Congenital mesoblastic nephroma: report of a Case with review of the most significant literature.

AIMS AND BACKGROUND: Congenital mesoblastic nephroma (CMN) is a rare pediatric tumor of the kidney with the highest peak of incidence during the first 3 postnatal months. It has previously been confused with Wilms' tumor (which, on the contrary, is rare during the first six months of age and is still considered a histogenetic congener). CMN almost always has a favourable prognosis. Therefore, CMN needs to be correctly diagnosed and differentiated from other pediatric renal neoplasms. Two morphological subtypes are currently distinguished histologically: the classical or leiomyomatous type and the atypical or cellular type. Mixed forms with a combination of the two patterns are also on record. Recurrence and even tumor-related death have been described in the literature and always related to the atypical form or to the mixed form, particularly in patients aged more than 3 months and in those cases in which the surgical removal was not complete. Opinions concerning post-surgical clinical management, especially in regard to adjuvant therapy, are not unanimous. METHODS: A case of CMN, predominantly of the classical histological subtype diagnosed in a baby with a follow-up of 6 years, is herein presented. The tumor was discovered at birth and surgically removed after one month. Since the tumor showed a high mitotic index (one of the characteristics of the cellular subtype) and the perirenal fat was focally involved with the tumor, the possibility of giving adjuvant chemotherapy was considered. Flow cytometric analysis was also performed which showed a diploid DNA content of neoplastic cells. RESULTS: The tumor was completely removed, surgical margins were free histologically, and no clear-cut histological features of the atypical subtype were noted. Flow cytometrically, it showed the euploid DNA content. Consequently no additional therapy was given. Six years after surgery the patient is developing well and is free of disease. He has regular follow-up examinations. CONCLUSIONS: CMN almost always pursues a benign clinical course if diagnosed under three months of age and if totally surgically excised independent of histological type. Criteria for management of atypical cases are not unanimous in regard to the benefit of additional therapy after surgery.

Female genito-urethroplasty and submucosal periurethral collagen injection as adjunctive procedures for continence in the exstrophy-epispadias complex. Preliminary results.

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.

[The treatment of nocturnal enuresis. A comparative study between desmopressin and acupuncture used alone or in combination]. / Trattamento dell'enuresi notturna. Studio comparativo tra desmopressina ed agopuntura, usate singolarmente o in associazione.

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.

Distal hypospadias repair by urethral sliding advancement and Y-V glanuloplasty.

Different techniques are available today for repairing distal (coronal and subcoronal) hypospadias but none is universally recommended. We developed the technique of a distal urethral advancement glanuloplasty operation that is specifically intended for distal hypospadias repair even with mild chordee. Mobilization of the distal urethra is performed for 1.0 to 1.5 cm. after subcoronal circumcision and a deep Y-shaped incision of the glans. The mobilized urethra is dorsally split and slid forward to the tip of the dart of the glans previously prepared. Glanuloplasty is performed using the 2 lateral flaps of glans tissue. From January 1987 to December 1989 we used this technique in 74 cases of distal hypospadias with mild or no chordee (patient age 18 months to 9 years, mean 3.5 years). A transurethral catheter was left indwelling for 3 to 4 days. Hospitalization time was 5 (plus or minus 1.5) days. Results after 4 to 40 months of followup are encouraging cosmetically and functionally. All patients were cured. In 3 cases (4%) meatal stenoses occurred requiring meatotomy and in 8 boys meatal dilations were performed on an outpatient basis. In 1 case (1.3%) a fistula developed, which was subsequently repaired without further complications. This operation may be successfully used in most cases of distal hypospadias, with a low complication rate and excellent cosmetic results. It also may be used if mild chordee is present, thus reducing the indications for flip-flap urethroplasty in those cases when meatal advancement procedures may not be effective.

[Prenatal diagnosis of anatomo-pathologic aspects of 2 cases of sirenomelia]. / Diagnosi prenatale e quadro anatomopatologico di 2 casi di sirenomelia.

The antenatal echographic diagnostic aspects and anatomopathological features of two foetuses suffering from sirenomelia, or caudal pole regression syndrome, are examined. Conduction of the two cases is compared. In the first case, observed at the 22nd week, antenatal diagnosis of sirenomelia led to the therapeutic interruption of pregnancy. The second case, seen close to term, closed with the birth of an already dead sirenomelic foetus.

Bladder instability and enuresis treated by acupuncture and electro-therapeutics: early urodynamic observations.

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 in children: etiopathogenesis and clinical aspects]. / L'incontinenza urinaria nelle bambine: etiopatogenesi e clinica.

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%).