Infra-areolar subcutaneous mastectomy in the surgical treatment of adolescent gynecomastia.

BACKGROUND: Despite being a common condition in puberty, only 5-10% of pubertal gynecomastia need surgical treatment. Here the authors present their experiences with infra-areolar subcutaneous mastectomy in the surgical treatment of adolescent gynecomastia. METHODS: The records of patients who underwent infra-areolar subcutaneous mastectomy for adolescent gynecomastia between January 2004 and December 2021 were reviewed retrospectively. The patients' demographic data, complaints and clinical presentation, physical examination, laboratory and radiological findings, surgical management, and postoperative follow-ups were evaluated. The patients were evaluated according to the localization of the gynecomastia (unilateral/bilateral) and according to the patients' body mass index (BMI) (normal/overweight). RESULTS: A total of 21 boys with a mean age of 15 ± 1.4 years were operated by the senior author and infra-areolar subcutaneous mastectomy by a semilunar incision was performed for adolescent gynecomastia. The mean duration of the complaint was 24.2 ± 10.9 months. Gynecomastia was bilateral in 15 (71.5%) and unilateral in 6 (28.5%) of the patients. Sixteen patients (76.2%) were normal weight, 5 (23.8%) were overweight. Chromosomal anomaly and hypogonadism were detected in two patients. Pseudoangiomatous stromal hyperplasia was detected in the pathology of one patient. The mean follow-up time was 2.7 ± 1 years, seroma developed in 2 patients at the early postoperative period. Their long-term follow-up was uneventful without complication. CONCLUSIONS: The results of infra-areolar subcutaneous mastectomy are very promising in pubertal gynecomastia due to good skin elasticity and without obesity in the adolescent age group. We believe that this method provides an almost invisible incision and a natural pectoral appearance for adolescent patients who have experienced serious emotional problems related to their body images.

The effect of postoperative ventilation strategies on postoperative complications and outcomes in patients with esophageal atresia: Results from the Turkish Esophageal Atresia Registry.

OBJECTIVES: Postoperative ventilatory strategies in patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) may have an impact on early postoperative complications. Our national Esophageal Atresia Registry was evaluated to define a possible relationship between the type and duration of respiratory support on postoperative complications and outcome. STUDY DESIGN: Among the data registered by 31 centers between 2015 and 2021, patients with esophago-esophageal anastomosis (EEA)/tracheoesophageal fistula (TEF) were divided into two groups; invasive ventilatory support (IV) and noninvasive ventilatory support and/or oxygen support (NIV-OS). The demographic findings, gestational age, type of atresia, associated anomalies, and genetic malformations were evaluated. We compared the type of repair, gap length, chest tube insertion, follow-up times, tensioned anastomosis, postoperative complications, esophageal dilatations, respiratory problems requiring treatment after the operation, and mortality rates. RESULTS: Among 650 registered patients, 502 patients with EEA/TEF repair included the study. Four hundred and seventy of patients require IV and 32 of them had NIV-OS treatment. The IV group had lower mean birth weights and higher incidence of respiratory problems when compared to NIV-OS group. Also, NIV-OS group had significantly higher incidence of associated anomalies than IV groups. The rates of postoperative complications and mortality were not different between the IV and NIV-OS groups. CONCLUSION: We demonstrated that patients who required invasive ventilation had a higher incidence of low birth weight and respiratory morbidity. We found no relation between mode of postoperative ventilation and surgical complications. Randomized controlled trials and clinical guidelines are needed to define the best type of ventilation strategy in children with EA/TEF.

COVID-19 pandemic and lockdown: what has changed in common home accidents such as foreign bodies and corrosive injuries?

