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A case of simultaneous heart and kidney transplantation is presented in a 12 years old girl. The patient suffered from dilated cardiomyopathy and end-stage kidney disease. Bilateral vesico-renal refluxes were corrected at the same time of the duplex transplantation. A multiorgan harvesting from a pediatric 8 years old donor was utilized. Follow-up is 40 months. Cytomegalovirus (CMV) infection and acute tubular necrosis from haemoglobinuria were transient complications. Hemolysis from bypass and extracorporeal circulation was involved as pathogenetic factor of the acute tubular necrosis. Cardiac and renal function remain excellent and the child is fully recovered and resumed a normal and active life. Combined heart and kidney transplantation may be of benefit in selected patients, also in pediatric age.

The gliding testis is a testicle located below the external ring, can be manipulated to the upper scrotum, but is prone to ascend to its original position. Histologic changes can be detected in these gonads by 7 years of age. We evaluated 427 consecutive prepubertal boys referred for cryptorchidism. One hundred and twenty-three had classical undescended, 71 ectopic, 55 retractile, and 178 (mean age 6 years 2 moths) gliding testes. The gliding testes were smaller than controlaterals in 24% of boys. All gliding testes were unilateral whereas bilaterality was 85% in the retractile group (p < 0.0001), 17.5% in the undescended (p < 0.001), and 10% in the ectopic group (p < 0.01). A history for one or more of the following: orchidopexy (3), hormonal treatment (5), late testicular descent (9), spermatic cord torsion (5), testicular pain (10), actual retractile testes (20) or actual gliding testis (58), was present in 93 (52.2%) of the fathers of the gliding group. Forty-seven (81%) paternal gliding testes were hypotrophic. Seventy-five boys with gliding testis underwent initial hormonal therapy with transient benefit, and 57 were operated on. Two anatomical findings are typical of the gliding testis: the absence of the gubernaculum and a processus vaginalis partially patent from the upper scrotum to the mid groin area. This feature explains the mobility of the gliding testis from the external ring to the upper scrotum. The absence of the gubernaculum may be responsible for a higher incidence of spermatic cord torsion in this population. The gliding testis is a distinct entity, representing the mildest degree of a true undescended testis. As hormonal treatment gives only transient results, orchidopexy should be considered before testicular damage occurs.

Congenital megaureter in a solitary kidney (CMSK) is a life-threatening disease. From January 1988 to December 1993, we treated 9 patients for CMSK (2 cases with unilateral renal dysplasia and 7 with unilateral renal agenesis). In 7 cases, the malformation was detected by prenatal ultrasonography and urological counseling. The ages at first postnatal observation ranged from 24 h to 5 years (mean = 8 months). The postnatal urological workup included: serum renal function screening, urinary system ultrasonography, a micturition cystogram, intravenous pyelography and a nuclear renal scan. The therapy of choice was decided on the basis of renal function. One emergency divertive procedure was performed after birth, in 3 cases of renal failure in the first 2 weeks of life. The remaining cases were operated electively at an average age of 20 months. In 2 cases that underwent delayed surgical elective correction and in 1 of the 3 cases that underwent an emergency diversion, some degree of renal failure still persists. In our opinion, prenatal diagnosis, accurate postnatal urological workup and strict postoperative follow-up are fundamental to avoid the devastating consequences of CMSK on the affected child.

The clinico-pathologic association of nodular renal blastema, multicystic kidney and obstructive uropathy has been recently identified. We report on a female patient diagnosed as having unilater multicystic dysplasia by prenatal ultrasonography. The patient was nephrectomized at the age of 6 1/2 months. Examination of the resected kidney revealed multiple unilocular cysts in the cortex and hypoplasia of the homolateral ureter; histological study confirmed the presence of multiple cysts limited to the renal cortex, and revealed, among them, multiple cortical metanephric blastema cells islands. Our case supports a relationship between nodular renal blastema, cortical cysts and obstructive uropathy; ureter hypoplasia could cause intraluminal back pressure, with consequent abnormal development of the ampullae, normally endowed in nephronic anlagens induction, cystic tubular ectasia and persistence of nodular renal blastema. The peripheral location of renal nodular blastema and cysts supports a late error in nephrogenesis, at the time of formation of the last generation of nephrons.

