Combined use of perimeatal-based flap urethroplasty (Mathieu) with midline incision or urethral plate in hypospadias repair.

Perimeatal-based flap urethroplasty is commonly used for the primary correction of distal hypospadias. The Mathieu repair provides excellent function and satisfactory cosmetic results, but the risk of devascularisation of the neourethral flap is an inherent problem of this technique. The midline incision of the urethral plate is part of the Snodgrass method, which offers good urethral calibre and tension-free closure. Our aim was to test the effectiveness of the use of the perimeatal-based flap combined with a midline incision of the urethral plate in hypospadias repair. 19 boys, aged 20 months to 5 years, with distal hypospadias, underwent a one-stage repair using the above-described modification of the Mathieu technique. The meatal-based flap with a midline incision of the urethral plate was similar in all cases. The hypospadias repair was successful in all patients, only one boy developed a urethrocutaneous fistula which required subsequent operation. There was no postoperative scarring, chordee or urethral stricture during follow-up. Hypospadias repair using a perimeatal-based skin flap and combined with an incision of the urethral plate reduces complication rates and offers good cosmetic results.

The response of balanitis xerotica obliterans to local steroid application compared with placebo in children.

PURPOSE: We evaluated the clinical effectiveness of topical steroid application for balanitis xerotica obliterans in children and analyzed the association of any clinical response with histological findings. MATERIALS AND METHODS: Our double-blind, placebo controlled, randomized study included 40 boys in whom balanitis xerotica obliterans was diagnosed clinically by cicatricial phimosis. The severity of phimosis was graded into 4 groups. Patients were randomized to receive the topical application of 0.05% mometasone furoate or placebo. After 5 weeks phimosis severity was reevaluated and all patients underwent circumcision. Surgical specimens were histologically typed as an early, intermediate or late form of balanitis xerotica obliterans. RESULTS: Seven patients were withdrawn from the study. In the steroid group 7 boys had clinical improvement and 10 had no change. Histological study showed an early, intermediate and late form of balanitis xerotica obliterans in 5, 5 and 7 cases, respectively. Of cases with clinical improvement 5 were the early and 2 the intermediate type. In the placebo group 5 cases worsened clinically and 11 did not change. Histological evaluation revealed an early, intermediate and late form of balanitis xerotica obliterans in 3, 7 and 6 boys, respectively. Of the 5 cases with histological worsening, disease was the early, intermediate and late type in 2, 2 and 1, respectively. CONCLUSIONS: Applying a potent topical steroid affects improvement in balanitis xerotica obliterans in the histologically early and intermediate stages of disease, and may inhibit further worsening in the late stage.

[Laparoscopic intervention in pediatric urology]. / Laparoszkópos beavatkozások a gyermekurológiában.

The authors summarise the short history of the laparoscopy, and main steps of its use in international and Hungarian practice of urological surgery. They evaluate the possibility of laparoscopy in paediatric urological practice. Finally, they present their experience with this minimally invasive technique from 1995. Between October 1995 and September 1998, 83 laparoscopic procedures were performed. In 59 patients the vena testicularis were ligated by laparoscopy and in 22 patients 24 non palpable testes were diagnosed and operated. In 9 of the cases laparoscopic orchidopexy was performed. The authors demonstrate their operative technique. All laparoscopic interventions were tolerated well by children and they were treated without any complication. Postoperative pain medication were given for 5 children. The operative time for varix ligation ranged from 15 and 20 minutes, and for laparoscopic orchidopexy from 30 and 40 minutes. The recurrence rate of varicocele was found to be 1.5%, and postoperative hydrocele developed in one patient (1.5%).

The same mutation affecting the splicing of WT1 gene is present on Frasier syndrome patients with or without Wilms' tumor.

Denys-Drash and Frasier syndromes are rare human disorders that associate nephropathy with gonadal and genital abnormalities. In DDS there is a predisposition to Wilms' tumor. Heterozygous point mutations in the Wilms' tumor, type1 gene (WT1), particularly those altering the zinc finger (ZF) encoding exons, have been reported in most DDS patients, while mutations in intron 9 of the same gene cause FS. This paper describes two cases of DDS, one FS and one patient with Wilm's tumor and intersex genitalia, in which mutations were searched by sequencing the exons 8 and 9 of WT1 gene. Patient 1 carried a missense point mutation in exon 8 (ZF2), converting a CGA-Arg codon to a TGA-stop codon. Patient 2 presented a single nucleotide deletion within exon 9 (ZF3) introducing a premature chain termination at codon 398. Patients 3 and 4 had a C-->T transition at position +4 of the second alternative splice donor site of exon 9 (this mutation was detected in peripheral blood and in tumor derived DNA of patient 3). However, patient 3 had previously developed a Wilms' tumor. This is the first case of Wilms' tumor development in a phenotypically and genetically confirmed case of FS.

Fusional anomalies of the testis and epididymis.

