Total knee arthroplasty in a patient with diastrophic dwarfism.
Diastrophic dwarfism is an autosomal recessive disease that predominantly occurs in the Finnish population (1 in 33,000) but has been known to occur worldwide. Affected patients present with multiple cartilaginous anomalies and early degeneration of weight-bearing joints. Once past infancy, life expectancy is favorable and patients may undergo multiple surgical procedures throughout their lifetime to repair .or replace affected joints. The characteristic short trunk of these patients in addition to scoliosis, cervical kyphosis, and involvement of articular cartilages can create unique ventilation and airway challenges for anesthesia providers involved in their care.
York College of Pennsylvania/WellSpan Health Nurse Anesthetist Program, USA.
Robin L
RL
Crowley
Richard E
RE
Haas
D000768:Anesthesia, General / Q000009:adverse effects / Q000379:methods*; D019645:Arthroplasty, Replacement, Knee; D004392:Dwarfism; D005260:Female; D006801:Humans; D007442:Intubation, Intratracheal / Q000009:adverse effects / Q000379:methods*; D008875:Middle Aged; D010612:Pharyngitis / Q000209:etiology / Q000517:prevention & control*