BACKGROUND: COVID-19 pandemic greatly affected our lives in all areas. Due to the social isolation policies implemented during this period, the majority of parents and all school-age children spent their lives at home. This study aims to investigate the effects of pandemic and isolation on home accidents treated in our center. METHODS: Foreign body ingestion (gastric foreign bodies: G.FB), foreign body aspiration (respiratory foreign bodies: R.FB), and corrosive substance (CS) ingestion cases admitted to our hospital between March 11, 2019, and March 10, 2021, were retrospectively analyzed. Demographic data, type and cause of home accidents, the time of the accident and the admission to the hospital, the location of the foreign body, and the follow-up data were recorded. The patients were divided into two groups: the pre-pandemic period (11 March 2019-10 March 2020) and the COVID pandemic period (11 March 2020-10 March 2021), and the data were evaluated between two groups as < 6 years old and 6-18 years old. RESULTS: During the 2 years, a total of 982 patients were admitted to our hospital for G.FB, R.FB, or CS. Four hundred and eighty-three of them (49.2%) were in the pre-pandemic period and 499 (50.8%) were in the pandemic period (p = 0.206). The mean age of the patients was 3.63 ± 3.32 years; 82.4% of the patients in the pre-pandemic group and 85.4% of the patients in the pandemic group were children < 6 years old. While the F/M ratio was 1/1.5 during the pre-pandemic period, it was 1/1.1 during the pandemic period. Of the cases, 73.3% were G.FB, 4.6% were R.FB, and 22.1% were CS. Almost half of the accidents occurred between the hours of 16 and 24. During the pandemic period, the accidents increased to occur between 0 and 8 am in children < 6 years old, and between 8 am and 4 pm in children 6-18 years old (p = 0.003). All of the home accidents in the 6-18 age group between 0 and 8 o'clock were girls (p < 0.0001). During the pandemic period, the frequency of button batteries and food products increased in G.FB. Also, the frequency of R.FB increased significantly (p = 0.006) and the most common R.FB was the food products. The frequency of CS increased in girls during the pandemic period, and CSs were brought to the hospital in a shorter time after the accident during the pandemic period (p = 0.007). CONCLUSIONS: It can be thought that the main reason why home accidents are common in the 0-6 age group is due to the developmental characteristics of the child rather than the longer time spent at home. The pandemic and isolation increase the frequency of foreign body aspirations and home accidents in girls.

The effects of hesperidin on stricture formation in corrosive esophageal burns: an experimental study.

AIM/BACKGROUND: Esophageal strictures in children that develop as a result of accidental ingestion of corrosive substances remain an important health problem. The purpose of this study is to determine the effects of Hesperidin, an effective bioflavonoid in the proliferative and exudative phase of inflammation, on the stricture formation in corrosive esophageal burns. METHODS: Experimental esophageal burns in rats were created using a modified Gehanno and Guedon model with 20% NaOH. Rats were divided into 5 groups. In the Sham group, the distal esophagus was prepared and cannulated according to the model, but no NaOH was administrated. The esophageal burn was created with NaOH in the other groups. The burned groups were divided into two groups as untreated (T14, T21) and treated with 100 mg/kg/day Hesperidin (H14, H21) intraperitoneally, and these groups were divided into two according to their sacrification periods (14 and 21 days). Inflammation, fibrosis, and necrosis were graded by histopathological evaluation in all groups. The efficacy of treatment was evaluated using the weight of rats, stenosis index, and histopathological parameters. RESULTS: Histopathologic damage scores such as inflammation, necrosis, and fibrosis were lower in the H14 and H21 groups and higher in the T14 and T21 groups. And also stenosis index was found higher in T14 and T21 groups (p < 0.05), while it was similar to the Sham group in H14 and H21 groups. No statistically significant difference was found between the H14 and H21 groups in terms of stenosis index. When weights of the rats at the beginning and end of the experiment were compared, weights of the H14 and H21 groups and the Sham group were similar. There was a significant decrease in the weight of the rats in the T14 and T21 groups (p < 0.001). CONCLUSIONS: This study is the first to use Hesperidin in preventing esophageal damage in an esophageal caustic burn model. It was shown that Hesperidin was effective in reducing macroscopic and microscopic histopathologic damage in the corrosive esophageal burn model, preventing the stricture formation, and has positive effects on nutrition in rats with an esophageal burn.

