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.

Laparoscopy-Assisted Percutaneous Cholangiography in Biliary Atresia Diagnosis: Comparison with Open Technique.

Introduction. Biliary atresia is a surgical cause of prolonged jaundice, which needs to be diagnosed with cholangiography that has traditionally been performed via laparotomy. Laparoscopic assistance has lately been introduced to avoid unnecessary laparotomy. We aim to evaluate the benefits of the laparoscopy-assisted cholangiography and compare it to the traditional procedure via laparotomy. Patients and Method. The medical records of the cases who had undergone cholangiography for prolonged jaundice between 2007 and 2014 were analyzed. The patients were grouped according to cholangiography technique (laparotomy/laparoscopy). The laparoscopy and laparotomy groups with patent bile ducts were focused and compared in terms of operation duration, postoperative initiation time of enteral feeding, and full enteral feeding achievement time. Results. Sixty-one infants with prolonged jaundice were evaluated between 2007 and 2014. Among the patients with patent bile ducts, operation duration, postoperative enteral feeding initiation time, and the time to achieve full enteral feeding were shorter in laparoscopy group. Conclusion. Laparoscopic cholangiography is safe and less time-consuming compared to laparotomy, with less postoperative burden. As early age of operation is a very important prognostic factor, laparoscopic evaluation should be an early option in work-up of the infants with prolonged jaundice with direct hyperbilirubinemia, for diagnosis/exclusion of biliary atresia.

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.

A case of uneventful ABO-incompatible liver transplantation from a deceased donor managed with routine immunosuppressive treatment.

ABO-incompatible liver transplantation (ILT) was formerly contraindicated because of the increased risk of antibody-mediated humoral graft rejection due to preformed anti-A/-B antibodies on recipient endothelial cells. A 2.5-year-old girl with end-stage liver disease underwent cadaveric donation ILT because of acute liver failure and esophageal variceal bleeding before transplantation. The patient's blood type was A Rh (-) and the donor's blood type B Rh (+). The operation and postoperative course were uneventful. The immunosuppression consisted of steroids, and tacrolimus was initiated on the day of the surgery. The patient's hemoglobin level did not change, and direct Coombs test performed daily was consistently negative. Anti-B titer was observed at a maximum of 1/8. The patient was followed up during the first year. This case of ILT from a cadaveric donor is significant because the 2.5-year-old recipient did not experience any complications after undergoing routine immunosuppressive treatment.

Renal sparing surgery using focus ultracision harmonic scalpel in patients with bilateral wilms' tumor: case report.

BACKGROUND: Bilateral Wilms' tumor is rare and surgical treatment requires an individual approach. Surgical approach to the tumors located in the central part of the kidney represents a major challenge and nephrectomy is usually essential. Renal sparing surgery is difficult in such cases. CASE REPORT: We describe a 3 year-old female patient with bilateral Wilms' tumor arising in the central localisation of the right and foci in the left kidney enucleated successfully with a simple and comfortable renal preserving operative technique using Focus Ultracision Harmonic Scalpel. CONCLUSION: In cases when tumors are located in the central part of the kidney and partial nephrectomy is consequently very difficult, the use of a Focus Ultracision Harmonic Scalpel provides easy dissecting, enucleation, and excision of the tumor.

Severe abdominal trauma involving bicycle handlebars in children.

OBJECTIVES: To emphasize the severity of the underlying injury which may not be realized during the initial patient admission to the emergency department. METHODS: A retrospective case note review of children admitted to our institution with the severe abdominal injury. RESULTS: Eight children were identified with the severe abdominal injury secondary to the trauma from a bicycle handlebar that needed special care in the intensive care unit. All injuries were due to blunt trauma. The mean delay from the time of the accident to the time of presentation was 34.5 hours. All patients had an imprint of the handlebar edge on the hypochondrium. There were 3 pancreatic lacerations, 1 duodenal laceration, 1 jejunal laceration, 1 liver laceration, 1 abdominoinguinal laceration that all required open surgery, and 1 duodenal hematoma that resolved in 4 weeks follow-up period. The patients who required open surgery were evaluated with computed tomographic scans before surgery. CONCLUSIONS: Children with an imprint made by the handlebar edge on the abdominal wall or give a clear history of injuries by a bicycle handlebar should be treated with great care. Early computed tomography evaluation may help to reduce the morbidity resulting from the delay in diagnosis of injuries to the internal organs.

Testicular adrenal rest hyperplasia due to 21-hydroxylase deficiency: a case report.

Bilateral testicular tumors are a rare complication of congenital adrenal hyperplasia. It can be extremely difficult to distinguish histologically between Leydig cell tumors and adrenocortical rest hyperplasia, which may lead in some cases to unnecessary orchidectomy. A 7-yr-old boy was admitted because of precocious puberty and enlargement of testicles. Hormonal studies established the diagnosis of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Testicular biopsy revealed interlacing strands, cords, and rests of cells resembling interstitial (Leydig) cells but with no Reinke crystalloids. Here we report a case of testicular adrenal rest hyperplasia in congenital adrenal hyperplasia and discuss the pathological and clinical features and origin of this rare lesion by using immunohistochemical evaluation.

Hairy polyp of the tongue: a case report.

Hairy polyps or dermoids of the oro- and nasopharynx are benign lesions containing elements of both ectodermal and mesodermal origin. Because of its rarity, we report a case of hairy polyp arising from the tongue in a 40-day-old infant. The lesion was covered by squamous epithelium and a central core of fibroadipose tissue, minor salivary glands, and cartilage. We discuss the clinicopathological features, terminology, etiology, and differential diagnosis of this condition.