Group of rare diseases characterized by the destruction or abnormal resorption of bone tissue that is not related to a tumor, trauma or infection.

The classification of the International Skeletal Dysplasia Society includes:

-        familial expansive osteolysis

-        mandibuloacral dysplasia

-        progeria or Hutchinson-Gilford syndrome

-        Torg-Winchester syndrome or MONA (Multicentric Osteolysis-Nodulosis-Arthropathy)

-        Hajdu-Cheney syndrome

-        pycnodysostosis

-        multicentric carpotarsal osteolysis

-        lipomembranous osteodystrophy with leukoencephalopathy (Nasu-Hakola disease)

One could add Gorham-Stout syndrome although it is not hereditary.

Anesthetic implications:

intubation difficulties worsening with age: micrognathia, deformation of the jaw, cervical lesions

References : 

-         Herd RS, Sprung J, Weingarten TN. 
Primary osteolysis syndromes: beware of difficult airway. 
Pediatr Anesth 2015; 25: 727-37.

Updated: October 2018