A non-catecholamine-producing sympathetic paraganglioma of the spermatic cord: the importance of performing candidate gene mutation analysis.

BACKGROUND: Catecholamine-producing tumours are called pheochromocytomas when they are located in the adrenal gland and sympathetic paragangliomas when they are located elsewhere in the abdomen. Rarely these tumours do not produce catecholamines and even more rarely they arise in the spermatic cord. Over the past decade, systematic mutation analysis of apparently sporadic cases of pheochromocytomas and paragangliomas has elucidated the frequent presence of germ line mutations in one of five candidate genes, including RET, VHL, SDHB, SDHC, and SDHD. CLINICAL HISTORY AND METHODS: We describe a 45-year-old man with a non catecholamine-producing paraganglioma of the spermatic cord. We performed SDHB immunohistochemistry and performed mutation analysis of the SDHB, SDHC, and SDHD genes. RESULTS: There was no staining of tumour cells with SDHB immunohistochemistry, indicative of an SDH mutation. Mutation analysis demonstrated a germ line SDHD mutation (p.Val147Met). CONCLUSIONS: Systematic mutation analysis is required in paraganglioma patients for the detection of germ line mutations. This should be preceded by SDHB immunohistochemistry to limit the number of genes to be tested.

Pyeloureteritis cystica.

Pyeloureteritis cystica is a rare situation of the renal pelvis and ureters, characterized by cystical formations of the epithelium. The etiology is unknown and there is no specific treatment. It is usually diagnosed accidently during imaging of the upper urinary tract for different reasons. We present a case of pyeloureteritis cystica.

Shockwave lithotripsy and endourological management of urinary calculi in children: a single-center 10-year experience.

PURPOSE: Shock wave lithotripsy (SWL) and endourological techniques revolutionized the management of pediatric urolithiasis. We sought to assess the impact of new technology and local practice in the treatment of pediatric urolithiasis during a 10-year period. MATERIALS AND METHODS: Between 1997 and 2006, 125 children (90 boys and 35 girls), aged 18 months to 15 years, were managed in our department for urolithiasis. Stone localization, stone composition, presence of anatomic abnormalities, and treatment modality were evaluated retrospectively. RESULTS: In 102 children, the stone was located in renal pelvis (0.5-45-mm diameter), in eight in the renal pelvis and one in the calyx; three had staghorn calculi; and 12 had ureteral stone (4-12-mm diameter). Ninety-three of 125 children underwent a total of 108 SWL sessions. Stone size ranged from 0.5 to 35 mm. The stone-free rates were 86%, 92%, and 96% after first, second, and third SWL session, respectively. Ureteroscopy was performed in 12/125 children, and 10/12 (83.5%) were rendered stone free. Nine of 125 children underwent percutaneous nephrolithotomy, and four of nine were stone free (44.5%), although five of the nine children (55.5%) required SWL for residual stone fragments. Open surgery was performed as initial procedure in 11 (9%) children. In 9 of 11 children, ureteropelvic junction obstruction was corrected simultaneously. Open surgery was followed by SWL in 3 of 11 patients. Two of three patients with staghorn calculi underwent nephrolithotomy and SWL and one of three with cysteinuria was managed with SWL. CONCLUSIONS: SWL and endourological techniques are safe and effective in managing urolithiasis in pediatric patients. These minimally invasive methods reduced dramatically the cases of open surgery, which should be undertaken mainly in coexisting anatomic abnormalities.

Transvaginal vs transobturator approach for synthetic sling placement in patients with stress urinary incontinence.

We present comparative short-term experience with the transvaginal (TVT) and the transobturator (TVT-O) approaches for the treatment of stress urinary incontinence (SUI). We studied 315 women with SUI, treated with a tension-free tape placement. The TVT approach was applied in 265 women, while 50 women were treated by the TVT-O approach. The mean operation time was 25 and 17 min in the TVT and TVT-O group, respectively (p<0.001). In the TVT group, continence rates were 87% after 1 year, while in the TVT-O group, the continence rate was 94%. Postoperative pain was reported in 14.4 and 28% of the TVT and TVT-O patients, respectively (p=0.02). Complications such as bladder perforation, retropubic hematoma, and urinary retention took place only in the TVT group. Urinary tract infections were recorded in 20 and 8% of the TVT and TVT-O patients, respectively (p=0.04), while vaginal erosion took place in 1.5 and 2% and de novo urgency in 14 and 8%. Both approaches show high rates of cure at the first postoperative year, while complications are less with the TVT-O procedure.

