Prenatal diagnosis of congenital megalourethra: case report and literature review.

BACKGROUND: Congenital megalourethra is an uncommon cause of lower urinary tract obstruction that is rarely prenatally diagnosed in second trimester sonographic examination as a cystic genital mass. CASE: In the presented case, the megalourethra was accompanied with bilateral mild pelviectasis. The newborn had no morbidity during follow-up period. To review the literature, electronic databases including PubMed, Web of Science and Google Scholar were searched up to February 15, 2021. In 51 prenatally diagnosed cases in the literature, most of the cases had accompanying congenital anomalies, especially structural abnormalities in the genitourinary. CONCLUSIONS: In the absence of associated abnormalities, the condition of the upper urinary tract is the main determinant of postnatal outcome. The outcome of congenital megalourethra may be good as in our case, but there may also be serious disorders such as renal failure, pulmonary hypoplasia, erectile dysfunction and fertility issues.

Can ultrasound guidance reduce radiation exposure significantly in percutaneous nephrolithotomy in pediatric patients?

In this study, we aimed to compare clinical and technical outcomes between pediatric patients who underwent percutaneous nephrolithotomy (PCNL) under fluoroscopy (FL) and those that underwent this procedure under FL with ultrasound assistance (FLUSA). The data of 66 PCNL patients were analyzed retrospectively. Renal puncture was successful in 22 patients in the FLUSA group and 44 patients in the FL group. In all cases, FL was used for tract dilation and confirmation of ureteral catheter positioning at the beginning of the procedure. The sample consisted of 46 males and 20 females with a mean age of 7.2 ± 2.1 years (range 1-17 years). Stone size varied from 8.0 to 75.4 mm, and 89% of patients achieved a completely stone-free state. The median puncture time was 130.5 ± 25.3 s for FLUSA and 295 ± 82.8 s for FL, the median fluoroscopic screening time was 95 ± 33 and 230 ± 116 s, respectively, and the median radiation dose was 19.04 ± 9.9 dGy/cm2 and 54 ± 21.4 dGy/cm2, respectively. The median puncture time, fluoroscopic screening time, and radiation dose were statistically lower in the FLUSA group (p = 0.001, Mann-Whitney U test). The greatest problem in PCNL is the use of fluoroscopy. Due to some anatomical differences from adults, applying PCNL in pediatric patients using only ultrasound may decrease the success rate. Puncture with ultrasound significantly reduces the radiation dose in children. Puncture with ultrasound and dilation under fluoroscopy is a successful and safe treatment method with low morbidity and high success rates and shorter hospital stay in pediatric patients.

A serious complication of liver hydatid cysts in children: cystobiliary fistulas.

AIM: We aimed to determine predictive factors for predicting cystobiliary fistulas (CBF) in children after treatment of liver hydatid cyst (LHD). METHODS: The records of patients who were treated for LHD between 01.06.2009 and 1.06.2019 were retrospectively reviewed. Age, sex, laboratory test results, size and number of cysts, method of first intervention (percutaneous or surgery), whether or not CBF developed and how it was treated were investigated. Among findings, those could be predictive were investigated. Data were evaluated with SPSS 21.0 program, p < 0.05 was considered significant. RESULTS: Of the 97 patients, 48 (49.5%) were male, 49 (50.5%) female, the mean age was 11.2 years, Eighty patients had right (82.5%), 13 had left, and 4 had bilobar involvement. As first intervention, surgery was performed in 39 (40.2%); percutaneous treatment was performed in 58 (59.8%) patients. In 8 patients (20.5%) in surgery group and in 6 patients (10.3%) in percutaneous group, totally in 14 patients (14.4%), CBF developed. The mean cyst diameter of CBF-developed group was 114.36 mm, and of CBF-undeveloped group was 74.30 mm. There was no statistically significant differences between groups in terms of age, sex, involved lobe, other organ involvement, and preoperative results (p > 0.5). There was a significant relationship between the cyst diameter and the rate of CBF development in both surgical and percutaneous groups (p < 0.05). ROC analysis was performed, and the cut-off value for the development of CBF detected as 69 mm for children. Since obstructive jaundice seen in adults is not common in children, an increase in liver function tests and bilirubin levels were not seen in our patients. CONCLUSION: A significant correlation was found only between the size of the cyst and developing CBF. Cysts greater than 69 mm have a higher risk of developing CBF after both percutaneous and surgical treatment and should be closely monitored.

Decision for surgery in the management of a rare condition, childhood gallbladder polyps, and the role of ultrasonography.

Gallbladder polyps are tumors or tumor-like protrusions of the gallbladder. They are rarely seen in the pediatric age. Most important issue about these mostly incidental lesions is the risk of malignant transformation. Size more than 10 mm is the classicalcutoff for determining this risk, but it is rarely valid in children. Ultrasonography is the method of choice for follow-up, but it rarely demonstrates change of size or malignant transformation. Hereby, we report 6 cases of childhood gallbladder polyps, none of which had a genetic risk factor. Follow-up was uneventful in 4 of them. Two patients had undergone surgery, but none of the lesions were neoplastic. In the follow-up, a single experienced radiologist should handle the patient, in order to prevent inter-observer variation. The cut-off size for deciding surgery should be 10 mm for those cases with genetic background creating malignancy risk (metachromatic leukodystrophy, pancreaticobiliary duct abnormalities, achondroplasia, Peutz-Jeghers syndrome) or with accompanying cholelithiasis, and 15 mm for those without any risk factors to prevent any unnecessary operations.

