Surgical management of ambiguous genitalia in infants and children: An SGPGI experience

• Main Authors: Subodh Kumar Das, Anant Kumar, N K Arvind, S Phadke
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2003-01-01
• Series: Indian Journal of Urology
• Subjects: Ambiguous genitalia; surgery.
• Online Access: http://www.indianjurol.com/article.asp?issn=0970-1591;year=2003;volume=19;issue=2;spage=145;epage=151;aulast=Das

Introduction: Assignment of a proper gender to a neonate born with ambiguous genitalia is a social emer-gency. Once a sex has been assigned the next critical step is performance, if needed, of a reconstructive procedure in a timely fashion. In an attempt to evaluate our experi-ence with this unique group of patients, we have retro-spectively reviewed the course of 31 children managed surgically at our institute between 1989 and 2000. Patients and Methods: This series consists of 16 geno-type females with congenital adrenal hyperplasia (CAH), 7 male pseudohermaphrodites, 5 children with mixed go-nadal dysgenesis (MGD), and 3 true hermaphrodites. All the 16 patients with CAH underwent vaginoplasty, and clitoral recession. Five of the male pseudohermaphrodites were raised as females. All of them underwent B/L gona-dectomy, and clitoral recession. Perineal vaginoplasty was done in 4 of these patients and I patient who was due for colo-vaginoplasty was lost to follow-up. 2 male pseudoher-maphrodites were raised as males. Both of them under-went B/L orchiopexy and hypospadias repair. Four of the 5 cases of MGD were given a female sex assignment and all 4 underwent gonadectomy due to high risk of gonado-blastoma. In the 5th patient a male gender assignment was given. There were 3 true hermaphrodites, 2 of whom were managed by clitoral recession and vaginoplasty. One pa-tient was diagnosed at the age of 15 years. This patient had been brought up as a male child and presented to us with the complaint of pain in the lower abdomen. Investigations revealed haematometra and haematocolpos and the patient had to undergo B/L salpingo-oophorectomy and hysterectomy. Results: The postoperative period in most of the pa-tients was uneventful except for 2 minor complications. The cosmetic results have been excellent. 2 patients were lost to follow-up. Follow-up in the rest of the patients ranged from 9 months to 6.3 years and all the patients were found well adjusted to the society as far as their gen-der assignment is concerned. However the functional re-sults are yet to be evaluated, as most of the patients have not yet achieved full sexual maturity. Conclusion: This retrospective review emphasizes the complexities of assessment and management of ambigu-ous genitalia in infants and children. It also confirms the data from other series that the vast majority of infants born with the 4 most common forms of ambiguous genitalia (CAH, MGD, male pseudohermaphroditism, and true hermaphroditism) may be raised as females as phallic in-adequacy usually makes it difficult to achieve a cosmeti-cally acceptable appearance. However the sex of rearing and parents′ intentions and wishes should be taken into consideration in the reconstruction of genitalia of a child.