Esophageal Atresia Associated with Congenital Duodenal Obstruction: Turkish Esophageal Atresia Registry (TEAR) Evaluation.

INTRODUCTION: Coexistent congenital duodenal obstruction and esophageal atresia (EA) is known to have significant morbidity and mortality. Management strategies are not well-defined for this association. The data from the Turkish EA registry is evaluated. MATERIALS AND METHODS: A database search was done for the years 2015 to 2022. RESULTS: Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2,104 (± 457) g with 6 babies weighing less than 1,500 g. Twenty-six (84%) had type C EA. The duodenal obstruction was complete in 15 patients and partial in 16. Other anomalies were detected in 27 (87%) patients. VACTERL-H was present in 15 (48%), anorectal malformation in 10 (32%), a major cardiac malformation in 6 (19%), and trisomy-21 in 3 (10%). Duodenal obstruction diagnosis was delayed in 10 (32%) babies for a median of 7.5 (1-109) days. Diagnosis for esophageal pathologies was delayed in 2. Among 19 babies with a simultaneous diagnosis, 1 died without surgery, 6 underwent triple repair for tracheoesophageal fistula (TEF), EA, and duodenal obstruction, and 3 for TEF and duodenal obstruction in the same session. A staged repair was planned in the remaining 9 patients. In total, 15 (48%) patients received a gastrostomy, the indication was long-gap EA in 8. Twenty-five (77%) patients survived. The cause of mortality was sepsis (n = 3) and major cardiac malformations (n = 3). CONCLUSION: Congenital duodenal obstruction associated with EA is a complex problem. Delayed diagnosis is common. Management strategies regarding single-stage repairs or gastrostomy insertions vary notably depending on the patient characteristics and institutional preferences.

Pneumatosis cystoides intestinalis mimicking free intraabdominal air following chemotherapy for relapsed acute myeloblastic leukemia in a transplanted neutropenic child: a case report.

BACKGROUND: Pneumatosis cystoides intestinalis (PI) is a rare but important condition in which widespread air sacs are found in the submucosa, and subserosa of the bowel wall. Although it has several etiologies, children receiving chemotherapy are at risk for PI. Preferred imaging tools for the diagnosis are abdominal direct radiography and computed tomography. In patients with PI, rupture of intramural air sacs is the source of benign pneumoperitoneum, causing free air without true intestinal perforation. Intestinal perforation or obstruction are indications for surgical intervention. CASE: Here, we present a 4-year-old patient diagnosed with acute myeloblastic leukemia (AML), who underwent allogeneic hematopoietic stem cell transplantation (HSCT) from a matched sibling donor (MSD) and developed PI after HSCT. The patient was consulted to the pediatric surgery department, and her oral feeding was stopped. Broad spectrum antibiotics (teicoplanin, metronidazol and vancomycin) were initiated. Her fever increased during the 24-hour monitoring, there was no stool passage, CRP ( > 25 mg/dL, normal value < 1 mg/dL) and abdominal distension increased and there was prolonged neutropenia and radiologic investigations could not rule out intestinal perforation, so the patient underwent exploratory laparotomy. No intestinal perforation was found. There was no sign in the intestinal wall and numerous gas-filled cysts of various sizes. CONCLUSIONS: PI is an uncommon complication, and direct radiography/computed tomography scans are very helpful in making the diagnosis in suspicious cases. PI, should be kept in mind, especially in transplanted or relapsed leukemia patients receiving intensive chemotherapy.

Can 18-years of data from a tertiary referral center help to identify risk factors in esophageal atresia?

