Congenital vesicovaginal fistula (VVF) with distal vaginal agenesis (DVA) is an extremely rare anomaly and isn’t easy to diagnose. To detect VVF in DVA, waiting until adolescence for the operation and performing cystoscopy during the menstrual period will ensure a correct diagnosis. Here, a girl with this anomaly is presented.

In this study, we aimed to present the imaging and surgical results of our pediatric UPJO patients and to discuss our findings in the context of the current literature. All medical records of children who underwent open pyeloplasty due to UPJO were reviewed retrospectively. Obtained data included patient demographics, clinical symptoms, ultrasonographic and scintigraphic imaging, intraoperative details, length of hospital stay and complications. 114 patients underwent 124 open dismembered pyeloplasties for UPJO. Anderson-Hynes dismembered pyeloplasty technique was used except Y–V plasty in a recurrent patient. Significant changes in mean hydronephrosis degree, pelvic anterior–posterior diameter and parenchymal thickness were observed. The mean preoperative ipsilateral differential renal function was 37

Simple renal cysts are not commonly found in children. They occur in a small percentage of cases, with an incidence of 0.2%-0.5%. These cysts are typically solitary and develop in the renal cortex. Pain, infection, hematuria, hypertension, or obstruction of the collecting system are indications for treatment. When intervention is necessary, there are several ways to reduce cyst volume. In this report, we discuss the pediatric case of a solitary giant renal cyst and its therapeutic approach. We present a symptomatic pediatric renal cyst patient treated with a sclerosing agent. A simple renal cyst is a rare condition in children, and its treatment includes conservative management, percutaneous sclerosing agent injection, and surgical approach. Choosing the appropriate treatment according to the patient's condition and clinical symptoms is essential. We think that sclerotherapy should be the first-line therapy before surgery in symptomatic simple renal cysts.

555 Chylous ascites is a rare condition in children characterized by leakage of triglycerides and low density lipoprotein (LDL)-rich lymphatic fluid into the peritoneal cavity. Primary chylous disorders are most frequently caused by congenital lymphatic dysplasias or lymphatic malformations. In the treatment strategy, it is essential to first drain the fluid with recurrent paracentesis, stop enteral nutrition, and start parenteral nutrition and then continue the nutrition with foods containing medium-chain fatty acids.1,2 CA-125 is a high-molecularweight glycoprotein, originating from the coelomic epithelium. It is elevated in the majority of patients with epithelial ovarian cancer. It has also been shown to be elevated in lymphoma, leukemia, and gastrointestinal system malignancies.3-5 In this paper, it was aimed to present a child with isolated chylous ascites and CA-125 elevation without any malignancy or additional disease.

Aim: Testicular tumors are rare in children and have a bimodal distribution. The first peak is at two years of age in boys and comprises mainly non-GCNIS derived tumors (pre-pubertal teratoma and yolk sac lesions). Here, the clinical features and treatment of testicular tumors in prepubertal children in our center are presented. Materials and Methods: The clinical records of those patients treated for testicular tumors younger than 18 years in our institution from January, 2006 to June, 2022 were reviewed retrospectively. Results: A total of 12 patients were included in this study. All of the patients were younger than 3 years, except for one patient, who was 8 years old. The median age at primary diagnosis was 17 months (1-107 months). The most common clinical presentation was testicular swelling (n=9). Three patients were diagnosed incidentally. Serum & alpha;-fetoprotein (AFP) was increased in 3 patients with yolk sac tumors (>1,000 ng/dL) and in one patient with mature cystic teratoma (1 month-old infant with an AFP concentration of 629 ng/dL). Preoperative & beta;-hCG levels were normal in all patients (<1.2 mIU/mL). Of the 11 patients whose preoperative scrotal ultrasound could be obtained, 5 solid-cystic lesions, 3 cystic lesions, and 2 solid lesions were reported. Calcification was detected in 4 patients. All 4 patients with malignant tumors and 3 patients with benign tumors underwent radical inguinal orchiectomy. Of the 5 tumors removed by testis preserving surgery, 2 were mature teratomas, 2 were epidermoid cyst and 1 was a benign multi-cystic lesion. There was a patient with yolk sac tumor who died in the fifth month postoperatively while receiving chemotherapy. The remaining patients had no metastatic or local primary testicular tumor recurrence during a mean follow-up of 92 months (2-198 months). Conclusion: Most pre-pubertal tumors are benign and testicular sparing surgery can be performed in patients with negative serum tumor markers. Inguinal radical orchiectomy is sufficient in the treatment of yolk sac tumor.

