Extra-medullary hematopoiesis causing bilateral optic atrophy in beta thalassemia/Hb E disease.
OBJECTIVE
To report a rare presentation of bilateral optic nerve compression from extra-medullary hematopoiesis in beta thalassemia/Hb E disease.


METHODS
Interventional case report.


METHODS
A 13-year-old Thai girl was reported with slowly progressive bilateral visual loss due to optic neuropathy as a result of compression from extra-medullary hematopoiesis. Computed tomography of orbit and brain revealed a homogeneous enhancing midline soft tissue mass originating from the ethmoid and sphenoid sinuses along with extensive involvement of the skull.


METHODS
Blood transfusion once every month and low-dose radiotherapy to sphenoid and ethmoid bones with some visual improvement.


CONCLUSIONS
Extra-medullary hematopoiesis in beta thalassemia can involve paranasal sinuses and cause compressive neuropathy. In patients with beta thalassemia that required multiple blood transfusions with progressive visual loss, ectopic marrow compression of optic nerve should be ruled out.
Ittipunkul
Nimitr
N
Department of Ophthalmology, Faculty of Medicine, Chiang Mai University, Thailand.
Martin
Tada
T
Siriwanasan
Rutchada
R
Olanratanachai
Kanokporn
K
Rootman
Jack
J
D000293:Adolescent; D005260:Female; D006411:Hematopoiesis, Extramedullary; D006801:Humans; D009900:Optic Nerve / Q000000981:diagnostic imaging / Q000503:physiopathology*; D014057:Tomography, X-Ray Computed; D017086:beta-Thalassemia / Q000150:complications*