Treatment of neurogenic bowel dysfunction using transanal irrigation: a multicenter Italian study.

STUDY DESIGN: Thirty-six patients with unsatisfactory treatment of neurogenic bowel dysfunctions (NBD) were enrolled from Spinal Units and Rehabilitation Centers in Italy. Treatment was for 3 weeks using a newly developed integrated system with an enema continence catheter for transanal irrigation (Peristeen, Coloplast A/S Kokkedal Denmark). OBJECTIVES: To evaluate the effects of Peristeen Anal Irrigation on NBD and patient quality of life (QoL). SETTING: Italy. METHODS: Lesion level, ambulatory status and hand functionality were determined in all patients. NBD symptoms and QoL were evaluated before and after treatment, using a specific questionnaire. Statistical analysis was performed using McNemar Test and Sign Test. RESULTS: Thirty-six patients were enrolled, and 32 patients completed the study. At the end of the treatment, 28.6% of patients reduced or eliminated their use of pharmaceuticals. Twenty-four patients became less dependent on their caregiver. There was a significant increase in patients' opinion of their intestinal functionality (P=0.001), QoL score (P=0.001) and their answers regarding their degree of satisfaction (P=0.001). A successful outcome was recorded for 68% of patients with fecal incontinence, and for 63% of patients with constipation. CONCLUSION: Peristeen Anal Irrigation is a simple therapeutic method for managing NBD and improving QoL. It should be considered as the treatment of choice for NBD, playing a role in the neurogenic bowel analogous to that of intermittent clean catheterization in bladder treatment.

Ureterovascular hydronephrosis in children: is pyeloplasty always necessary?

OBJECTIVE: To evaluate the efficacy of the vessel transposition technique in ureterovascular hydronephrosis in children. METHODS: Over a 25-year period, we treated 111 patients with 112 instances of ureterovascular hydronephrosis. In order to determine the obstructive effect of the vessels, we performed an intraoperative diuretic test. Using this approach, 61 patients judged to have only vascular pyeloureteral junction obstruction underwent vessel transposition. However, 50 patients in whom the intraoperative diuretic test proved doubtful needed pyeloplasty. RESULTS: Surgical success was achieved in 98% of the patients. Only 1 child treated by vessel transposition had an unsatisfactory outcome which necessitated a subsequent pyeloplasty for persistent hydronephrosis. This was due to a previously unrecognized intrinsic pyeloureteral junction obstruction. CONCLUSION: Based on our clinical experience, the intraoperative diuretic test has proven to be a safe and effective diagnostic tool in children with ureterovascular hydronephrosis. Its use may contribute to treating some cases of ureterovascular hydronephrosis without resorting to pyeloplasty.

Management of giant omphalocele by progressive external compression: case report.

The management of giant omphalocele remains a major surgical challenge. A staged approach is mandatory to achieve an uncomplicated reduction. The case of a child conservatively and successfully treated by progressive external compression of the herniated organs and viscera using an elastic bandaging is described here. The complete integration of the omphalocele content and the closure of the abdominal wall were obtained in 9 days. No ventilatory support was required. The procedure is easy, safe, effective and inexpensive. Large base and intact amniotic sac are prerequisites for feasibility.

[Non-palpable testis: modern diagnostic and therapeutic trends]. / II testicolo non palpabile: moderni orientamenti diagnostici e terapeutici.

Management of the nonpalpable testis is controversial and can be difficult as shown by the multiple modalities for evaluation and proposed treatment. In our opinion, laparoscopy is the ideal first step in the management of patients with a nonpalpable testis, having a great reliability in locating or confirming absence of an occult testis. Accurate preoperative assessment and localization will assist in selecting the appropriate surgical approach either laparoscopic, laparoscopic-assisted, or open procedure. Laparoscopy was performed on 74 patients with 86 nonpalpable testis. The technique has been demonstrated to be extremely safe and lead to diagnosis in 100% of cases. Surgical management of nonpalpable testis was directly performed by laparoscopy identifying intra-abdominal vanishing testis and locating an intra-abdominal or inguinal testis. The two-stage Fowler-Stephens orchiopexy was performed in 41 patients. The first stage consisted of laparoscopic clip ligation of the spermatic vessels. Of these, 32 underwent the second stage by vas-based orchiopexy; all testes, but one, showed a normal size and consistency. To conclude, we advocate the use of laparoscopy in all boys with nonpalpable testis.

[The non-palpable testis: modern diagnostic and therapeutic approaches]. / II testicolo non palpabile: moderni orientamenti diagnostici e terapeutici.