Earlier, the cause of infertility in undescended testis (UT) had been widely accepted as a consequence of the higher temperature of the inguinal/abdominal region. Observations made in the past two decades, however, gave new evidences. The most important of these is that UT is often associated with the fusional anomalies (FA) of the testis and epididymis. FA is the consequence of pathological intrauterine hormonal processes and many authors believe FA to be the primary cause for infertility in UT. Since 80% of UT cases are of endocrine origin, it would be suspected that the very same factors are responsible for both UT and FA. FA and other anomalies of the epididymis often occur in testicular torsion (TT) as well. It is remarkable that infertility could follow the unilateral forms of UT and TT despite the presence of a "healthy" contralateral gonad. In both entities contralateral FA (probably associated with testicular dysgenesis) is suspected. These observations could influence the primary surgical treatment of patients with UT and TT, as well as the mode of further management of these cases.

Laparoscopic varicocele operation: a chance to prevent the recurrence.

There are recurrences and postoperative hydrocele of varicocele after any kind of surgical treatment. Laparoscopic clipping and dissection of internal spermatic vessels was performed without any complication in 73 children to treat varicocele in our department between 1995 and 1998. We have used a new method to detect etiological factors at laparoscopic surgery. The well-known Linton and Trendelenburg test was adapted to detect incidental collateral veins in 73 patients. Using these test, collateral veins were detected in 16 boys. The testicular artery identified in most of the cases as a pulsatile vessel. The operating time was 10-25 minutes. Laparoscopic varicocelectomy is a safe, effective treatment causing minimal discomfort and allowing patients an early to return to activity. These results suggest this technique a viable alternative to open ligation in paediatric urological practice.

Intrascrotal and testicular solid masses in childhood.

A retrospective study was performed between 1985 and 1994 on paediatric patients operated for asymptomatic intrascrotal or testicular palpable masses. Tumour was suspected in each case and it was surgically explored. Twenty-six children were affected, their age ranging between 9 days and 14 years. In 11 cases testicular torsion, in 4 epididymitis and in another 2 dystrophic calcification were found. Tumours, including rather rare alterations, were observed in only 9 children. The present results draw attention to the tumour-like occurrence of testicular torsions and other benign alterations of the scrotum.

[Non-surgical approach to the treatment of vesico-ureteral reflux in childhood]. / A gyermekkori vesicoureteralis reflux konzervatív kezelésének lehetöségeiröl.

The authors report about their point of view, based on their experience treating children with vesicoureteric reflux (VUR) in the last two decades. 812 children were treated for VUR between 1973-1992. In 62 (7.6%) due to the parenchymal damage, primary nephrectomy was made, antireflux operations were done in 227 (30.3%). Without surgery, recovery or significant improvement was achieved in 523 children (69.7%). The basis of the good results with conservative therapy is in their opinion the thorough knowledge of the function of the urinary organs, especially of the urinary tract, and the precise detection of the pathophysiological processes leading to VUR. The conditions in which antireflux surgery is avoided, are discussed in details, emphasizing the situations and methods eventually leading to mistakes, e. g. the improper judgement of VUR detected during acute pyelonephritis or during an incorrectly performed micturition cystography, and the undiagnosed damaged bladder function. Essential is therefore to clarify the etiology of the VUR and the compensated/uncompensated state of the urinary tract, the clinical condition of the child. Antireflux operation is necessary only if in the renal parenchymal damage the role of the VUR is obvious and it can be prevented only with surgery.

Results of surgical treatment in children with bladder exstrophy.

A series of 42 children (30 boys and 12 girls) underwent surgery for bladder exstrophy between 1972 and 1989. Primary bladder closure was performed in 11 patients and was successful in 6. Four of these children are about 2 years old and so it is not yet possible to assess their continence. Ureterosigmoidostomy was performed in 35 children, one of whom was converted to cutaneous ureterostomy. Follow-up ranges from 2 to 20 years (average 9 years 3 months) and 50% of the patients are symptom-free. The most frequent problems were acidosis and urinary tract dilatation. Other complications, such as hypokalaemia and pyelonephritis, were seldom seen. Although ureterosigmoidostomy has some disadvantages, our patients have adapted well and lead a normal life.

Testosterone secretion in children with undescended testis.

Testicular testosterone (T) production was examined in thirty boys with undescended testes (UT) following the administration of 4500 U gonadotropic hormone. Twenty boys had bilateral UT and ten had UT plus hypospadias. As for possible causes of reduced Leydig cell activity it was investigated whether the testis was (1) hypoplastic; (2) abnormally fused with the epididymis; (3) located in the abdomen; (4) or UT was associated with hypospadias. Average T values were significantly lower when the testicle was hypoplastic or its fusion with the epididymis was imperfect; but remained largely undiminished when the testicle was located in the abdomen or when UT was combined with hypospadias. The occurrence of both pathologic and physiologic T reactions in each of the four groups suggests that the population of UT children is heterogeneous, probably due to differences in aetiology and in intrauterine hormonal processes. In the case of UT and hypoplasia the time and method of operation (orchidopexy) must be selected with utmost care, bearing in mind that an originally small testicle with impaired T secretion may become physiologic by the time of puberty.

