Year : 2003 | Volume
: 19 | Issue : 2 | Page : 145--151
Surgical management of ambiguous genitalia in infants and children: An SGPGI experience
Subodh Kumar Das, Anant Kumar, NK Arvind, S Phadke
Department of Urology and Renal Transplanation and Department of Medicine Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
Introduction: Assignment of a proper gender to a neonate born with ambiguous genitalia is a social emergency. 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 experience with this unique group of patients, we have retrospectively reviewed the course of 31 children managed surgically at our institute between 1989 and 2000.
Patients and Methods: This series consists of 16 genotype females with congenital adrenal hyperplasia (CAH), 7 male pseudohermaphrodites, 5 children with mixed gonadal 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 gonadectomy, 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 pseudohermaphrodites were raised as males. Both of them underwent 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 gonadoblastoma. 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 patient 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 patients 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 gender assignment is concerned. However the functional results 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 ambiguous 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 inadequacy usually makes it difficult to achieve a cosmetically acceptable appearance. However the sex of rearing and parents«SQ» intentions and wishes should be taken into consideration in the reconstruction of genitalia of a child.
Department of Urology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow - 226 014
|How to cite this article:|
Das SK, Kumar A, Arvind N K, Phadke S. Surgical management of ambiguous genitalia in infants and children: An SGPGI experience.Indian J Urol 2003;19:145-151
|How to cite this URL:|
Das SK, Kumar A, Arvind N K, Phadke S. Surgical management of ambiguous genitalia in infants and children: An SGPGI experience. Indian J Urol [serial online] 2003 [cited 2020 Jan 27 ];19:145-151
Available from: http://www.indianjurol.com/article.asp?issn=0970-1591;year=2003;volume=19;issue=2;spage=145;epage=151;aulast=Das;type=0