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2015 ; 2
(2
): 80-84
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gab.com Text
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English Wikipedia
Surgical Treatment of Gorham s Disease with Massive Osteolysis of the Skull and
Cervical Spine: A Case Report and Review of Literature
#MMPMID28663971
Kohno M
; Aota Y
; Kawai T
; Murata H
; Saito T
NMC Case Rep J
2015[Apr]; 2
(2
): 80-84
PMID28663971
show ga
Gorham's disease is a rare disorder of unknown etiology and variable clinical
presentation and is characterized by the proliferation of lymphatic vessels
associated with massive regional osteolysis. Although 10 cases involving the
skull and cervical spine have been reported in the literature, little is
available concerning the surgical treatment of either atlantoaxial dislocation or
basilar impression. Most cases have experienced universally unsuccessful
treatment with bone grafts, which have led to dissolution. This case report
describes the clinical course, and radiotherapeutic, medical, and surgical
treatment for Gorham's disease with basilar impression and massive osteolysis of
the skull and upper cervical spine. The case of a 27-year-old man with
progressive massive osteolysis of the skull and cervical spine is reported.
Multiple surgical treatments to decompress the spinal cord and stabilize the
skull and upper cervical spine with autologous fibular grafts were performed in
order to prevent the progression of atlantoaxial dislocation and basilar
impression. Pathologically, radiotherapy failed to show any effect. The efficacy
of antiresorptive therapy with bisphosphonates could not be confirmed either
clinically or radiologically. Although solid bone fusion was not obtained, the
patient has achieved a satisfactory functional outcome and remains completely
active after repeated surgeries. Surgical treatment is extremely difficult in
cases of Gorham's disease involving the skull and upper cervical spine. Fibular
bone grafts seem to show resistance to erosion to osteolytic tissue.