BACKGROUND: Esophageal atresia is a complex esophageal malformation with an incidence of 1 in 3,500-4,000 live births, and it usually occurs together with anomalies in other systems or chromosomes. This study aimed to investigate the short-term and long-term results of cases of esophageal atresia retrospectively in our institution and to analyze the factors affecting the outcome. METHODS: Charts of the patients managed for esophageal atresia in our tertiary pediatric surgery department were investigated retrospectively. Statistical analysis was performed to determine the risk factors for morbidity and mortality. RESULTS: One hundred and thirteen (95.8%) of 118 cases underwent a single-stage or staged esophagoesophagostomy procedure. In only five of the 40 patients with a long gap between the two atretic ends was an esophageal replacement procedure required. The most common early and late complications were anastomotic stenosis (41.6%) and gastroesophageal reflux (44.9%). In logistic regression analysis, the birthweight (OR [95% CI] = 0.998 [0.997, 0.999], P = 0.001) and preoperative inotrope requirement (OR [95% CI] = 13.8 [3.6-53.3], P < 0.001) were the two risk factors in the mortality prediction model obtained by multivariate analysis. The gap length between the two atretic ends (OR [95% CI] = 1.436 [1.010, 2.041], P = 0.044) and the number of sutures for anastomosis (OR [95% CI] = 1.313 [1.042, 1.656], P = 0.021) were the two risk factors in the gastroesophageal reflux prediction model obtained by multivariate analysis. CONCLUSIONS: Our study's early and late complication rates were like those found in other studies. Identifying risk factors would be beneficial and might help reduce the severity of potential complications in esophageal atresia patients. Prospective studies on large patient series would help develop registry-based, standardized management protocols.

Comparative analysis of reliability and clinical effects of the critical view of safety approach used in laparoscopic cholecystectomy in the pediatric population.

PURPOSE: With the increase in indications for cholecystectomy in children, the frequency of laparoscopic cholecystectomy procedures and related problems has increased. The Critical View of Safety (CVS) is the target-specific method for identifying the cystic duct during laparoscopic cholecystectomy to prevent common bile duct injury. We hypothesize that the use of CVS is reliable in the pediatric population. METHODS: Data of 91 patients under 18 years of age who underwent elective laparoscopic cholecystectomy were retrospectively analyzed. Patients were divided into two groups depending on whether CVS was applied. The descriptive characteristics and complications of the two groups were compared. RESULTS: CVS was applied to 41 patients. When the groups were compared in terms of operation time, postoperative length of stay, idiopathic gallbladder perforation, dropping stone, and presence of surgical site infection, no statistical significant difference was found. However, the time to start oral feeding was shorter in CVS group (p = 0.01). CONCLUSION: We believe CVS is a reliable method to ensure safe cystic channel identification in the pediatric population. New studies are warranted on the effectiveness of CVS for safely performing laparoscopic cholecystectomy in children, as the procedure is being performed more frequently in the pediatric population.

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.

Outcome of Very Low and Low Birth Weight Infants with Esophageal Atresia: Results of the Turkish Esophageal Atresia Registry.

INTRODUCTION: The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the outcome of very low birth weight (VLBW) and low BW (LWB) infants with esophageal atresia (EA). MATERIALS AND METHODS: The data registered by 24 centers between 2014 and 2018 were evaluated for demographic features, prenatal findings, associated anomalies, surgical treatment, and outcome. Patients were enrolled in three groups according to their BWs (VLBW <1,500 g), LWB = 1,500-2,500 g), and normal BW (NBW; >2,500 g). RESULTS: Among the 389 cases, there were 37 patients (9.5%) in the VLBW group, 165 patients (42.4%) in the LBW group, and 187 patients (48.1%) in the NBW group. Prenatal diagnosis rates were similar among the three groups (29.7, 34.5, and 24.6%, respectively). The standard primary anastomosis was achieved at a significantly higher rate in NWB cases than in the other groups (p < 0.05). In patients with tracheoesophageal fistula (TEF), patients of the NBW group had significantly higher rates of full oral feedings, when compared with VLBW and LBW cases (p < 0.05). At the end of the first year, when we evaluate all patients, the number of cases with fistula recanalization and esophageal anastomotic strictures (AS) requiring esophageal dilatation was similar among the groups. The weight and height measurements at 6 months and 1 year of age of the survivors were similar in all the groups. The overall mortality rate was significantly higher in the VLBW and LBW groups, when compared with the NBW patients, even in patients with tension-free anastomosis (p < 0.05). The incidence of the associated anomalies was 90.6% in cases with mortality, which was significantly higher than in survivors (59.6%; p < 0.05). According to Spitz's classification, the survival rate was 87.1% in class I, 55.3% in class II, and 16.7% in class III. The most common causes of mortality were associated with cardiovascular diseases, pneumonia, and sepsis. CONCLUSION: The national data of TEAR demonstrates that the developmental and feeding parameters are better in NBW patients. Although VLBW patients have higher risk of developing fistula canalization than the LBW and NBW groups, long-term complications, such as anastomotic strictures, weight, and height values, after 1 year are similar in both groups. According to our results, associated anomalies and LBWs are still significant risk factors for mortality in cases with EA.

