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Case Reports in Anesthesiology
Volume 2013 (2013), Article ID 716756, 3 pages
http://dx.doi.org/10.1155/2013/716756
Case Report

Pycnodysostosis: An Anaesthetic Approach to This Rare Genetic Disorder

1Department of Anaesthesia and Critical Care, S. N. Medical College, Agra, India
2Department of Orthopaedics, S. N. Medical College, Agra, India

Received 21 December 2012; Accepted 10 February 2013

Academic Editors: A. Apan, E. Farag, A. Han, C.-H. Hsing, E. W. Nielsen, and D. A. Story

Copyright © 2013 Rajeev Puri et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.