Management of the nonpalpable testis is controversial and can be difficult as shown by the multiple modalities for evaluation and proposed treatment. In our opinion, laparoscopy is the ideal first step in the management of patients with a nonpalpable testis, having a great reliability in locating or confirming absence of an occult testis. Accurate preoperative assessment and localization will assist in selecting the appropriate surgical approach either laparoscopic, laparoscopic-assisted, or open procedure. Laparoscopy was performed on 74 patients with 86 nonpalpable testis. The technique has been demonstrated to be extremely safe and lead to diagnosis in 100% of cases. Surgical management of nonpalpable testis was directly performed by laparoscopy identifying intra-abdominal vanishing testis and locating an intra-abdominal or inguinal testis. The two-stage Fowler-Stephens orchiopexy was performed in 41 patients. The first stage consisted of laparoscopic clip ligation of the spermatic vessels. Of these, 32 underwent the second stage by vas-based orchiopexy; all testes, but-one, showed a normal size and consistency. To conclude, we advocate the use of laparoscopy in all boys with nonpalpable testis.

[A flowmetric study after a Mathieu-Righini intervention in medial-distal hypospadias]. / Studio flussometrico dopo intervento di Mathieu-Righini nelle ipospadie medio-distali.

Uroflowmetric long-term data (mean follow-up 4.5 years) of 64 nonselected patients successfully treated for mid-distal hypospadias with Mathieu-Righini procedure were analyzed. None of our patients needed meatal dilation after primary procedure. Four patients (6.3%) presenting a plateau pattern and a maximum flow rate less than -2 standard deviations were considered to have meatal obstruction. These patients were successfully treated with meatoplasty. We conclude that functional characteristics of the neourethra are quite equivalent to a normal urethra in most of boys and adolescents after meatal based flap urethroplasty. On the other hand, in order to prevent the possibility of unknown strictures postoperative uroflowmetric controls are strongly recommended.

[Renin-dependent arterial hypertension in polycystic kidney operated on at a late period]. / Ipertensione arteriosa renino-dipendente in rene multicistico operato tardivamente.

A case of a 17 year-old male patient developing renin dependent hypertension 3 years after nephrectomy for multicystic dysplastic kidney is reported. The risks of arterial hypertension in adolescence and adulthood as well as malignancy strongly suggest, at least in our opinion, precocious prophylactic nephrectomy.

[Malignant sacrococcygeal teratoma: report of a case of "explosive" intrauterine growth]. / Teratoma maligno sacro-coccigeo a sviluppo addomino-pelvico: descrizione di un caso ad "esplosiva" crescita intrauterina.

An exceptional case of fetal sacrococcygeal teratoma with an exclusive abdominopelvic growth is described. The tumor was not detected on a "routine" ultrasound study obtained at 31 weeks of gestation showing findings of a severe congenital uropathy. It remained unrecognized until the 34 weeks of gestation when the mass began an explosive growth causing a rectouretral fistula. Despite appropriate obstetrical and surgical management the baby died 24 hours later because inadequate pulmonary maturity. The istological findings showing malignant elements such as embryonal carcinomas.

Evolution of urodynamic patterns in posterior urethral valves.

A retrospective, long-term urodynamic study was performed in order to follow the evolution of the urodynamic patterns in 55 unselected patients previously affected by posterior urethral valves (PUV). The mean maximum cystometric capacity (MCC) values progressively increased over time and, on long-term follow-up, were just above 2 standard deviations (SD). The mean compliance values were clearly reduced in the first urodynamic studies after valve ablation, and only after a mean of 5 years follow-up did they approach the lower limits of normal. The small compliance and capacity (SCC) group showed two trends of evolution: a more numerous subgroup tended toward progressive normalization while a second subgroup (<20%) showed mean compliance values below normal limits, with reduced (-2 SD) MCC persisting at long-term follow-up. The number of patients in this group decreased over time. In contrast, we were able to show a significant increase in patients with myogenic failure. In this group scheduled voiding using the Valsava maneuver in conjunction with a regimen of double or triple micturition was usually succesful in modifying the course, normalizing MCC, reducing residual urine, and also eliminating incontinence. Finally, initial urodynamic investigations in the fulguration and vesicostomy groups showed a much higher percentage of SCC bladders in the latter group (83.5% vs. 35%). However, at long-term examinations the urodynamic parameters were nearly identical in both groups, showing that temporary bladder defunctionalization does bot adversely affect future detrusor activity. No direct relationship between urodynamic abnormalities and renal insufficiency could be shown, however, the majority of patients with reduced glomerular filtration rates still showed urodynamic dysfunction at long-term follow-up. In the authors' opinion, serial urodynamic investigations in association with serial evaluation of the evolution of upper urinary tract and renal function are mandatory for correct PUV management and provide useful guidelines for avoiding incorrect treatment and obtaining better long-term results.

Evolution of upper urinary tract and renal function in patients with posterior urethral valves.

