Juxta-adrenal malignant schwannoma.

A case of malignant schwannoma occurring in an 11-year-old boy is described. To our knowledge, this is the third case of malignant juxta-adrenal schwannoma reported in the literature. It was misdiagnosed at the onset of the disease as a post-traumatic renal hematoma. Angiography revealed that the tumor derived its blood supply from collateral vessels of the aorta, splenic, and left gastric arteries. An analysis of the diagnosis, clinical course, and prognostic factors of this rare tumor is performed.

[Primary varicocele: an underestimated pathology in childhood and adolescence]. / Varicocele primitivo: una patologia sottostimata in età pediatrico-adolescenziale.

Idiopathic varicocele is a neglected entity in classic pediatric textbooks. Yet vast epidemiologic studies revealed that the incidence of varicocele gradually increases in patients between 10 and 15 years old and is comparable to that of the adult male population. Clinically, the pediatric varicocele is often associated with smaller and hypotrophic testis; histologically it also is associated with pathological and progressive changes of the testis. We report on the preliminary results of a personal epidemiologic study and a comparative follow-up to value the efficacy of surgical correction in two groups of adolescent and adult patients. Early recognition and treatment of a severe varicocele with testicular hypotrophy may help to prevent in adults the infertility associated with this condition.

[Cardiosurgical intervention in extracorporeal circulation and intellectual performance in childhood. Study and follow-up of 20 cases]. / Intervento cardiochirurgico in C.E.C. e performance intellettiva in età pediatrica. Studio e follow-up di 20 casi.

6 and 12 months before and after surgery in E.C.C., twenty subjects, at the age of development between 6 and 14 years, suffering from congenital heart disease, were subjected to psychometric observations with tests of level and others (wisc, raven, pm 38, rey, bender), in order to analyse the intellective level, specific mental capacities and eventual modifications in the mental and cognitive performances of the patients. No psychometric, statistically meaningful variations were generally noticed during the 3 observations. No meaningful correlations resulted between the duration of E.C.C. and psychometric observations. The patients subjected to the E.C.C. longer than the average of the total group generally didn't show any statistically meaningful variations in the scores of the 3 observations. To sum up the authors think that the experimental group showed and maintained before and after the cardiac surgery, a normal developing and maturative level and anyway that after the follow-up, no changes in the several intellective capacities of the patient were noticed.

[Current status of Senning's physiologic correction of transposition of the great vessels. Personal experience]. / Stato attuale della correzione fisiologica secondo Senning della trasposizione dei grossi vasi. Esperienza personale.

From January 1978 to December 1985, 70 children affected by TGA have been operated with the Senning operation. Among the 70 cases, 64 were simple TGA and 6 were TGA + VSD. The mean age at operation was 6 months (range 2 day - 3 years). Of 64 cases with simple TGA, two (respectively of 2 days and 40 days) died with an operative mortality rate of 3.1% of 6 cases with TGA and VSD, one died with a mortality rate of 16.6%. The total mortality was 4.2%. Since 1981 in a continuative series of 47 patients there were no deaths. Our current policy is now the following: after the diagnosis of simple TGA by ECHO, if the Rashkind septostomy is successful, we perform Senning operation over 3 months; if the Rashkind is unsuccessful, we give prostaglandin (PGE1) for a long period (25-30 days) and in case we do a Blalock-Hanlon septectomy. In conclusion we think that in our hands, the policy followed in the treatment of simple TGA is justified by the results achieved but this policy does not prevent us from using different techniques if the results are better.

[Current possibilities of the surgical correction of intersexual states]. / Le attuali possibilità di correzione chirurgica delle condizioni intersessuali.

The diagnosis of genital anomalies must be approached with the utmost care, for the results will decide the future sexual life of the patient. Before corrective surgery is performed, it is absolutely necessary to complete all endocrinologic and genetic studies. It is possible to predict with a high degree of confidence the hormonal and sexual development of intersexual children during puberty and thus to choose the "right" operation for a given patient. So occasionally situations will arise in which the sex that is selected for a child differs from its gonadal or chromosomal sex. In author's experience almost all genital anomalies can be surgically corrected with a very good cosmetic result. It is recognised that in a great number of cases surgery alone cannot be sufficient treatment, endocrinological and psychological management are equally important. It is current opinion that early corrective surgery must be advised.

[Anesthesiologic management of surgery in the 1st month of life. Review of the literature and clinical contribution]. / Condotta anestesiologica nella chirurgia del primo mese di vita. Revisione della letteratura e contributo clinico.

This paper reports observations on 160 patients, aged between 1 and 30 days, submitted to general anaesthesia for surgery in the period within 1/1/81 and 31/12/84, at the Pediatric Surgery Department - Vicenza Regional Hospital. First we mentioned the main anatomo-physiological features in newborns, and on the grounds of the most recent bibliography and of our own experience, we propose a general anaesthesia record, as much as possible univocal, still bearing in mind the difficulty of this piece of advice, related to the various neonatal malformations, to the very poor conditions, and sometimes to dramatic emergency. This record as come out to be as much as possible corresponding to the main physiopathological features in newborns and doesn't modify the weak balance in these young patients.

