[Intermittent hydronephrosis in childhood]. / Intermittáló hydronephrosis gyermekkorban.

INTRODUCTION: Intermittent pelviureteric junction obstruction, and its consequence,intermittent hydronephrosis is a difficult condition to identify. The renal collecting system is not dilated between the episodes of abdominal pain attacks and a prompt investigation is hard to carry out during the time of painful crisis. Therefore, most of the patients are initially misdiagnosed. AIM: The aim of the study was to assess the occurrence and the clinical characteristics of this phenomenon in children operated in the Department of Urological Surgery, Heim Pál Children Hospital, Budapest, Hungary. PATIENTS AND METHODS: Medical records of children operated for hydronephrosis between 2008 and 2012 were reviewed. The occurrence rate and clinical features of intermittent hydronephrosis were analyzed. RESULTS: 76 children were operated for pelviureteric junction obstruction, of which 10 met the criteria of intermittent hydronephrosis. The average interval between the onset of symptoms and the final diagnosis was 2 years and 4 months. In 7 patients pyeloplasty, and in 3 patients nephrectomy were performed. CONCLUSIONS: Intermittent hydronephrosis represents a well-defined proportion among cases operated for hydronephrosis. The delay in diagnosis led to the loss of the kidney in one third of the patients, and this finding urges for the awareness of health professionals for this phenomenon. In case of recurrent abdominal pain of unknown etiology one must suspect a urological origin, even if a previous abdominal ultrasound showed normal kidneys.

Long-term psychological and sexual outcomes of severe penile hypospadias repair.

INTRODUCTION: Hypospadias is the most common penis malformation, and there exist a variety of surgical approaches used to correct the abnormal position of the meatus. Although the long-term outcomes of surgery are considered important for psychosexual development, only a few attempts have been made to evaluate patient satisfaction. AIM: The aim of our study was to evaluate surgical results and psychosocial adaptations in a homogeneous group of subjects with severe penile hypospadias who underwent the same types of surgical repairs during childhood and compare the results to data obtained from age-matched healthy controls. METHODS: In this cross-sectional study, 104 men (between 24 and 42 years old) who underwent an uncomplicated two-stage hypospadias repair in their childhood and 63 age-matched healthy men without genital malformations completed the questionnaire. MAIN OUTCOME MEASURES: Difference in self-perception assessed by a 15-item questionnaire regarding psychosexual well-being and penile appearance between subjects with corrected hypospadias and healthy participants. RESULTS: On average, subjects with a hypospadias repair were less satisfied with their genital appearance; however, they were more satisfied with their sex lives compared to healthy controls. The meatus distance was approximately 1.5 cm from the tip of the penis after surgical correction. None of the postoperative surgical results correlated with patient satisfaction. Furthermore, the small percentage of patients (11%) who were very unsatisfied with their surgical outcomes had no significant differences in surgical outcomes compared to satisfied patients. However, there was a significant difference between the two groups in almost all psychological outcome measures. CONCLUSIONS: In adults who underwent an uncomplicated ventral repair of a severe penile hypospadias 20-30 years earlier, healthy psychosexual development was achieved despite the lack of a glanular meatus. Early identification of unsatisfied patients is important for appropriate long-term follow-up and counseling.

The leucine-rich repeat-containing G protein-coupled receptor 8 gene T222P mutation does not cause cryptorchidism.