Comparison of Open Repair and Laparoscopic Percutaneous Internal Ring Suturing Method in Repairing Inguinal Hernia in Children.

Introduction An inguinal indirect hernia is one of the most frequent surgical conditions in children. In this study the experience with laparoscopic percutaneous internal ring suturing (PIRS) and open inguinal hernia surgery and their relations evaluated. Methods All children over 90 days of age and without having prior inguinal region surgery with a diagnosis of indirect inguinal hernia underwent surgical repair with open or laparoscopic PIRS technique. Patients' gender, age at surgery, inguinal hernias side, surgery duration, recurrence, complications, and follow-ups were collected. Results A total of 488 inguinal hernias of 405 patients were repaired. The diagnoses were unilateral inguinal hernia in 360 (88.9%) and it was bilateral in 33 (8.1%) patients. The operative technique was laparoscopic PIRS for 227 and open inguinal hernia surgery for 178 patients. In the PIRS group, a contralateral hernia was found in 48 of 205 children (23.4%). The surgery times were 23.3 (PIRS) and 33.7 (open) min for unilateral and 28 (PIRS) and 53.1 (open) min on average for bilateral inguinal hernia surgery. Mean follow-up was 30.4 months for PIRS and 24.4 months for open technique. Recurrence was observed in seven (3%) patients in PIRS and one (0.5%) in the open group and postoperative complications in three (1.3%) in PIRS and four (2.2%) in the open group. Conclusion PIRS method has the advantage to evaluate contralateral hernia at the same session, minimal scar related to surgery, and preserve the spermatic cord from manipulation. PIRS is an alternative feasible method and easy to perform to repair the inguinal hernia with/without communicating hydrocele in children.

Food Safety When Eating Out-Perspectives of Young Adult Consumers in Poland and Turkey-A Pilot Study.

Food safety is perceived differently by consumers in different countries. The objective of this study was to examine the experience of young adults regarding the safety of meals eaten outside the home in Poland and Turkey. Questionnaire surveys were conducted on a group of 400 young adults. The findings provided new insights into cross-cultural consumer perceptions of the food safety of meals eaten out. Differences in the perception of the safety of the meals eaten out concerned both the manner in which consumers chose an eating establishment, the frequency with which they ate out, their experience of the meals consumed, and their practice of lodging complaints. Consumers in Poland and Turkey experienced different problems with the health quality of meals eaten out. The experience of consumers in Turkey reflected the occurrence of numerous cases of meals of poor quality, while in Poland it was smaller. This suggests that meals eaten out in Poland (an EU country) may have a lower health risk than in Turkey (a non-EU country). The method described in this study could be an additional tool for checking the operation of food safety systems in eating out establishments.

Comparison of laparoscopic percutaneous internal ring suturing method and open inguinal hernia repair in children under 3 months of age.

OBJECTIVES: Laparoscopic inguinal hernia repair in younger infants has not been completely accepted worldwide. The aim of this study was to evaluate the safety and feasiblity of laparoscopic percutaneous internal ring suturing method in children aged younger than 3 months and compare the recurrence and complication rates with open repair; which may still be mentioned as the gold standard procedure. MATERIAL AND METHODS: A total of 387 children underwent inguinal hernia repair in the clinic between 2016 and 2019. One hundred and forty of them were under 3 months old and divided into two groups; children who underwent laparoscopic percutaneous internal ring suturing (Group 1) and open surgery (Group 2). Selection of the surgical method was regardless of weight, sex or any patient characteristics other than surgeon's choice. Operation durations, complications and recurrences were compared between the two groups. RESULTS: A total of 140 patients underwent surgery due to inguinal hernia. Group 1 included 85 and Group 2 included 55 children. There were two recurrences in each group (p> 0.05). Operative durations were shorter in Group 1 for both; unilateral and bilateral repairs (p <0.0001). There were no intraoperative complications in any group. There was one major postoperative complication in Group 2: iatrogenic undescended testis, and none was observed in Group 1. In the laparoscopic group, 47% of the children who were diagnosed to have unilateral hernia were revealed to have bilateral inguinal hernias (n= 31). CONCLUSION: Laparoscopic percutaneous internal ring suturing method seems favourable in terms of operative time. It also has the advantage of detecting contralateral patent processus vaginalis or asymptomatic contralateral inguinal hernia.

