Semaglutide and laparoscopic sleeve gastrectomy in an adolescent with congenital adrenal hyperplasia due to 21-hydroxylase: a case report

Abstract

Abstract Background Classic congenital adrenal hyperplasia, primarily due to 21-hydroxylase deficiency, leads to impaired cortisol and aldosterone production and excess adrenal androgens. Lifelong glucocorticoid therapy is required, often necessitating supraphysiological doses in youth to manage androgen excess and growth acceleration. These patients experience higher obesity rates, hypertension, and glucose metabolism issues, complicating long-term health management. Despite this, there is limited literature on effective obesity management strategies in congenital adrenal hyperplasia patients, emphasizing the need for comprehensive care approaches. Case presentation We present the case of an 18-year-old Hispanic male with classic congenital adrenal hyperplasia and class III obesity, who underwent a multimodal obesity treatment plan. Diagnosed shortly after birth, he experienced rapid weight gain starting at the age of 2 years, with his body mass index escalating to 52.5 kg/m2 by age 15. Initial interventions included lifestyle modifications and pharmacotherapy with metformin and topiramate, which were ineffective alone. Subsequently, he was treated with semaglutide, achieving an 11% body mass index reduction. Owing to ongoing metabolic dysregulation, he underwent laparoscopic sleeve gastrectomy at the age of 17 years. The surgery was well tolerated, with careful intraoperative glucocorticoid management. Post-surgery, he experienced significant improvements in body mass index, hunger, and satiety, along with a reduction in emotional overeating. Conclusions This case highlights the potential of an integrative, multidisciplinary approach to address severe obesity and its associated comorbidities in patients with classic congenital adrenal hyperplasia. The successful outcomes from both pharmacotherapy and bariatric surgery suggest that tailored obesity management strategies can optimize health in this unique population, reinforcing the need for further research into comprehensive obesity care in individuals with congenital adrenal hyperplasia.

Keywords

• Congenital adrenal hyperplasia
• Pediatric obesity
• Laparoscopic sleeve gastrectomy