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.

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.

Analysis of Growth, Nutritional Status and Hospital Visitation Scores Associated with Reflux After Nissen Fundoplication in Neurologically Impaired Children with Gastroesophageal Reflux.

BACKGROUND: Neurologically impaired children (NIC) often experience swallowing difficulties and gastroesophageal reflux disease (GERD). Although these conditions could place children in a state of poor nutritional status and prevent them from thriving, there is insufficient research evaluating growth and nutritional status following fundoplication in these patients. METHOD: This is a retrospective study of patients who were neurologically impaired and underwent Nissen fundoplication between April 2001 and March 2015. Seventy-six patients were enrolled, and the follow-up period was 12 months or longer. Growth was measured by the change in body weight and height. Nutritional status was measured by the change in body mass index, serum albumin and protein level. RESULTS: Median age at operation was 1.85 years old, and median body weight was 10 kg. The respective Z scores for weight and height showed significant improvements after 1 year since the operation compared to 1 year within the operation (-2.42 ± 2.19 vs. -1.31 ± 1.96, P < 0.001) (-1.6 ± 2.16 vs. -1.05 ± 1.69, P = 0.002). The respective Z scores for body mass index, albumin and protein also showed improvements after 1 year since the operation compared to 1 year within the operation (-2.07 ± 2.99 vs. -0.89 ± 2.1, P < 0.001) (3.55 ± 0.48 vs. 3.86 ± 0.45, P < 0.001) (6.22 ± 0.76 vs. 6.65 ± 0.51, P < 0.001). Hospital visitation scores associated with reflux were significantly lower after the operation (4.1 ± 3.43 vs. 1.18 ± 1.67, P < 0.001). CONCLUSIONS: In summary, after Nissen fundoplication in NIC with GER, growth and nutritional status improved significantly. Also, hospital visitation scores associated with reflux decreased after the operation.

Analysis of clinical features of lipoblastoma in children.

OBJECTIVE: A lipoblastoma is pathologically benign but often recurs. Due to its rarity, studies are scarce. The purpose of this study was to investigate the clinical characteristics of lipoblastoma occurring in children and to detect any correlations with the expression of Ki-67. PARTICIPANTS: From 1998 to 2010, 33 patients were diagnosed with lipoblastoma at Seoul National University Children's Hospital. METHODS: Ki-67 immunohistochemistry staining of the tumor tissue was performed. RESULTS: A total of 33 patients (64% males) were enrolled in the study, with a mean age of 28 month. Eleven and 22 lesions were deep and superficial, respectively. Complete excisions were performed for 30 patients, and three underwent incomplete excisions. Two patients who underwent incomplete excision subsequently underwent a second operation due to tumor regrowth, and one patient had a recurrence despite complete excision. There was no statistically significant correlation observed between the tumor size or recurrence and the expression of Ki-67. CONCLUSIONS: Lipoblastoma requires an accurate diagnosis and operative resection to alleviate the symptoms induced by its growth. Incompletely resected tumor may regrow; therefore, complete excision is the treatment of choice. Continuous follow-up is needed to monitor for recurrence of disease, even after a complete excision.

Long term outcomes of pediatric liver transplantation according to age.

Liver transplantation (LT) has been the key therapy for end stage liver diseases. However, LT in infancy is still understudied. From 1992 to 2010, 152 children had undergone LT in Seoul National University Hospital. Operations were performed on 43 patients aged less than 12 months (Group A) and 109 patients aged over 12 months (Group B). The mean age of the recipients was 7 months in Group A and 74 months in Group B. The patients' survival rates and post-LT complications were analyzed. The mean Pediatric End-stage Liver Disease score was higher in Group A (21.8) than in Group B (13.4) (P = 0.049). Fulminant hepatitis was less common in Group A (4.8%) than in Group B (13.8%) (P = 0.021). The post-transplant lymphoproliferative disorder and portal vein complication were more common in Group A (14.0%, 18.6%) than in Group B (1.8%, 3.7%) (P = 0.005). However, the 1, 5, and 10 yr patient survival rates were 93%, 93%, and 93%, in Group A and 92%, 90%, and 88% in Group B (P = 0.212). The survival outcome of pediatric LT is excellent and similar regardless of age. LTs in infancy are not riskier than those of children.

Efficacy and Safety of IDegAsp in a Real-World Korean Population with Type 2 Diabetes Mellitus.

