Pycnodysostosis : An Anaesthetic Approach to This Rare Genetic Disorder

Abstract EN

Pycnodysostosis (the Toulouse-Lautrec syndrome) is a rare autosomal-recessive disorder of osteoclast dysfunction.

This disorder was first described by Maroteaux and Lamy in 1962.

We describe anaesthetic management of a 35-year-old female having pyknodysostosis with fracture shaft left femur with anticipated difficult intubation.

Therefore, spinal anesthesia was planned for her fracture fixation.

The intra- and postoperative period remains uneventful.