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BACKGROUND: Priapism is a rare condition in children and the treatment algorithm is controversial in this age group. Herein, we report eight cases with low-flow priapism and our stepwise treatment approach in light of literature. METHODS: We present a simple stepwise treatment for low-flow priapism including five steps. Step 1: Cold compress and analgesia while evaluation the priapism and its etiology. Step 2: Corporal aspiration and adrenaline infusion in the ward. Step 3: Modified Winter shunt in the same place. Step 4: Ketamine application and caudal block in the operating room. Step 5: Sapheno-cavernous (Grayhack) shunt. Eight cases with low-flow priapism were reviewed retrospectively. Symptoms, duration of tumescence, the interventions, and step that provide detumescence were recorded. RESULTS: The mean age of patients was 8.5 years (1-17 y). The median time of the priapism before admission was 15 h (4-165 h). The etiological factors were sickle cell disease, hemodialysis due to chronic renal failure, and factor V Leiden mutation in three patients. Detumescence was achieved in one patient at Step 2, in two patients at Steps 3, 4, and 5, respectively. Rigidity of cavernous body was observed in one patient in long-term follow-up. CONCLUSION: Low-flow priapism is a urological emergency that may cause erectile dysfunction. Treatment options should be selected according to a protocol that prevents time loss and avoids more invasive treatment in unnecessary situations. Our algorithm with simple nature and its steps from less invasive to more invasive procedures may be an alternative for the treatment of low-flow priapism.

Patients with nutcracker syndrome (NCS) associated left varicocele are at high risk of varicocele recurrence when treated only with routine surgical approach for varicocele. An eleven year-old boy who had mild symptoms for NCS and left grade 3 varicocele referred to our clinic. To cure varicocele and to decrease left renal vein pressure, an end to end anastomosis was performed between left spermatic vein and inferior epigastric vein after inguinal microscopic varicocelectomy. His six months follow-up was uneventful. In this case report, we present that this method in children with NCS is a safe and effective method.

Aim: The most important stage of percutaneous nephrolithotomy (PNL), which is an effective and reliable method in the treatment of large and complex kidney stones, is to create a suitable and effective tract after the accessory is provided. For this purpose, different techniques such as Amplatz, baloon and Alken dilators have been described. We aimed to compare the efficacy and safety of single-step Amplatz dilatation technique with stepwise Amplatz dilatation technique in pediatric PNL patients. Methods: This is a retrospective cohort study. We evaluated the data from 75 PNL operations performed on 72 pediatric patients in our center within the last decade. The data from single-step dilation technique patient group (group 1, n: 41) and the stepwise dilatation technique patient group (group 2, n: 34) was compared in terms of durations of fluoroscopy and surgery, stone-free and complication rates, pre and postoperative hematocrit levels and blood transfusions rates. Results: There was no significant difference between the groups in terms of demographic data, mean stone burden, duration of surgery, decrease in hematocrit, blood transfusion rates, stone-free rate and complication rates. The median fluoroscopy durations of group 1 and group 2 were 120 and 220 seconds, respectively. Duration of surgery and fluoroscopy were significantly shorter in the single-step dilatation group Conclusion: PNL is a safe and effective procedure for pediatric stone diseases. Performing this procedure with a single-step dilatation technique ensures that children are less exposed to radiation.

Abscess, hemangiomas and soft tissue masses are present in the differential diagnosis of genital masses in children. We aimed to present an adolescent patient with labial angiomyxoma. Fourteen years-old girl had a hard, immobile, painless mass in her right labium which has been growing progressively for last 9 months. Magnetic resonance imaging revealed a 42x33x38 mm sized, multiloculated and lobulated contoured mass in right labium. The mass was excised and the pathological examination was compatible with angiomyxoma. There are no intraoperative or postoperative complications. She had no local recurrence in postoperative 1 year-follow-up. Genitourinary angiomyxomas are rarely seen in adolescent patients. Close follow-up after surgical excision for local recurrence is essential., Çocuklarda genital bölge kitlelerinin ayırıcı tanısında abseler, hemanjiomlar ve yumuşak doku kaynaklı kitleler yer alır. Labial anjiomiksomalı adolesan hastayı sunmayı amaçladık. On dört yaşında kız hasta 9 ay içinde progresif büyüyen sağ labiumda sert, immobil, ağrısız bir kitle ile başvurdu. Yapılan manyetik rezonans incelemede sağ labium majus düzeyinde 42x33x38 mm boyutlu, multilokule, lobule konturlu kitlesel lezyon saptandı. Eksize edildi ve patolojisi anjiyomiksoma ile uyumlu saptandı. Hastanın lokal nüks açısından takipleri devam etmekte olup ameliyat sırasında ve sonrasında herhangi bir sorun yaşanmadı. Genitoüriner anjiomiksomalar adolesanlarda nadiren görülür. Cerrahi eksizyonu ve lokal nüks açısından postoperatif yakın takibi önemlidir.

