Current practice of diagnosis and treatment for rectourethral fistula in male patients with anorectal malformation: a multicenter questionnaire survey in Japan.

PURPOSE: Surgical procedures for anorectoplasty for anorectal malformations (ARMs), particularly rectourethral fistula (RUF), depend on the institution. We investigated the diagnosis and treatment of RUF in male patients with ARMs in Japan using a questionnaire survey. METHODS: An online survey inquiring about the diagnosis and treatment (diagnostic modalities, surgical approaches, fistula dissection devices, and fistula closure techniques) of each type of ARM in male patients was conducted among institutional members of the Japanese Study Group of Anorectal Anomalies. Fisher's exact test was used to compare surgical methods between posterior sagittal anorectoplasty (PSARP) and laparoscopy-assisted anorectoplasty (LAARP). RESULTS: Sixty-one institutions (100%) completed the survey. LAARP was the preferred approach for high-type ARM (75.4%). PSARP was preferred for intermediate-type ARM (59.0%). Monopolar devices were most commonly used (72.1%) for RUF dissection. Blunt dissection was more frequent in the PSARP group (PSARP vs. LAARP: 55.6 vs. 20.0%, p < 0.005). Cystoscopy/urethroscopy to confirm the extent of dissection was used more frequently in the LAARP group (70.0% vs. 25.0%, p < 0.005). Clips and staplers were used more frequently in the LAARP group (p < 0.05). CONCLUSION: Distinct fistula management strategies for PSARP and LAARP were revealed. Further studies are needed to investigate the postoperative outcomes associated with these practices.

Factors Associated With Adverse Outcomes Following Duodenal Atresia Surgery in Neonates: A Retrospective Study.

Objectives There is limited evidence on the infants' postoperative complications who have undergone surgical repair of duodenal atresia and stenosis. This study aimed to identify the factors associated with poor surgical outcomes after the initial repair. Methods We retrospectively reviewed the data of 82 patients who underwent surgery for duodenal atresia and stenosis between January 1994 and December 2013 at our institution. Gestational age, birth weight, fetal growth, and other associated anomalies were recorded. Multivariate regression analyses were used to identify the factors associated with surgical outcomes, including postoperative complications and time to full oral intake. Results The median gestational age was 37.6 weeks, with 30 (37%) preterm (<37 weeks) and 11 (13%) early preterm (<34 weeks) infants. The median birth weight was 2531 g, with 27 (33%) patients < 2000 g and 10 (12%) patients < 1500 g. Postoperative surgical complications were identified in 18 (22%) cases, of which 12 (15%) required additional operations. Multivariate regression analysis revealed that a combination of very low birth weight (<1500 g) and early preterm was significantly associated with both surgical and non-surgical postoperative complications (p = 0.0028 and 0.021, respectively) and a prolonged time to full oral intake postoperatively (p = 0.013). Conclusion Very low birth weight and early preterm were significantly associated with postoperative complications and a prolonged time to full oral intake.

Beta-human chorionic gonadotropin-producing neuroblastoma: an unrecognized cause of gonadotropin-independent precocious puberty.