Tension-free vaginal tape for the treatment of stress urinary incontinence in geriatric patients.

OBJECTIVE: To evaluate the safety and efficacy of tension-free vaginal tape (TVT) for the treatment of stress urinary incontinence (SUI) in geriatric patients. PATIENTS AND METHODS: Fifty-one women, aged 65-80 (mean 72.3) years, underwent a TVT procedure for genuine SUI from 2001 to 2004. A urodynamic test together with uroflowmetry were performed. The patients' SUI bother score was assessed using a visual analog scale (VAS). RESULTS: The mean operative time was 25 (range 14-29) min and mean hospitalization time was 1.2 (range 1-2) days. Bladder perforation occurred in 3 cases (5.8%) and was managed conservatively. Pre- and postoperative maximum flow rate were not significantly different (p > 0.05). The patients' SUI bother score estimated by the VAS was statistically significantly improved (p < 0.0001). After a mean follow-up of 35.6 (range 14-60) months, 49 patients (96%) had no SUI, while 2 patients (3.9%) had persistent SUI. Also, during the follow-up 5 patients (9.8%) were diagnosed with de novo urgency due to detrusor overactivity and 1 patient (1.9%) had persistent dysuria that was resolved with urethrolysis. CONCLUSIONS: SUI in elderly women can be safely treated with the TVT procedure in the vast majority of the patients. However, bladder perforation during surgery and de novo urgency postoperatively should be taken into account.

Continent urinary diversion using Mainz II modified ureterosigmoidostomy.

OBJECTIVE: To assess the surgical technique of Mainz II urinary diversion and to discuss its simplicity, quickness of performance, short postoperative period, few postoperative complications, as well as its effectiveness allowing patients to have a very good quality of life. PATIENTS AND METHODS: Over the last six and a half years, 47 patients aged between 65 and 76 years (mean age 69.9 +/- 2.5 years) underwent radical cystectomy and Mainz II ureterosigmoidostomy for invasive bladder cancer. All patients were followed according to a standard protocol including assessment of continence, renal function and acid-base balance. RESULTS: Most of the patients retained normal renal function, complete continence and acid-base balance. There were no perioperative complications. In three patients there was urine leakage. Four patients presented with pyelonephritis and needed hospitalization. Mild hyperchloraemic acidosis was seen in nine patients. Most of them were pleased from their new quality of life. CONCLUSION: The modified Mainz II ureterosigmoidostomy is a simple, quick, easy and safe procedure to achieve urinary diversion in invasive bladder cancer resulting in a very good quality of life for patients without altering their appearance and making them capable of performing all kinds of work.

Management of nocturnal enuresis in Greek children.

Our experiences of managing nocturnal enuresis in Greek children at our Outpatient Clinics of Pediatric Urology are described. Between March 2001 and October 2003, 142 children with primary nocturnal enuresis (93 boys and 49 girls), aged 7-18 years old (mean: 9.0+/-0.5) were included in this prospective study. Initially, behavioral conditioning therapy, using a body-worn urinary alarm, was instructed in all cases. If no improvement was recorded, 40 microg of intranasal desmopressin was administered, initially for three months. If urodynamic studies demonstrated pure detrusor instability, anticholinergics (5 mg oxybutinine or 2 mg tolterodine) were given instead. Combination medication (desmopressin and anticholinergics) was administered for coexisting diurnal enuresis, which was present in 8 children. Among the 142 children the overall response rate was 51.41%. Successful response was recorded in 16 children practicing conditioning behavioral therapy, in 47 receiving desmopressin (with or without anticholinergics), and in 10 children receiving only anticholinergics. During the follow-up period (mean: 6.2 months), no serious side effect was recorded. The use of desmopressin, and anticholinergics in specific subgroups, was found to be effective and safe for the management of nocturnal enuresis in children.

Nocturnal enuresis in children. A four-year experience in outpatient clinics of pediatric urology.