© 2020, Yuzuncu Yil Universitesi Tip Fakultesi. All rights reserved.The scar tissue occurring in operating area after the repair of hypospadias which is one of the common congenital anomalies of male children has part in formation of post-operative complications. Many surgical techniques and materials have been used to reduce complications. The purpose of this study is to research the effects of contractubex® (allantoin, heparin, extractum cepae [AHEC]) gel on development of scar in the area repaired after urethrotomy. In the study, 45 male White New Zealand rabbits were used. The rabbits were divided into three groups randomly. Group 1(K): The group to which urethrotomy and primary closure were applied Group 2(A): The group to which urethrotomy, primary closure + 15-day AHEC were applied Group 3(B): The group to which urethrotomy, primary closure + 30-day AHEC were applied Urethras of the rabbits in the groups were calibrated with angiocath (24 Fr) once a day for 30 days. Along with calibration, AHEC was applied to the urethras of the rabbits in 2nd group for 15 days and urethras of the rabbits in the 3rd group for 30 days. To evaluate scar tissue, macroscopic and histopathological evaluations were carried out. Although inflammation was much more in the groups to which AHEC was applied, fibrosis was determine d to be less. It is thought that the application of AHEC may be effective in reducing scar formation after operation.

Presence of multiple calculi in a single system ureterocele is a rare condition. A 3-year-old boy presented with recurrent urinary tract infections in whom multiple calculi were noted in the urinary bladder on x-ray and ultrasound scan. In addition, ultrasound showed the presence of ureterocele in the size of 18x15x12 mm. Open surgery revealed an ureterocele with multiple stones. Here, we present a boy with multiple stones in the left ureterocele diagnosed intra-operatively due to its rarity, etiology and treatment options.

It is well established that testicular differentiation of the human embryonic gonad depends on the action of the Y-chromosomal gene SRY. However, exceptional cases such as SRY-negative cases of 46,XX testicular disorder of sexual development (DSD), and of 46,XX ovotesticular DSD document that testicular tissue can develop in the absence of the SRY gene. These SRY-negative XX sex reversal cases are very rare and usually sporadic, but a few familial cases have been reported. We present a large, consanguineous family with nine affected individuals with phenotypes ranging from 46,XX testicular DSD to 46,XX ovotesticular DSD, with predominance of male characteristics. Absence of SRY in peripheral blood was documented by fluorescence in situ hybridization (FISH) and PCR analysis in all nine affected individuals, and by FISH analysis on gonadal sections with testicular tissue in four affected individuals. By quantitative PCR, a duplication of the SOX9 gene was excluded. In addition, as linkage analysis showed that the nine affected members of the family do not share a common SOX9 haplotype, any mutation at the SOX9 locus could be ruled out. Together, these findings implicate a mutation at a sex-determining locus other than SRY and SOX9 as the cause for the XX sex reversal trait in this family.

Growth and development were monitored for up to 42 months in nine neonates to whom ciprofloxacin, a fluoroquinolone, was given in the neonatal period at a dosage of 20 mg/kg/day. Ciprofloxacin was used only as a ‘life-saving’ therapy in cases of sepsis produced by bacterial agents resistant to other antibiotics. Two other groups of nine neonates, matched by birth weight and gestational age, were studied as controls: one group with sepsis, which was effectively treated with cefotaxime and a group of healthy neonates. No statistically significant differences in growth and development between the groups were found during follow-up for 42 months. No osteoarticular problems or joint deformities were observed in the ciprofloxacin group. Ciprofloxacin appears to provide a therapeutic option as a ‘life-saving’ therapy for newborns with sepsis produced by multiply resistant organisms.

A total of 24 neonates and infants, aged from 2 days to 10 months, received treatment with intravenous fluconazole for microbiologically documented or presumed fungal infection. The mean fluconazole dosage was 6 mg/kg/day (range 2 –16 mg/kg/day) and the mean duration of therapy was 25 days (range 5–72 days). Efficacy was evaluated in neonates with proven fungal infections, as documented by the presence of pathogen at baseline. A positive clinical response was achieved in 23 of the 24 clinically evaluable patients (96%); eradication of the fungal organism was also achieved in 23 of the 24 evaluable patients (96%). Adverse events occurred in two patients (8%) but therapy was not discontinued in either patient. The present results confirm the efficacy and safety of fluconazole in the treatment of neonates and infants with severe fungal infections.

The oromandibular limb hypogenesis syndromes (OLHS), which are congenital malformations involving the tongue, mandible, and the limbs are extremely rare with a few sporadic cases reported in the literature. In this report, the authors report a case of OLHS with gastroschisis, that has not been described previously according to the medline search.