Evaluation of the early outcomes of partial urogenital sinus mobilization in females with congenital adrenal hyperplasia (original) (raw)
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Frontiers in pediatrics, 2016
Genital reconstruction in female patients with virilizing congenital adrenal hyperplasia (CAH) is very challenging. Our aim was to evaluate the techniques employed to treat complications after failure of primary urogenital sinus (UGS) surgery, as well as the result of these reoperations. Twenty girls with virilizing CAH who were previously submitted to genitoplasty in our service and elsewhere had recurrent UGS stenosis and vaginal introitus stenosis that required surgical treatment. The main symptoms were recurrent urinary tract infection (UTI) in nine, dyspareunia in six, and hematocolpos in three (two associated with sepsis). The anatomical findings were the persistence of UGS with stenosis in 17 patients and vaginal introitus stenosis in 3. The mean age at procedure was 15.2 years, averaging 13.1 years after the first surgery. The surgical techniques employed were isolated perineal flap in 17 patients and perineal flap with partial mobilization of UGS in 3. The mean follow-up af...
The Journal of Urology, 2009
The ideal surgical technique and appropriate age for performing feminizing genitoplasty are debatable, and few long-term outcome studies have been reported. PURPOSE: To report a retrospective study on anatomical and functional outcomes of feminizing genitoplasty in patients with virilizing congenital adrenal hyperplasia. METHODS: We selected 34 patients (mean age = 3.4 ± 2.5 yr) with genital ambiguity classified according to Prader stage. Follow-up ranged from 2 to 16 years. Clitoral length ranged from 1.9 to 5.0 cm; 28 patients had a single perineal orifice, and 6 had a double orifice. The surgical technique included clitorovaginoplasty in a single procedure and was carried out before 2 years of age in 18 patients. Clitoroplasty was performed with glans preservation in all patients. Blood supply was exclusively maintained by the frenular pedicle in 97% of the cases, whereas clitoral dorsal nerves and vessels were preserved in the remaining 3%. The opening of the urogenital sinus was performed using either the Y-V perineal flap procedure (25 patients) or the cutback incision procedure (8 patients). RESULTS: Good morphological and functional results were achieved in 68% of the patients; 21% of the patients had surgical complications, such as incision bleeding (2 cases), glans necrosis (1 girl with Prader V), and vaginal introitus stenosis (4 cases). Three of the latter underwent dilation with acrylic molds in the post-pubertal period with good functional results. CONCLUSIONS: We conclude that single-stage feminizing genitoplasty consisting of vulvoplasty, clitoroplasty, and Y-V perineal flap produced good cosmetic and functional results in virilized girls with congenital adrenal hyperplasia, with few complications. In addition, this surgical approach prevented the need for neovaginaplasty even in patients with high vaginal insertion.
One -Stage Feminizing Genitoplasty in Patients with Congenital Adrenal Hyperplasia
2006
Background/ Purpose: A growing interest has been noted recently in one -stage Feminizing genitoplasty for patients with congenital adrenal hyperplasia (CAH). The timing of surgery is currently controversial. This study was carried out to investigate the feasibility and outcome of one –stage feminizing genitoplasty at various ages. Materials & Methods: Eighteen female patients with CAH were treated during a six year-period. All patients undergone one- stage clitoroplasty, vaginoplasty, and labioplasty. Fifteen with high or intermediate confluence of vagina and urethra underwent total urogenital mobilization (TUM). A perineal posterior flap vaginoplasty and minimal mobilization of the urogenital sinus (UGS) was adequate in 3 cases with short UGS. Each patient was evaluated as regard to age at surgery, degree of virilization, preoperative diagnostic studies, operative technique, and outcome. Follow up ranged from 2 to 72 months. Results: Patients aged 5 weeks to 6 years. Mean operative...
Clinics, 2006
The ideal surgical technique and appropriate age for performing feminizing genitoplasty are debatable, and few long-term outcome studies have been reported. PURPOSE: To report a retrospective study on anatomical and functional outcomes of feminizing genitoplasty in patients with virilizing congenital adrenal hyperplasia. METHODS: We selected 34 patients (mean age = 3.4 ± 2.5 yr) with genital ambiguity classified according to Prader stage. Follow-up ranged from 2 to 16 years. Clitoral length ranged from 1.9 to 5.0 cm; 28 patients had a single perineal orifice, and 6 had a double orifice. The surgical technique included clitorovaginoplasty in a single procedure and was carried out before 2 years of age in 18 patients. Clitoroplasty was performed with glans preservation in all patients. Blood supply was exclusively maintained by the frenular pedicle in 97% of the cases, whereas clitoral dorsal nerves and vessels were preserved in the remaining 3%. The opening of the urogenital sinus wa...
Background and Rationale: Congenital adrenal hyperplasia in females leads to virilization of the external genitalia and an anomalous lower genitourinary tract. Hormonal therapy controls the endocrinopathy. Surgical repair of the anomaly is indicated to allow raising a female child with external genitalia matching her gender and to avoid psychological complications. Surgery at an earlier stage is believed to be easier and of better results than when deferred to adolescence. Objectives: To study the anatomical abnormalities of genotypic females with virilized atypical genitalia due to congenital adrenal hyperplasia and their impact on surgery and to review the surgical techniques of feminizing genitoplasty and their early short-term outcomes. We aim to highlight the multidisciplinary management for these complex cases and to discuss the difficulties and challenges met during this. Patients & Methods: Genotypic prepubertal females with virilized atypical genitalia due to congenital adr...
Urology, 2018
To analyze nationwide information on the timing of surgical procedures, cost of surgery, hospital length of stay following surgery, and surgical complications of female genital restoration surgery (FGRS) in females with congenital adrenal hyperplasia (CAH). We used the Pediatric Health Information System database to identify patients with CAH who underwent their initial FGRS in 2004-2014. These patients were identified by an International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code for adrenogenital disorders (255.2) in addition to a vaginal ICD-9 procedure code (70.x, excluding vaginoscopy only) or perineal ICD-9 procedure code (71.x), which includes clitoral operations (71.4). A total of 544 (11.8%) females underwent FGRS between 2004 and 2014. Median age at initial surgery was 9.9 months (interquartile range 6.8-19.1 months). Ninety-two percent underwent a vaginal procedure, 48% underwent a clitoral procedure, and 85% underwent a perineal procedure (non-clit...
Urology Annals, 2013
Congenital adrenal hyperplasia (CAH) is a rare condition that results in the disorder of sexual differentiation characterized by virilization of 46XX females. [1,2] The affected female children are often treated by reconstruction of their genitalia by single or staged surgical procedures. [3] Few studies followed women with CAH into adulthood. The number of patients reported from the western world is limited. [4-9] In addition, there is a paucity of data on the surgical management of patients with CAH in our region and none on the long-term outcome in adult women. [10] It is not known whether the outcome in women native to the Middle East is affected by the different ethnic make-up, racial background, psychosocial structure or the local clinical setup.
2020
Background: Objectives: 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 onestage feminizing genitoplast...
Pediatric Surgery International, 2006
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.