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Background: Omphalocele and gastroschisis are the two most common congenital abdominal wall defects; however, no previous study has focused on gastrointestinal and hepatobiliary tract malformations in these two conditions. This study aimed to investigate the demographic characteristics, coexisting congenital gastrointestinal and hepatobiliary tract anomalies, hospital course, and outcomes of patients with gastroschisis and omphalocele., Methods: This is retrospective chart review of all patients admitted to one tertiary medical center in Taiwan between January 1, 2000 and June 30, 2020 with a diagnosis of gastroschisis or omphalocele. The medical records were reviewed to obtain demographic data regarding coexisting gastrointestinal and hepatobiliary tract anomalies and outcomes., Results: Of the 51 patients included, 21 had gastroschisis and 30 had omphalocele. Gastroschisis was associated with a significantly younger maternal age and a higher incidence of small for gestational age. Of the 30 patients with omphalocele, twelve had associated gastrointestinal and hepatobiliary anomalies. Seven of the 21 patients with gastroschisis had gastrointestinal anomalies, and none had hepatobiliary anomalies. Among the omphalocele patients, three (10%) had documented malrotation, and one developed midgut volvulus. Among gastroschisis patients, four patients (19%) had malrotation, and two developed midgut volvulus. There were no statistically significant differences in postoperative complications or mortality rates between those with and without gastrointestinal/hepatobiliary tract anomalies., Conclusion: The diversity of coexisting gastrointestinal and hepatobiliary tract anomalies is higher in the omphalocele than in gastroschisis. In addition, we demonstrate that patients with gastroschisis or omphalocele have a higher rate of intestinal malrotation and midgut volvulus., (Copyright © 2022 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved.)

Background: Hepatoblastoma (HB) is the most common childhood primary hepatic malignancy. The overall survival rate in patients with HB has reached more than 80% over the past decades. The poor prognostic and high-risk HB have been defined, but the treatment and cure of refractory or relapsed HB is still an arduous task., Methods: The complete records of HB in patients under the age of 18 at the MacKay Memorial Hospital between 1990 and 2019 were examined., Results: The treatment results for 11 patients with refractory or relapsed HB are presented. The multi-modality treatment records were reviewed and the clinical characteristics associated with poor outcome included multifocal lesions, low α-fetoprotein, great vessel invasion and metastases. Delayed liver tumor surgery was carried out in eight cases. The median duration of follow-up for the 11 patients was 48.6 months (range 1.9 to 316.8 months). The 5-year and 10-year overall survival rate were 62.3% ± 15% (SE) and 49.9% ± 16.4% (SE), respectively. Most treatment-related toxicities were tolerable. The major concern during long term follow-up was irreversible high-frequency hearing loss., Conclusion: Patients with refractory/relapsed HB are still a thorny issue and more research is needed to improve the outcome., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2021. Published by Elsevier B.V.)

Background: The survival of children with malignant germ cell tumor (GCT) increased over the past 2 decades with platinum-based chemotherapy. This report has three objectives: (1) comparison of PVB (cisplatin, vinblastine, and bleomycin) with JEB (carboplatin, etoposide, and bleomycin) regimens; (2) treatment modality of vaginal GCT; and (3) management of stage I testicular yolk sac tumor (YST) in boys under 2 years old., Methods: From January 1, 1987 to December 31, 2010, 81 patients with malignant extracranial GCT were treated. Two consecutive protocols, PVB followed by JEB, were used. Girls with vaginal YST received minimal surgery and chemotherapy. Boys under 2 years old with Stage I testicular YST received surgery with or without chemotherapy., Results: As of June 30, 2012, the 10-year overall survival (OS) was 95 ± 3% (standard error) and the event-free survival (EFS) was 88 ± 4%. With PVB, 35 patients had 10-year OS of 91 ± 5% and EFS of 89 ± 5%. With JEB, 25 patients had 7-year OS of 96 ± 5% and EFS of 96 ± 5%. All five girls with vaginal YST were cured with vagina-preserved strategy. In 32 boys age under 2 years old with stage I YST, 16 with light chemotherapy were all in EFS, whereas two of 16 patients without chemotherapy relapsed. After PVB, six patients developed nephrotoxicity and one had pulmonary fibrosis., Conclusion: Girls with vaginal YST who received minimal surgery and chemotherapy had excellent prognosis and sexual organs were preservable. Light chemotherapy after surgery is a treatment option for boys under 2 years old with stage I YST to decrease relapse rate. Both JEB and PVB are effective. JEB resulted in more myelosuppression but otherwise less serious long-term toxicity than PVB., (Copyright © 2015. Published by Elsevier B.V.)