The state of the testicle and the epididymis associated with exstrophy of the bladder in undescended testes.

In undescended tests, the large number of testicular dysplasias/hypoplasias and of that of the fusion abnormalities of the epididymis, and the joint occurrence of the two, can achieve even 50%. The frequency of the above anomalies were studied in undescended testes associated with exstrophy of the bladder. Bilateral undescended testes were observed in 5 out of 26 boys born with vesical exstrophy. In 3 of them bilateral orchiopexy had already been performed. In 5 of the 6 operations intact testicles and intact epididymis were found. Fusion abnormality was observed only in one case, but the testicle was intact also in that case. This can be attributed to the fact that in exstrophy of the bladder, the testicles have failed to descend not due to the insufficient effect of fetal androgen, but due to anatomical causes. This is also supported by the fact that where the disorder of descension has been caused by mechanical factors, there was a much lower ratio of testicular-epididymal fusion abnormality.

States causing infertility in adulthood in children with undescended testis.

A total of 1386 operations for undescended testis was made in 1250 boys. In 774 of them an apparently intact testis and epididymis were found. In 612, such epididymal developmental abnormalities were detected which exclude or do not make possible the passage of sperms from these organs to the deferent duct. It is believed to be plausible that the large number of cases with infertility is due, also in individuals with a unilateral cryptorchidism, to the abnormal fusion of the epididymis in both testes.

Testicular-epididymal fusion abnormality in undescended testis.

In the course of 1195 operations, performed in children with cryptorchidism, the testes were studied for size, turgor and fusion with the epididymis. Inquiries were made into the reasons of fusion disorders which were conspicuously numerous. Attention is called to this not too familiar condition which seems to play an important role in the emergence of fertility disorders in men with congenital cryptorchidism.

Neurofibromatosis of the bladder.

Neurofibromatosis of the urinary tract is estimated to account for 20 per cent of the visceral manifestations of the generalized disease. Among its urological forms involvement of the bladder is the most common. Clinically, neurofibromatosis resembles a tumour, therefore, its early histologic diagnosis is of utmost importance. In the present case a tumour involving the posterior bladder wall was found in a 6 years old boy subjected to examination because of a terminal haematuria. First the tumour was resected, later cystectomy with implantation of the distended ureter into the sigmoid was undertaken because of progression of the tumour. Postoperative hypokalaemia responded to the usual medication. Ureteral distension was reversed by the operation. Though neurofibromatosis (NF) is histologically a benign process, it tends to recur, is invasive, and impairs urinary functions. Therefore, it may endanger life and thus requires cystectomy with surgery for urinary diversion.

Ureterocystoplasty for megaureter: a technique practicable in bladder wall fibrosis.

The cause of megaureter (hydroureter) is inadequacy of the ureterovesical junction. The only possible means of its therapy consists in a surgical reconstruction of the ureterovesical junction. A technical modification of ureterocystoplasty for megaureter, having been employed since 1976, is described. It basically consists in the construction of a tunnel from the ureter within the bladder, the ureter being sutured to the wall of the bladder only loosely. In 150 children 182 ureteroneocystoplastic operations were done. As confirmed by the postoperative course, as well as by the results of the follow-up studies, the technique may be used with benefit even in case of infection, impaired renal function or bladder wall fibrosis.

[Pyeloplasty in infants and children]. / Pyelumplastik bei Säuglingen und Kindern.

Pyeloureteral obstructions of various pathogeneses and their complications can be visualized satisfactorily be means of intravenous urography. Of the other diagnostic methods angiography was found to be the most useful, no retrograde examination was carried out. When distension of the end of the calyx or obvious parenchymal lesion appears, plastic operation must be performed irrespective of the patient's age, the presence of infection or anomaly of the localization. Only kidneys which have lost their functions should be removed. In the operation the pyeloureteral boundary is always excised, the new transition is secured by splinting and a transrenal drain is used. Secondary nephrectomy had to be performed in a single case. One-year and three-year intravenous urographic check-ups showed in 72 of the 100 cases good, in 19 satisfactory results, in 8 cases the pyeloureteral boundary was funnel-shaped, but the dilatations of the calyx-ends remained unchanged. Pyeloplasty is no longer a high-risk operation.

Blood supply of the rat amygdala.

The cerebral vessels of the rat were filled with inks of different colours. The topography of the vessels of the amygdala were reconstructed from serial sections. The circulation of the individual amygdaloid nuclei was studied in detail. The arteries of the amygdala arise from the deep and cortical branches of the internal carotid and middle cerebral arteries. Eight major arteries were found to supply blood to the amygdala. All amygdaloid nuclei receive branches from both arterial trunks. The vast majority of the veins are collected by the middle cerebral and basal veins. Only a small fraction drains into the hippocampal vein. Of particular importance are the veins ending in the basal vein and those cortical ones that run in the rhinal sulcus. All amygdaloid nuclei have a multidirectional drainage.