock) under general anesthesia. Measured outcomes of efficacy included anorectal manometry, Wexner constipation score and the quality of life score for defecation, measured at baseline and at 2 weeks and 3 months after injection. The Holschneider incontinence score and an assessment of pain, bleeding, heating sensation, and swelling were also performed at follow-up as outcomes of safety.There was no significant change in measured outcomes with Botox treatment. Botox did decrease the number of patients who experienced abdominal distension at 3 months, compared to 2-weeks, post-injection. No major complications were identified, with only 2 cases of anal bleeding that resolved spontaneously. Local tenderness at the injection site was reported by 4 patients, recovering without treatment.The efficacy of Botox, injected into the IAS, for the treatment of achalasia is questionable on short-term follow-up. Larger studies with a longer follow-up period and the use of repeated injections are required to evaluate the evidence for this treatment.

Comparison of Acute Abdominal Surgical Outcomes of Extremely-Low-Birth-Weight Neonates according to Differential Diagnosis.

BACKGROUND: Improvements in perinatal intensive care have improved survival of extremely-low-birth-weight (ELBW) neonates, although the risk of acute abdomen has increased. The differential diagnosis resulting in abdominal surgery can be categorized into necrotizing enterocolitis (NEC), spontaneous intestinal perforation (SIP), meconium-related ileus (MRI), and meconium non-related ileus (MNRI). The purpose of this study was to review our experience with abdominal surgery for ELBW neonates, and to evaluate characteristics and prognosis according to the differential diagnosis. METHODS: Medical records of ELBW neonates treated between 2003 and 2015 were retrospectively reviewed. RESULTS: Of 805 ELBW neonates, 65 (8.1%) received abdominal surgery. The numbers of cases by disease category were 29 for NEC, 18 for SIP, 13 for MRI, and 5 for MNRI. Ostoma formation was performed in 61 (93.8%) patients; primary anastomosis without ostoma was performed in 4 (6.2%). All patients without ostoma formation experienced re-perforation of the anastomosis site. Seven patients had 30-day postoperative mortality (6 had NEC). Long-term survival of the surgical and non-surgical groups was not statistically different. NEC was a poor prognostic factor for survival outcome (P = 0.033). CONCLUSION: Abdominal surgery for ELBW neonates is feasible. Ostoma formation can lead to reduced complications compared to primary anastomosis.

Pediatric minimally invasive surgery for malignant abdominal tumor: Single center experience.

This study examined the safety and usefulness of minimally invasive surgery (MIS) for malignant abdominal tumors in pediatric patients and analyzed the factors affecting the resection margin, operative time, and hospital stay of neuroblastoma (NBL) patients.We retrospectively reviewed data of pediatric patients who underwent MIS for malignant abdominal tumors from January 2011 to June 2017 at the Seoul National University Children's Hospital. Sex; age at operation; diagnosis; tumor location; operation-related data, such as operation time and transfusion; and follow-up data were reviewed. We divided patients into an excision group and a biopsy group. Detailed pathologic data were reviewed to analyze factors affecting the resection margin of NBL. Median value and range were calculated for all continuous variables. Mann-Whitney test and χ test were used as appropriate. P values of <.05 were considered significant.Thirty-four pediatric patients were included; 21 were boys. The median age was 4 (0.2-18) years. The most common diagnosis was NBL (17 patients; 50.0%). Three patients each were diagnosed with lymphoma, solid pseudopapillary tumor of the pancreas, and teratoma. The median tumor size was 3.4 (0.5-10.2) cm. The median operation time was 108 (55-290) minutes, and the median hospital stay was 5 (2-11) days. The number of conversions to open surgery was 4. There were no postoperative complications or mortality. There were 18 patients in the excision group and 16 in the biopsy group. Diagnosis and the number of patients receiving preoperative chemotherapy differed between the 2 groups. R0 resection of NBL was significantly higher in patients with stage 1 disease and those aged >2 years. There were no clinical factors influencing operative time or hospital stay.MIS was feasible and safe in pediatric patients with malignant abdominal tumors. R0 resection of NBL was related to age and stage.

