Surgical treatment of cloacal anomalies.

From 1989-1998 14 patients were treated with cloacal anomalies: 5 typical cloacas (PC), 5 posterior cloacas, and 4 cloacal exstrophies (CE); 12 underwent surgery. Four typical cloacas were resolved with posterior sagittal anorectovagino-urethroplasty (PSARVUP), whereas in the 5th total urogenital mobilization (TUM) was used. Three PCs were managed with transanorectal TUM and 2 with anterior TUM without opening the anal canal and rectum (without a protective colostomy). Two CEs were treated with atypical procedures. Two patients with CE died without surgery and 2 died after surgery due to complex associated anomalies. During postoperative follow-up of 1-8 years, 5 children had voluntary bowel movements and no soiling while the others had soiling with or without enemas; 1 had stress incontinence; 3 were on clean intermittent catheterization due to neurogenic bladder and were dry. PSARVUP provides a satisfactory result if there is no sacral anomaly. TUM makes this operation easier to perform. In patients with a PC it is sometimes possible using TUM to separate the urinary from the genital tract and remove the accessory urethra without opening the anus and rectum.

Surgical treatment of the Müllerian duct remnants.

BACKGROUND: Persistent Müllerian duct tissue in male individuals may result in an enlarged prostatic utricle (utricular cysts and utricle) or a Müllerian duct cysts, either distinctively or synonymously. In intersex patients Müllerian duct remnants (MDR) are an usual occurrence. Surgical excision is the definitive treatment of symptomatic remnants, as well as during the reconstruction of intersexual genitalia. Many approaches have been described. The authors review their experience in intersex patients. METHODS: From 1986 to 1999, the authors treated 111 patients with intersex disorders. The records of 47 patients raised as boys with MDR were reviewed. Based on the symptoms and the size of the remnants, in 32 patients the structures were removed. In 13 patients extirpation was done by perineal approach, in 9 by transperitoneal approach, and in the remaining 9 patients the combined abdominal and perineal approach were undertaken. In one patient the large prostatic utricle was extirpated by a posterior sagittal pararectal approach. Perineal approach was mainly used in younger asymptomatic children, with the prostatic utricle disclosed incidentally during genitography because of intersex disorders. Operation was performed only in cases in which the prostatic utricle was observed by cystoscopy or identified by Fogarty balloon catheter introduction into the prostatic utricle. In older patients these structures were discovered frequently after failed urethroplasty, or after symptoms of urinary infection, urinary retention, or epididymitis. We elected to use the transperitoneal approach based on the extension of these structures into the pelvis. The average age of patients at the time of surgery was 8.6 years, with a range of 1 to 30 years. RESULTS: There were no rectal or bladder injuries during surgery. An older patient had temporary impotence after abdomino-perineal extirpation. The lack of ejaculation, seen in 5 patients, was related to frequent intra-utricular termination of the vas deferens. Posterior sagittal pararectal approach certainly enabled complete exposure and exact visualization of all structures, with considerably decreased bleeding. If gonadal biopsy or gonadectomy were necessary, the transperitoneal approach could not be avoided. CONCLUSIONS: Surgical treatment of MDR in intersex patients varies according to the size of the utricle, and a double approach is often necessary. A high degree of success may be achieved with minimal morbidity. J Pediatr Surg 36:870-876.

True hermaphroditism: 10 years' experience.

True hermaphroditism (TH) is the rarest variant of intersex malformations. By definition, these patients have both ovarian and testicular tissue. Most present due to ambiguous genitalia and/or gynecomastia. From 1986 to 1996, we treated 97 patients with intersex disorders; there were 4 with TH. In all cases testicular and ovarian tissue was separate. In 3 patients the external genitalia were ambiguous and 1 had hypoplastic male genitalia. Three patients had a 46, XY and 1 a 46, XX karyotype. Three patients had been listed as males and 1 as a female. The number of operations required varied from 3 to 9 (mean 5.7 per patient). Two patients who had been raised as males finished puberty with hypogenitalism and hypoplastic testes. One married and could have erections, but no ejaculation. Two male patients have been on supplementary androgen therapy. The only patient who had been raised as a female after clitorovaginoplasty had a 6-cm-deep, blind-ending vagina that was deepened to 12 cm with bougienage, so that she was capable of having normal intercourse. It is felt at present that most patients with TH should be raised as females. This was confirmed in our patients as well, which underlines the significance of early diagnosis.