The pathogenesis of gonadotropin-independent precocious puberty (PP) includes both congenital and acquired forms, the latter of which may be associated with neoplasms, such as sex-steroid hormone-producing tumors. Beta-human chorionic gonadotropin (ß-hCG)-producing tumors also cause gonadotropin-independent PP by stimulating the production of testosterone in Leydig cells. Germ cell tumors and hepatoblastoma both produce ß-hCG; however, there is limited evidence to show that gonadotropin-independent PP is caused by other ß-hCG-producing tumors. We herein report the first case of ß-hCG-producing neuroblastoma associated with the development of gonadotropin-independent PP. A 2-year-old boy presented with an increased penile length, enlargement of the testes, pigmentation of the external genitalia, and growth acceleration. Imaging, blood, and urinary examinations revealed the presence of neuroblastoma in the right adrenal region. Decreased levels of luteinizing hormone and follicle-stimulating hormone with an increased testosterone level were indicative of gonadotropin-independent PP. Since serum ß-hCG was elevated, ß-hCG-producing neuroblastoma was suspected. Histological findings of the resected tumor were compatible with neuroblastoma. An immunohistochemical analysis using serial sections revealed staining for ß-hCG in synaptophysin-positive cells. Furthermore, immunofluorescence showed the co-staining of ß-hCG with neuron-specific enolase. These results suggested that ß-hCG was produced by tumor cells. Surgical removal of the tumor promptly normalized serum ß-hCG and testosterone levels. In conclusion, we propose the addition of neuroblastoma to the list of differential diagnoses of gonadotropin-independent PP with ß-hCG positivity in serum that includes germ cell tumors and hepatoblastoma.

Safety and Feasibility of Laparoscopic Resection of Neuroblastoma Without Image-Defined Risk Factors Performed by Pediatric Surgical Trainees: A Multicenter Comparison Study.

Introduction: Laparoscopic resection has gradually been adopted for neuroblastoma patients; however, some authors reported that, due to its technically demanding procedures, this operation should be performed only by highly experienced surgeons. The aim of this study was to evaluate the safety and feasibility of laparoscopic resection of abdominal neuroblastoma by pediatric surgical trainees. Subjects and Methods: In this multicenter, retrospective study, including 18 children with abdominal neuroblastoma indicated for 19 laparoscopic resections (1 with bilateral neuroblastomas) from 1999 to 2018, the clinical data were retrospectively reviewed and compared between trainee and attending surgeons. Results: None of the cases had image-defined risk factors (IDRFs) at surgery. All patients successfully underwent complete laparoscopic resection without blood transfusion, open conversion, or intraoperative or postoperative complications. Of the 19 cases, 6 were performed by pediatric surgical trainees under the guidance of attending surgeons, and 13 were performed by attending surgeons. With comparable tumor, largest diameter, operative time, and bleeding amount were not significantly different between the two groups. In the trainee surgeon group, one local recurrence occurred at a secondary resection site in the bilateral neuroblastoma patient with Stage L2. Conclusions: Laparoscopic resection of neuroblastoma could be safe and feasible when limited to absent IDRFs at surgery by pediatric surgical trainees under the guidance of experienced attending surgeons, as well as by attending surgeons.

Anterior chest wall regression after Nuss bar removal in adult patients with pectus excavatum.

OBJECTIVE: Pectus excavatum repair with the Nuss procedure can be successfully performed in adults. After removing the pectus bars, the anterior chest wall may regress to some degree. The purpose of this study was to clarify the amount of improvement and regression of the chest wall after bar removal. METHODS: In 45 adult patients who underwent the Nuss procedure, the sternovertebral distance (SVD) on lateral chest X-ray was measured (A) before the Nuss procedure, (B) before bar removal, and (C) after bar removal. The average SVD was compared, and the difference between A and C suggesting final sternal elevation and B and C suggesting regression was calculated. The correlation between the duration of bar in situ and the amount of regression was analyzed. RESULTS: The average period of bar in situ was 34.9 ± 5.0 (range 23-45) months. The average SVD-A, SVD-B, and SVD-C values were 58.9 ± 20.0, 89.3 ± 19.1, and 81.6 ± 20.1 mm, respectively, with significant differences among them. Final sternal elevation was 22.7 ± 17.4 mm, and average regression was 7.6 ± 8.6 mm. The correlation coefficient between the duration of bar in situ and the amount of regression was 0.119, suggesting no clear correlation. CONCLUSIONS: In spite of some degree of chest wall regression after bar removal, the Nuss procedure was effective for adult patients with pectus excavatum. The period of bar in situ and chest wall regression had little correlation.

The risk factors for cholestasis in patients with duodenal atresia in a single institutional cohort.

