Excision of an Anovestibular Fistula with a Normal Anus Using a Biopsy Punch.

Among anorectal malformations (ARM), the isolated H type anovestibular fistula (AVF) with a normal anus is a rarity, affecting only approximately 3% of patients with anorectal malformations. AVFs are abnormal, epithelial-lined connections between the anal canal and vestibulum, and their management is a challenge for surgeons. Complete excision of the entire fistula tract is the goal of treatment, but this can often be difficult. Moreover, recurrences are not so rare. Fistulas can occur as a result of a congenital malformation, but most often have an acquired etiology. Here, we report AVF with a normal anus in a newborn. The fistula repair was performed using a biopsy punch and layered closure without overlapping suture lines, when the patient was 4 months old.

Internal Ileal Diversion as Treatment for Progressive Familial Intrahepatic Cholestasis Type 1-Associated Graft Inflammation and Steatosis after Liver Transplantation.

BACKGROUND: Progressive Familial Intrahepatic cholestasis type I (PFIC1) is a rare congenital hepatopathy causing cholestasis with progressive liver disease. Surgical interruption of the enterohepatic circulation, e.g., surgical biliary diversion (SBD) can slow down development of liver cirrhosis. Eventually, end stage liver disease necessitates liver transplantation (LT). PFIC1 patients might develop diarrhea, graft steatosis and inflammation after LT. SBD after LT was shown to be effective in the alleviation of liver steatosis and graft injury. CASE REPORT: Three PFIC1 patients received LT at the ages of two, two and a half and five years. Shortly after LT diarrhea and graft steatosis was recognized, SBD to the terminal ileum was opted to prevent risk for ascending cholangitis. After SBD, inflammation and steatosis was found to be reduced to resolved, as seen by liver biochemistry and ultrasounds. Diarrhea was reported unchanged. CONCLUSION: We present three PFIC1 cases for whom SBD to the terminal ileum successfully helped to resolve graft inflammation and steatosis.

The feasibility of routine use of distal stoma refeeding method in newborns with enterostomy.

Objective: Temporary enterostomies are life-saving in neonatal surgery, however, causing loss of fluid electrolyte, delays in distal bowel adaptation and thus delayed growth of newborns. In this study, we aimed to present the method and clinical results of distal stoma refeeding in premature and mature neonates.Methods: Between January 2012 and December 2017, records of newborn patients who underwent enterostomy and distal stoma refeeding were retrospectively analyzed. Premature and full term neonates who had distal stoma refeeding were evaluated by stoma indications, resection status, duration of total parenteral nutrition use, time of stoma closure operation and surgery findings.Results: Distal stoma refeeding was performed to 23 newborns in study period. Surgical diagnosis of newborns were meconium ileus, focal intestinal perforation, volvulus, necrotizing enterocolitis, ileal atresia, jejunal atresia, and gastroschisis. The bowel resection was performed in 14 patients. Stoma closure was done on the average day 77th. Total parenteral nutrition was given average 28 d and no total parenteral nutrition complication was seen. The stoma closure operations were performed easily.Conclusions: Distal stoma refeeding is safe and successful method. The success of distal stoma refeeding depends on expert team, time, and equipment.

Treatment of Congenital Gastric Outlet Obstruction due to a Web: A Retrospective Single-center Review.

BACKGROUND: Congenital gastric antral web and congenital pyloric atresia are rare reasons of congenital gastric outlet obstruction (CGOO). Depending on the different forms, the symptoms vary from unspecific epigastric pain to nonbilious "projectile" vomiting, ileus and failure to thrive. Throughout the years, many different treatment options are described, like simple surgical dilatation, incision or excision of the membrane, possibly combined with a pyloroplasty. PATIENTS AND METHODS: In this retrospective single-center observation study, we present 9 cases with different kinds of webs leading to CGOO. In a time period of 45 years (1970 to 2015) different treatment strategies and limitations of minimal invasive endoscopic treatment on the basis of complications and outcome are discussed. RESULTS: Six patients had an incomplete antrum web. One patient suffered from a complete antrum web. In addition, in 2 patients a complete pyloric web, as well as an incomplete pyloric web was found. During the past 45 years, the treatment options for CGOO due to webs changed, and with the development of smaller and more flexible video endoscopes an endoscopic treatment has become feasible. The treatment strategy and follow-up examination was specific to every patient. CONCLUSION: On the basis of our review, minimal endoscopic procedures in small newborns and toddlers require a high level of experience. They are technically challenging and need suitable equipment. Overall, endoscopists and pediatric surgeons should equally be prepared for all possible complications in every treatment step. The endoscopic approach to managing this unusual disease may require multiple interventions and is associated with a risk of perforation. Therefore, we are convinced that centers undertaking this treatment modality should be equipped with the proper instrumentation, prepared to perform multiple endoscopic interventions, and supported with an ability to emergently convert to surgery if required.

Retroperitoneal cystic lymphangioma: a diagnostic and surgical challenge.

A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. The incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Due to rarity, preoperative diagnosis is often difficult.

Pure esophageal atresia with normal outer appearance: case report.

Isolated esophageal atresia is characterized by a long segment between the 2 esophageal pouches. This article presents a case of pure esophageal atresia with a 1-cm-long segment at the midportion without discontinuity that resembled the subtype II3 according to the Kluth atlas. Resection of the atretic segment and primary anastomosis were performed successfully.