Endoscopic septum division of tubular esophageal duplication in two children and systematic review.

Tubular esophageal duplication is a rare congenital malformation The surgical treatment of this lesion can be challenging. We aimed to present our experience in two cases with tubular esophageal duplication. Both cases were endoscopically treated by a dual knife. The first patient underwent a single, while the second patient required three sessions of endoscopic septum division using an electrosurgical knife. Both showed satisfactory clinical, radiological and endoscopic response to treatment. Moreover, a systematic literature review has been performed. To identify all available studies, a detailed search on tubular esophageal duplication was performed according to the PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) guidelines. Tubular esophageal duplication was reported in 16 pediatric cases in 14 studies in the literature. Surgery was applied to 10 of these cases and endoscopic treatment was applied to two of them. In the follow-up, nine patients who underwent surgical treatment and two patients who underwent endoscopic treatment were uneventful. Tubular esophageal duplications can be successfully treated endoscopically. Endoscopic septum division is a minimally invasive procedure that allows full anatomical recovery and satisfactory therapeutic response.

The effect of azygos vein preservation on postoperative complications after esophageal atresia repair: Results from the Turkish Esophageal Atresia Registry.

AIM: Preservation of the azygos vein (AV) maintains normal venous drainage of the mediastinum and decreases postoperative congestion. The modification of esophageal atresia (EA) repair by preserving AV may prevent postoperative complications and may lead to better outcomes. The data from the Turkish Esophageal Atresia Registry (TEAR) were evaluated to define the effect of AV preservation on postoperative complications of patients with EA. METHODS: Data from TEAR for a period of five years were evaluated. Patients were enrolled into two groups according to the preservation of AV. Patients with divided (DAV) and preserved AV (PAV) were evaluated for demographic and operative features and postoperative complications for the first year of life. The DAV and PAV groups were compared according to the postoperative complications, such as fistula recanalization, symptomatic strictures, anastomotic leaks, total number of esophageal dilatations, and anti-reflux surgery. In addition, respiratory problems, which required treatment, were compared between groups. RESULTS: Among 502 registered patients; the data from 315 patients with the information of AV ligation were included. The male female ratio of DAV (n = 271) and PAV (n = 44) groups were 150:121 and 21:23, respectively (p > 0.05). The mean body weight, height, gestational age, and associated anomalies were similar in both groups (p > 0.05). The esophageal repair with thoracotomy was significantly higher in DAV group, when compared to the PAV group (p < 0.05). The rates of primary anastomosis and tensioned anastomosis were similar in both groups (p > 0.05). There was no difference between DAV and PAV groups for anastomotic leaks, symptomatic anastomotic strictures, fistula recanalization, and the requirement for anti-reflux surgery (p > 0.05). The rate of respiratory problems, which required treatment, was significantly higher in the DAV group (p < 0.05) CONCLUSION: The data in the TEAR demonstrated that preserving the AV during EA repair led to no significant advantage on postoperative complications, with exception of respiratory problems. AV should be preserved as much as possible to maintain a normal mediastinal anatomy and to avoid respiratory complications.

Adrenal masses in children: Imaging, surgical treatment and outcome.

BACKGROUND/OBJECTIVE: This study aims to evaluate the current surgical approach to adrenal masses in the pediatric age group. METHODS: We retrospectively analyzed cases that underwent surgery for adrenal masses between 2007 and 2017. Patients were assessed regarding age, sex, primary diagnosis, image defined risk factors (IDRF), surgical treatment method, complications, duration of hospital stay, and follow-up. RESULTS: We examined 50 patients who underwent surgery for adrenal mass (mean age: 4.8 years; range: 5 days-14 years). For IDRF assessment, Ultrasonography was used in 42, Computed Tomography in 36, and Magnetic Resonance Imaging in 36 patients. Lesions were present on the right in 25, left in 21, and bilateral in 4 patients. Histopathological findings were neuroblastoma (n = 29), ganglioneuroma (n = 6), adrenal cortex tumor (n = 5), ganglioneuroblastoma (n = 4), pheochromocytoma (n = 3), cyst (n = 1), and adrenal hematoma (n = 2). Laparotomy was performed on 37 patients, and laparoscopy on 13 patients. None of the cases had any operative complications. CONCLUSION: The crucial factors determining the surgical approach to adrenal masses in pediatric cases are the histopathology of the mass, volume, and IDRF. Minimally invasive procedures could be reliably performed in appropriate cases.

Congenital generalized lipodystrophy: The evaluation of clinical follow-up findings in a series of five patients with type 1 and two patients with type 4.

Congenital generalized lipodystrophy (CGL) is a rare disorder characterized by lipoatrophy affecting the face, limbs and trunk, acromegaloid features, hepatomegaly, hypertriglyceridemia, and insulin resistance. The aim of this study is to evaluate the long-term follow-up findings including gastrointestinal and cardiac manifestations of the patients with CGL1 and CGL4, caused by mutations in the AGPAT2 and CAVIN1 genes, respectively. Two patients aged 2 and 9 years with the same biallelic CAVIN1 mutation and five patients aged between 6 months and 11 years 4 months with AGPAT2 mutations have been followed up for 3-9 years. The patients were between 7 and 20 years of age at their last examination. One of the two patients with CGL4 had congenital pyloric stenosis. The other patient with CGL4 have developed recurrent duodenal perforations which have not been reported in CGL patients previously. The pathological examination of duodenal specimens revealed increased subserosal fibrous tissue and absent submucosal adipose tissue. None of the five CGL1 patients had gastrointestinal problems. Two patients with CGL4 developed hypertrophic cardiomyopathy (HCMP) and severe cardiac arrhythmia, only one patient with CGL1 had HCMP. Hyperinsulinemia was detected in one patient with CGL4 and three patients with CGL1, these three CGL1 patients also had acanthosis nigricans. Hepatic steatosis was detected in one patient with CGL4 and two patients with CGL1 by ultrasonography. In conclusion, these findings suggest that CGL4 patients should also be carefully followed up for gastrointestinal and cardiac manifestations.

