Anaesthetic Concerns In A Child With 21-Hydroxylase Deficiency For A Day Care Surgery.

摘要

Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is one of the most common autosomal recessive hereditary diseases. The impairment of cortisol synthesis leads to excessive stimulation of adrenal gland by adrenocorticotropic hormone(ACTH), adrenal hyperplasia and excessive androgen synthesis. More than 90% of congenital adrenal hyperplasia is caused by 21-hydroxylase deficiency which is found in 1:10,000 to 1:15,000 live births. Children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency presenting for surgeries is very unusual. Here we are presenting a fifteen year old male child with classical 21-hydroxylase deficiency, who was successfully managed as a day case for elective orchidopexy under general anaesthesia. The consent for publication for the case was obtained by mother of child after surgery.