Pycnodysostosis: An Anaesthetic Approach to This Rare Genetic Disorder

Author:

Puri Rajeev1ORCID,Saxena Arpita1ORCID,Mittal Awak2,Arshad Zia1,Dwivedi Yogita1,Chand Trilok1,Mittal Apurva1,Agrawal Archna1,Prakash Jay1,Pilendran Sathiyanarayanan1

Affiliation:

1. Department of Anaesthesia and Critical Care, S. N. Medical College, Agra, India

2. Department of Orthopaedics, S. N. Medical College, Agra, India

Abstract

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.

Publisher

Hindawi Limited

Subject

Anesthesiology and Pain Medicine

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