Gender Identity and Assignment Recommendations in Disorders of Sex Development Patients: 20 Years' Experience and Challenges

Objective: Gender assignment in infants and children with disorders of sex development (DSD) is a stressful situation for both patient/families and medical professionals. Methods: The purpose of this study was to investigate the results of gender assignment recommendations in children with DSD in our clinic from 1999 through 2019. Results: The mean age of the 226 patients with DSD at the time of first admission were 3.05±4.70 years. 50.9% of patients were 46,XY DSD, 42.9% were 46,XX DSD and 6.2% were sex chromosome DSD. Congenital adrenal hyperplasia (majority of patients had 21-hydroxylase deficiency) was the most common etiological cause of 46,XX DSD. In 46,XX patients, 87 of 99 (89.7%) were recommended to be supported as a female, 6 as a male, and 4 were followed up. In 46,XY patients, 40 of 115 (34.8%) were recommended to be supported as a female, and 70 as male (60.9%), and 5 were followed up. In sex chromosome DSD patients, 3 of 14 were recommended to be supported as a female, 9 as a male. The greatest difficulty in making gender assignment recommendations were in the 46,XY DSD group. Conclusion: In DSD gender assignment recommendations, the etiologic diagnosis, psychiatric gender orientation, expectation of the family, phallus length and Prader stage were effective in the gender assignment in DSD cases, especially the first two criteria. It is important to share these experiences among the medical professionals who are routinely charged with this difficult task in multidisciplinary councils.

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.

Factors Affecting the Prevalence of Gastro-oesophageal Reflux in Childhood Corrosive Oesophageal Strictures.

BACKGROUND: Gastro-oesophageal reflux may accompany the corrosive oesophageal damage caused by the ingestion of corrosive substances and affect its treatment. The factors that affect the development of reflux in these cases and their effects on treatment still remain unclear. AIMS: Our aim is to investigate the prevalence of gastro-oesophageal reflux in children with corrosive oesophageal strictures, the risk factors affecting this prevalence and the effects of gastro-oesophageal reflux on treatment. STUDY DESIGN: Case-control study. METHODS: We enrolled 52 patients with oesophageal stricture due to corrosive substance ingestion who were referred to our clinic between 2003 and 2010. Groups, which were determined according to the presence of gastro-oesophageal reflux (GER), were compared with each other in terms of clinical findings, results of examination methods, characteristics of the stricture and success of the treatment. RESULTS: The total number of patients in our study was 52; 30 of them were male and 22 of them were female. The mean age of our study population was 4.2±2.88 years. Thirty-three patients had gastrooesophageal reflux (63.5%). Patients who had strictures caused by the ingestion of alkali substances were 1.6-times more likely to have reflux. There were no differences between patients with or without reflux in terms of number and localisation of strictures. Mean distance of stricture was longer in patients with reflux (3.7±1.8 cm) than in patients without (2.2±1.0 cm) (p<0.005). Only one patient among 17 who had a long stricture (≥4 cm) did not suffer from reflux. Patients with long stricture were 1.9-times more likely to have reflux. Dilatation treatment was successful in 69.6% of patients with reflux and in 78.9% of patients without. The mean treatment period was 8.41±6.1 months in patients with reflux and 8.21±8.4 months in the other group. There was no significant difference between groups in terms of frequency of dilatation and dilator diameters (p>0.05). CONCLUSION: Corrosive oesophageal stricture was usually accompanied by gastro-oesophageal reflux and the length of stricture is an important risk factor. Negative effects of reflux over dilatation treatment have not yet been demonstrated in the short-term. Nevertheless, this frequent rate of reflux may eventually increase the risk of oesophagitis and Barrett's oesophagus; therefore, we suggest that these effects should be prospectively evaluated in a large number of patients and these patients should be followed-up routinely in terms of the long-term effects of reflux.

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.

Fetal urinary ascites in a neonate without detectable obstructive uropathy or neurogenic bladder etiology.

Fetal urinary ascites is usually secondary to an obstructive uropathy or neurogenic bladder. We present such a case in the absence of these conditions, but the presence of ipsilateral vesicoureteral reflux with Hutch diverticula. The patient was a 5-day-old boy presenting with distension of the abdomen and impairment of renal function. Tests revealed urinary ascites and renal insufficiency which spontaneously resolved after transurethral urinary drainage was established. This rare complication should be considered in neonates with high intrapelviureteric and intrarenal pressure as a result of high-grade vesicoureteral reflux with paraureteral diverticula.

Giant renal hydatid cyst resembling a simple cyst: an intraoperative diagnosis.

An interesting case of a giant renal hydatid cyst is presented. The big cystic mass detected at ultrasonography (US) and computerized tomography (CT) in a ten-year-old girl looked like a simple cyst. No germinative membrane or any other radiological sign of a hydatid cyst was present. The operation was planned for a simple cyst; but was altered, after an intraoperative diagnostic needle sampling brought out the characteristic fluid of hydatid cyst. We came to the conclusion that hydatid cystic masses may not always present with their characteristic radiological findings; and that extreme caution should be practiced by the radiologist and the surgeon in order to prevent iatrogenic echinococcal dissemination.