Which treatment modality for pediatric pilonidal sinus: Primary repair or secondary healing?

OBJECTIVE: To evaluate the outcome and characteristics of patients who were operated for sacrococcygeal pilonidal sinus (SPS) treatment using primary repair or secondary healing technique. METHODS: Forty-seven patients (female: 25, male: 22) diagnosed with pilonidal sinus operated between 2009 and 2015 were retrospectively analyzed. The cases were evaluated for age, gender, body mass index (BMI), surgical technique, hospital stay, recovery time and recurrence. Mainly two types of surgical techniques were applied: primary repair and secondary healing. RESULTS: Mean age of the patients was 15.6 (±1.2) years. Excision and primary repair was performed in 36, excision and secondary healing was in 11. There was no statistically significant difference between the groups regarding length of hospital stay and duration of postoperative pain (p > 0.05). There was a statistically significant difference between groups regarding recovery time (p < 0.05). Recurrence was detected in a total of 11 (23.4%) patients. CONCLUSION: We did not find any significant difference concerning length of hospitalization, duration of postoperative pain and recurrence rate. However we determined that the primary repair group recovery time is significantly shorter compared to our secondary healing group. Therefore we recommended primary repair in the treatment of primary repair PS in children.

A rare cause of vomiting: annular pancreas.

Annular pancreas is a rare congenital anomaly that consists of a ring of pancreatic tissue partially or completely encircling the second part of the duodenum. It can affect anyone from neonates to adults, and is difficult to diagnose because it can present in a wide range of clinical conditions. Although cases have also been reported in adults, symptomatic cases are often referred in infancy or early childhood. A 17-year-old female patient who was diagnosed as having annular pancreas is reported. The patient had had non-bilious vomiting accompanied by abdominal pain, especially 5-10 minutes after meals, for seven years. Annular pancreas, which may be seen at any age, should be considered in the differential diagnosis of patients with non-bilious vomiting, particularly after meals, over a long period.

Management of esophageal perforation secondary to caustic esophageal injury in children.

PURPOSE: To review our management of esophageal perforation in children with caustic esophageal injury. METHOD: We reviewed the medical records of 22 children treated for esophageal perforations that occurred secondary to caustic esophageal injury. RESULTS: There were 18 boys and 4 girls (mean age, 5 years; range, 2-12 years). Three children were treated for perforation during diagnostic endoscopy and 19 were treated for a collective 21 episodes of perforation during balloon dilatation. One child died after undergoing emergency surgery for tracheoesophageal fistula and pneumoperitoneum. Another patient underwent esophagostomy and gastrostomy. Twenty patients were treated conservatively with a nasogastric tube, broad spectrum antibiotics, and tube thoracostomy, 16 of whom responded but 4 required esophagostomy and gastrostomy. Although the perforation healed in 21 patients, 20 were left with a stricture. Two children were lost to follow-up, 8 underwent colonic interposition, and 10 continued to receive periodic balloon dilatations. Two of these 10 patients underwent colonic interposition after a second perforation. The other 8 became resistant to dilatations: 4 were treated by colon interposition; 2, by resection and anastomosis; and 2, by an esophageal stent. CONCLUSIONS: Esophageal perforation can be managed conservatively. Because strictures tend to become resistant to balloon dilatation, resection and anastomosis is preferred if they are up to 1 cm in length, otherwise colonic interposition is indicated.

Intestinal rotation anomalies in childhood: review of 22 years' experience.

PURPOSE: We review our experience of treating intestinal rotation anomalies in infants and children in the 22-year period between 1978 and 2000. METHODS: The type of operation performed, postoperative complications, and mortality were compared in three age groups. Group 1 consisted of neonates <1 month old, Group 2 consisted of infants aged <1 year old, and Group 3 consisted of children aged >1 year old. RESULTS: There were 101 infants and children, with a female : male ratio of 2 : 1. Of the 101 patients, 72 (71%) were neonates, with a mean age of 11.8 days (range 1-28 days); 20 (19.8%) were under the age of 1 year, with a mean age of 6.7 months (range 1-12 months); and 9 (8.9%) were >1 year of age, with a mean age of 6 years (range 1-9 years). Eighty-five (84%) patients underwent emergency procedures. Ladd's operation was performed in all patients, with various additional procedures. The most frequent postoperative complications were adhesive intestinal obstruction, stoma necrosis, evisceration, and short bowel syndrome. The mortality rate was 36% in Group 1, 20% in Group 2, and 0% in Group 3. CONCLUSIONS: In this series surgery was usually performed as an emergency procedure, with higher morbidity and mortality in newborns than in older infants and children.