Objective: Bovine collagen has been successfully used for the endoscopic treatment of urinary incontinence (UI) and vesico-ureteric reflux (VUR) in children for more than 8 years, although its long-term efficacy has sometimes been questioned. Its failure is generally ascribed either to technical difficulties or to instability of collagen at the site of the implant. To assess the efficacy of the procedure we evaluated the children treated at this hospital between 1990 and 1993., Patients and Methods: A total of 156 children, aged 5 months to 13 years, were treated for VUR. Over the same period, 25 patients aged between 2 and 14 years (nine with neuropathic bladder and 16 with exstrophy-epispadias complex who were still incontinent after bladder neck reconstruction) had periurethral or pericervical glutaraldehyde cross-linked bovine collagen injection for UI. Five more children with exstrophy-epispadias complex (aged 1-3 years) underwent periurethral collagen injection to stimulate bladder enhancement and allow subsequent bladder neck reconstruction., Results: In children treated for VUR, a single injection proved successful in 72.2% of cases (127 ureters); a second collagen injection raised the success rate to 81%. Continence improved in all nine neuropathic bladders and in 10 of 16 children with exstrophy-epispadias complex treated for UI after bladder neck reconstruction. In four of the five exstrophy-epispadias complex patients who were treated to stimulate bladder enhancement, bladder capacity increased by 25%., Conclusions: Endoscopic treatment of VUR seems to be a valid alternative to open surgery, even though concerns remain about the long-term efficacy of collagen implantation. An important distinction should be made between early and late failure of the procedure. Early failure, which we define as persistence of reflux, is usually due to incorrect technique or technical difficulties. Late failure, or recurrence of reflux, which has previously been attributed to the biodegradability of collagen, seems to be due to the displacement of the injected collagen. Micturition itself or high bladder pressure (such as detrusor instability) could be responsible for the displacement of the injected collagen medially and distally, where it can no longer support the submucosal ureteric tunnel. In the treatment of urinary incontinence, both the implant technique and the choice of the site of injection seem to have a considerable effect on the results. In our experience, endoscopic collagen injection is effective in the treatment of both urinary incontinence and VUR in paediatric patients. Accurate selection of patients and technical adjustments and refinements are essential to obtain the best results.

The 6-year follow-up of a patient affected by Wolfram's syndrome, a rare disease characterized by diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy (OA), neurosensory deafness (D), atony of the urinary tract and other abnormalities (DIDMOAD or Wolfram's syndrome), is described. Our patient has diabetes insipidus, diabetes mellitus, abnormal audiograms, without subjective evidence of hearing loss, and dilatation of the urinary tract. Diagnosis was suspected at the age of 8 years. Diabetes mellitus was the first manifestation and treatment with insulin was necessary. Desmopressin therapy decreased dramatically the daily urinary output. In view of the significant morbidity and mortality from renal failure associated with recurrent urinary infections, we have drawn special attention to the urological manifestations of the syndrome. During the follow-up, the patients underwent some investigations, such as renal ultrasound and echotomography and cystourethroscopy. Outstanding results of these studies are severe bilateral hydronephrosis with dilatated ureters and loss of renal tissue. The particular finding is the presence of posterior urethral valves with obstructed bladder. The anatomical outlet obstruction are variable and may be disastrous. There may be failure to thrive, sepsis, anemia be disanal failure. In such instances corrective surgery could improve bladder and ureteral functions.

Müllerian duct congenital anomalies such as Rokitansky-Mayer syndrome, Urogenital Sinus, Vaginal Atresia and Cloacal Malformation are relatively uncommon (1-5% of born female newborns). The complexity of these malformations has taken great interest regarding mainly the surgical procedure available for correction of genital abnormalities. However, the problem of urinary incontinence is still underestimated, and continence is often a goal difficult to achieve. Authors report 11 cases of congenital anomalies of Müllerian duct differentiation which have been observed in the last decade in pediatric age with special regard to preoperatory diagnostic procedures, urinary continence valuation after surgical correction, therapy chances for continence, considering the psychological implication of this unresolved abnormality in everyday-lifetime.