Background: This study investigated the real-world efficacy and safety of insulin degludec/insulin aspart (IDegAsp) in Korean adults with type 2 diabetes mellitus (T2DM), whose insulin treatment was switched to IDegAsp. Methods: This was a multicenter, retrospective, observational study comprising two 26-week treatment periods, before and after switching to IDegAsp, respectively. Korean adults with uncontrolled T2DM treated with basal or premix insulin (±oral antidiabetic drugs) were enrolled. The primary objective was to compare the degree of glycosylated hemoglobin (HbA1c) change in each 26-week observation period. The analyses included changes in HbA1c, fasting plasma glucose (FPG), body weight, proportion of participants achieving HbA1c <7.0%, hypoglycemic events, and total daily insulin dose (ClinicalTrials.gov, number NCT04656106). Results: In total, 196 adults (mean age, 65.95 years; mean T2DM duration, 18.99 years) were analyzed. The change in both HbA1c and FPG were significantly different between the pre-switching and the post-switching period (0.28% vs. -0.51%, P<0.001; 5.21 mg/dL vs. -23.10 mg/dL, P=0.005), respectively. After switching, the rate of achieving HbA1c <7.0% was significantly improved (5.10% at baseline vs. 11.22% with IDegAsp, P=0.012). No significant differences (before vs. after switching) were observed in body weight change, and total daily insulin dose. The rates of overall and severe hypoglycemia were similar in the two periods. Conclusion: In real-world clinical practice in Korea, the change of insulin regimen to IDegAsp was associated with an improvement in glycemic control without increase of hypoglycemia, supporting the use of IDegAsp for patients with T2DM uncontrolled with basal or premix insulin.

The Burden of Undiagnosed Adults With Attention-Deficit/Hyperactivity Disorder Symptoms in Japan: A Cross-Sectional Study.

Background Symptoms experienced by adult patients with attention-deficit/hyperactivity disorder (ADHD) frequently result in functional impairment across academic/occupational functioning, daily life, and social functioning. A substantial proportion of undiagnosed and untreated ADHD has been suggested in Japan. This study aims to better understand the potential undiagnosed ADHD population in Japan by quantifying the burden associated with ADHD symptoms through a comparison of the prevalence of comorbidities, health-related quality of life (HRQoL), work productivity and activity impairment (WPAI), and healthcare resource utilization (HRU) between undiagnosed potential ADHD respondents who were screened positive and negative using Adult ADHD Self-Report Scale (ASRS)-v1.1. Methodology Respondents from Japan National Health and Wellness Survey 2016 who answered ASRS-v1.1 without an ADHD diagnosis were included. Respondents checking ≥4 items from ASRS-A and ≥9 from ASRS-A+B were classified as ASRS A+ (n = 309) and ASRS AB+ (n = 227), respectively. ASRS negative (n = 9,280) were respondents who were neither ASRS A+ nor ASRS AB+. Data on the presence of comorbidities, HRQoL, WPAI, and HRU were compared. Results ASRS A+ and ASRS AB+ respondents reported higher coexistence of mental comorbidities (depression, generalized anxiety disorder, bipolar disorder, obsessive-compulsive disorder, etc.), sleep problems (insomnia, narcolepsy, sleep apnea, etc.), and physical comorbidities (non-alcoholic steatohepatitis, allergy, and asthma). They also reported greater WPAI and HRU and lower HRQoL than matched ASRS-negative respondents. Conclusions A significantly higher burden was identified among undiagnosed adults with potential ADHD symptoms. Appropriate diagnosis may help those at risk or those who present with symptoms overlapping with ADHD.

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.

Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features.

BACKGROUND: Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF. OBJECTIVE: To evaluate the clinical and radiologic features of congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula. MATERIALS AND METHODS: We retrospectively reviewed postoperative esophagograms and medical records of 187 children (107 boys, 80 girls) who had primary repair of EA/TEF from 1992 to 2009 at our institution. We evaluated the incidence of CES, clinical findings, radiologic features and management of CES in these children. RESULTS: CES was diagnosed in 22 of 187 EA/TEF children (12%); one child had double CES lesions, for a total of 23 lesions. Ten of those 22 children (45%) had presented with significant symptoms of esophageal obstruction. The diagnosis of CES was delayed in 10 children (45%) until 1-10 years of age. On esophagogram, CES (n = 23) was located in the distal esophagus (n = 20, 87%) or mid-esophagus (n = 3, 13%). The degree of stenosis was severe (n = 6, 26%), moderate (n = 10, 43%), or mild (n = 7, 30%). Eight children, including two with unsuccessful esophageal balloon dilatation of CES, were treated surgically. Histologic examination revealed tracheobronchial remnant (n = 7) or fibromuscular hyperplasia (n = 1). One child with surgically treated CES developed achalasia at the age of 3 years 9 months. CONCLUSION: Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed.

Falciform ligament abscess after omphalitis: report of a case.

A falciform ligament abscess is a rare type of intra-abdominal abscess. A 2-yr-old male, who had omphalitis two months previously, presented with a fever and right upper quadrant abdominal pain. The ultrasound and CT scan showed an abdominal wall abscess located anterior to the liver, which was refractory to conservative management with percutaneous drainage and antibiotics. On the third recurrence, surgical exploration was performed and revealed an abscess arising from the falciform ligament; the falciform ligament was excised. A follow up ultrasound confirmed complete resolution of the abscess with no further recurrence.

Social burden of people with the migraine diagnosis in Japan: evidence from a population-based cross-sectional survey.