[Sound alarms and conditioning therapy in the treatment of childhood enuresis: a study of 180 cases]. / L'allarme sonoro e la terapia del condizionamento nel trattamento dell'enuresi infantile: uno studio su 180 casi.

The Authors present the study and results in 180 cases of infantile enuresis, treated with a new bell alarm associated to conditioning psychological treatment (toilet training, token economy, motivational counseling, urine stop exercises, etc.). The cases have been subdivided into the type of enuresis, age and sex. 140 (77.8%) subjects aged from 6 years to 16 years with primary enuresis, and 40 (22.2%) aged from 6 years to 15 years with secondary enuresis have been treated. The following results were obtained in the primary enuresis with this type of treatment after a follow-up of 6-12 months: permanent recovery in 85.71% of the cases, relapse after 6 months in 11.42%, improvement in 5% and failures in 9,29% of the cases. In secondary enuresis the following results were obtained: permanent recovery after 12 months in 75% of the cases, relapse after 6 months in 10%, improvement in 7.5% and failures in 17.5% of the cases. The differences in the results between the two types of enuresis with respect to the age of the cases up to 12 years are proved and relatively less with respect to the average time of treatment for the total remission. Regarding the sex of the cases no significant therapeutic answer was noted neither in the final results nor in the time of treatment in both types of enuresis. Finally, it is not the substitution of symptoms which are proved but the benefits of a general and psychological level.

[Trans-atrial correction of ventricular septal defects in infancy and childhood (author's transl)]. / Trattamento chirurgico dei difetti interventricolari per via trans-atriale in età pediatrica.

The Authors reviewed their surgical experience in correction of ventricular septal defects through tricuspid valve. 57 patients out of 59 operated for surgical closure of V.S.D. underwent this technique. All patients remained in sinus rhythm after surgical repair, 40% showed a right bundle block, and only in a case there was a right bundle block plus left anterior emiblock. Clinical conditions of all the patients greatly improved after intervention. Overall mortality in this series was 6.7%. The Authors conclude that trans-atrial approach is safe and advisable technique in surgical management of ventricular septal defects.

[Free autogenous muscle grafts in nine children with total and incontinence (author's transl)]. / Autotrapianto muscolare libero in nove bambini con incontinenza anale totale.

Nine children with total anal incontinence after surgery for anorectal agenesis were successfully operated on by free autogenous muscle transplantation. Skeletal muscles were first denervated and 3 weeks later transplanted to the anal region as a U--sling around the rectum, imitating the normal position and function of the puborectalis muscle. The grafts were placed in contact with the normal muscles of the region to make reinnervation possible. Eight children were completely evaluated after operation. Two years after surgical procedure six children were "continent" and only two were still incontinent, but improved. In these cases the indication for muscle transplantation was not quite correct. The return of the function of the muscle grafts completely changed the lives of these children. The results of this new surgical procedure are encouraging and invite further trials.

[Portal hypertension and bleeding esophageal varices in children. Esophageal transection with paraesophagogastric devascularization versus shunting procedures (author's transl)]. / Ipertensione portale e varici esofagee sanguinanti in età pediatrica: sezione esofagea e devascolarizzazione esofago-gastrica o "shunts" derivativi?

It is known that portasystemic shunts in the treatment of portal hypertension causes in the long term a high rate of complications in children. The most severe are encephalopathy, postoperative hepatic failure in intrahepatic hypertension and occlusion or poor functioning of the shunts. The incidence of the last mentioned complication is high in children, especially in those operated in their early years of life. The most severe danger of portal hypertension is the haemorrhage from bleeding esophageal varices. During the last few years the technique of esophagogastric devascularization with esophageal transection and ligation of varices (Sugiura's procedure) has given a very high percentage of success in the long term. The Authors report their experience with Sugiura's procedure in 9 children with portal hypertension (average age at operation of four and half years). In six cases there was a prehepatic and in the other three an intrahepatic hypertension. The follow-up varied from six months to seven and half years with an average of almost four years. In all the children the surgical treatment was successful with the complete disappearance of haemorrhage and esophageal varices. For their own experience and for Sugiura's long-term results, the Authors think that Sugiura's procedure offers the most effective alternative to portasystemic shunts in the surgical treatment of portal hypertension, especially in early childhood.

Ureteral reimplantation in children with neurogenic bladder.

The treatment of urologic complications from myelomeningocele and especially of vesico-renal reflux is a controversial problem. A series of 26 reimplanted ureters in 17 children, with good results in more than 85%, is reported. Ureteroneocystostomy, carried out with a few technical innovation, may represent a useful method for the treatment of vesico-renal reflux and obstruction of the uretero-vesical junction in neurogenic bladder associated with myelomeningocele. This surgical approach leads to the disappearance of the reflux, decrease of dilatation of the upper urinary tract and preservation of renal function in most cases; moreover, infection can be more easily controlled. Ureteral reimplantation should be preceded by periodic urethral dilatation, external transurethral sphincterotomy, and pharmacologic regulation in order to attempt to decrease urethral resistance. After successful surgery, it is possible to try to reeducate the bladder. Reimplantation should be preferred to permanent urinary diversion even if there is gross reflux.