Purpose: In the past many female patients with congenital adrenal hyperplasia and atypical genitalia were surgically treated with clitoral recession or incomplete reduction of erectile bodies. We report the results of repeat clitoral surgery performed for clitoral pain or enlargement using a nerve sparing reduction clitoroplasty technique.
Materials And Methods: We identified 6 female patients with congenital adrenal hyperplasia who had undergone prior clitoral recession or incomplete reduction elsewhere. They then presented to our center with clitoral pain or enlargement, where they were treated with nerve sparing clitoroplasty between 2000 and 2010. We collected patient reported data relating to clitoral sensation and sexual function outcomes.
Results: Mean ± SD age at evaluation for repeat clitoral surgery was 21 ± 7 years and mean age at clitoroplasty was 22 ± 8 years. Median postoperative followup was 9 months (IQR 32-6). All patients showed improvement with resolution of clitoral pain or enlargement.
Conclusions: Clitoral pain and enlargement upon arousal can be a major concern for women with clitoromegaly and congenital adrenal hyperplasia after clitoral recession is performed. Our series suggests that clitoral recession or incomplete reduction in childhood may be an inadequate initial solution in the congenital adrenal hyperplasia population due to the potential for future androgen elevation and the possibility of later symptom development. In addition we found that patients may be successfully treated with nerve sparing clitoroplasty, resulting in resolution of pain and ability to engage in sexual activity.
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http://dx.doi.org/10.1016/j.juro.2015.12.053 | DOI Listing |
Int J Neonatal Screen
December 2024
Laboratory of Genomic, Epigenetics, Precision and Predictive Medicine, School of Medicine, Mohammed VI University of Sciences and Health, Casablanca 82403, Morocco.
Unlabelled: Newborn screening (NBS) represents an important public health measure for the early detection of specified disorders; such screening can prevent disability and death, not only from metabolic disorders but also from endocrine, hematologic, immune, and cardiac disorders. Screening for critical congenital conditions affecting newborns' health is a great challenge, especially in developing countries such as Morocco, where NBS program infrastructure is lacking. In addition, the consanguinity rate is high in Morocco.
View Article and Find Full Text PDFFront Endocrinol (Lausanne)
December 2024
Centre for Endocrinology, William Harvey Research Institute, Barts & the London School of Medicine & Dentistry, Queen Mary, University of London, London, United Kingdom.
J Clin Res Pediatr Endocrinol
December 2024
Department of Pediatric Endocrinology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey.
Central adrenal insufficiency (CAI) occurs due to a pituitary gland disorder (secondary AI) or hypothalamic dysfunction (tertiary AI). It is a potentially life-threatening condition that has many congenital and acquired causes. Adrenocorticotropic hormone (ACTH) deficiency may be isolated or more commonly it can be accompanied by other pituitary hormone deficiencies or midline defects.
View Article and Find Full Text PDFJ Clin Res Pediatr Endocrinol
December 2024
Ondokuz Mayıs University, Faculty of Medicine, Department of Paediatric Endocrinology, Samsun, Turkiye.
Primary adrenal insufficiency (PAI) is a critical condition that requires prompt diagnosis and initiation of treatment. Diagnosis can be challenging due to various underlying causes, including defects in adrenal gland development, resistance to adrenocorticotropic hormone (ACTH), autoimmune causes, and metabolic problems. A specific diagnosis is essential for developing a treatment plan and identifying other possible accompanying pathologies.
View Article and Find Full Text PDFJ Clin Res Pediatr Endocrinol
December 2024
Ankara University, Pediatric Endocrinology, Ankara, Turkey.
Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency accounts for approximately 95% of all CAH cases and is one of the most common inborn errors of metabolism. While glucocorticoid therapy has significantly improved patient outcomes, the focus has shifted towards managing the long-term effects. Numerous adverse outcomes have been associated with CAH, including those resulting from supraphysiological doses of glucocorticoid and mineralocorticoid replacement, excessive adrenal androgen secretion, and elevated levels of steroid precursors and adrenocorticotropic hormone (ACTH).
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!