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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., (Copyright © 2019. Published by Elsevier Inc.)

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., (Copyright © 2019. Published by Elsevier Inc.)

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 ± 1.49 weeks, mean birth weight 3117.5 ± 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., (Copyright © 2019. Published by Elsevier Inc.)

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., (Copyright © 2018. Published by Elsevier Inc.)

Background: Mesenteric lymphatic malformations (MLs) are a heterogeneous group of benign diseases of the lymphatic system that present with cystic dilated lymphatics of the mesentery. MLs are rare and represent less than 5% of all lymphatic malformations. The aims of this study were to analyze the characteristics of MLs in children and to suggest a modified classification., Patients and Methods: We investigated 25 patients who underwent ML surgery. The clinical data and pathological findings were reviewed retrospectively. We divided the patients into 4 groups according to the operative findings. Group 1 included patients with MLs involving the intestinal walls. Group 2 included patients with pedicle-type MLs with no relationship to the mesenteric vessels. Group 3 patients presented with MLs located in the mesenteric boundaries near the mesenteric vessels. Group 4 patients had multicentric and diffusely infiltrated MLs., Result: The male-to-female ratio was 11:14, and the median age at diagnosis was 5years of age. The most common symptom was abdominal pain. The jejunal mesentery was the most frequently involved site in this study. Five patients showed the macrocystic type and 20 patients showed the mixed cystic type. With the exception of one patient with a large mixed cystic-type ML who underwent incomplete mass excision, 24 patients underwent complete mass excision. The group 1 patients (n=14) underwent mass excision performed with segmental resection of the bowel. The group 2 patients (n=3) only underwent mass excision surgery. The patients in group 3 (n=7) underwent mass excision with segmental resection of the intestine because ML excision altered the blood supply of the adjacent intestines. The group 4 patients (n=1) presented with MLs involving the entire mesentery and underwent incomplete excision., Conclusion: The relationships between MLs and the neighboring organs determine the surgical strategy, and the size and location of MLs affect the operative methods. The modified classification based on these findings can facilitate effective treatment planning., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to analyze the long-term outcomes, such as nutritional status, pancreatic function, gastrointestinal (GI) function, and quality of life (QOL), in children who underwent pylorus-preserving pancreaticoduodenectomy (PPPD)., Methods: Between 1992 and 2013, there were 15 children who underwent PPPD at Seoul National University Children's Hospital, and 10 of them participated in this study. A retrospective review of the patients' medical records and follow-up was done. Their nutritional statuses were estimated by height, body weight, weight for age Z-score, body mass index (BMI), and serum protein, albumin levels. The endocrine and exocrine functions of the pancreas were estimated by diabetes mellitus (DM), steatorrhea, and Bristol stool chart. The GI function and QOL were evaluated via questionnaires. The follow-up period ranged from 3 to 18years., Results: There were no severe growth disturbances, 6 patients experienced mild steatorrhea and 3 showed above the category 6 in Bristol stool chart. All the patients experienced mild GI symptoms. As for the QOL, there were no significant negative answers, except for one patient with DM., Conclusions: Almost all the study subjects, who underwent PPPD in their childhood, did not present significant problems except for one patient with DM., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Esophageal duplication cysts are rare congenital cystic masses that may induce various symptoms. Complete surgical resection is the standard treatment. Although thoracoscopic resection has recently become more popular, cervical esophageal duplication cysts located at the upper third esophagus are difficult to excise via thoracoscopic approach. Here we present a case of a cervical esophageal duplication cyst in an infant, which could be resected via thoracoscopic approach only., (Copyright © 2013. Published by Elsevier Inc.)

Gastric adenomyoma in children is a rare benign tumor composed of a mixture of duct-like epithelial structures with smooth muscle bundles. It has been considered as a subtype of ectopic pancreas because the epithelial component resembles that in pancreatic ducts. However, it is now recognized as an independent pathologic lesion. Gastric adenomyoma is usually asymptomatic but could cause obstruction, inflammation, ulceration, and malignant transformation. Hence, to achieve diagnostic certainty and to control symptoms, complete operative resection is recommended. To the best of our knowledge, this is the first case of gastric adenomyoma mimicking a gastric duplication cyst in children., (Crown Copyright © 2012. Published by Elsevier Inc. All rights reserved.)

