Pycnodysostosis and severe laryngomalacia complicating general anesthesia: A case report.

Abstract

Pycnodysostosis is a very rare autosomal recessive disease. This disorder presents with osteosclerosis, leading to fragile bones, short stature, craniofacial abnormalities, laryngomalacia, sleep apnea syndrome, and many other findings. Difficulty intubation is very common. In our case, we have a patient scheduled for a plastic surgery on her skull under general anesthesia (GA), but she could not lie in a flat position as this could lead to cyanosis and cardiac arrest. So, we have to intubate her in 45° back-up position. Intubation in positions different from the usual supine one probably because of severe laryngomalacia in this patient is possible but needs skilled anesthesiologists and trained personnel.