From January 1972 to June 1993, 166 patients with posterior urethral valves (PUV) were treated in our surgical department, 59 with a milder form of PUV (upper urinary tract [UUT] complication rate 29%) and 107 with a severer form (UUT complication rate 96.3%). Only the latter group was studied for long-term (mean 9.3 years) evaluation of the UUT and renal function. A temporary vesicostomy was the primary treatment in 25 patients. Indications for temporary diversion were very young age and/or low birth weight, severe and bilateral UUT complications, and severe renal damage. All the other patients were treated by primary endoscopic valve fulguration. After removal of the lower urinary tract obstruction, vesicorenal reflux (VRR) resolved spontaneously or was ameliorated in 59.2% of the renal units. Spontaneous normalization or evident amelioration were found at long-term follow-up in nearly 70% of dilated, non-refluxing ureters. Ureteral reimplantation was performed on 41 of the 202 dilated or refluxing ureters (surgical rate 20.3%). The surgical failure rate requiring reoperation was 5% (2/41). The prerequisite for successful reimplantation was a large-capacity, stable, and compliant bladder. Ten nephroureterectomies were carried out for unilateral, massive VRR and renal dysplasia; 1 late nephrectomy was performed for arterial hypertension. The evolution of renal function showed statistically significant overall improvement, which was more evident in patients diagnosed and treated in the 1st month of life (P = 0.000) than in those treated between 1 and 12 months (P = 0.004) or after 1 year of age (P = 0.025). Renal function considerably improved in the vesicostomy group (P = 0.000). Thirteen patients (12.4%) are now either dead (2) or have end-stage renal disease (6) or chronic renal insufficiency evolving toward end-stage renal disease (5); 5 of these 13 were treated by vesicostomy in the first days or months of life, and at presentation the glomerular filtration rate (GFR) was less than 25 ml/min . 1.73 m(2). Determination of basic GFR and, even more, functional renal reserve is relevant in predicting the long-term evolution. In the author's opinion, vesicostomy is the procedure of choice in very ill newborns or infants. Aggressive management with early surgical reconstruction is rarely justified, because frequently UUT complications resolve spontaneously or clearly improve, and their surgical treatment has limited and very precise indications.

Long-term (over 22 years) follow-up of four familial cases of Caroli's disease.

The authors report a long-term (over 22 years) follow-up of four familial cases, 1 brother and 3 sisters, affected by classic Caroli's disease. The male patient experienced acute cholangitis at the age of 6 and 1 of the 3 sisters at 12 years. The other two sisters were asymptomatic at the time of diagnosis. At the last control, all the patients were alive and have remained symptom-free for more than 22 years. Nevertheless, some data suggest a slowly evolving impairment of the disease even in the absence of a clinical expression. In this paper, the ways in which Caroli's disease is inherited and managed are also discussed.

[Hemangioendothelioma of the liver in the newborn: description of 2 cases]. / Emangioendotelioma del fegato in età neonatale: descrizione di due casi.

The infantile hemangioendothelioma of the liver is a rare benign vascular tumor that accounts for some 10% of all primary hepatic masses. Two cases were seen at the Department of Pediatric Surgery of Vicenza's Hospital in the last ten years. Both of patients were females and presented in neonatal period asymptomatic. The first patient was found to have hepatomegaly by pediatrician at six weeks of age, the second was an incidental finding following ultrasound neonatal screening. The Authors outline some difficulties met with differential diagnosis between benign versus malignant hepatic tumors. Explorative laparotomy was considered necessary in both the patients. Liver biopsy was the only surgical procedure performed in the nonresectable tumor. Treatment with corticosteroids finally proved to be successful to determine progressive involution of the tumor. In the resectable form, left hepatic lobectomy was successfully performed because of the progressive size increase of the tumor.

[Ureteral ectopy. The residual stump syndrome]. / Ectopia ureterale. La sindrome del moncone residuo.

Anatomy and symptoms of ureteral ectopy in childhood can greatly vary. In most cases the site of ectopies in the urethral tract, so that a complete excision of the distal ureter is difficult and may cause damage to the sphincteric structures. When lower ureterectomy is performed below iliac vessels, symptoms secondary to residual ectopic ureter are rare. In our experience, residual ureteral stumps were symptomatic in two cases, with continence and micturation disturbances, which were codified as "residual ureteral stump syndrome". Clinical features and treatment are herein reported.

[Valves of the anterior urethra without diverticulum. Description of 3 cases]. / Valvole dell'uretra anteriore senza diverticolo. Descrizione di tre casi.

Anterior urethral valves are rare entities, especially in case they are not associated to urethral diverticulum. Because of their obstructive effects on the urinary tract, they must be searched in case of clinical evidence of lower obstructive uropathy. Three cases of anterior urethral valves without urethral diverticulum are herein reported. Differential diagnosis with urethral diverticula, endoscopic features and treatment are discussed and emphasized.