A communicating bronchopulmonary foregut malformation is a rare anomaly characterized by a fistula between an isolated portion of respiratory tissue and the esophagus or stomach. We describe the thoracoscopic management of a communicating bronchopulmonary foregut malformation in a 23-month-old boy by division of an anomalous bronchus and right upper lobectomy., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Purpose: This study was performed to investigate whether compression/stretching of the spermatic cord or blunt dissection influences testicular development and fertility. In addition, the authors evaluated whether the extents of testicular damage differ between these 2 surgical manipulations., Methods: Forty-four prepubertal male Sprague-Dawley (SD) rats (Harlan Sprague-Dawley Inc, Indianapolis, Ind) were divided into 3 groups: (1) the control group (CG) animals underwent a sham operation in the right groin, (2) the experimental group 1 (EG1) underwent compression/stretching of the right spermatic cord, and (3) the experimental group 2 (EG2) underwent dissection around the right spermatic cord structures. Testicular volumes, weights, mean seminiferous tubular diameters (MSTDs), mean testicular biopsy scores, and numbers of offspring and of pregnant females were evaluated., Results: Right (operative) and left (nonoperative) testicular volumes were smaller in the EG2 group than in the CG or EG1 groups. Left MSTDs in the EG1 and EG2 groups increased more than in the CG group. Numbers of Sertoli cells in left testes differed in the 3 groups, in the order EG1 < CG < EG2. Mean testicular biopsy scores, offspring numbers, and pregnant female numbers were no different in the 3 groups., Conclusions: Both surgical manipulations influenced testicular growth, but they did not compromise spermatogenesis or fertility in SD rats.

Massive bleeding from the porta hepatis is a rare complication after a Kasai portoenterostomy and usually requires urgent surgical intervention. Among the causes of the porta hepatis bleeding, bleeding from the injured caudate arterial branches has never been reported. Here, we describe 2 cases of caudate arterial branch bleeding after a Kasai portoenterostomy, which were successfully treated with transcatheter arterial embolization without laparotomy.

Background/purpose: The purpose of this report was to review the results of the antegrade continence enema (ACE) procedure and to compare the outcomes of right and left colon ACEs in children., Methods: Thirty patients who underwent an ACE between 1998 and 2005 were analyzed. Data were obtained based on the following parameters: postoperative soiling, catheter insertion time, colonic washout time, quality of life, and abdominal pain during and after the ACE. Twenty-nine patients were followed for an average of 3.8 years (range, 4 months-7.3 years)., Results: Right colon ACEs were performed in 23 patients, and left colon ACEs were performed in 7 patients. The common complications of the ACE included abdominal pain during and after the ACE (51.7%) and stoma strictures (41.4%). The overall ACE success rate was 24/29 (82.8%; right colon ACE, 18/29; left colon ACE, 6/29). Twenty-three patients (95.8%) believed their quality of life was improved. There were no significant differences in complications or outcomes between the right and left ACEs., Conclusions: An ACE is an effective treatment for children with fecal incontinence. A left colon ACE has similar efficacy as a right colon ACE in managing fecal incontinence in children.

Vasoactive intestinal polypeptide (VIP) is known to cause the watery diarrhea, hypokalemia, and achlorhydria syndrome. A 14-year-old girl was admitted with a 4-year history of persistent uncontrollable diarrhea and hypokalemia. Computed tomographic evaluation of the neck, chest, and abdomen were normal. Numerous polyps covering the entire colon and rectum were noted on colonoscopy. The serum VIP level was 143 pg/mL. The patient underwent a total proctocolectomy with an ileal-J-pouch. The pathologic examination revealed ganglioneuromatosis. Postoperatively, the symptoms resolved, and the serum VIP level fell to lower than 5 pg/mL. This is an unusual case of the watery diarrhea, hypokalemia, and achlorhydria syndrome caused by ganglioneuromatosis of the entire colon and rectum.

Choristoma is a tissue or mass with a normal histology at an abnormal location, and cystic choristoma has rarely been reported in the head and neck region. Cervical cystic masses in neonates are usually diagnosed as cystic lymphangioma. The authors present a case of a congenital laterocervical cystic mass that appeared to be lymphangioma, but which turned out to be a cystic choristoma.

