Analysis of gap length as a predictor of surgical outcomes in esophageal atresia with distal fistula: a single center experience.

PURPOSE: Long-gap esophageal atresia (LGEA) is still a challenge for pediatric surgery. No consensus exists as to what constitutes a long gap, and few studies have investigated the maximum gap length safely repairable by primary anastomosis. Based on surgical outcomes at a single institution, we aimed to determine the gap length in LGEA with a high risk of complications. METHODS: The medical records of 51, consecutive patients with esophageal atresia (EA) with primary repair in the early neonatal period between 2001 and 2021 were retrospectively reviewed. Three, major complications were found in the surgical outcomes: (1) anastomotic leakage, (2) esophageal stricture requiring dilatation, and (3) GERD requiring fundoplication. The predictive power of the postsurgical complications was assessed using receiver operating characteristic analysis, and the area under the curve (AUC) and the cutoff value with a specificity of > 90% were calculated. RESULTS: Sixteen patients (31.4%) experienced a complication. The AUC of gap length was0.90 (p < 0.001), and the gap length cutoff value was ≥ 2.0 cm for predicting any complication (sensitivity: 62.5%, specificity: 91.4%). CONCLUSION: A gap length ≥ 2.0 cm was considered as defining LGEA and was associated with an extremely high complication rate after primary repair.

Surgery for hepatoblastoma in children with trisomy 18: a monocentric study.

PURPOSE: Recently, children with trisomy 18 have been receiving more active treatment for malignancies. We report herein seven cases complete resection was achieved, and discuss multidisciplinary treatment for hepatoblastoma in patients with trisomy 18. METHOD: The medical records of children with trisomy 18 who were treated at the study center between 2010 and 2023 were reviewed. RESULT: Six of 69 patients had hepatoblastoma development, and three of these underwent multidisciplinary treatment. In addition, 6 patients had been referred by another hospital for treatment, and four of these underwent multidisciplinary treatment. Among the seven patients who underwent multidisciplinary treatment, three, two, and two were categorized in Pre-treatment Extent of Disease (PRETEXT) classification group I, II, and III, respectively. Neoadjuvant chemotherapy resulting in tumor reduction was performed in three cases. In all the cases, complete resection was achieved with pathologically safe margins. Perioperative complications included circulatory failure in one case and bile leakage in two cases. Adjuvant chemotherapy was administered in four cases. The postoperative observation period ranged from 3 months to 11 years, and all the patients are recurrence-free. CONCLUSION: Children with trisomy 18 complicated with hepatoblastoma whose cardiopulmonary conditions are stable may be good candidates for chemotherapy and surgery.

Treatment Strategy for Currarino Syndrome Complicated With Anorectal Stenosis.

PURPOSE: The present study aimed to review the treatment experience and outcomes of Currarino syndrome (CS) complicated with anorectal stenosis to evaluate the current treatment strategies. METHODS: Seven cases of CS complicated with anorectal stenosis, treated at our hospital between 1998 and 2021, were retrospectively investigated. This is a case series article from a single institution. RESULTS: In six and three cases and one case, the presacral mass was a mature teratoma, meningocele, and lipoma, respectively. Resection of the lesion was performed in all six cases of mature teratoma, and duraplasty was performed before resection in all three cases of meningocele. Moreover, surgery for anorectal stenosis was performed simultaneously in four patients. Surgery was performed for six cases of anorectal stenosis, with the remaining case relieved by dilation using a metal bougie. The surgical methods used were a partial resection with end-to-end anastomosis, anorectal strictureplasty, pull-through, posterior sagittal anorectoplasty, and cutback after mass resection. Pathological analysis of the anorectal stenoses revealed disorganized and rough smooth muscle fibers and the replacement of the stroma by an increased quantity of collagen fibers. CONCLUSIONS: The clinical outcomes of CS can be improved by establishing a treatment flow chart and understanding the complicated pathophysiology of the disease.