Why Do the Patients with Hirschsprung Disease Get Redo Pull-Through Operation?

INTRODUCTION: The treatment of Hirschsprung disease (HD) is pull-through (PT) surgery. Redo PT can be performed in 1 to 10% of patients after initial PT. In this study, we reviewed the causes and associated factors of redo PT. MATERIALS AND METHODS: We retrospectively reviewed medical charts of 657 patients with HD who underwent surgeries between September 1979 and January 2016. The indications for redo PT are as follows. First, there were persistent obstructive symptoms after the first operation, (1) with transition zone shown definitely on contrast study, (2) with anatomic problems, and (3) obstructive symptoms persist despite conservative or nonredo surgical treatment without (1) and (2). We analyzed the causes and associated factors of redo PT. RESULTS: A total of 49 (7.5%) patients underwent redo PT. Among them, 41 and 8 patients underwent PT twice and three times, respectively. Among 57 cases of redo, the causes of redo included pathologic problem (n = 28)-aganglionosis (n = 20), hypoganglionosis (n = 4), immature ganglion cell (n = 4)-or anatomic problem (n = 21)-stricture (n = 13), fistula and/or abscess (n = 8) at anastomosis. Comparing associated factors between the nonredo and redo groups, the redo group had longer initial PT operation time (p = 0.001), more postoperative complications (p < 0.001), and more transanal endorectal PT (TERPT) approach as initial PTs (p < 0.001). According to causes of redo, the anatomic problem group underwent more third PTs than the pathologic problem group (p = 0.010). CONCLUSION: Approximately 7.5% of patients experienced redo PT. The cause of redo included pathologic (n = 28) or anatomic problem (n = 21). Longer operation time, more complications, and TERPT were associated with redo. The anatomic problem group underwent more third PTs than the pathologic problem group.

Predicting Survival of Congenital Diaphragmatic Hernia on the First Day of Life.

BACKGROUND: This study aimed to determine perinatal risk factors for 30-day mortality of congenital diaphragmatic hernia (CDH) patients and develop a prognostic index to predict 30-day mortality of CDH patients. Identifying risk factors that can prognosticate outcome is critical to obtain the best management practices for patients. METHODS: A retrospective study was performed for patients who were diagnosed with CDH from November 2000 to August 2016. A total of 10 prenatal risk factors and 14 postnatal risk factors were analyzed. All postnatal variables were measured within 24 h after birth. RESULTS: A total of 95 CDH patients were enrolled in this study, including 61 males and 34 females with mean gestational age of 38.86 ± 1.51 weeks. The overall 30-day survival rate was 63.2%. Multivariate analysis revealed that five factors (polyhydramnios, gestational age at diagnosis <25 weeks, observed-to-expected lung-to-head ratio ≤45, best oxygenation index in 24 h >11, and severity of tricuspid regurgitation ≥ mild) were independent predictors of 30-day mortality of CDH. Using these five factors, a perinatal prognostic index for 30-day mortality was developed. Four predictive models (poor, bad, good, and excellent) of the perinatal prognostic index were constructed, and external validation was performed. CONCLUSIONS: Awareness of risk factors is very important for predicting prognosis and managing patients. Five independent perinatal risk factors were identified in this study. A perinatal prognostic index was developed for 30-day mortality for patients with CDH. This index may be used to help manage CDH patients.

Prospective evaluation of clinical outcomes and quality of life after gastric tube interposition as esophageal reconstruction in children.