The evaluation of the adrenal and thyroid axes and glucose metabolism after burn injury in children.

INTRODUCTION: Burn injury is an important trauma and can cause many hormonal and metabolic changes in the human body. In contrast to the situation with adults, there are inadequate number of studies on children with this condition. Our aim in this study was to determine the changes in the adrenal, thyroid axes and glucose metabolism in the acute and subacute period and relation to the percent total body surface area (TBSA) burned in children with major burn injury. PATIENTS AND METHOD: A total of 90 hospitalized children aged 0-13 years (mean: 3.44 +/- 2.90 years) with major fluid burn injury were included in this study. Serum ACTH, cortisol, fasting glucose and insulin and thyroid hormones (free T3 and T4, TSH) were evaluated in the first 24 hours after the burn injury and on day 3 and month 3 consequently in all of the cases. An ACTH stimulation test was performed when necessary. Continuous variables were compared with analysis of variance and categorical variables were compared with the chi-square test. The Pearson correlation was used to determine the relation between ACTH and cortisol and between blood glucose and insulin (on day 1 and 3 and month 3). Also the correlation with the TBSA burned and the hormonal values were evaluated. A p value <0.05 was considered statistically significant. FINDINGS: ACTH and Cortisol level increased in first 24 hours and decreased significantly by time. The change of ACTH and Cortisol level with time was significantly related to the TBSA burned. ACTH and cortisol levels were found significantly correlated in each time-point, but the correlation was highest in 3rd month. The glucose level in the first 24 hours was significantly higher than day 3 and month 3 levels in all of the children. There was again a significant increase in the serum insulin level in the first 24 hours. However there was no difference between day 1 and 3 serum insulin levels. A correlation was found between serum glucose and insulin on the first day but not on day 3 or month 3. Free T3 and T4 levels increased by time. Pairwise comparisons indicated that 1st day free T3 and T4 level was significantly lower than both 3rd day and 3rd month levels. CONCLUSION: ACTH, Cortisol and fasting blood glucose level increased significantly however serum insulin level was slightly higher in first 24 hours after burns in children. Thyroid hormones were found to be decreased acute period following burns but staying in normal levels.

Urological problems or fecal continence during long-term follow-up of patients with anorectal malformation.

INTRODUCTION: Anorectal malformations comprise a wide spectrum of diseases. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. The aim of this study was to evaluate fecal continence together with additional urinary anomalies in patients with anorectal malformations (ARM) and determine which of them is more troublesome for life quality in long-term follow-up. MATERIALS AND METHODS: A total of 93 cases followed up and treated at our clinic between 1983 and 2009 were evaluated for urinary pathologies, fecal continence, and their quality of life (QOL). Patients were classified according to the international system (Krickenbeck), and were evaluated on the basis of voluntary bowel movement, constipation, and fecal soiling. They were also classified as good, fair, and poor regarding fecal continence. The urinary system pathologies, the medical and surgical treatments received, and the pediatric nephrology follow-up results were all evaluated. The patients were grouped by age and their QOL scored using a telephone interview. RESULTS: The mean age (+/-SD) was 8.47 +/- 4.85 (3-25) years, and the mean follow-up period was 6.96 +/- 4.55 (1-23) years. Evaluation of the patients in relation to their fecal continence revealed that 35 (37.6%) had constipation, 22 (23.6%) had fecal soiling, and voluntary bowel movements were absent in 7 (7.5%). Fecal continence was evaluated using the Krickenbeck classification, and it was good in 74 (79.6%), fair in 12 (12.9%), and poor in 7 (7.5%). QOL evaluation of these cases showed markedly decreasing QOL as the cases changed from good to poor fecal continence. Urinary system pathology was detected in 35 (37.6%) of the cases with 22 (23.7%) having severe uropathology. We compared the 22 patients with severe uropathology and 71 cases without severe uropathology for QOL and found the ARM group with severe uropathology to have significantly lower physical QOL, psychosocial QOL, and total QOL values. Reviewing the patients by fecal continence and urinary pathologies together regarding QOL showed that patients with good fecal continence and no additional urinary abnormality had the best QOL. The QOL decreased considerably in both the group with a fecal incontinence problem but no additional urinary abnormality and the group with good fecal continence but serious additional urinary abnormality. The QOL was worst in patients with urinary pathology and fecal incontinence. DISCUSSION: Anorectal malformations constitute a wide spectrum of disease. Urinary anomalies and their complications significantly increase the morbidity in these children even after the correction of the ARM. In the evaluation of the published series, we noted that the main issue was fecal continence, and there was not enough data concerning the urinary system pathologies of the patients when they reach adult ages. Anorectal dysfunction has a negative effect on QOL in ARM patients, but is not life threatening. Such patients can lead normal life although the QOL is affected. However, vesicoureteral dysfunction can cause permanent damage in other organs. This damage may cause mortality depending on the level of the malformation. Evaluating the patients considering fecal continence and urinary system pathology together showed that nearly 10% of the ARM patients had serious problems affecting their long-term QOL related to fecal control, whereas nearly a fourth of the cases had additional anomalies of the urinary system that will affect the QOL despite all treatments used, as the development of pyelonephritis, hypertension, and end-stage renal disease should be expected in these patients. CONCLUSION: Urinary system anomalies in patients with ARM are at least as serious and complex as gastrointestinal system anomalies and create more problems than fecal incontinence during long-term follow-up.