Nowadays enuresis is a problem that pediatric urologists are often called to treat, since it affects 15 to 30% of school-age children. In 85% of affected children bedwetting is monosymptomatic, not accompanied by other voiding disorders or daytime incontinence. Treatment of choice is still highly controversial, as the physiopathology is not yet fully understood and the pathogenesis is multifactorial: genetic and psychological factors, sleep disorders, urinary reservoir abnormalities, urine production disorders can all play a part. Behavioural treatments (psychotherapy, bladder training and biofeedback, electric alarm) and pharmacological therapy (tricyclic antidepressants, anticholinergics, DDAVP) have been used with variable results. In our 1 year experience (54 enuretic children) DDAVP proved to be effective in reducing the number of wet nights per week in 79% of cases. Acupuncture, which we have been using for many years, also gave good results in 55% of treated patients. Long term success of DDAVP and acupuncture was respectively 50 and 40%. We discuss the probable pathophysiology and present our own results and those reported in the literature. It has to be stressed that an accurate diagnostic selection of patients and a better understanding of physiopathology are the basis of effective treatment of enuresis.

A rare case of asymptomatic pancreatic lipoma in an eleven-month-old child is presented. The abdominal mass was discovered occasionally during a grip episode. Preoperative tests proved the presence of a neoformation arising from the pancreas. It was multilobed, capsulated and located between colon transversus, spleen and stomach without infiltration of surrounding structures. The histological picture was that of a mature lipoma. The authors discuss the differential diagnosis versus other fat tumors.

Continence is the main goal in the treatment of patients with bladder exstrophy and prognosis is necessarily long-term. Over an 8-year period, 44 patients presented with bladder exstrophy and/or incontinent epispadias. Twenty-five bladder closures (7 secondary), 23 male urethroplasties, 19 bladder neck (BN) reconstructions and 6 bladder augmentations were performed in these patients. In 18 children 2 additional procedures were employed in the last 3 years: female genito-urethroplasty (15) and submucosal periurethral collagen injection (11). Eight children underwent both procedures. Genito-urethroplasty and collagen injection were performed before BN reconstruction in 6 and 5 cases respectively, in order to increase bladder outlet resistance. The submucosal injection was performed at 3, 9 and 12 o'clock in the BN or sphincteric urethra, using 0.75 to 2.5 ml of cross-linked bovine collagen. Continence in the children who underwent the complete staged reconstruction was good in 58%, fair in 32% and poor in 11%. Following female genito-urethroplasty and periurethral collagen injection, bladder capacities increased by 25%. These complementary procedures are effective in increasing outlet resistance and bladder capacity in patients with exstrophy and/or epispadias; they may improve continence and lessen the need for further bladder augmentation.

During the period from March to September 1989, 40 children suffering from primary nocturnal enuresis, aged between 5 and 14 years, were included in a study to assess the comparative therapeutical efficacy of DDAVP and acupuncture. Children were divided into four groups of 10: group A was treated with DDAVP, group B was treated with acupuncture, group C was treated with DDAVP and acupuncture and group D was treated with placebo (control). The trial design included 3 periods: observation (2 weeks), treatment (8 weeks) and follow-up (4 weeks). Nineteen children completed the study. The efficacy of treatment, which was expressed as a percentage of dry nights, was high in both the DDAVP and acupuncture groups, when used separately. The combined treatment of DDAVP and acupuncture appeared to be the most efficacious both in terms of the percentage of dry nights at the end of treatment and in relation to the stability of results, even after the end of the study. The paper gives a detailed analysis of correlations between type of treatment and urinary osmolarity.