Background:Laryngotracheobronchial anomalies in children are rare, and their clinical manifestations are diverse. In this study, we report the clinical aspects and prevalence rates of tracheal bronchus (TB) and congenital subglottic stenosis (CSS) in a select group of patients at our institution and briefly review and discuss the literature to draw attention to these rare anomalies. Case series:We retrospectively reviewed the clinical records of 249 children for whom senior surgeons performed rigid bronchoscopy for suspected or confirmed food choking or foreign body aspiration in children between August 2013 and January 2020. Bronchoscopic findings and demographics of the patients with laryngotracheobronchial anomalies were documented. Four among the 249 patients (1.6 %) had right-sided TB (two males aged 24 and 42 months and two females aged 14 and 60 months), while three (1.2 %) had grade 1 CSS (one male aged eight months and two females aged 11 and 13 months). There was no previous history of endotracheal intubation for any subglottic stenosis (SS), though all three patients were admitted with sudden onset of respiratory difficulty. One of the TB cases had congenital cardiac anomalies, and two were symptomatic before their admission to the emergency department. Conclusions:The prevalence of CSS and TB in the healthy population is low. Physicians dealing with the pediatric airways should consider such anomalies for prompt diagnosis, proper instrumentation, management, and follow-up of these cases. Our data also correlate with previous data, indicating that these anomalies' prevalence rates have not increased during the last few decades. HIPPOKRATIA 2023, 27(2):59-63.

Objective: Laparoscopy has been accepted as the best diagnostic tool and suggested as the treatment of choice for non palpable testes cases. However; its use in unilateral non palpable testis cases has been previously debated. Methods: The clinical records of the non-palpable testis cases that were managed with laparoscopy between January 2011 and December 2013 were retrospectively reviewed. Results: Laparoscopy was performed in 29 non-palpable testis cases. The cases were divided into three groups according to the laparoscopic findings. Orchiopexy was performed in cases with viable testes, and the internal inguinal ring was left open in these cases. Conclusion: Laparoscopy provided definitive diagnosis and was helpful in the treatment of unilateral, non-palpable testis cases. Leaving the internal inguinal ring open did not result in subsequent indirect inguinal hernia in our cases.

Arteriovenous malformation (AVM) of urinary bladder is a very rare condition in which a section of blood vessels lacks capillary vessels resulting in blood from an artery being delivered directly to a vein. We report a rare case of AVM of the bladder wall mimicking a bladder tumour presenting with acute abdomen.

Objective: The aim of this study was to scintigraphically investigate the preoperative and postoperative renal functions of patients operated due to ureteropelvic junction obstruction, discuss the factors affecting the indication of surgery and emphasize the important points to consider during scintigraphic examination. Method: We retrospectively analyzed pediatric cases who underwent pyeloplasty due to ureteropelvic junction obstruction. Patients operated for primary ureteropelvic junction obstruction whose preoperative and postoperative dynamic scintigraphy results could be obtained were included in the study. The patients were divided into two groups as those with <50% and ≥50% ipsilateral differentiated renal function. The difference between the groups was considered statistically significant when p<0.05. Results: The median ages of 36 cases in <50% DRF group and 24 cases in ≥50% DRF group were 7.7 months and 8.5 months, respectively. The mean ipsilateral differentiated renal function values of the main groups were significantly different from each other both in the preoperative and postoperative periods. The changes within the two main groups were also analyzed, yielding significant differences. Ipsilateral differentiated renal function changes on a general, group and subgroup basis, and preoperative ultrasound findings of the ipsilateral kidney in subgroups were revealed. Conclusion: Increased hydronephrosis degree and renal pelvis anterior-posterior diameter are closely related to overestimated ipsilateral kidney function. For deciding on surgery in patients with ureteral obstruction, scintigraphic examinations make sense with the help of US and the clinical condition of the patient.