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.

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.

Sentinel nubbin: A potential pitfall in the management of undescended testis secondary to epididymo-testicular nonunion.

INTRODUCTION: Epididymal anomalies are common in boys with undescended testis. Epididymo-testicular non-union with a nubbin in the scrotum is a rare anomaly and may be confusing during management of cryptorchidism. The non-fused epididymis and vas deferens which are descended to the scrotum, may be regarded as nubbin tissue secondary to testicular atrophy and proximal testis may be overlooked. OBJECTIVE: To evaluate a relatively rare anomaly of epididymo-testicular non-union in which vas deferens and epididymis are descended to the scrotum resembling a nubbin tissue, while a separate testis remains in a proximal peritoneal fold (hernia or processus vaginalis)which could potentially be missed during the management of patients with undescended testis. STUDY DESIGN: The charts of the fourteen patients with epididymo-testicular non-union and a scrotal nubbin are retrospectively evaluated. RESULTS: Median age of the patiens was 1.5 years (0.5-12), 11 were left and 3 were right. No connection could be demonstrated between the testis and vaso-epididymal unit in any of the patients. Orchidopexy was performed in 13 patients while orchidectomy in1 patient. The scrotal nubbin tissue is excised in 4 patients and histological examination revealed sections of vas deferens and epididymis. One of the patients underwent inguinal exploration and orchidectomy for assumed testicular atrophy elsewhere but histological examination revealed vas deferens and epididymis without any sign suggesting an atrophic testis. An intrabdominal testis was found incidentally in this patient and he underwent 2 additional procedures for orchidopexy. DISCUSSION: Although epididymo-testicular anomalies are common in boys with undescended testis, nonunion is the rarest type of this anomaly. Similar cases are reported in the literature as simple fusion anomalies with indefinite clinical significance or even polyorchidism although no accessory testis could be demonstrated. However, the scrotal nubbin tissue which actually contains epididymis and vas is a potential decoy and may result in missing of the proximal testis if regarded as a sign of testicular atrophy and further evaluation is obviated. As this is retrospective study, actual incidence of the condition is yet to be determined. CONCLUSION: A scrotal nubbin tissue may be secondary to epididymo-testicular nonunion as well as testicular atrophy. As feasibility of radiological imaging studies are limited, a laparoscopic exploration may be considered to confirm or to exclude a separate testis in patients with scrotal nubbin. Prospective studies are needed to evaluate the actual incidence of the condition and gain-loss analysis of laparoscopic exploration.

Management of perinatal testicular torsion: experience of two tertiary centers.

PURPOSE: Perinatal testicular torsion is a rare condition in which testicular salvage rates are significantly low and management is controversial. We retrospectively evaluated our patients and aimed to go through our management approach. METHODS: The newborn patients who underwent surgery for testicular torsion in two tertiary referral centers between 2000 and 2019 are enrolled. Radiological and clinical findings are retrospectively evaluated. RESULTS: Thirty-two newborns are enrolled in the study. All of the cases were unilateral and 30 ended up with immediate orchidectomy while 2 received orchidopexy but both underwent atrophy on follow-up. No bilateral torsion is demonstrated neither in 16 patients with bilateral exploration nor in the follow up of the rest 16 with unilateral exploration. Intravaginal torsion was encountered in five patients (16%). No morbidity or mortality related to anesthesia is documented. No malignancy is encountered in any of the patients. CONCLUSION: Although ipsilateral testicular salvage rates are low, urgent bilateral exploration in a multidisciplinary center seems reasonable to give a chance to the ipsilateral testis and to avoid the rare but catastrophic bilateral torsion, after discussing with the family.

Decreased second to fourth digit ratios in female multiple sclerosis patients.

