Abdominoplasty in Prune Belly Syndrome.

INTRODUCTION: Many patients with Prune Belly Syndrome (PBS) require abdominoplasty alone or in combination with correction of any urogenital abnormalities. This video presents a simplified technique with which to treat the abdominal flaccidity in PBS. METHODS: A longitudinal xypho-pubic fusiform figure is drawn on the abdomen, based on the area of skin and subcutaneous tissue to be removed. This is performed with preservation of the musculo-fascial layer and the umbilicus. A lateral elliptical single xypho-pubic line is drawn in the most lax side of the fascia, which is incised along this line. After urinary tract reconstruction and orchidopexy, closure is initiated by suturing the medial edge of the wider fascial flap laterally to the peritoneal side of the contralateral flap. Next, the now outer fascial flap is laid over the inner flap, and a buttonhole is made to expose the umbilicus. The subcutaneous tissue of the inner flap is laterally undermined to gain extra distance for the suture of the outer flap over the inner flap. The subcutaneous tissue and skin are sutured in the midline, incorporating the umbilicus. RESULTS: In a 30-year period, 43 PBS patients underwent this procedure with good cosmetic and long-term functional results. CONCLUSION: This abdominoplasty technique is simple and presents good functional and cosmetic results in PBS patients.

Diagnostic work-up and laparoscopic correction of an ectopic ureter.

INTRODUCTION: A duplex renal collecting system is a common congenital anomaly in children. Continuous dribbling (especially if after the toilet-training period) should raise suspicion of the presence of an ectopic ureter, which is most often associated with ureteral duplication. This video will demonstrate the complete diagnostic work-up necessary in these cases. CASE REPORT: A 10-year-old girl presented with continuous dribbling. Ultrasonography and computerized tomography depicted a duplex system on the left side, with the upper pole ureter ectopically inserting into the vaginal cavity and good upper pole renal parenchyma. A careful urethrocystoscopy showed a topic right ureteral orifice and a topic lower pole left ureteral orifice. Retrograde pyelography was performed and displayed normal left lower pole anatomy. A vaginography was performed, which showed reflux to the ectopic ureter. Vaginoscopy clearly identified the ectopic ureteral orifice. A guide wire was introduced through this meatus and retrograde contrast injection confirmed the diagnosis of an ectopic ureter. RESULTS: At laparoscopy, a larger upper pole ureter and a normal lower pole ureter on the left side were identified. A termino-lateral ureteroureteral anastomosis was performed. After the procedure, the child reported immediate resolution of urinary dribbling. CONCLUSION: In order to optimize its surgical correction, efforts should be made to appropriate localization of the ectopic ureter.

New tissue bulking agent (polyacrylate polyalcohol) for treating vesicoureteral reflux: preliminary results in children.

PURPOSE: We report the preliminary results of endoscopic treatment of vesicoureteral reflux in children using polyacrylate polyalcohol copolymer. MATERIAL AND METHODS: We performed a prospective multicenter review of pediatric patients treated with subureteral injection of a new nonabsorbable substance. Only patients with 1 year of followup were included. RESULTS: A total of 83 patients underwent injection of polyacrylate polyalcohol copolymer at our institutions between 2005 and 2006. Among this group 18 males and 43 females with a median age of 58 months (range 9 months to 18 years) completed 1 year of followup. Reflux was bilateral in 27 patients (44.3%) and unilateral in 34 (55.7%). Number of injected ureters was 88. Reflux grade was V in 3 ureters (3.4%), IV in 12 (13.6%), III in 41 (46.6%) and II in 32 (36.4%). Mean +/- SD injected volume per unit was 0.76 +/- 0.43 ml. Median followup was 20 months (range 16 to 24). Complications after injection included dysuria in 6 patients (9.8%), fever in 3 (4.9%) and lumbar pain in 4 (6.6%). Reflux was eliminated in 78 renal units (88.6%), decreased to grade I in 6 (6.8%) and persisted in 4 (4.5%). Ureteral obstruction developed in 1 patient and was treated operatively. Overall success rate was 83.6%. CONCLUSIONS: Polyacrylate polyalcohol copolymer can be used to treat vesicoureteral reflux with comparable efficacy to other substances currently used, with a low rate of complications.