yond the standart location of the bladder trigone is called ectopic ureter.1 More than 80% of patients with ectopic ureter also have a complete duplex system.2 The fact that affected individuals are asymptomatic often prevents them from being diagnosed with ectopic ureter. In females, the ectopic ureter may present with urinary incontinence. In both sexes, it may be diagnosed in the antenatal period while it may present with urinary tract infection or urinary obstruction in the postnatal period.

Vaginal agenesis is a congenital anomaly which occurs as isolated developmental defect or as a part of complex anomalies. Vaginal anomalies associated with anorectal malformations are usually diagnosed and repaired in time of surgical correction of anorectal malformations during the infancy period. Herein a case with vaginal agenesis and recto-vestibular fistula, who underwent a neo-vaginal reconstruction with distal segment of fistula during the posterior sagittal anorectoplasty was presented. The use of recto-vestibular fistula as a neo-vagina in cases with vaginal agenesis combined imperforate anus seems to be a feasible and effective approach that has satisfactory anatomical and functional outcomes for vaginal reconstructions.

Purpose: The aim of this study was to assess the safety and the efficiency of pyeloplasty in infants with ureteropelvic junction obstruction (UPJO) in the first six weeks of their life. Materials and Methods: Clinical records of the patients who had surgery during first six weeks of life for UPJO between June 2009 and June 2014 were analysed retrospectively. Results: In this period, twenty-six dismembered pyeloplasties were performed in twenty-four patients on mean operation age of 27.3 +/- 10.2 days (range 8-42 days). On the first postnatal ultrasound all twenty-six renal units had SFU-4 hydronephrosis. Mean preoperative and postoperative anterior-posterior pelvic diameter and parenchymal thickness were 33.1 +/- 8.9mm (range 14-49mm), 3.2 +/- 1mm (range 1-4,6mm) and 14.7 +/- 6.6mm (range 6-27mm) and 7.8 +/- 1.9mm (range 3.0-10.4mm), respectively. The differences between preoperative and postoperative parenchymal thickness and anterior-posterior pelvic diameter were statistically significant (P. 0.0001). Preoperative MAG3 dynamic renal scintigraphy showed obstructive pattern on the diuretic renogram in 26 units. Mean preoperative and postoperative differential renal function on dynamic renal scintigraphy of the affected renal units were 46 +/- 15 and 44 +/- 15, respectively. Postoperative drainage was normal on dynamic renal scintigraphy in 25 (96.2%) of the 26 units, redo-pyeloplasty was needed in only one unit (3.8%). Conclusion: In conclusion, patient selection and timing of surgery are very important in the protection of renal function in newborn with UPJO. In our opinion, if there is indication for surgery, early surgical intervention should not postpone in this period. Surgical treatment of UPJO during first six weeks of life is safe and effective.

OBJECTIVE: Androgen (AR), Estrogen (ER) and Progesterone (PR) hormones play an important role in the prenatal and postnatal development of urogenital tract and especially the penis. The expressions of AR, ER and PR receptors in penile tissues in children with hypospadiases had also been shown previously. In this leading study, to demonstrate of the sex hormone receptor expression in cases with different types of hypospadias were aimed. METHODS: This study was designed in children operated due to hypospadiases without DSD. Biopsy samples of 3 mm’s were obtained from three different sytes as the lateral parameatal tissue and the anterior corner of the prepuce, and inner layer of posterior prepuce. The presence of AR, ER and PR receptors was investigated immunehistochemically. RESULTS: Mean age was 5.4 years in 18 children with hypospadiases; in totally 33 specimens were taken in 5 subcoronal as 5 specimens, and 7 penile as 15 specimens, and 6 penoscrotal as 13 specimens. According to sytes of samples; 13 samples were from lateral para-meatal tissues, and 13 were from anterior corners of prepuces, and 7 were from inner layers of posterior prepuces. In regard to receptor expression; ER and AR receptors were positive in 29 (87.8%) and 12 (36.4%) respectively; PR receptors were negative. CONCLUSION: This study emphasized the dominant expression of estrogen receptors in penile tissues of children with hypospadias. Although there was not a manifest correlation of androgen receptors absence in regard to the severity of hypospadias patients, there was a marked estrogen receptors presence in penile tissues. These findings suggest that the disrupted androgen and estrogen receptor interaction and/or balance could play a role during the development of external genitalia in hypospadias patients. Progesterone receptor was not present and therefore the active role in the postnatal development of hypospadias is still debatable.