CONTEXT: Insulin-like 3 and its receptor, leucine-rich repeat-containing G protein-coupled receptor 8 (LGR8), are essential for the first phase of testicular descent. Homozygous loss of either of the two genes in mice leads to cryptorchidism. Although mutations in both homologous human genes are not a common cause of cryptorchidism. To date, only one missense mutation at codon 222 (T222P) of the LGR8 gene has been proposed as a causative mutation for cryptorchidism. This conclusion was based on both functional in vitro studies and the lack of mutation in a large group of controls. The geographical origin of the mutation carriers suggested a founder effect in the Mediterranean area. OBJECTIVES: We sought to define the frequency of the T222P mutation in four different countries to assess whether the screening for this mutation could be of use as a diagnostic genetic test. MATERIALS AND METHODS: A total of 822 subjects (359 with a history of cryptorchidism and 463 controls) from Italy, Spain, Hungary, and Egypt were genotyped for the T222P mutation by direct sequencing. RESULTS: The phenotypical expression of the mutation also included normal testicular descent. The mutation frequency was not significantly different in cryptorchid patients vs. noncryptorchid controls (3.6 vs. 1.7%, respectively). No significant geographical differences were observed in mutation frequencies. The haplotype analysis allowed us to predict three distinct haplotypes, i.e. three possible mutation events. CONCLUSIONS: Our results suggest that the T222P mutation cannot be considered either causative or a susceptibility factor for cryptorchidism. A true causative mutation in the LGR8 gene still remains to be identified.

[Is premature induced labour justified in case of fetal urinary tract dilatation?]. / Indokolt-e a szülés elorehozása magzati veseüregi tágulat miatt?

UNLABELLED: There is a decreasing trend in the number of intrauterine interventions performed due to urinary tract dilatations of the foetus diagnosed ultrasonographically. This is due to the low efficacy of the interventions, the high rate of complications and a better knowledge of the foetal physiology. However, the demand for early corrective surgery is still present. In Hungary pre-term caesarean section is performed in many institutions in order to enable the operation of the neonate, despite the fact that the benefits of this intervention have not been established. AIM: In order to test the correctness of this practice, the authors conducted a retrospective analysis of the patients of two large case-volume paediatric institutions. PATIENTS: In a period of 5 years, 329 neonates were followed due to intrauterine diagnosis of urinary tract dilatations (197 at the Department of Urology, Heim Pál Children's Hospital in Budapest, whereas in Miskolc 132), including 13 cases where the urological anomaly was the indication for pre-term induction of labour. RESULTS: Of the 13 neonates who had been delivered prematurely, none were operated before the postconceptional age of 40 weeks, and 3 subsequently required no surgical intervention. Two neonates developed life-threatening conditions that presumably could have been avoided without the premature caesarean section. CONCLUSIONS: The authors conclude that there are several arguments against pre-term delivery: 1. Prenatal diagnostics does not always yield as accurate information as the diagnostic procedures performed in infancy; 2. Intrauterine urinary tract dilatation often resolve spontaneously, superseding the need for both pre- and post-natal surgical interventions. 3. Scientific observations have confirmed that there is no difference between the efficacy of postnatal and pre-term interventions. Based on the above-mentioned, authors emphasize that this outdated practice can no longer be accepted, and call for good cooperation between the obstetrician, the paediatric urologist and nephrologist, to coordinate the management of the case.

Congenital lymphovascular malformations with urological symptoms: a report of two cases and review of the literature.

OBJECTIVES: To collect and review the diagnostic and therapeutic solutions for primary and congenital lymphovascular malformations leading to urological symptoms in childhood and also to find the most efficient therapeutic algorithms managing such conditions. METHODS: In our work, we assemble all the diagnostic and therapeutic tools for lymphovascular malformations with urological-urogenital symptoms and demonstrate the interventional therapeutic algorithms through two of our cases where surgery (laparoscopic intervention and clipping of the lymph vessel) had to be performed to stop lymphoid leakage and restore anatomy. RESULTS: In cases, where lymphovascular malformations and urological-urogenital symptoms are both present, therapeutical success is graded by Browse's scoring system. According to that, our choices of management achieved the best possible outcome in both cases below. CONCLUSIONS: Although conservative ways of therapy are known and widely used, in more advanced cases surgical help is often needed to reach long-term improvement. In situations where significant mass of chyle has accumulated causing severe complaints, conservative therapy should not to be started. Surgical solutions provide fast and lasting improvement for patients suffering from congenital lymphovascular malformations.

Treatment of posttraumatic high-flow priapism in 8-year-old boy with percutaneous ultrasound-guided thrombin injection.

