Post-operative complications following feminizing genitoplasty in moderate to severe genital atypia: Results from a multicenter, observational prospective cohort study

Autor: Cortney Wolfe-Christensen, Elizabeth B. Yerkes, Kristy J. Scott Reyes, Yegappan Lakshmanan, Allyson Fried, Thomas F. Kolon, Earl Y. Cheng, Laurence S. Baskin, Yee-Ming Chan, Avi Baskin, David A. Diamond, Amy C. Tishelman, Theresa Meyer, Dix P. Poppas, Natalie J. Nokoff, Brian A. VanderBrink, Blake W. Palmer, Larry L. Mullins, Sabrina Meyer, Christopher E. Aston, Paul F. Austin, Alethea Paradis, Bradley P. Kropp, Pierre Williot, Amy B. Wisniewski
Rok vydání: 2020
Předmět:
Male
Pediatrics
medicine.medical_treatment
030232 urology & nephrology
Disorders of Sex Development
Cohort Studies
Congenital
0302 clinical medicine
Atypia
Prospective Studies
Prospective cohort study
Glans
Child
Pediatric
Urology & Nephrology
medicine.anatomical_structure
Child
Preschool
Cohort
Female
Patient Safety
Urogenital sinus reconstruction
6.4 Surgery
medicine.medical_specialty
Atypical genitalia
Disorders of sex development
Urology
Sexual and Gender Minorities (SGM/LGBT*)
Article
Paediatrics and Reproductive Medicine
03 medical and health sciences
Rare Diseases
Clinical Research
030225 pediatrics
medicine
Genetics
Humans
Orchiopexy
Sex organ
Genitalia
Preschool
Adrenal Hyperplasia
Adrenal Hyperplasia
Congenital
business.industry
Congenital adrenal hyperplasia
Cosmesis
Evaluation of treatments and therapeutic interventions
medicine.disease
Urogenital Surgical Procedures
Hypospadias
Pediatrics
Perinatology and Child Health
Congenital Structural Anomalies
business
Zdroj: Journal of pediatric urology, vol 16, iss 5
J Pediatr Urol
Popis: Summary Introduction Differences of sex development (DSD) are congenital conditions in which there is atypical chromosomal, gonadal and/or phenotypic sex. A phenotype of severe genital atypia in patients raised as male is a relatively rare occurrence and standards for management are lacking. Decision making for early surgical planning in these rare cases includes, but is not limited to, degree of atypia, location of testes, and presence of Mϋllerian remnants. In this study we describe surgical approaches and short-term outcomes for masculinizing genitoplasty in moderate to severe genital atypia in young patients raised male, for whom parents opted for early surgery. Methods This NIH-sponsored study is an ongoing, observational, multicenter investigation assessing medical, surgical and psychological outcomes in children and their parents affected by atypical genitalia due to DSD. Participants were prospectively enrolled from 12 children's hospitals across the United States that specialize in DSD care. Criteria for child enrollment were a Quigley score of 3–6 in those with a 46, XY or 45,X/46, XY chromosome complement, age Results Of the 31 participants, 30 underwent hypospadias repair and 1 patient did not undergo a genitoplasty procedure. The majority of participants (22) received a staged hypospadias repair. Seventeen complications were identified in 12 of the 31 children (41%) at 12 months of follow up. Glans dehiscence and urethrocutaneous fistula were the most common complications. Orchiopexy was performed in 14 (44%) and streak gonads were removed in 4 (13%) participants. Both parents and surgeons reported improved cosmesis after surgery when compared to baseline. Conclusion Genitoplasty was chosen by parents for the majority of children eligible for study. No single surgical approach for masculinizing moderate to severe genital ambiguity in young patients with 46, XY or 45,X/46, XY DSD was adopted by all surgeons. Complications occurred in 41% of those who underwent genitoplasty for severe hypospadias. Overall, appearance of the genitals, as determined by parents and surgeons, improved following genitoplasty. Outcomes of early genitoplasty are needed to guide families when making decisions about such procedures for their young children. Download : Download high-res image (286KB) Download : Download full-size image Summary Figure . River plot comparing Likert cosmesis score over time for mothers, fathers, and surgeons. Panel A includes all patients in the cohort.
Databáze: OpenAIRE
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