PURPOSE: The etiology of cholestasis in neonates is associated with several factors including gastrointestinal disease and surgery. We aimed to identify the potential risk factors for perioperative cholestasis in patients with duodenal atresia and determine specific cutoff values for the risk factors. METHODS: This retrospective cohort study included 76 neonates diagnosed with duodenal atresia surgically treated during the neonatal period at our institution between January 1990 and March 2017. The neonates were categorized into two groups: those with and without cholestasis. Univariate and multivariate analyses were performed to identify the possible risk factors for cholestasis. RESULTS: Among the 76 neonates with duodenal atresia, 21 (27%) developed cholestasis. The duration of total parenteral nutrition was identified as a risk factor in univariate analysis; however, it was not an independent risk factor for cholestasis. Gestational age and highest C-reactive protein (CRP) values were independent risk factors, with adjusted odds ratios of 0.53 and 1.25, respectively. To predict the occurrence of cholestasis, the cutoff value for gestational age was 35.0 weeks, and highest CRP value was 2.4 mg/dL. CONCLUSIONS: The occurrence of cholestasis in patients with duodenal atresia was associated with preterm delivery and severity of the inflammatory response during the perioperative period.

Intussusception Caused by Percutaneous Endoscopic Gastrostomy With Jejunal Extension in Patients With Severe Motor and Intellectual Disabilities.

The risk of intussusception related to percutaneous endoscopic gastrostomy with jejunal extension (PEG-J) in patients with severe motor and intellectual disabilities (SMID) remains unknown. In a cross-sectional study, a review of 26 patients (mean age, 11.6 ± 6.4 years) with SMID who underwent PEG-J was performed. During the follow-up period, 6 of 26 (23%) patients developed intussusception. The median period from PEG-J to the onset of intussusception was 364 (range, 8-1344) days. No significant difference was observed in the Cobb angle between the intussusception and nonintussusception groups; however, body mass index at the time of PEG-J was significantly lower in the intussusception group. Intussusception related to PEG-J occurs relatively frequently in patients, and it is possibly attributable to factors such as deformity caused by undernutrition and weight loss. If enteral nutrition via PEG-J has been established, earlier enterostomy can be recommended because of the high risk of intussusception in patients with SMID.

A Rare Case of Immature Sacrococcygeal Teratoma With Lymph Node Metastasis in a Neonate.

This is the first report of an immature sacrococcygeal teratoma with inguinal lymph node metastasis, providing the histologic transformation of an immature teratoma in association with chemotherapy. Incomplete tumor resection with coccygectomy was performed, and the histopathologic diagnosis was a grade 3 immature teratoma. Following the initial surgery, the residual tumors enlarged and the tumors metastasized to the inguinal lymph node, demonstrating immature teratoma without yolk sac tumor components. Although the tumor markers normalized after chemotherapy, the residual tumors had enlarged significantly. Therefore, a complete resection of the residual tumors was performed, and they were found to be mature teratomas.

Long-term functional outcome of sacrococcygeal teratoma after resection in neonates and infants: a single-center experience.

PURPOSE: This study aimed to evaluate the incidence and factors associated with long-term functional outcomes of sacrococcygeal teratoma (SCT) after resection in neonates and infants. METHODS: Twenty-nine patients with a minimum of 3 years of follow-up who underwent resection and were histologically diagnosed with SCTs between 1982 and 2017 at our institution were included. RESULTS: The median age at the time of the study was 10.0 years. Functional disorders occurred after surgery in 6 (20.7%) patients. Anorectal dysfunction, urologic dysfunction, and lower-extremity motor disorders occurred in 6 (20.7%), 4 (13.8%), and 3 (10.3%) patients, respectively. One patient with all three types of functional disorders developed intestinal perforation due to ileus and died of sepsis at 13 years of age. The overall mortality rate after tumor resection was 3.4%. The patients who developed functional disorders presented a low 1-min Apgar score, larger tumors requiring abdominosacral resection, surgical injury to the pelvic organs, and immature or malignant histological findings. CONCLUSION: Although the mortality rate was low, the long-term rate of functional disorders after SCT resection was approximately 20%. SCT patients with large tumors, surgical injury to the pelvic organs, and immature or malignant histological findings require thorough follow-up.