Clues to the diagnosis of biliary atresia in neonatal cholestasis.

BACKGROUND/AIMS: The purpose of this study was to identify important clues in differentiating biliary atresia (BA) from causes of neonatal cholestasis other than BA (non-BA) and establishing the reliability of current tests. MATERIALS AND METHODS: Thirty-four patients with BA and 27 patients with non-BA cholestasis being monitored at the Çukurova University Medical Faculty, the Pediatric Gastroenterology Department and the Pediatric Surgery Department between 2009 and 2015 were retrospectively assessed. RESULTS: Cases of early onset jaundice, acholic stool, gamma-glutamyl transferase (GGT) elevation, and absent or small gallbladder on ultrasonography (USG) were greater in the BA group, while the levels of consanguinity and splenomegaly were higher in the non-BA group. The highest positive predictive value and specificity was determined for a GGT level greater than 197 in addition to absent or small gallbladder on USG and acholic stool in the BA group. Moreover, the presence of acholic stool (97%) exhibited the highest sensitivity and accuracy in the diagnosis of BA. CONCLUSION: Pale stool, GGT elevation, and absent or small gallbladder on USG are the most reliable tests for diagnosing BA. We recommend that intraoperative cholangiography should be performed without waiting for further test results when a neonate or infant presents with acholic stool, high GGT values, and absent or small gallbladder on abdominal USG.

Factors affecting survival in neonatal surgery unit in a tertiary care university hospital during 26 years.

This clinical study was designed to evaluate mortality rate and the factors that may affect survival in neonatal surgery unit. Randomly chosen 300 (ß: 0.20) patients among 1,439 patients treated in neonatal surgery unit during years 1983 to 2009, were evaluated retrospectively. The patients were separated into three groups according to date of treatment; Group A: 1983 - 1995, Group B: 1996 - 2005 and Group C: 2005 - 2009. M/F ratios did not differ between non-survived and survived patient populations. Mortality rates were 37%, 22% and 13% in Group A, B, and C respectively (p < 0.001). Parenteral nutrition, maternal age, time until admission and gestational age did not affect mortality rate, however median age of newborn was lower in non-survived cases (1 day vs. 3 days, p < 0.001). Associating abnormality, low birth weight ( < 1,500 g), associating sepsis, need of globulin and requirement of respiratory support were determinants of lower survival (p < 0.001). The mortality rate for patients that underwent thoracotomy (42%) and laparotomy (41%) were higher than patients that underwent other operations (8%) and observation (10%) (p < 0.001). Diaphragmatic hernia had higher mortality rates than the other pathologies (p < 0.001). Survival rate is increasing to date in newborn pediatric surgery unit; it is independent from parenteral nutrition, maternal age, time to admission and gestational age however it is affected adversely by the age of patient, associating abnormality, low birth weight, presence of sepsis and requirement of respiratory support. Increase in survival could be related to various additional factors such as development of delicate respiratory support machines, broad spectrum antibiotics, hospital infection control teams, central venous catheters, use of TPN by central route, volume adjustable infusion pumps, monitoring devices, neonatal surgical techniques, prenatal diagnosis of pediatric surgical conditions and developments of environmental control methods in neonatal surgical units.

Epididymal leiomyoma: an unusual intrascrotal tumor in a child.

A 13-year-old boy presented with a large painless scrotal mass and underwent radical orchiectomy with the presumptive diagnosis of testicular tumor. The diagnosis was epididymal leiomyoma (EL). The treatment of paratesticular tumors is excision of the tumor without orchiectomy whenever possible. Even when the epididymal origin of the tumor is detected during operation, it may be impossible to spare the testis because of the tumor's large size or incorporation of the testis into the tumor mass. This case is presented because of the extremely rare incidence of epididymal tumors and of EL and to emphasize the diagnosis and treatment of epididymal tumors.