TITLE:
Long term treatment of recurring pathological fractures due to Mccune Albright Syndrome: Case report and literature review
AUTHORS:
Yoshvin Sunnassee, Yuhui Shen, Rong Wan, Jianqiang Xu, Weibin Zhang
KEYWORDS:
McCune Albright Syndrome; Surgical Treatment; Polyostotic Fibrous Dysplasia
JOURNAL NAME:
Case Reports in Clinical Medicine,
Vol.2 No.9,
December
20,
2013
ABSTRACT:
McCune Albright
syndrome is a rare genetic disorder which is characterized by café au lait skin
pigmentation, precocious puberty and polyostotic fibrous dysplasia. Treating
recurring pathological fractures due to Albright syndrome is a very challenging
endeavor, and more so when it is accompanied by poor bone quality and deformity.
We hereby present the case of a 23-year-old male patient who is treated several
times for recurrent pathological fractures of the femur at our center. We
analyze the difficulties associated with treating a patient with poor bone quality
over several years, discuss our treatment options, review the literature for
similar cases and look at what we could have done differently. We weigh in on
the difficulties in treating a severely deformed shepherd’s crook, the ways of
achieving proper internal fixation and the dangers of using plating instead of
an IM nail as suggested in the literature. Our main goal in reporting this case
is to bring forth the unusual challenges encountered when treating patients
with Albright syndrome and discussing the options of the orthopedic surgeons
when treating these types of patients.