BACKGROUND: Sex-related differences in multiple sclerosis (MS) suggest a possible role of prenatal sex hormones in the pathogenesis. The aim of this study was to investigate whether the 2D:4D ratio, considered a predictor of prenatal hormonal exposure, in MS patients differ from that in healthy controls. METHODS: Two hundred MS patients and one hundred seventy healthy control subjects with similar age and sex distribution enrolled. All participants were right-handed. The right hands of all participants were scanned using a digital scanner. Measurements of second and fourth digit lengths were made from digital scans. The 2D:4D ratio was calculated by dividing the length of the second digit by the length of the fourth digit. RESULTS: A total of 171 MS patients' and 159 healthy controls' digit scans included in the study. The MS group consisted of 94 females (mean age 38.32 ± 10.5) and 77 males (mean age 42.06 ± 11.8) and the control group of 86 females (mean age 40.24 ± 9.7) and 73 males (mean age 38.49 ± 11.6). 2D:4D ratios of female MS patients were significantly lower than those of healthy females (p=0.004). Although 2D:4D ratios of male patients with MS were lower than those of healthy males, this difference was not statistically significant (p=0.33). There was no significant correlation between the 2D:4D ratio, EDSS levels or duration of the disease in male or female MS patients (p<0.05). CONCLUSION: Although our results suggest that a prenatal hormonal balance in favor of androgenic activity may be a risk factor for MS, complex factors mediating the actions of sex hormones on target cells should always be considered when evaluating the effects of sex hormones.

Non-Targeted Authentication Approach for Extra Virgin Olive Oil.

The aim of this study is to develop a non-targeted approach for the authentication of extra virgin olive oil (EVOO) using vibrational spectroscopy signatures combined with pattern recognition analysis. Olive oil samples (n = 151) were grouped as EVOO, virgin olive oil (VOO)/olive oil (OO), and EVOO adulterated with vegetable oils. Spectral data was collected using a compact benchtop Raman (1064 nm) and a portable ATR-IR (5-reflections) units. Oils were characterized by their fatty acid profile, free fatty acids (FFA), peroxide value (PV), pyropheophytins (PPP), and total polar compounds (TPC) through the official methods. The soft independent model of class analogy analysis using ATR-IR spectra showed excellent sensitivity (100%) and specificity (89%) for detection of EVOO. Both techniques identified EVOO adulteration with vegetable oils, but Raman showed limited resolution detecting VOO/OO tampering. Partial least squares regression models showed excellent correlation (Rval ≥ 0.92) with reference tests and standard errors of prediction that would allow for quality control applications.

Characteristics, diagnosis, and treatment modality of pediatric patients with cystic echinococcosis: a single centre experience.

Kaman A, Tanir G, Çakmakçi E, Demir P, Öz FN, Aydin Teke T, Metin Ö, Gayretli Aydin ZG, Karaman A. Characteristics, diagnosis, and treatment modality of pediatric patients with cystic echinococcosis: a single centre experience. Turk J Pediatr 2019; 61: 704-713. Cystic echinococcosis (CE) is among the most common zoonotic infections worldwide. Studies about CE are limited in childhood. The aim of this study was to evaluate clinical, radiological and laboratory characteristics of childhood CE at a tertiary care pediatric hospital. Medical records of children with CE were analyzed between January 2005 and January 2015. A total of 130 patients with a median age of 10.4 years (IQR= 7.2-years-13.2 years) were evaluated. The anatomic locations of cysts were as follows; liver (76.9%), lung (36.9%), spleen (6.2%), pelvic region (3.8%) and kidney (2.3%). The most common symptoms were abdominal pain and cough in the patients with liver cysts and lung cysts, respectively. The indirect hemagglutination (IHA) test positivity was 58%. Elevated serum total immunoglobulin E levels were detected in 59% of the patients. Fourty-four patients with liver CE, 33 patients with lung CE were treated surgically and 23 patients with liver CE were treated with percutaneous aspiration, injection and re-aspiration (PAIR) along with medical treatment. The recurrence was observed in five patients with liver CE. It was demonstrated that CE mainly involves liver but lung cysts are more frequently symptomatic and prone to be complicated than liver cysts in children. IHA test positivity together with abdominal ultrasonography are useful to diagnose liver CE but thorax CT is usually needed to diagnose lung CE. Liver cysts that are sized greater than 5 cm are more frequently treated with PAIR or surgery but smaller liver cysts can be treated medically.