Objective: To determine the optimal timing of surgery for recurrent intussusception., Methods: We retrospectively reviewed medical records of patients aged from 0 to 18 years old with diagnosis of intussusception in the Pediatric Department at Mackay Memorial Hospital between January 1995 and May 2010., Results: During the study period, there were 686 children (divided into three age groups: 367 < 2 years, 289 aged 2 to 5 years, 30 > 5 years) with diagnoses of intussusception. Eighty-five of the 686 patients had recurrent intussusception, of whom 56 had two, 16 had three, 11 had four, and 2 had five episodes. The recurrence rate after the first, second, third, and fourth barium enema reductions were 15.7%, 37.7%, 68.4%, and 100.0%, respectively. The incidence of recurrence and failure rate of barium enema reduction did not differ significantly among these three age groups. Surgery was performed in 177 children (146 during the first episode and 31 in recurrent cases). The probability of eventual surgery after first enema reduction was 21.8%, after the second 35.7%, and after the third 70.0%. Lead points were found in 15 children, and all of them were found during surgery for the first episode of intussusception., Conclusion: The probability of recurrence was 100% after the fourth episode of intussusception in our study. After the third episode of intussusception, the probability of recurrence and eventual surgery were 68% and 70%, respectively. From this study, surgical intervention should be considered at the third episode of intussusception., (Copyright © 2012. Published by Elsevier B.V.)

Background: To assess whether constipation or fecal incontinence is a major late complication after posterior sagittal anorectoplasty in patients with anorectal malformation (ARM)., Methods: We retrospectively enrolled 188 children, 85 low-type ARM (L-ARM) and 103 high-type ARM (H-ARM), who had complete medical records of bowel habits and medication histories after posterior sagittal anorectoplasty for anorectal malformation in Mackay Memorial Hospital. Stool characteristics as well as physical and medication history were evaluated. The symptom severity (SS) scoring system was used to assess changes in bowel habits., Results: During a mean follow-up period of 4.3 years, constipation was found to be the most common late complication in both groups of patients (64.5% in the L-ARM group and 78.6% in the H-ARM group). Compared to constipation, stool incontinence was much less frequent, with 4.7% in L-ARM and 3.9% in H-ARM. There was no significant difference in mean SS scores between the two groups., Conclusion: Constipation was the most common late sequela in children after correction of ARM in our study., (Copyright © 2012. Published by Elsevier B.V.)

Background: To classify and evaluate the clinical spectrum of congenital webs in the gastrointestinal (GI) tract, including clinical courses and related factors., Methods: A retrospective chart review was performed on 37 patients with congenital GI webs at a pediatric care teaching hospital in north Taiwan. All of the related parameters were collected and analyzed., Results: Twelve patients had gastric webs, 22 had duodenal webs, and three had jejunal webs. The mean time to diagnosis was 1576 days for gastric webs, 116 days for duodenal and 230 days for jejunal webs. There was a statistically significant difference between the gastric and duodenal groups (p = 0.001). The major symptom was vomiting (78%). Patients with duodenal webs had a high association with congenital anomalies (50%). The major anomalies included cardiac (27%) and GI anomalies (18%). Endoscopy was performed in 10 gastric cases, and all of them were noted to have positive findings, including a fixed nonfolded stenotic ring following a second gastric chamber and a real pylorus. All of the patients received surgery except for three with gastric webs, and no mortality was noted. The mean postoperative days of tolerated feeding was 6 for those with gastric webs, 10 for those with duodenal and 11 for those with jejunal webs., Conclusion: The clinical course of gastrointestinal webs may be chronic or obscure. A delay from onset of symptoms to treatment may exist, especially in gastric webs. We suggest that prompt endoscopic confirmation and surgical intervention for these lesions, when suspected due to clinical and radiologic abnormalities, will decrease the morbidity of unexplained recurrent symptoms or signs of GI obstruction in these patients., (Copyright © 2012. Published by Elsevier B.V.)