Purpose: Although the incidence of choledochal cyst (CC) diagnosed in the prenatal or neonatal period is increasing, excision of CC in early infancy is not universally recommended. To validate the rationale of excision of CC in the neonatal period, operative complications and the histologic grade of liver fibrosis at the time of excision of CC were compared in three age groups., Methods: 198 patients who underwent excision of CC, were divided into three age groups, i.e., within 30 days old (group 1, n = 11), 1 to 12 month-old (group 2, n = 40) and over 12 month-old (group 2, n = 147). Retrospective review of their medical records was performed., Results: The overall operative complications were 12 (7 cholangitis, 4 bleeding, 1 ileus). No complication occurred in the group 1. The grade of liver fibrosis had statistically significant positive correlation among groups., Conclusion: Excision of CC in the neonatal period is a safe procedure. The grade of liver fibrosis increased with age. These results support the rationale of early excision of CC.

Background/purpose: The aim of the study was to review the gastrointestinal abnormalities occurring in association with situs anomalies., Methods: Patients with situs anomalies were identified from the medical records of pediatric patients of Seoul National University Children's Hospital from January 1980 to July 2004. Retrospective study was undertaken. Diagnosis was made on the basis of the information obtained from a combination of echocardiography, angiography, abdominal ultrasonography, liver scan, upper gastrointestinal study, or abdominal computed tomography., Results: A total 67 patients diagnosed as having situs anomalies were identified. There were 40 males and 26 females (1.54:1). Of these 67 patients, 45 patients (67%) were diagnosed as having situs inversus, 16 patients (24%) as having right isomerism, and 6 patients (9%) as having left isomerism. Of 45 patients with situs inversus, there were 26 patients (58%) who had intraabdominal abnormalities. These were duodenal atresia, biliary atresia, gastroschisis with malrotation, congenital hepatic fibrosis, tracheoesophageal fistula (type C), Currarino's triad, and pheochromocytoma. Of 16 patients with right isomerism, there were 14 patients (88%) who had intraabdominal abnormalities. These were hiatal hernia and diaphragmatic hernia. Of 6 patients with left isomerism, there were 4 patients (67%) who had intraabdominal abnormalities. These were malrotation and biliary atresia., Conclusion: When a patient is noted to have congenital heart disease as part of situs anomalies, or if an atypical position of organs is noted at imaging evaluation, we recommend that the patient undergo chest radiography, abdominal ultrasonography, upper gastrointestinal study, and abdominal computed tomography.

Background/purpose: Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignancy found in older children, and the most appropriate treatment strategy has been controversial. The authors report the tumor's clinical characteristics and the results of management in 6 children and recommend the best treatment plans based on these findings., Methods: Six USL pathologically verified at Seoul National University Children's Hospital between 1990 and 2000 were reviewed retrospectively., Results: The mean age at diagnosis of USL was 10.3 years (range, 7 to 13 years). There were 2 girls and 4 boys; 5 of 6 children presented with large abdominal mass, one with weight loss. Tumors ranged in size from 8.5 x 9.0 cm to 15.0 x 15.0 cm. Location of the tumor varied from right lobe in 4 to left lobe in one, and both lobes in one. Three children underwent primary complete resection without preoperative chemotherapy. Partial resection of the tumor was done in one child because of encasing inferior vena cava and hepatic vein. However, complete resection was possible at second-look operation after chemotherapy. Two children underwent complete resection after chemotherapy. Preoperative chemotherapy allowed successful resection of 3 USL, which were initially considered unresectable. Postoperative recovery for all children was uneventful. Chemotherapy according to the the Third Intergroup Rhabdomyosarcoma Study (IRS III) was administered to all except one child who refused further postoperative chemotherapy after having had severe complications during the first cycle of chemotherapy. One child with partial resection died of sepsis at 22 months after diagnosis during postoperative chemotherapy after complete surgical removal of the tumor. Five children are alive without recurrence at 40, 45, 48, 60, and 122 months, respectively, after diagnosis., Conclusion: The combined therapy of surgery and chemotherapy in USL can improve the prognosis., (Copyright 2002, Elsevier Science (USA). All rights reserved.)