Treatment of high-flow priapism varies and has included transcatheter embolization when conservative approaches fail. We present the case of an 8-year-old boy with high-flow priapism, who was treated with ultrasound-guided thrombin injection because of encouraging experience with the method obtained when treating aneurysms and pseudoaneurysms. This procedure appears to be a useful alternative to transcatheter embolization and results in no radiation exposure.

High incidence of balanitis xerotica obliterans in boys with phimosis: prospective 10-year study.

This prospective study was designed to address the incidence and clinical and histologic characteristics of balanitis xerotica obliterans in a large random pediatric population with phimosis. We investigated 1178 boys who presented consecutively with phimosis between 1991 and 2001. All patients who underwent complete circumcision and surgical specimens were typed histologically as early, intermediate, or late forms of this disorder or as nonspecific chronic inflammation. Patients with balanitis xerotica obliterans were controlled at 1, 6, and 12 months postoperatively, then yearly. Balanitis xerotica obliterans was found in 471 of the 1178 patients (40%), with the highest incidence in boys aged 9 to 11 years (76%). Secondary phimosis occurred in 93% of boys with balanitis xerotica obliterans and in 32% of those without the disorder. In six instances of balanitis xerotica obliterans, meatotomy and in one meatoplasty was performed, as well as circumcision. On histologic evaluation, we found 19% had early, 60% intermediate, and 21% late form of balanitis xerotica obliterans. Glanular lesions disappeared completely within 6 months in 229 out of 231 patients. Our data strongly suggest that the true incidence of childhood balanitis xerotica obliterans is higher than previously assumed. Its incidence peaks in the 9 to 11 years age group, in whom secondary phimosis was almost exclusively caused by balanitis xerotica obliterans.

Use of buccal mucosa patch graft for recurrent large urethrocutaneous fistula after hypospadias repair.

INTRODUCTION: To assess the effectiveness of buccal mucosa patch graft in the treatment of recurrent large urethrocutaneous fistula after hypospadias repair. MATERIALS AND METHODS: A free graft of buccal mucosa was used for closure in 7 boys (mean age 4.8 years) with large (>4 mm) urethocutaneous fistula. Four fistulas were in the midshaft, 2 of them penoscrotal and 1 coronal type. All patients had undergone at least two previous unsuccessful fistula repairs, and 3 of them had undergone three attempts for closure. Fistula repairs were similar in all cases. RESULTS: The repair was successful in 6 out of 7 cases, and in these cases the urinary stream was good after the removal of the catheter. The unsuccessful case was the coronal one. CONCLUSION: Based on our experience it seems that in cases with recurrent large fistula after hypospadias reconstruction, the use of buccal mucosa patch graft for closure is a good treatment choice.

Evaluation of 100 laparoscopic varicocele operations with preservation of testicular artery and ligation of collateral vein in children and adolescents.

OBJECTIVES: To introduce a useful technique for identifying any collateral veins during laparoscopic varicocele operation and to evaluate our long-term results with this technique after 100 cases. METHODS: A new method was used to detect an incidental collateral vein. During surgery the patient was placed in anti-Trendelenburg position after introducing laparoscopic instruments until the dilated scrotal varicose filled up with blood. The main spermatic vein was then grasped atraumatically and the blood was pressed out manually from the scrotum. During this manoeuvre any existing collateral vein/veins became dilated and could be easily identified. The dilated spermatic vein and also any collateral vessel were first prepared then clipped. RESULTS: Out of the 100 patients, collateral veins were detected and ligated in 19 cases. In 15 cases a single collateral, in 4 cases two collaterals, and in the remaining 81 children no collateral veins were found. The testicular artery was identified in all operations as a pulsatile vessel. At a mean of 24 months (range 6-60 months) follow-up recurrence occurred in one patient and in five children hydrocele developed postoperatively. Testicular atrophy and severe intra- or postoperative complication did not occur in any patient of this series. CONCLUSION: The identification of incidental collateral vein by this method and preservation of the testicular artery resulted in a very good success rate in children and adolescents treated by laparoscopic varicocele operation.