Clinical outcomes and protocol for the management of isolated congenital diaphragmatic hernia based on our prenatal risk stratification system.

BACKGROUND/PURPOSE: The aim of this study was to evaluate our prenatal risk stratification system for risk-adjusted management in fetuses with isolated congenital diaphragmatic hernia (CDH). METHODS: Ninety-four infants prenatally diagnosed with isolated CDH treated between 1998 and 2017 at our institution were included in this retrospective single-center cohort study. RESULTS: The patients were prenatally classified into four risk groups: Group A (n = 54), which consisted of infants with neither liver-up nor a contralateral lung-to-thorax transverse area (L/T) ratio <0.08. The infants in group A were divided into two subgroups: Group A-1 (n = 24) consisted of mild conditions; and Group A-2 (n = 30) consisted of severe conditions; Group B (n = 23), which consisted of infants with either liver-up or L/T ratio <0.08; and Group C (n = 17), which consisted of infants with both liver-up and L/T ratio <0.08. The rates of survival to discharge in Groups A-1, A-2, B, and C were 100.0%, 100.0%, 87.0%, and 58.8%, respectively. The rates of intact discharge were 91.7%, 90.0%, 52.1%, and 23.5%, respectively. CONCLUSIONS: Our prenatal risk stratification system demonstrated a significant difference in the severity of postnatal status and clinical outcomes between the groups. STUDY TYPE: Case Series, Retrospective Review. LEVELS OF EVIDENCE: LEVEL IV.

Clinical outcomes of ex utero intrapartum treatment for fetal airway obstruction.

PURPOSE: The aim of this study was to evaluate the indications and the clinical outcomes of the fetuses managed with ex utero intrapartum treatment (EXIT) procedures. METHODS: We retrospectively reviewed the medical records of all fetuses who underwent EXIT procedures between 2003 and 2018. RESULTS: EXIT procedures were performed in nine cases. The prenatal diagnosis of the neonates was congenital high airway obstruction syndrome in four cases, the neck masse in five cases. Although the airway management under the EXIT procedure was successful in eight cases, the airway management failed in one case. During the EXIT procedures, the airway was managed by endotracheal intubation in two cases, whereas six cases underwent tracheostomy. Six cases with fetal airway obstruction survived to discharge, whereas three cases died due to airway management failure or complications of the underlying disease. A case with a cervical teratoma underwent tumor resection the day after birth due to rapid enlargement of the neck mass. Long-term survival was achieved in five cases. CONCLUSIONS: We concluded that the EXIT procedure was effective and could be performed safely in the airway management of fetuses with suspected airway obstruction. The treatment strategy for the neck masses should be planned before birth.

Learning curve for the thoracoscopic repair of esophageal atresia with tracheoesophageal fistula.

AIM: Thoracoscopic repair (TR) of esophageal atresia with tracheoesophageal fistula (EA/TEF) remains a considerable challenge, even for the most experienced pediatric surgeons. The aim of this study is to report the outcomes of our experience with TR of EA/TEF and to determine the learning curve for this procedure. METHODS: Eleven consecutive cases that had undergone TR of EA/TEF at our institutes were included in this study. The medical charts were reviewed retrospectively. To determine the learning curve for TR of EA/TEF, a logarithmic curve-fitting analysis was performed. The data were expressed as medians with ranges. RESULTS: The median age and birth weight were 1 day (range, 1-3 days) and 2.8 kg (range, 2.5-3.7 kg), respectively. TR was completed in all cases without any complications. The median operative time was 230 min (range, 164-383 min). There were no cases of anastomotic leakage. One patient with a long gap required repeated balloon dilatation for refractory anastomotic stricture. No mortality or recurrence of tracheoesophageal fistula occurred. The operative time was significantly longer in patients with a long gap (>20 mm) than in those with a shorter gap. Once the three cases with a long gap had been excluded, the operative time decreased as the number of treated cases increased. The relationship between the operative time and case number fit a logarithmic function curve well (operative time in minutes = 300 - 62 × log (case number), R2  = 0.8359, P = 0.0015). CONCLUSIONS: Our results suggest that TR of EA/TEF is a safe procedure. It has a considerable learning curve, but requires advanced endoscopic surgical skills.

