Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and Management

摘要

Prader-Willi syndrome is a rare congenital disorder characterized by obesity, hypotonia, hypogonadism and developmental delay. The genetic disorder is caused by microdeletion on chromosome 15 on the paternal chromosome. The syndrome is a 2-stage disorder with hypotonic early infantile phase and an obese childhood phase. These individuals have abnormal physiologic response to hypoxia and hypercapnia, narrow oropharyngeal space, thick secretions and exaggerated response to sedatives posing increased anaesthetic risk. We describe the anaesthetic management of a patient with Prader-Willi syndrome where in laparoscopic orchidopexy was performed successfully under general anaesthesia.