Abstract
An 18-year-old female patient diagnosed with McCune–Albright syndrome
(MAS) developed postoperative acute heart failure with negative findings
during preoperative cardiac screening and restricted fluid infusion.
Latent cardiovascular involvement of MAS could be a reasonable
explanation for the present case.
INTROUCTION
McCune-Albright syndrome (MAS) is a rare genetic disease characterized
by polyostotic fibrous dysplasia, skin hyperpigmentation, and endocrine
dysfunction, with the prevalence ranging between 1/100 000 and 1/1 000
0001, 2. Due to the severe deformity of lower limbs
caused by fibrous dysplasia in the proximal femur or tibia, substantial
numbers of patients diagnosed with MAS are admitted to orthopedics
clinics for surgical interventions3. Although the
skeletal and endocrine abnormalities of MAS are well recognized by most
clinicians, rare but fatal cardiovascular involvement is seldom
reported. It is also reported that cardiac abnormality such as myocyte
hypertrophy, visible microscopically in a macroscopically normal heart,
strongly suggests that Gs mutation may be present in the heart with
inconspicuous macroscopic findings4. Thus,
the cardiovascular involvement of
MAS patients is prone to be overlooked in clinical practice and related
to potential risk of medical disputes. To the best of our knowledge,
cardiovascular involvement of MAS patients who underwent orthopedic
surgery has not yet been presented. Here, we report the case of a young
woman with MAS who suffered from acute heart failure after proximal
femur osteotomy for lower limb deformity correction, although she had no
positive cardiovascular abnormality or history of cardiovascular
disease.
CASE PRESENTATION