Characteristics of gastroesophageal reflux in pediatric patients with neurological impairment.

PURPOSE: To clarify the characteristics of gastroesophageal reflux (GER) in pediatric patients with neurological impairment (NI). METHODS: Twenty-six patients with NI, 1-17-year-old, fed via a nasogastric tube were categorized based on the absence (group I) or presence (group II) of abnormal GER, defined with multichannel intraluminal impedance-pH monitoring as %time with an esophageal pH <4.0 being ≥5.0% or number of liquid/mixed bolus reflux events(RE) >70 in 24 h. RESULTS: %Time and number of distal and proximal bolus REs were not significantly different between acid and nonacid REs. %Time of acid bolus RE and that of acid and total number of distal and proximal bolus REs were significantly higher in group II than in group I. Mean acid clearance time was significantly longer in group II. Median bolus clearance time was similar between the groups. No bolus RE parameters were significantly different between acid and nonacid REs in group I. Acid bolus RE was significantly higher than nonacid RE in terms of %time and number of proximal bolus RE in group II. CONCLUSION: Acid REs significantly increased in pediatric patients with NI with abnormal GER. The number of acid and nonacid REs was comparable in patients without abnormal GER.

Fluoroscopy-guided Percutaneous Endoscopic Gastrostomy in Children: A Simple and Safe Technique.

Percutaneous endoscopic gastrostomy (PEG) is a widely used minimally invasive procedure. However, PEG is difficult in neurologically impaired patients due to scoliosis with a high-riding stomach and intestinal dilatation. Fluoroscopy-guided PEG (F-PEG) is expected to avoid PEG-related complications such as intestinal injury and malpositioned tube placement. The aim of this retrospective study is to evaluate the feasibility and safety of F-PEG in children. Twenty-one handicapped pediatric patients underwent F-PEG from January 2012 to December 2014 at our hospital. Among them, 6 had scoliosis, 7 had a high-riding stomach, and 3 had gastric volvulus. F-PEG was performed in all 21 patients without any major complications. The median procedure duration was 22 minutes. In 1 patient with severe scoliosis (Cobb angle, 118 degrees) and a high-riding stomach, F-PEG was safely performed. In conclusion, F-PEG is a simple and feasible technique, and can reduce the risk of PEG-related complications in handicapped pediatric patients.

Long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal reconstruction.

PURPOSE: The aim of this study was to evaluate the long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal/alimentary reconstruction. MATERIALS AND METHODS: We reviewed the medical records of four long-term survivors of tracheal agenesis and collected the following data: age, sex, type of tracheal agenesis, method of reconstruction, nutritional management, and physical and neurological development. RESULTS: The patients consisted of three boys and one girl, who ranged in age from 77 to 109months. The severity of their condition was classified as Floyd's type I (n=2), II (n=1), or III (n=1). Mechanical respiratory support was not necessary in any of the cases. Esophageal/alimentary reconstruction was performed using the small intestine (n=2), a gastric tube (n=1), and the esophagus (n=1). The age at esophageal reconstruction ranged from 41 to 55months. All of the cases required enteral nutrition via gastrostomy. Three of the patients were able to swallow a small amount of liquid and one was able to take pureed food orally. The physical development of the subjects was moderately delayed-borderline in childhood. Neurological development was normal in two cases and slightly delayed in two cases. CONCLUSIONS: None of the long-term survivors of tracheal agenesis required the use of an artificial respirator, and their development was close to normal. Future studies should aim to elucidate the optimal method for performing esophageal reconstruction to allow tracheal agenesis patients to achieve their full oral intake.