Few studies on gastric tube interposition for esophageal reconstruction in children have assessed the long-term outcomes and quality of life (QoL). The aim of this study is to evaluate the long-term outcomes and QoL after a gastric tube interposition by reviewing our experiences with esophageal reconstruction.Twenty-six patients were included who underwent gastric tube interposition from 1996 to 2011 at our institution. We reviewed the medical records and conducted telephone surveys, prospectively performed esophagography, endoscopy, 24-hour pH monitoring, and esophageal manometry. The median follow-up period of 12 (range, 3-18) years.Median age at the time of surgery and survey were 9 (range, 2-50) months and 12.4 (range, 3.1-19.0) years, respectively. There were 14 cases of reoperation of gross type C and B esophageal atresia (EA) and 10 cases of long gap pure EA. The z scores of anthropometric data at the survey did not increase after the operation. Severe stricture in esophagography was observed in 20% of patients, but improved with balloon dilation with intact passage. Gastroesophageal reflux was able to be treated with medications. Esophageal peristalsis was observed in 1 of 8 patients in manometry. No Barrett esophagus or metaplasia was not found from endoscopy. QoL was similar to the general population and did not differ between age groups.Gastric tube interposition could be considered for esophageal reconstruction in pediatric patients when native esophageal anastomosis is impossible. Nutritional evaluation and support with consecutive radiological evaluation to assess the anastomosis site stricture are advised.

The Optimal Timing of Enterostomy Closure in Extremely Low Birth Weight Patients for Acute Abdomen.

There are few reports on enterostomy closure (EC) timing for acute abdomen in extremely low birth weight (ELBW) patients. We retrospectively reviewed ELBW patients who underwent enterostomy formation (EF) and subsequent EC. We investigated baseline characteristics, surgical outcomes, and follow-up data of 55 patients and analyzed optimal timing by age at EC, enterostomy duration, and body weight (Bwt) at EC. The minimum p-value approach (MPA) using the Chi-squared test was used to determine each cut-off value. Mean gestational age was 25+3 weeks, while mean age and Bwt at EF were 10 days and 660 g. Enterostomy duration and Bwt at EC were 102 days and 2400 g. Fourteen surgical complications were related to EC. The MPA identified a cut-off of 2100 g (p = 0.039) at EC but no significant cut-off age or enterostomy duration. The 18 patients <2100 g had more enterostomy-related problems at EC than the >2100 g group (66.7% vs 10.8%, p < 0.001). No other characteristics were significantly different. Operation time, ventilator period, hospital stay, parenteral nutrition duration, and full feeding day were significantly longer in <2100 g patients. Follow-up Bwt did not differ (11.55 kg vs 13.95 kg, p = 0.324). Our findings suggest EC can be safely performed when Bwt is over 2100 g.

Inguinal hernia in preterms in neonatal intensive care units: Optimal timing of herniorrhaphy and necessity of contralateral exploration in unilateral presentation.

BACKGROUND: We sought to determine the optimal timing of IH repair in preterms and the need for routine contralateral exploration. METHODS: Medical records of 3690 pediatric patients who underwent unilateral IH repair between January 1998 and December 2009 were reviewed. We assessed medical record review and telephone interviews. In total, 1990 patients were enrolled in the study. Early, early-delayed, and late repair were defined as herniorrhaphy performed within 7 days of diagnosis, later than 7 days of diagnosis, and after discharge from the NICU, respectively. RESULTS: Of 1990 patients, 90 preterms and 1900 full-terms were included. Among these, 7, 11 and 72 preterm patients received early, early-delayed and late IH repairs, respectively. Preoperative incarceration and postoperative complication rates were not different, but the recurrence rate was higher in the early repair group. Two group analysis of early and early-delayed vs. late repairs indicated similar results. The rates of synchronous and metachronous bilateral IH (SBIH, MBIH) were observed to be higher and the diagnostic interval of MBIH was shorter in preterms than in full-terms (35.6% vs. 15.9%, P < 0.001; 12.2% vs. 6.3%, P < 0.001; 5.2 vs. 41.8 months, P = 0.003). CONCLUSION: Our results indicate that IH repair is safe to perform in preterm babies in the NICU at a delayed or late stage since the preoperative incarceration and recurrence rates were not different. Contralateral exploration could be considered in preterms because the rates of SBIH and MBIH were significantly higher and the MBIH diagnosis interval was shorter than in full-terms. LEVEL OF EVIDENCE: III, treatment study.