Symptomatic cycling Cushing disease managed by simultaneous bilateral laparoscopic adrenalectomy in a 11-year-old boy
The present case is about a 11-year-old Saudi boy who was referred to our service for bilateral adrenalectomy. The patient had been diagnosed by the pediatric endocrinologist with cycling Cushing disease. He presented with a history of rapid weight gain, decreased school performance and emotional disturbances in the previous 18 months. These complaints came in cycles, each lasting 2-4 months before the patient returned to normal. Adrenal ultrasound and computed tomography scan showed normal adrenal glands, magnetic resonance imaging of the brain also showed normal pituitary and hypothalamic area. During one of these cycles, his weight increased by 4 kg, blood pressure was 140/100 and he exhibited the typical signs of Cushing's disease. Blood work confirmed Cushing's disease. His serum cortisol at 1 PM was elevated at 16.6 mcg/dL. Electrolytes showed sodium 143 mEq/L, potassium 4 mEq/L, chloride 103 mEq/L, total CO2 27, blood urea nitrogen 9 mg/dL and creatinine 0.3 mg/dL. 24 h urine for free cortisol was high at 542 mcg/24 h (normal 5-55 mcg/24 h). At 2 months later, the patient presented with a third episode and was admitted for a full work-up. Urine free cortisol was elevated at 350 mcg/24 h, at midnight serum cortisol was 19.9 mcg/dL and serum adrenocorticotropic hormone (ACTH) was <4 pg/ml. Am cortisol was 15.7 mcg/dL and ACTH was 5.4 pg/ml. The patient was subjected to small and high doses of dexamethasone suppression tests (1 mg and 5 mg respectively) but there was no change in cortisol or ACTH serum levels. These findings confirmed a diagnosis of Cushing's disease of adrenal origin and its cyclic nature presented Carney's complex syndrome as a possibility. Further investigations to exclude Endocrine and cardiac tumors as part of Carney's complex included ultrasound of the thyroid and echocardiography which were normal. Ultrasound of the testes showed bilateral diffuse macro-calcification as well as 0.8 × 0.8 cm hypo-echoic mass within the left testicle, which strengthened the diagnosis of Carney's complex. The patient had no abnormal skin rash and the family history was negative for any endocrine problems. Deoxyribonucleic acid analysis on our patient did not show the known mutation in Carney's complex (PRKAR1A) which is found in only 50% of those patients.\nBilateral adrenalectomy was the treatment of choice in this patient. The plan was to start with right laparoscopic adrenalectomy and proceed to left adrenalectomy with the possibility of converting to open. The patient underwent a successful simultaneous bilateral adrenalectomy; the operative time was 3 h and 30 min. The estimated blood loss was 50 ml. The patient resumed his diet and ambulated on the 1st post-operative day and was discharged from hospital on the 2nd post-operative day. The post-operative course was smooth and uneventful and he was started on replacement therapy post-operatively. At 3 months later, he was doing well with a remarkable improvement in his behavior and school performance. Histopathology report of his adrenal gland was consistent with micronodular adrenocortical hyperplasia.