OBJECTIVES: To quantify the social burden among Japanese migraine patients in the context of currently available migraine treatments, by comparison with non-migraine controls, and comparison of migraine patients currently taking prescription medication versus not taking prescription medication. DESIGN: Cross-sectional analysis. SETTING: Data from the population-based online self-administered Japan National Health and Wellness Survey (NHWS) 2017. PARTICIPANTS: Respondents to the NHWS (n=30 001) were ≥18 years. Migraine patients were respondents with self-reported experience and physician diagnosis of migraine. Non-migraine controls reported no migraine experience. Migraine patients were subgrouped into currently taking prescription medication for migraine (Rx) and currently not taking prescription medication (non-Rx). METHODS: One-way analysis of variance tests were performed to compare health-related quality of life (HRQoL), work productivity and activity impairment and healthcare resource utilisation between migraine patients and matched non-migraine controls selected by 1:1 propensity score matching. Generalised linear models were used to compare outcomes and migraine related characteristics between Rx and non-Rx. RESULTS: Compared with matched controls, migraine patients (n=1265) had significantly lower HRQoL in terms of lower Physical Component Summary (48.36 vs 51.29, p<0.001), Mental Component Summary (44.65 vs 48.31, p<0.001), Role/Social Component Summary (41.78 vs 46.18, p<0.001) and mean EuroQol 5-Dimension index (0.77 vs 0.86, p<0.001) scores. Migraine patients experienced significantly higher absenteeism (6.95% vs 3.07%, p<0.001), presenteeism (32.73% vs 18.94%, p<0.001), work productivity loss (34.82% vs 20.03%, p<0.001) and daily activity impairment (35.70% vs 22.04%, p<0.001) and visited healthcare professionals more often (8.38 vs 4.57, p<0.001) than controls. No significant differences in these outcomes were found when comparing Rx (n=587) and non-Rx (n=678) patients. CONCLUSIONS: There is an unmet need for improved HRQoL and work productivity in Japanese migraine patients despite the currently available prescription medications, which are important factors to consider for future development of migraine therapies.

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.

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.

Increasing comorbidities in a South Korea insured population-based cohort of patients with chronic hepatitis B.

BACKGROUND: It is controversial whether chronic hepatitis B (CHB) patients have more non-liver comorbidities than non-CHB subjects. AIM: To characterise the demographics, comorbidity and health utilisation of CHB patients in South Korea and compare them to matched controls. METHODS: Using the Health Insurance Review & Assessment Service (HIRA) 2007-2016 database, adult patients with claims for CHB analysed. CHB cases and non-CHB controls matched in a 1:4 ratio using propensity score matching method. RESULTS: The age of CHB patients significantly increased from a mean 46.9 years in 2007 to 52.3 years in 2016. The proportions of persons having both liver-related and non-liver related comorbidities were higher in CHB patients compared to matched controls (dyslipidaemia [37.23% vs 23.77%, P < 0.0001], hypertension [29.39% vs 25.27%, P < 0.0001] chronic kidney disease (CKD) [3.02% vs 1.14%, P < 0.0001] and osteoporosis/fracture [OF] [4.09% vs 3.23%, P < 0.0001]). Approximately 50% of CHB patients had more than one comorbidity among CKD, diabetes, DLP, and OF. The odds of CKD in CHB patients were 1.42 times higher, and the odds of OF in CHB patients were 1.09 times higher than matched controls after adjustment for confounders (P < 0.0001). CONCLUSION: Prevalence of liver as well as non-liver comorbidities in patients with CHB was higher than matched controls and increased over time.

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.

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.

Neuroblastoma: treatment outcome after incomplete resection of primary tumors.

PURPOSE: For International Neuroblastoma Staging System (INSS) stages III or IV neuroblastoma (intermediate or high risk), complete excision of the primary tumor is not always feasible. Most current studies on the treatment outcome of these patients have reported on the complete excision status. The aim of this study is to review the treatment outcome after the incomplete resection. METHODS: The medical records of 37 patients that underwent incomplete resection between January 1986 and December 2005 were reviewed retrospectively. Incomplete resection was assessed by review of the operative notes and postoperative computerized tomography. Age, gender, tumor location, INSS stage, N-myc gene copy number, pre- and postoperative therapy, and treatment outcome were reviewed. The treatment outcome was evaluated according to the postoperative treatment protocol in the high-risk group. RESULTS: Intermediate-risk patients were treated with conventional chemotherapy, isotretinoin (ITT) and interleukin-2 (IL-2). High-risk patients were treated with peripheral blood stem cell transplantation (PBSCT), ITT, and IL-2 (N = 11). Before the introduction of PBSCT, the high-risk patients were also treated with the conventional chemotherapy (N = 19). Intermediate-risk patients (N = 5) currently have no evidence of disease (NED). For the high-risk patients (N = 32), 19 patients were treated with chemotherapy alone; 15 patients died of their disease while four patients currently have an NED status. Eight of 11 patients that underwent PBSCT are currently alive. CONCLUSIONS: For intermediate risk, conventional chemotherapy appears to be acceptable treatment. However, for high-risk patients, every effort should be made to control residual disease including the use of myeloablative chemotherapy, differentiating agents and immune-modulating agents.

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.

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.