Background

Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder associated with deficiencies in 21-,11-, or 17-hydroxylase (OHD). More than 90% of CAH is caused by 21OHD. Clinical manifestations are mainly due to adrenocortical insufficiency, hyperandrogenism and adverse effects of glucocorticoids supplementation. Anesthetic management surrounds the complications of ACTH suppression due to steroid therapy, mainly hypertension, hyperglycemia, electrolyte abnormalities, adrenal and pituitary suppression and immune suppression.


Case report
	
20 month old female with masculinization due to CAH secondary to 21OHD. Medicated with hydrocortisone and fludrocortisone since birth. 
Proposed to elective reductive genitoplasty under general-epidural anesthesia. After the induction of the general anesthesia we placed an epidural lumbar catheter (liquid column method); ropivacaine 0.2% was used according to anesthetic needs. She followed the adrenal insufficiency protocol of our center in the perioperative period, which consists in 2 mg/kg of hydrocortisone in the anesthetic induction and every 4 hours during surgery. In the first 24 post-operative hours, the dose is 20 mg of intravenous hydrocortisone every 6 hours, and the habitual dose is resumed in the second post-operative day. 
Procedure lasted for 6 hours and 45 minutes. Vital parameters and glucose levels were stable. Successfully extubation on operating room at the end of the surgery followed by transfer to pediatric intensive care unit. Postoperative recovery was uneventful. 
Multimodal analgesia with epidural ropivacaine 0.1% infusion for 48 hours was performed, with low pain scores.

Discussion
Steroid supplementation is crucial in perioperative period and was performed with an initial bolus of hydrocortisone 2mg/kg, repeated at each 4 hours, during all the procedure.
Hemodynamic stability and good pain control were achieved during and after the procedure. Epidural analgesia may contribute to this success.


Learning Points

Children with CAH can be managed successfully during anesthesia after understading the patophysiology of disease with proper history and perioperative steroid supplementation.
Hemodynamic and metabolic stability, as well a good pain control are major concerns in the anesthetic management of this patients.

Anaesthetic management of congenital adrenal hyperplasia in children - a case report