Anatomical and cosmetic outcomes following feminizing genitoplasty in patients with disorders of sexdevelopment
Wael Fathi badawy Abosena, M.D degree in paediatric surgery, Hisham AlMohamady Almetaher, M.D, Ashraf Ahmed El Attar, M.D, Essam Abdelaziz Elhalaby, M.D, Ahmed Hassan Nofal, M.D.
faculty of medicine, tanta university, TANTA, Egypt.
BACKGROUND: Ambiguous genitalia in the newborn is the commonest presentation of disorders of sexual development (DSD), most commonly secondary to congenital adrenal hyperplasia (CAH), Those patients require feminizing genital reconstruction aiming at separation of the urinary and genital tracts allowing for normal voiding, creation of an adequate vaginal introitus and achievement of a near normal appearance of the external genitalia. The aim of this work was to evaluate the anatomical and cosmetic outcome of feminizing genitoplasty in Patients with disorders of sex development.
METHODS: Twenty patients presented with disorders of sex development admitted to the Pediatric Surgery Unit in Tanta University Hospital, over a period of two years,from June 2016 to June 2018. Genital examination was performed for all patients and the degree of virilization in cases of CAH was assessed according to the Prader scoring system. All patients undergone one- stage feminizing genitoplasty and the Postoperative cosmetic and anatomical outcome was evaluated according to the criteria described by Creighton and colleagues. Follow-up period ranged from 6 to 24months.
RESULTS: Out of the twenty cases in this study, 18 cases had congenital adrenal hyperplasia, two cases had complete androgen insensitivitysyndrome (CAIS). All cases of CAH had a 21-hydroxylase deficiency. The Age of patients ranged between 6 months and 16 years. Seven patients were less than one year of age at time of surgery (7/20[35%]). While6 patients were older than 3 years (6/20 [30%]). The urogenital sinus mobilization which was used in 17 cases (17/18[94.5%]), The partial urogenital mobilization (PUM) was used in 3 cases (3/18 [16.7%]) and the total urogenital mobilization (TUM) was used in 14 cases (14/18 [77.7%]) while colon vaginoplasty was used in the two cases ofCAIS and one case of CAH. Our anatomic and cosmetic outcomes were good in 17(85%) for those patients initially treated at our institute by our specialized team, satisfactory in 2(10%) and poor in1(5%).
CONCLUSIONS: Feminizing genitoplasty can be used safely and effectively with good anatomical and cosmetic outcome provided that the pediatric surgeon is familiar with the procedure. The feminizing genitoplasty is technically much easier in young infants. Sigmoid vaginoplasty is a good choice in cases of CAIS and CAH where the vaginal confluence was high close to the bladder neck and the vagina was very short.
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