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan.

BACKGROUND: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan. METHODS: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan. RESULTS: Of the 28 patients registered as having a certain diagnosis of MMIHS, 19 (male/female, 3/16) patients were analyzed. All of the patients developed functional bowel obstruction in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all patients. Although various medications were given, the patients did not show significant improvement. Drainage stomas were created in the jejunum (n=11) and colon (n=5). Sixteen patients were maintained by parenteral nutrition (PN). Nine patients died of sepsis or liver failure. The five- and ten-year survival rates were 63% and 57%, respectively. CONCLUSIONS: MMIHS patients developed severe functional intestinal obstruction in the neonatal period and had no specific therapeutic intervention. The majority of MMIHS patients required long-term PN. Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition.

Causal relationship between delayed gastric emptying and gastroesophageal reflux in patients with neurological impairment.

PURPOSE: To investigate the causal relationship between delayed gastric emptying (DGE) and gastroesophageal reflux (GER) in patients with neurological impairment (NI). METHODS: Subjects included 30 NI patients (age, 1-34 years; median, 6 years). Combined multichannel intraluminal impedance-pH (MII-pH) monitoring and (13)C-breath test evaluated acid/non-acid reflux episodes (RE) and gastric emptying rates, respectively. Values are shown with medians and ranges. RESULTS: Percentage time for esophageal pH < 4.0 (reflux index: RI) and numbers for total RE and RE > 5 min were 8.3 (0-35.7), 44 (0-129), and 5 (0-22), respectively. Total percentage time and bolus RE numbers were 1.4 (0.0-6.9) and 49 (2-159), respectively. Median bolus clearance time was 16 s (9-45). T 1/2, T lag, and GEC were 103 s (75-204), 54 s (18-97), and 3.4 (2.3-4.3), respectively. Gastric emptying rates and acid/non-acid reflux parameters were not significantly correlated, except T 1/2 correlated significantly with the numbers of RE > 5 min (p = 0.04). T 1/2 inversely correlated with bolus clearance time (p = 0.01). Reflux parameters between DGE and non-DGE patients were not significantly different, except median bolus clearance time was significantly shorter in DGE patients (p = 0.01). CONCLUSIONS: NI patients showed a wide range of gastric emptying rates without any significant causal relationship between DGE and GER.

Does laparoscopy-aided gastrostomy placement improve or worsen gastroesophageal reflux in patients with neurological impairment?

BACKGROUND/PURPOSE: The purpose of this study was to retrospectively investigate whether laparoscopy-aided gastrostomy placement (LGP) improved or worsened gastroesophageal reflux (GER) in neurological impairment (NI) patients. METHODS: Subjects included 26 NI patients nourished via nasogastric tubes (age, 1-17years; median, 6years). They were divided into groups based on the percentage of time with an esophageal pH <4.0 (reflux index: RI) before LGP: Group 1 (GI, n=13), RI <5.0%; Group II (GH, n=13), RI ≥5.0%. Acid/nonacid reflux episodes (RE) were evaluated using combined pH-multichannel intraluminal impedance (pH-MII) monitoring, and gastric emptying was measured with the C breath test before and after LGP. RESULTS: RI and number of RE evaluated with pH analyses and number of total/acid distal and proximal bolus RE with pH-MH increased significantly in GI. RI and acid clearance time with pH analyses and number of total bolus RE with pH-MII decreased significantly in GH. Gastric emptying parameters did not change significantly in GI, whereas the half-gastric emptying time and gastric emptying coefficient improved significantly in GH. CONCLUSION: LGP reduces GER in NI patients with pathological GER by improving gastric emptying, although it has a paradoxical influence on those without pathological GER.