Comparison of videolaparoscopic versus open surgery for benign renal diseases in children.

INTRODUCTION: Laparoscopic nephrectomy in children has become a reasonable alternative to open nephrectomy and has replaced open surgery for many kidney diseases. The aim of our study is to evaluate transperitoneal videolaparoscopic procedures in benign renal disease compared with the open surgical approach. PATIENTS AND METHODS: 34 children aged between 17 days and 15 years (mean, 6.14 years) were divided into two nephrectomy groups. The first underwent transperitoneal videolaparoscopic nephrectomy and was composed of 21 patients (12 female and 9 male) aged between 2 months and 15 years (mean, 7.42 years). The second group underwent open nephrectomy and was composed of 13 patients (6 female and 7 male) aged between 17 days and 11 years (mean, 3.91 years). The groups were compared for time of anaesthesia, operating time, length of hospital stay, postoperative pain and time to restore oral intake. Short and long term complications were also evaluated. Statistical analysis was performed by a Student's t test, with a p value < 0.05 being considered significant. The study was approved beforehand by the Scientific Ethics Committee in our institution. RESULTS: Statistically significant differences were observed only for the length of hospital stay. No cases in the laparoscopic group were converted into open surgery. There were no immediate or late complications. Blood loss was negligible and so it was not necessary to administer any transfusions. CONCLUSIONS: In our experience, transperitoneal videolaparoscopic nephrectomy has similar results to those of open nephrectomy, except for hospitalisation times.

Laparoscopic management of intersexual states.

Laparoscopy has gained acceptance as the ideal method of surgical treatment of the internal [figure: see text] genital organs in patients with intersex disorders. The intersexual states for which laparoscopy is needed are female and male pseudohermaphroditism, true hermaphroditism, and Turner's syndrome. In these patients, the indications for laparoscopy are the removal of normal gonads and ductal structures that are contrary to the assigned gender and the removal of dysgenetic gonads that are nonfunctional and that present potential for malignancy. In addition to being a minimally invasive surgery, one of the main advantages of this method is the lack of scars, a fact much appreciated by patients and their parents. Generally, gonadectomy is a straightforward operation because the gonads present with an accessible pedicle. Laparoscopic orchidopexy has been standardized and can be performed in patients in whom the testis must be relocated to the scrotum. The removal of ductal structures is also easily performed in most cases, whereas hysterectomy with resection of the vagina may present some difficulties owing to the location of these structures. In patients with a long vaginal component of the urogenital sinus, the distal segment must be removed by a retrograde perineal access, usually performed simultaneously with genitoplasty. Endocrinologists must be aware of the application of this method of treatment in intersex patients, and urologists proficient in laparoscopic techniques must extend their field of work in this area.

Male pseudohermaphroditism due to 17 beta-hydroxysteroid dehydrogenase 3 deficiency. Diagnosis, psychological evaluation, and management.

Ten male pseudohermaphrodites with 17 beta-hydroxysteroid dehydrogenase 3 (17 beta-HSD3) deficiency were evaluated in 1 clinic with an average follow-up of 10.1 years. The diagnoses were made by demonstrating low to normal serum testosterone levels, high androstenedione levels, and high ratios of serum androstenedione to testosterone in the basal state or after treatment with human chorionic gonadotropin. The molecular features of the underlying mutations were identified in all 7 families. Two additional males in the same families are believed to be affected on the basis of history obtained from family members. All of the 46,XY individuals in these families were registered at birth and raised as females (despite the presence of ambiguous genitalia in all or most), and all virilized after the time of expected puberty due to a rise in serum testosterone to or toward the normal male range. The age at diagnosis varied from 4 to 37 years. Ten individuals were studied by the same psychologist, and change of gender role (social sex) from female to male occurred in 3 subjects and in the 2 presumed affected subjects not studied. The individual with the highest serum testosterone level maintained female sexual identity, and in 2 families some of the affected males changed gender role and others did not. Thus, while androgen action plays a role in the process, additional undefined psychological, social, and/or biologic factors must be determinants of gender identity/role behavior. Management of the 7 individuals who chose to maintain female sex roles included castration, clitoroplasty, vaginal enlargement procedures when appropriate, treatment of hirsutism, cricoid cartilage reduction, and estrogen replacement. Three of the 7 are married (2 twice), 1 is involved in a long-term heterosexual relationship, 1 is engaged to be married, and the other 2 are not married and not believed to be sexually active. The 3 subjects who changed gender role behavior to male underwent hypospadias repair, and 1 was given supplemental testosterone therapy. One of these men is divorced, and the other 2 (aged 29 and 35 years) are unmarried. The diagnosis in 8 of these subjects was made after the time of expected puberty; it is unclear whether the functional and social outcomes would have been different if the diagnosis had been made and therapy begun earlier in life.