If patients had a unilateral inguinal hernia and contralateral cryptorchidism, TTE with PMDS should be considered. The mutation detected in the AMH gene is associated with PMDS and its phenotype is variable. A 7-month-old male infant presented to the pediatric surgery department with a right-sided inguinal hernia and bilateral undescended testis. During herniotomy, tissues suggestive of a rudimentary uterus with fallopian tubes and testes like structures on both sides of uterus were found [Fig-1-2]. A biopsy of the gonads and structure located midline to the gonads was performed and patient was referred to our pediatric endocrinology department with a preliminary diagnosis of sexual development disorder. At physical examination, weight was 8.4 kg [standard deviation score (SDS): -0.09] and height was 71 cm (SDS: -0.51). Blood pressure was 98/60 mm/Hg. Both testes were not palpable. Penis size and appearance were normal. Parents were first-degree relatives. Serum follicle-stimulating hormone (FSH) level was 0.92 IU/L (reference value 1.5-12.4 IU/L), luteinizing hormone (LH) was 1.17 IU/L (1.7-8.6 IU/L), total testosterone (TT) 0.025 ng/mL (0.120.21 ng/mL), estradiol (E2) was <5 pg/mL (<12 pg/mL), and AMH was <0.02 ng/mL (24.2-275.4 ng/mL). Karyotype analysis revealed a XY karyotype. Histopathological examination of the hernia contents was consistent with a rudimentary uterus with bilateral edematous tubal structures. Gonad biopsies showed immature seminiferous tubule. AMH gene sequence analysis performed with a preliminary diagnosis of AMH deficiency revealed a previously undescribed homozygous IVS2-3C>G (c.556-3C>G) mutation. The parents had the same mutation in heterozygous form. The patient was evaluated as a case of AMH deficiency and presented to our “Sex Development Disorders Council”. Upon their decision, orchiopexy was performed. Figure2

According to additional anomalies, transverse testicular ectopia (TTE) is classified into three groups. Type-2 TTE, accompanied by persistent mullerian duct syndrome, constitutes approximately 20% of the patients. Surgical treatment should be planned after careful physical examination, ultrasonography, and genetic/endocrinologic evaluation. Herniorrhaphy, orchiopexy with testicular biopsy, and excision of the mullerian structures are the most appropriate surgical approaches in cases of TTE with persistent mullerian duct syndrome. We aimed to share our approach to the diagnosis and treatment of a patient with type-2 TTE. Possibility of TTE should be kept in mind in children with nonpalpable testis on one side and inguinal hernia on the other side.

Previous reports have described complications resulting from inadvertent intravenous administration of breast milk: hyperosmolarity, microembolism, hypersensitivity and septicaemia.We present the case of a premature infant who was accidentally given expressed breast milk (EBM) intravenously.The non-fatal outcome of the patient in the first month of the incident may have been related to pulmonary embolism and supportive treatment, which was started from the first instance of the milk administration.The fatal outcome of the patient was related to severe septicaemia in the second month of the administration.Particular care must be taken in the case of the patient who is receiving synchronous enteral and intravenous infusion therapy.Caregivers as well as healthcare professionals should be enlightened on the importance of this subject matter.