Nonclassic Congenital Adrenal Hyperplasia Metabolic Resolution Post Roux-en-Y Gastric Bypass and Associated Weight Loss

TL;DR

A woman with nonclassic congenital adrenal hyperplasia and severe obesity achieved metabolic improvement and weight loss after Roux-en-Y gastric bypass surgery.

Contribution

This case suggests bariatric surgery may resolve NCCAH symptoms in patients with class III obesity.

Findings

The patient lost 160 pounds and no longer required glucocorticoid therapy after surgery.

Metabolic markers like ACTH and androstenedione normalized post-surgery.

Bariatric surgery may serve as an alternative treatment for NCCAH with severe obesity.

Abstract

Nonclassic congenital adrenal hyperplasia (NCCAH) is characterized by mild cortisol deficiency, excess androgens and adrenocorticotropin (ACTH) production, and often with various features of dysmetabolic syndrome. Elective bariatric surgery is one of the most effective long-term management strategies for severe obesity. Our case presents a 34-year-old woman with symptomatic NCCAH and class III obesity who status post Roux-en-Y gastric bypass (RYGB) had significant weight loss with metabolic resolution of NCCAH, and no longer required glucocorticoid (GC) therapy. At 11 months post operation and off GC therapy, she had a weight deficit of approximately 160 pounds (72.57 kg) with continued metabolic resolution of NCCAH markers including ACTH, 17-hydroxyprogesterone, and androstenedione. Presently, GC therapy remains one of the few available treatments for symptomatic NCCAH; however, long-term GC therapy has the potential for various complications and side effects. Our case presents elective bariatric surgery as a potential and unique treatment option for patients with NCCAH with associated class III obesity. The exact pathophysiologic basis for this effect and its potential role in long-term management of appropriate NCCAH patients requires further study.

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