Copyright 2017 The Korean Society of Neuro-Ophthalmology http://neuro-ophthalmology.co.kr S43 ISSN: 2234-0971
A Case of the Orbital Apex Syndrome as Initial Presentation of Acute Myeloid Leukemia
Jae-Chan Ryu
1, Ji-Yun Park
1, Ju-Hyang Lee
2Departments of
1Neurology,
2Ophthalmology, Ulsan University Medical School, Ulsan University Hospital, Ulsan, Korea
Background: Patients with leukemia often have ocular manifestations. Although nearly all ocular structures can be affected, leu- kemic retinopathy is often the most clinically apparent manifestation. They sometimes may precede systemic diagnosis or may be a sign of leukemia recurrence. The optic nerve can reveal leukemic involvement of the CNS, which occurs more frequently in acute compared with chronic leukemia, children compared with adults and acute lymphocytic leukemia compared with acute myeloid leukemia. We report a rare case of superior orbital syndrome as initial presentation of adult acute myeloid leukemia.
Case report: A 54-year-old man presented with 4 days history of acute onset of ptosis and diplopia in the left eye. He was diag- nosed with diabetics on medication 20 years ago. He had been diagnosed with frontal lobe epilepsy by repeated episodes of sei- zures during sleep 17 years ago, but he did not take antiepileptics and he had same symptoms intermittently. He was scheduled to undergo surgery-navigation guided endoscopic sinus surgery at ENT with chronic rhinosinusitis two days later. On examination, his right eye was nearly complete ophthalmoplegia with ptosis, protrusion and decreased visual acuity (BCVA 0.3). CT of the paranasal sinuses showed mucosal thickening of the maxillary, ethomoid, sphenoid sinuses and the mainly the right nasal cavity suggesting chronic sinusitis. His brain MRI and MRA with enhancement showed mild thickening of the medial rectus muscle in the right orbit and pansinusitis without intracranial focal stenosis. Initial laboratory test showed normal CBCs and chemistry. He was initially diag- nosed with orbital apex syndrome due to inflammatory or infectious cause. ESS with culture and biopsy were performed and em- pirical antibiotics (ampicillin/sulbactam) with intravenous steroid pulse therapy were received. After steroid and antibiotics therapy, his ocular pain and gaze limitation was partially improved and he discharged. After 20 days of steroid therapy, he visited the emer- gency department because of aggravating eye pain and diplopia. At that time his CBCs showed pancytopenia and his bone mar- row biopsy showed increased lymphocytic histiocytes with erythroid precursor and RBCs that suggested pure erythoid leukemia.
Although he received chemotherapy and conservative care, he died three months after the onset of painful diplopia.
Conclusion: Orbital apex syndromes may result from a variety of inflammatory, infectious, neoplastic, iatrogenic/traumatic, and vascular condition. In this patient, orbital apex syndrome of adult leukemia is rarely the initial sign of the disease even initial CBCs were normal.
대한안신경의학회지: 제7권 Supplement 1 Clin Neuroophthalmol 7(Suppl 1):S43-43, May 2017
