Congenital adrenal hyperplasia: long-term evaluation of feminizing genitoplasty and psychosocial aspects.
Analyzing the long-term outcome in females with congenital adrenal hyperplasia (CAH) is crucial to evaluate effectiveness of treatment strategies. The aim of the study was to evaluate the long-term results in patients with CAH after feminizing surgery from the pediatric intersex clinic. Of 163 patients of CAH being followed (1980-2005), 50 responded for review. The patients had undergone feminizing genitoplasty and hormonal therapy. Evaluation included filling a detailed questionnaire along with physical examination and a structured interview in privacy. Assessment was performed for cosmetic results (50), psychosocial adjustment (42) above 5-year age, and functional outcome in 19 cases above 14-year age. Mean age at clitoroplasty was 3.6 years (1-16 years) and at time of the study was 14.6 years (4-23 years), with a mean post-op follow up of 6 years after the final surgery (6 months-17 years). The cosmetic outcome of clitoroplasty was excellent in 37, satisfactory in 10, and poor in 3. Gender identity was female, male, and mixed in 45, 4, and 1, respectively. The attitude to self and life was positive in 36 and negative in 6. The functional outcome of vaginoplasty was satisfactory, unsatisfactory, and undetermined in 11, 4, and 4, respectively. Endocrine control was satisfactory in 36/50. A novel assessment system has been adopted for analyzing the results of clitoroplasty and vaginoplasty. Endocrine control and surgical treatment are complimentary to each other to achieve satisfactory results in majority of CAH patients.
Gupta
D K
DK
Department of Paediatric Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India. devendra6@hotmail.com
Shilpa
Sharma
S
Amini
A C
AC
Gupta
M
M
Aggarwal
Gautam
G
Deepika
Gupta
G
Kamlesh
Khatri
K
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000523:psychology* / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005835:Genitalia / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D013997:Time Factors; D016896:Treatment Outcome; D013519:Urogenital Surgical Procedures / Q000379:methods