Literature DB >> 16832553

Anatomical and functional outcomes of feminizing genitoplasty for ambiguous genitalia in patients with virilizing congenital adrenal hyperplasia.

Maria Helena Palma Sircili1, Berenice B de Mendonca, Francisco Tibor Denes, Guiomar Madureira, Tânia Aparecida Sartori Sanchez Bachega, Frederico Arnaldo de Queiroz e Silva.   

Abstract

UNLABELLED: 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 cut-back 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.

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Year:  2006        PMID: 16832553     DOI: 10.1590/s1807-59322006000300005

Source DB:  PubMed          Journal:  Clinics (Sao Paulo)        ISSN: 1807-5932            Impact factor:   2.365


  13 in total

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Journal:  Ther Adv Urol       Date:  2011-12

2.  Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline.

Authors:  Phyllis W Speiser; Ricardo Azziz; Laurence S Baskin; Lucia Ghizzoni; Terry W Hensle; Deborah P Merke; Heino F L Meyer-Bahlburg; Walter L Miller; Victor M Montori; Sharon E Oberfield; Martin Ritzen; Perrin C White
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Journal:  Clin Pediatr Endocrinol       Date:  2022-04-10

4.  [Congenital adrenal hyperplasia in girls/young women].

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Journal:  Urologe A       Date:  2014-02       Impact factor: 0.639

Review 5.  Approach to the patient: the adult with congenital adrenal hyperplasia.

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Review 7.  Congenital Adrenal Hyperplasia: Review from a Surgeon's Perspective in the Beginning of the Twenty-First Century.

Authors:  Lisandro Ariel Piaggio
Journal:  Front Pediatr       Date:  2014-01-02       Impact factor: 3.418

8.  Surgical Treatment after Failed Primary Correction of Urogenital Sinus in Female Patients with Virilizing Congenital Adrenal Hyperplasia: Are Good Results Possible?

Authors:  Maria Helena Palma Sircili; Tania Sartori Sanchez Bachega; Guiomar Madureira; Larissa Gomes; Berenice Bilharinho Mendonca; Francisco Tibor Dénes
Journal:  Front Pediatr       Date:  2016-10-27       Impact factor: 3.418

9.  Clinical evaluation study of the German network of disorders of sex development (DSD)/intersexuality: study design, description of the study population, and data quality.

Authors:  Anke Lux; Siegfried Kropf; Eva Kleinemeier; Martina Jürgensen; Ute Thyen
Journal:  BMC Public Health       Date:  2009-04-21       Impact factor: 3.295

10.  Preliminary report: Surgical outcomes following genitoplasty in children with moderate to severe genital atypia.

Authors:  K J Bernabé; N J Nokoff; D Galan; D Felsen; C E Aston; P Austin; L Baskin; Y-M Chan; E Y Cheng; D A Diamond; R Ellens; A Fried; S Greenfield; T Kolon; B Kropp; Y Lakshmanan; S Meyer; T Meyer; A M Delozier; L L Mullins; B Palmer; A Paradis; P Reddy; K J Scott Reyes; M Schulte; J M Swartz; E Yerkes; C Wolfe-Christensen; A B Wisniewski; D P Poppas
Journal:  J Pediatr Urol       Date:  2018-02-02       Impact factor: 1.921

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