Spinal dysraphism represents a very rare congenital anomaly of the spinal cord and cauda equina, often associated with cutaneous sacral lesions. This condition results in distorsion of neural tissue, possibly leading to vesical dysfunction and urinary incontinence. A series of 12 children with spinal dysraphism (4 lipomeningoceles with tethered cord, 3 tight filum terminale, 2 dermal sinuses, 2 arachnoid cysts, 1 anterior meningocele) observed during the last 6 years is presented. The patients are divided into two groups: group A includes 5 infants (age range 4 months-2 years) and group B 7 older children (4-12 years). All 7 patients of group B were referred for urinary incontinence as their chief complaint; only 1 had evidence of a skin lesion while 3 had a club foot. In group A, 4 had skin lesions (2 asymptomatic and 2 dermal sinuses referred after several episodes of meningitis) and the 5th child had a club foot. The diagnosis was made by myelo-CT scan in the earlier 4 and by magnetic resonance imaging in the more recent 8 cases. All 12 children were operated. In group B, the patients' urinary incontinence persisted but did not worsen (3 were stable and 4 had slightly improved); in group A, 2 were dry (follow-up greater than 2 years), 1 could not be accurately evaluated because he was less than 2 years of age, and the 2 who suffered from meningitis were incontinent. Early diagnosis and neurosurgical treatment of spinal dysraphism may prevent urinary incontinence. Accurate urodynamic, neurophysiologic, and neuroradiologic evaluation of children with severe voiding anomalies or club foot is recommended even if no lumbosacral cutaneous lesions are present.

The authors report the results of a study on 20 children suffering from a particular type of enuresis, associated with bladder instability, characterized by uninhibited contractions of the detrusor muscle. The children selected showed symptoms of enuresis, frequency, urinary urgency and a positive urodynamic test. This test was performed on 11 patients before and after acupuncture. In 16 out of 20 children, the authors observed a net increase in the intensity and frequency of uninhibited bladder contractions 30 minutes after acupuncture. At 60 minutes the contractions decreased and at 24 hours they had practically disappeared. Clinically, a gradual elimination of enuresis has been observed in 11 cases and an improvement in the other 7. From these early observations, the efficiency of acupuncture in suppressing uninhibited bladder contractions seems to be proved, even though the therapeutic mechanism has yet to be clarified.

Urinary incontinence can only be considered a disease after a child has completely acquired spinal mechanisms of vesico-sphincteric nervous control. This normally happens for the majority of children between two and two-and-a-half years of age regarding voluntary daytime control, while complete night-time control is obtained in 90% of cases within the fourth year. By urinary incontinence we mean an involuntary leak of urine through the bladder neck. This can vary from an occasional leakage consisting of a few drops to a continuous and uncontrollable stream of urine. From 1981 to 1986 we evaluated, in the Pediatric Urological Service at Bambino Gesù Hospital, Rome, 426 children suffering from urinary incontinence. According to the etiology we divided the patients into two large groups: 1) patients with incontinence due to anatomic causes 56 (13%); 2) patients with incontinence due to dysfunctional causes 370 (87%).

The presentation of a rare case of leiomyoma of Meckel's diverticulum in a boy of 5 yr forms the starting point for a discussion of such cancers. In adult age, the complication is not so rare as the sporadic publications would suggest, incidence of tumoral forms being around 1 : 50 M.D. with a high malignancy frequency. In paediatric age, on the other hand, the tumoral degeneration of a M.D. is an exceptional occurrence: a review of more than 1,200 reported cases and personal experience (48 cases of M.D. in 15 yrs) confirm the rarity of the observation. The need to seek M.D. on each occasion and remove it to prevent the risk of tumoral degeneration in adult age is stressed. M.D. surgical removal tecniques are commented on and emphasis laid on the desirability of ample intestinal resection in cases of cancer of the diverticulum.

Prenatal ultrasound has set the stage for new trends in the diagnosis and treatment of many urinary tract malformations, in particular duplication of the renal collecting system with ureterocele. The authors have reviewed their experience with 41 ureteroceles in 7 years. The 36 patients were divided into two groups: those with prenatal diagnosis and subsequent early treatment (group A, 14 cases), and those with diagnosis later after birth (group B, 27 cases) showing various degrees of renal impairment as assessed by a 99Tc-diethylenetriamine pentaacetic acid scan. The surgical approach carried out on the patients of each group with particular regard to the endoscopic incision of the ureterocele as the treatment of choice, especially in neonatal period, is discussed.