Is color Doppler a reliable method for the diagnosis of malrotation?

PURPOSE: The gold standard for the diagnosis of malrotation is barium contrast study of the upper gastrointestinal system (UGCS), while color Doppler ultrasonography (CDUS) is another method used in the diagnosis. We investigated the value of CDUS for the diagnosis of malrotation in this study. METHODS: UGCS images, CDUS images, plain abdominal images, demographic data, and symptoms of 82 patients who were investigated for presumed malrotation during a 7-year period were evaluated, retrospectively. RESULTS: All patients underwent CDUS, and 18% of these patients were diagnosed with malrotation as the superior mesenteric vein was seen to be on the left of the superior mesenteric artery. We found that 16% of the 75 patients who underwent UGCS were diagnosed with malrotation. The sensitivity and specificity of CDUS in the diagnosis of malrotation was found to be 93.8 and 100%, respectively. The respective values for UGCS were 91.7 and 98.4%. CONCLUSION: Current data in the literature and our results underline that UGCS may yield false-positive and false-negative results. Although CDUS was found to be a reliable method for the diagnosis of malrotation in our study, the limitations of UGCS are also recognized for CDUS. Prospective studies are needed to determine the more valuable method.

Vaginal bleeding and a giant ovarian cyst in an infant with 21-hydroxylase deficiency.

BACKGROUND: Increased adrenal androgen hormones in congenital adrenal hyperplasia (CAH) can rarely cause giant ovarian cysts in the neonatal period. Although the exact mechanism of the development of ovarian cysts is unknown, it is thought that increased androgen levels stimulate folicle development by increasing follicle stimulating hormone (FSH) levels. CASE PRESENTATION: A 16-day-old newborn with ambiguous genitalia was presented to our clinic. Laboratory test results were as follows: sodium: 126 mEq/L, potassium: 5.4 mEq/L, renin: 132 pg/mL, adrenocorticotropic hormone (ACTH): 207 pg/mL, cortisole: 7.8 µg/dL, basal 17OH progesterone: 21 ng/mL, androstenedione: 5.1 ng/mL, testosterone: 1188 ng/dL and dehydroepiandrosterone sulfate (DHEAS)>1500 µg/dL. Karyotype analysis resulted in 46,XX. A homozygous mutation of R356W was detected in the CYP21A2 gene. The classical severe form of salt wasting 21 hydroxylase deficiency was diagnosed and treatment was started with hydrocortisone and fludrocortisone. Good metabolic control was ensured by monthly visits but the baby presented with vaginal bleeding as soiling at 4 months. The cystic lesion which extended to the epigastric area from the pelvis in the midline abdomen, had a size of 90×80×60 mm and medially, thin ovarian parenchyma was detected in ultrasonography. CONCLUSIONS: The findings in our patient suggest that a decline in adrenal androgens after glucocorticoid treatment resulted in an increase in gonadotropin levels and the giant cyst is developed by activation of gonadotropin cascade and increased gonadotropin receptors, instead of androgens.

Isolated thoracoschisis: Case report.

Ardiçli B, Karaman A, Özyazici A, Zenciroglu A, Okumus N. Isolated thoracoschisis: Case report. Turk J Pediatr 2017; 59: 217-220. Thoracoschisis is a rare congenital anomaly that refers to a congenital fissure of the chest wall. It is frequently accompanied with other congenital defects of the limbs and the abdominal wall as part of the limb-body wall complex, which is exencephaly/encephalocele and facial clefts, thoracoschisis and/or abdominoschisis and limb defects. Isolated thoracoschisis is a rare entity. We present a case of isolated thoracoschisis. A 24-week gestational age boy presented with a 3 cm chest wall defect in the left lateral 10th intercostal space and intestines herniating through the defect. There was no history of maternal drug use during pregnancy. Birth weight was 500 g. He underwent surgery. The intestines were reduced via the thoracic wall defect.

