Endocrine Abstracts

Cushing’s syndrome (CS) is a rare endocrine problem in children. Most of the cases in children are iatrogenic in nature due to extensive usage of topical, inhaled and oral corticosteroids. Endogenous hypercortisolism in children is mainly because of ACTH-dependent pituitary adenomas and constitutes ~70–80% of CS in children. ACTH-independent CS is rare in children above 5 years of age. Here, we are presenting a rare case of ACTH-independent macronodular adrenal hyperplasia.

A 7-year-old Iraqi boy presented to our OPD with a few years history of increased body weight, growth failure, facial swelling, and a few months history of pubic and facial hair growth and hypertension. On examination, weight 45 kg, height 114 cm, he had moon–like face, buffalo-hump, scanty terminal hair over face and pubic area and pinkish purple striae over abdominal wall. Blood pressure was high and he was on three antihypertensives. Biochemistry revealed low ACTH and high cortisol, non-suppression of cortisol on ODST. CT scan revealed bilateral bulky adrenals with nodular hyperplasia with one large lesion on the left side. Initially, he underwent laparoscopic left adrenalectomy. Post-operatively, he had persistently high cortisol with suppressed serum ACTH. Subsequently, he underwent right adrenalectomy. Post-surgery, he had very low serum cortisol. He was started on maintenance dose of oral hydrocortisone and fludrocortisone. All anti-hypertensives were stopped. He lost around 10–12 kg body weight 3 months post-surgery.

Conclusion: CS can cause serious long-term morbidity and effect the physical and mental development of the child. Treating physician can prevent these problems if he has high index of suspicion regarding endocrine problems whenever the child has obesity, high BP, and growth failure.