Pheochromocytoma treated by laparoscopic surgery.

OBJECTIVE: To evaluate the results of the laparoscopic technique in the treatment of adrenal pheochromocytoma. METHOD: Ten patients, 7 men and 3 women, between 10 and 67 years of age (mean 48) with pheochromocytoma underwent transperitoneal laparoscopic adrenalectomy and were evaluated retrospectively, based on clinical, laboratory, and pathological diagnosis. In all cases there was a solid unilateral adrenal tumor, 5 on the left side and 5 on the right side, whose greater diameter varied from 7 to 80 mm (mean 32). Nine of the 10 patients were chronically hypertensive or had already had hypertensive crises. One patient was normotensive, but presented metabolic alterations suggestive of adrenergic hyperfunction. RESULTS: No deaths occurred in this series. There were two (20%) conversions to open surgery, one due to venous bleeding and one due to the difficulty of dissection behind the vena cava in a patient presenting a partially retro-caval tumor. Surgical time in the 8 non-converted cases ranged from 70 to 215 minutes (mean 136). One patient (10%) received blood transfusion, and another (10%) presented two complications - acute renal failure and a subcutaneous infection. Both had been converted to open surgery. None of the non-converted cases was transfused or presented complications. Hospital discharge occurred between the 2nd and 11th post-operative day (mean 3). The pathological exam of the surgical specimens confirmed the diagnoses of pheochromocytoma in all 10 cases, one of them associated with an aldosterone-producing cortical tumor. CONCLUSIONS: Laparoscopic adrenalectomy for selected patients presenting pheochromocytoma is feasible and provides good results.

[Vesicoureteral reflux in children]. / Refluxo vésico-ureteral em crianças.

The association between vesicoureteral reflux and urinary tract infection represents a significant threat to the kidney, in the form of pyelonephritic scarring. The physiopathologic events that allow the upward migration of bacteria and their inoculation in the renal tissue, causing irreversible damage if not adequately treated, are reasonably understood. Reflux nephropathy is a major cause of childhood hypertension and renal insufficiency. Early diagnosis of reflux, and identification of the pyelonephritic aggression are important for successful therapy. Long-term chemoprophylaxis,to prevent urinary tract infection and renal scarring is the adequate form of treating low-grade vesicoureteric reflux, since spontaneous cure is expected in most of these cases. Although the same approach can be employed in high-grade reflux, surgery is a favorable alternative, with high rates of primary success,reducing the need of long-term medication and the exposure of the kidney to recurring pyelonephritic aggression. The retrograde flow of urine from the bladder to the upper urinary tract is an abnormal situation in the human being, known as vesicoureteral reflux (VUR). It results either from an intrinsic anatomical deficiency of the vesicoureteral junction or from an increased bladder pressure, due to mechanical or dysfunctional vesicourethral obstruction. The recognition of the association between VUR, urinary tract infection (UTI) and renal scars has led to an increased study of this disease in the last two decades.

Transureterocalicostomy: an alternative internal diversion technique.

We report a case of an infiltrating retroperitoneal tumor that completely enveloped and obstructed the mid third of the left ureter. After resection of the tumor, including a 10 cm. long segment of ureter, the upper left ureter was successfully diverted to the upper calix of the right ectopic kidney (transureterocalicostomy). To our knowledge our report represents the first case of such surgery.