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| Sexual development : genetics, molecular biology, evolution, endocrinology, embryology, and pathology of sex determination and differentiation. 2021 Jul 21. pii: 000517055. doi: 10.1159/000517055 |
| Surgical Practice in Girls with Congenital Adrenal Hyperplasia: An International Registry Study. |
| Hebenstreit D1, Ahmed FS2, Krone N3, Krall C4, Bryce J5, Alvi S6, Ortolano R7, Lima M8, Birkebaek N9, Bonfig W10, Claahsen van der Grinten H11, Costa EC12, Poyrazoglu S13, de Vries L14, Flück CE15, Guran T16, Bugrul F17, Güven A18, Iotova V19, Koehler B20, Schröder JT21, Konrad D22, Gevers E23, Krone R24, Milenkovic T25, Vieites A26, Ross R27, Tadokoro Cuccaro R28, Hughes I29, Acerini C30, Springer A31 |
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Abstract In this article international trends in surgical practice in girls with congenital adrenal hyperplasia (CAH) are evaluated. All cases that had been classified in the I-CAH/I-DSD registry as 46,XX CAH and who were born prior to 2017 were identified. Centers were approached to obtain information on surgical decision making. Of the 330 included participants, 208 (63.0%) presented within the first month of life, and 326 (98.8%) cases were assigned female. Genital surgery had been performed in 250 (75.8%). A total of 64.3, 89.2, and 96.8% of cases residing in Europe, South America and Asia, respectively, had at least one surgery. In a logistic regression model for the probability of surgery before the second birthday (early surgery) over time an increase of probability for early vaginal surgery could be identified, but not for clitoral surgery or both surgeries combined. Genitoplasty in girls with CAH remains controversial. This large international study provides a snapshot of current practice and reveals geographical and temporal differences. Fewer surgeries were reported for Europe, and there seems to be a significant trend towards aiming for vaginal surgery within the first 2 years of life. |
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© 2021 The Author(s). Published by S. Karger AG, Basel. |
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KEYWORDS: Congenital adrenal hyperplasia, Disorders of sexual development, Intersex, Surgery |
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Publikations ID: 34350892 Quelle: öffnen |
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