Tuba-ovarian auto-amputation caused by ovarian teratoma in an adolescent girl.

Atici A, Yilmaz E, Karaman A, Apaydin S, Afsarlar ÇE. Tuba-ovarian auto-amputation caused by ovarian teratoma in an adolescent girl. Turk J Pediatr 2017; 59: 90-92. Ovarian auto-amputation is an extremely rare condition commonly encountered in the perinatal period. Spontaneous or secondary torsion of the ovary caused by an ovarian lesion may result in infarction and subsequent auto-amputation of the ovary. This paper demonstrates a case that underwent laparoscopic appendectomy with an incidental calcified auto-amputated right ovary. A 16-year-old adolescent girl was admitted to our department with a history of one-day abdominal pain. Physical examination of the patient revealed abdominal tenderness and rigidity on right lower quadrant. Her white blood cell count was 11x103/mL, and C-reactive protein was 69 mg/L. The patient underwent a laparoscopic appendectomy with a provisional diagnosis of acute appendicitis, and further exploration revealed a 2x2 cm white ovoid mass floating freely in the pelvis. The left ovary was clearly identified in its usual localization, but the right tuba was blindly ending without any fimbria or ovary. Postoperative course of the patient was uneventful, and she was discharged on postoperative day 2. The histopathological examination revealed a necrotic calcified ovarian teratoma. Auto-amputated ovary is a rare occasion mostly encountered during perinatal period, and it may be unilateral or bilateral. An auto-amputated ovarian mass may rarely be a teratoma although the most common cause of auto-amputation during perinatal and adolescent period is ovarian torsion due to an ovarian cyst.

Gonadoblastoma in a patient with 46, XY complete gonadal dysgenesis.

46, XY complete gonadal dysgenesis (Swyer syndrome) is a rare cause of 46, XY sexual development disorder. The patient presented to our clinic with absence of breast development and lack of periods at the age of 17 years. Her history and familial history involved no relevant conditions. She had Tanner stage 1 thelarche, and Tanner stage 2 pubic hair development with no axillary hair development. External genital structure appearance was consistent with female phenotype and the patient had no palpable gonad. The patient diagnosed as 46, XY complete gonadal dysgenesis after evaluation of laboratory analyses, radiological methods and karyotype. The Sexual Orientation and Gender Identity Committee concluded that gonadectomy should be performed. Histopathologic analysis demonstrated gonadoblastoma. Gonad structures should be sought laparoscopically and once diagnosed, streak gonads should be removed prophylactically in patients with 46, XY complete gonadal dysgenesis.

Complete androgen insensitivity syndrome associated with bilateral sertoli cell adenomas and unilateral paratesticular leiomyoma: A case report.

Complete androgen insensitivity syndrome (CAIS) disorder of sex development due to mutations that cause function loss in androgen receptors in 46, XY individuals. The risk of malignancy in CAIS is 5-10% until the age of 25 years. A 17-year-old patient raised as a female presented to our clinic complaining of amenorrhea. She had a history of surgery for inguinal hernia at the age of 2 years. The patient's niece of the same age had been diagnosed with CAIS at our department and gonadectomy had been performed six months ago. She had four other nieces with the same diagnosis. Her external genital appeared phenotypically female. On physical examination, breast development was Tanner stage 5 and pubic hair Tanner stage 2 with scarce axillary hair. The gonad was palpated in the left inguinal region. Chromosome analysis revealed 46, XY and sex determining region Y (SRY) was positive. The patient was diagnosed as CAIS with laboratory and radiology results. The Sexual Orientation and Gender Identity Committee decided on gonadectomy. Histopathological evaluation of the gonad revealed bilateral Sertoli cell tumor and right paratesticular leiomyoma.