1. No.141
Early childhood feminizing genitoplasty for congenital adrenal hyperplasia: Does the age at surgery matter?
MS Ansari , Rakesh Kapoor
Department of urology and renal transplantation
Sanjay Gandhi postgraduate institute of medical sciences
Raebareli Road, Lucknow-U.P. INDIA , Fax:
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Introduction
Congenital adrenal hyperplasia (CAH) is the most common cause of ambiguous genitalia in newborns.
The issues like optimal timing for surgical correction i.e. early versus delayed and long term out come in terms of cosmetic, functional, psychosocial and psychosexual well-being are often debated1-2
Results
The age at diagnosis ranged from new born to 14 yrs (mean=17 months) and the age at operation ranged from 3 months to 18 yrs (mean=3.5 years)
The grade of ambiguity ranged from grade III to IV according to Prader classification6.
Seven patients underwent male genitoplasty, and laparoscopic excision of mullerian structures with bilateral gonadectomy, staged urethroplasty for hypospadias and testicular implantation (figure 1b and1c)
Thirty three patient underwent female genitoplasty. All patients underwent one-stage genitoplasty i.e. clitoroplasty and/or vaginoplasty.
In all 33 such patients, clitoral recession (30) or clitoral reduction (3) was done along with
Perineal vaginoplasty (25),
Vaginal pull through (Figures 1a,b and c) (5),
Colovaginoplasty (3),
Good endocrine control was achieved in 30 (85.7%) patients.For clitoroplasty cosmetic outcome was good in 26 (74%), satisfactory in 7 (20%) and poor in 2 (5.7%) patients
At a follow up of 9.2 Years (range1.5 – 15 ), 20 patients have been married and the functional outcome of vaginoplasty was good 11(55%), satisfactory 3 (15%), poor 5 (25%) and indeterminate in 1 (5%)
The psychosocial adjustment (i.e. body image and social behavior) was studied in 30 patients above 5 years of age showed positive body image in 26 (86.70%) and negative in 4 (13.3%),
Psychosexual well-being (i.e. attraction to opposite sex, sexual arousability and sexual intercourse) was assessed in 22 patients above 14 years of age showed good response in 12 (54.5%), satisfactory in 6 (27.30%) and indeterminate in 4 (18.2%) patients
Early complications were; excessive intraoperative bleeding (3), clitoral sloughing (2) and clitoral atrophy (3), while 5 (14.3%) patients developed vaginal stenosis as late complication. Of these five, 3 patients required Y-V introitoplasty (skin flap labiointroitoplasty) and 2 labioscrotal island flap introitoplasty.
Follow up ranged from 3 months to years. Post operative complications were seen in 6 patients in the form of clitoral sloughing in two, clitoral atrophy in one and bleeding in three patients.
Aim
To evaluate the long term results of feminizing genitoplasty in girls with ambiguous genitalia due to congenital adrenal hyperplasia (CAH) achieved through one-stage reconstruction
Methods
Between January 1990 to January 2012, 41 females with genital ambiguity (Prader III-V) due to CAH underwent one-stage feminizing genitoplasty along with supportive endocrine treatment
The results were reviewed with respect to age at surgery, operative procedures performed and final outcome (cosmetic, functional, psychosocial and psychosexual well-being) which was assessed by using an interview, a written questionnaire and physical examination in privacy
Fig 1a.Genitogram showing high urogenital confluence
Conclusions
One-stage female genitoplasty performed in early childhood in cases of CAH gives satisfactory long term cosmetic, functional, psychosocial and psychosexual results. It has few complications with acceptable rate of secondary surgical interventions
Fig. 1b. Vaginal pull through for high urogenital confluence
Acknowledgements
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Fig 1c. Same patient after pull through vaginoplasty
References
1.Burgu B, Duffy PG, Cuckow P, Ransley P, Wilcox DT. Long-term outcome of vaginal reconstruction: comparing techniques and timing. J Pediatr Urol. 2007;3(4):
2. Graziano K, Teitelbaum DH, Hirschl RB, Coran AG. Vaginal reconstruction for ambiguous genitalia and congenital absence of the vagina: a 27-year experience. J Pediatr Surg Jul;37(7):955-60