Laparoscopic pyeloplasty in children: Is the outcome different in children under 2 years of age?

PURPOSE: To report the outcomes of a series of children younger than 2 years with ureteropelvic junction obstruction (UPJO) who underwent laparoscopic pyeloplasty (LP), compared to children older than 2 years. PATIENTS AND METHODS: Twenty-three children (18 male) with UPJO underwent 24 Anderson-Hynes transperitoneal LPs between March 2003 and July 2007. The diagnosis of UPJO was confirmed on renal sonography and diuretic renogram. Nine children were younger than 2 years (Group 1) and 14 were older (Group 2). One child had bilateral UPJO and underwent two non-simultaneous procedures. All children were investigated with postoperative diuretic renogram and renal sonography, and the results were statistically analysed. RESULTS: LP was feasible without conversions or intraoperative complications in all 24 cases. Median age in Groups 1 and 2 was 11 months (4-24) and 74 months (27-204), respectively, (P<0.00001). No differences were found between the groups regarding median operative time, complications, technical difficulties, discharge home and follow-up. All patients in both groups experienced resolution of symptoms or obstruction. CONCLUSIONS: The LP in children younger than 2 years is highly successful with a low-rate of complications, and has the same outcomes as in older children.

The laparoscopic management of intersex patients: the preferred approach.

UNLABELLED: In the world's largest series of patients with intersex treated by laparoscopy, authors from Sao Paulo found that this technique allowed easy identification and removal of gonads. They also found that other organs could be removed and genitoplasty performed. OBJECTIVE: To present possibly the largest series of the use of laparoscopy for treating intersex patients. PATIENTS AND METHODS: Fifty intersex patients (34 with male and two with female pseudohermaphroditism, nine with gonadal dysgenesis, four with true hermaphroditism, and one with complex hypospadias), aged 0.5-46 years (mean 18.3), underwent laparoscopy to remove gonads and/or ductal structures incompatible with the social gender, or for gonadal tumour or a potential risk for malignancy. When necessary, genitoplasty was performed concomitantly. RESULTS: At the laparoscopic evaluation, 10 gonads of six patients were absent, while four were identified as 'vanishing'; 72 gonads (46 dysgenetic, 17 normal testes, one normal ovary, one ovotestis, seven gonadoblastomas or dysgerminomas) were removed; two ovotestes were replaced in the scrotum after removing the ovarian segment, as was one normal testis. Twelve patients with a urogenital sinus had its vaginal component removed, 11 including a hysterectomy. Three of these patients had a combined perineal approach to complete its removal, together with masculinizing genitoplasty. There were no intraoperative complications or conversions; two patients had complications after surgery. CONCLUSIONS: Laparoscopy allows the straightforward identification and removal of gonads. All abnormal ductal structures must be removed, as this increases the chance of resecting unidentified gonads. Removing the uterus and vaginal component of the urogenital sinus in patients with male social sex is feasible, with low morbidity. Genitoplasty, according to the social sex, can be performed in the same procedure.

Comprehensive surgical treatment of prune belly syndrome: 17 years' experience with 32 patients.

OBJECTIVES: To describe the results of comprehensive surgical management of prune belly syndrome. METHODS: Thirty-two patients were evaluated and treated for abdominal and genitourinary abnormalities. The surgical procedure, including simultaneous abdominoplasty, bilateral orchiopexy, and urinary tract reconstruction, was performed according to individual needs. At follow-up, the urinary tract, abdominal, and genital anatomy and function were evaluated. RESULTS: The upper urinary tract anatomy and function were stabilized in 30 patients, and no vesicoureteral reflux was found postoperatively. The bladder drainage was adequate in 29 patients, and 4 presented with recurrent bacteriuria. Abdominoplasty improved flaccidity in 29 patients. Adequate testicular position and consistency were obtained in 54 testes. Complications included acute tubular necrosis (1 patient) and ureterovesical obstruction (3 patients). CONCLUSIONS: Individualized comprehensive surgical management of prune belly syndrome is effective, with an acceptable incidence of complications and excellent long-term results.

Clinical, hormonal, behavioral, and genetic characteristics of androgen insensitivity syndrome in a Brazilian cohort: five novel mutations in the androgen receptor gene.

Androgen insensitivity syndrome (AIS) is caused by mutations in the androgen receptor gene and is associated with a variety of phenotypes in 46,XY individuals, ranging from phenotypic women [complete form (CAIS)] to men with minor degrees of undervirilization or infertility [partial form (PAIS)]. We studied 32 subjects with male pseudohermaphroditism from 20 families (9 CAIS, 11 PAIS) with the following criteria for AIS: 46,XY karyotype, normal male basal and human chorionic gonadotropin-stimulated levels of serum testosterone and steroid precursors, gynecomastia at puberty, and, in prepubertal patients, a family history suggestive of X-linked inheritance. The entire coding region of the androgen receptor gene was analyzed, and mutations were found in all families with CAIS and in eight of 11 families with PAIS. Fifteen different mutations were identified, including five (S119X, T602P, L768V, I898F, and P904V) that have not been described previously. Detailed clinical and hormonal features were compared with genotype in 25 subjects with AIS and confirmed by mutational analysis. LH hormone levels and the LH x testosterone product were high in all postpubertal subjects with AIS. All subjects with PAIS maintained at postpubertal age the gender identity and social sex that was assigned to them in infancy, in contrast to other forms of pseudohermaphroditism.