Background: Congenital Bochdalek diaphragmatic hernia (CDH) is easily recognized if it is present shortly after birth. However, cases of delayed CDH presentation are more subtle and therefore less easily diagnosed., Methods: We retrospectively analyzed 85 patients who were under 18 and diagnosed with CDH between June 1987 and May 2007. Those diagnosed before 1 month of age were categorized as having early-presenting CDH and those diagnosed after 1 month were categorized as having late-presenting CDH., Results: Of the 85 cases, 68 (80%) were early-presenting and 17 (20%) were late-presenting CDH. Respiratory symptoms were more common in early-presenting CDH, while gastrointestinal complaints were more dominant in the late-presenting group, particularly in patients with left diaphragmatic defects. Late-presenting CDH, which was more likely to be associated with chronic gastrointestinal symptoms, was associated with a lower mortality than acute early-presenting CDH with respiratory symptoms, despite the more prompt diagnosis of the latter. The small bowel was the organ most commonly herniated into the thorax. Congenital heart disease was the most commonly associated malformation, but the presence of associated anomalies did not increase mortality., Conclusion: Early-presenting CDH shows acute symptoms, is readily diagnosed, and requires prompt intervention to prevent death. In contrast, late-presenting CDH shows more subtle symptoms and is more difficult to diagnose. Although the late-presenting CHD has better prognosis, it still requires surgery intervention to alleviate the symptoms. Clinicians must maintain a high index of suspicion for CDH, regardless of its presentation.

Background: Sonography is used to diagnose infantile hypertrophic pyloric stenosis, but Little information is available about the appearance of postoperative sonographs. The purpose of this study was to evaluate the morphology of the pylorus in association with an obstruction before and after pyloromyotomy., Methods: Pyloric length, diameter, muscle thickness and intermuscular space were measured sonographically at diagnosis and daily after pyloromyotomy until discharge in 12 infants with infantile hypertrophic pyloric stenosis. The ratios of pyloric wall thickness and intermuscular space to the entire pyloric diameter were measured., Results: The pylorus still appeared hypertrophied after pyloromyotomy on the sonograms. The traditional measurement of linear dimensions of the pylorus was not significantly changed from the preoperative values by the time of discharge, except for muscle thickness. The intermuscular space increased from 4.8+/-0.8 mm preoperatively to 7.3+/-2.1 mm by postoperative day 3 (p=0.10). Lowe's pyloric ratio at diagnosis was a mean of 0.32, decreasing to 0.29 on postoperative day 3 and 0.29 on the day of discharge (p=0.82). The alternative pyloric ratio increased significantly by postoperative day 2 (0.24+/-0.09 on day 2 vs. 0.11+/-0.07 preoperatively, p=0.02)., Conclusion: The pyloric ratio appears to be a reliable parameter in evaluating the regression of pyloric stenosis after pyloromyotomy, and also aids in the diagnosis of pyloric stenosis.

Background: Gastric perforation among neonates is a rare but frequently fatal condition of uncertain etiology. The aim of this study was to review the clinical course of neonatal gastric perforation and to evaluate possible prognostic factors., Methods: We retrospectively analyzed the medical records of 15 patients with neonatal gastric perforation over a 19-year period. Another 97 patients described in the medical literature, for whom the gestational ages and birth weights were clearly stated, were also reviewed., Results: In our series, there were three girls and 12 boys, nine of whom were full-term infants and six preterm infants. The most common initial manifestations were poor activity, abdominal distension, and respiratory distress. The overall mortality was 47% (7/15). Prematurity was the only statistically significant risk factor; 83% (5/6) of premature infants died compared with 22% (2/9) of term babies (p < 0.05). Combining our series with the patients reported in the literature, there were a total of 50 premature infants and 62 term infants. Gastric perforation occurred on postnatal days 2-7 and presented with nonspecific manifestations. The mortality was significantly higher in premature than in term infants (31/50, 62% vs. 16/62, 26%; p < 0.001). A trend towards higher mortality in infants with lower birth weights was observed (>2500 g, 28%; 1501-2500 g, 52%; 1000-1500 g, 60%; <1000 g, 100%). Infants with birth weights <2500 g had a significantly higher mortality than infants with birth weights >2500 g (32/58, 55% vs. 15/54, 28%; p<0.05)., Conclusion: Neonatal gastric perforation is associated with high mortality, particularly in premature infants. There is also a trend towards higher mortality in lower-birth-weight infants.