AIM: We present our experience in the treatment of enuresis at the Pediatric Urology Outpatient Office over a period of four years. We report pertinent epidemiological data, diagnostic workup, as well as routine treatment protocol. MATERIAL AND METHODS: Between April 1998 and May 2002, 142 healthy children, aged between 6.5 and 18 years (mean: 9 +/- 0.5 years), were referred to us for bedwetting. Ninety three of them were boys and 49--girls. Eight of them had also concurrent daytime enuresis. According to our protocol, the type of enuresis was identified (primary or secondary) and then we administered the respective treatment. Sixteen children underwent behavioural therapy only. Fifteen children with detrusor instability received oxybutinine or tolterodine. Twenty children with diurnal and nocturnal enuresis were given desmopressin and oxybutinine or desmopressin and tolterodine. The remaining 91 children received monotherapy with desmopressin (individualized dose). The initial follow up ranged from 3 to 6 months. RESULTS: Out of 111 children receiving desmopressin, 66 stopped wetting, but 28 relapsed in two weeks and treatment continued for 3 more months. Nine children became dry. In the other groups there was almost complete response to treatment. CONCLUSION: Enuresis continues to be a suppressed problem for both children and parents; however, effective treatment is possible.

Percutaneous drainage of simple cysts of the kidney: a new method.

OBJECTIVE: The experience of our department on the treatment of solitary simple kidney cysts with continuous percutaneous drainage for 24 h and instillation of pure alcohol as a sclerotic agent is reported. The results are compared with those of previous years when the treatment consisted of percutaneous drainage and injection of pure alcohol. PATIENTS AND METHODS: During the period 1992-2001, 252 patients (136 male, 116 female; aged 22-74 years) were treated at our department, and all had a solitary kidney cyst. They were treated by percutaneous drainage and then injection of pure alcohol for 20 min via a nephrostomy tube (which remained in position for 24 h). The mean follow-up period was 5 years. This cohort of patients was compared to another one of 238 patients who were treated with a previous method (126 male, 124 female; aged 28-79 years, mean 59 years). RESULTS: In 71% of the patients the symptoms and the cyst both disappeared, in 22% there was no significant recurrence (i.e. cyst diameter <5 cm), and the remaining 7% presented significant recurrence (i.e. cyst diameter >5 cm). Most of the latter cases were treated again using the same method. Of the 73 patients with impaired kidney function, in 61 (83%) this appeared to have improved as a result of our treatment. Of the 61 patients with hypertension, in 29 (47%) this appeared to have improved. There was only 1 case with complications, which presented purulence of the cyst that required open surgery. In previous case series, which were treated with percutaneous drainage and injection of pure alcohol just after the puncture of the cyst--without continuous drainage of the cyst for 24 h--only 10% of the patients had no recurrence, 30% had no significant recurrence (volume of the cyst <20% of the pre-operative volume), and 60% of the patients had significant recurrence (volume of the cyst >20% of the pre-operative volume). CONCLUSIONS: Percutaneous drainage of solitary kidney cysts for 24 h followed by injection of pure alcohol, as a sclerotic agent, is an effective therapeutic method with only a few complications. It was therefore considered being the proffered method for the treatment of solitary kidney cysts.

Testicular tumors manifested as inferior vena cava thromboses. Case reports.

Four cases of asymptomatic testicular tumors with inferior vena cava (IVC) involvement are reported. All patients were referred to our hospital with non-specific abdominal pain and abdominal US revealed an inferior vena cava thrombosis. CT showed an IVC thrombus extending from the first to second lumbar vertebrae and also retroperitoneal lymphadenopathy in 3 patients. Scrotal US demonstrated intratesticular tumors. IVC thrombosis may result from asymptomatic intratesticular tumors (single or multiple). Because of that, scrotal US is of paramount importance as a routine screening test in patients who radiographically demonstrate caval thrombosis.

Idiopathic retroperitoneal fibrosis: a long-term follow-up study.

Eleven patients (8 male) with idiopathic retroperitoneal fibrosis (IRPF) were reviewed. The mean age of the patients was 44 years. Five patients (group 1) who had moderate obstruction were treated only by steroids. Six patients (group 2) who presented with severe obstructive uropathy (and/or serious metabolic disturbances) were treated with a combination of surgery and steroid administration. Unilateral or bilateral ureterolysis was performed in 5 patients. It was associated with nephrostomy in 2 cases and intraperitoneal disposition of the ureters in 1 case. Left nephrostomy without ureterolysis was performed in 1 patient. The mean follow-up period was 5.5 years (5 months to 20 years). All patients of group 1 now have normal renal function. In 5 patients of group 2, renal function improved significantly after operation; one of them was started on regular dialysis 16 years later. In another patient, IRPF recurred in the ureter of a living related renal graft 6 months after transplantation. In conclusion, steroid treatment alone offers a long-term survival in patients with IRPF of moderate severity. Combined treatment must be recommended for patients who present with severe obstruction and advanced uremia.