Pycnodysostosis: an anaesthetic approach to this rare geneti
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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. This disorder is characterized by short stature, increased bone density, short and stubby fingers, fragile bones that may fracture easily, and craniofacial abnormalities caused by delayed suture closure. Patients usually present with frequent fractures even after minor trauma.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....

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