Anaesthetic considerations in Aarskog Scott Syndrome

Abstract

Aarskog Scott syndrome is a rare genetic disorder characterised by facial, limb and genital abnormalities first described in 1970. Its evolving nature in terms of associated features and increased surgical interventions necessitates anaesthesiologists to have a thorough knowledge about this syndrome for a better preparedness. Although multiple case reports have been published in literature since its discovery, no case report regarding its anaesthetic considerations and challenges have been described in literature till now. We report challenges encountered and successful anaesthetic management of a seven-year-old girl with Aarskog Scott Syndrome posted for a corneal repair in view of traumatic corneal perforation.

Aortic root aneurysm in a patient with Aarskog–Scott syndrome

“…Aarskog–Scott syndrome is primarily known to be a cause of short stature, craniofacial dysplasia, digital anomalies, and genital malformations 7 . Nevertheless, in the course of time, various degrees of cardiovascular involvement have been reported in literature.…”