CASE REPORTS

Non-Traumatic Subperiosteal
Orbital Hematoma as a Presenting
Sign of Chronic Myelogenous
Leukemia
Michael K. Yoon, M.D.*, and Timothy J. McCulley, M.D.†‡

Abstract: We present a case with a nontraumatic

subperiosteal orbital hematoma as a presenting sign of
chronic myelogenous leukemia. A previously healthy 34 year-
old man presented with a 2-day history of right upper eyelid
swelling. CT scan revealed a subperiosteal mass, and routine
blood tests revealed a white blood cell count of 290,000/l
with normal platelet count. Chronic myelogenous leukemia
was diagnosed based on a subsequent bone marrow biopsy.
After orbitotomy and evacuation of the hematoma, the
patient’s visual acuity and motility returned to normal.
In conclusion, chronic myelogenous leukemia should be
considered in the differential diagnosis of nontraumatic
subperiosteal orbital hematoma.

S ubperiosteal hematomas of the orbit are typically associated

with orbital or facial trauma.1 Nontraumatic subperiosteal
orbital hemorrhages have been reported in conjunction with
hematologic abnormalities and Valsalva maneuvers.2–4
Chronic myelogenous leukemia (CML) is a myelopro-
liferative neoplasm characterized by an abnormal proliferation
of granulocytes. This condition can be associated with bleeding
episodes from platelet dysfunction. We report a case of subperi-
osteal hematoma as a presenting sign of chronic myelogenous
leukemia in association with asymptomatic sinusitis.

CASE REPORT
An otherwise healthy 34-year-old Caucasian man
reported a 2-day history of increasing dull headache with the
development of right upper eyelid swelling. Examination
revealed normal visual acuity, no afferent pupillary defect,
2-mm right proptosis, limited elevation of the right eye, and
mild ecchymosis of the right upper eyelid. He had no fever, and
vital signs were within normal limits.
CT revealed a 2.8 × 2.5 × 0.8-cm right superior subperi-
osteal orbital mass (Fig.). The scan was otherwise normal with
the exception of partial opacification of the adjacent ethmoid
and frontal sinuses. Complete blood count revealed a white blood
count of 290,000/l and a platelet count of 345,000/l. Abnormal
values included international normalized ratio of 1.4 (normal
range 0.9–1.2) and prothrombin time of 16.8 (12.2–15.7). Partial
thromboplastin time, platelet count, factor VIII, factor IX, ABO
antibody titer, and Rh antibody titer were all normal.
*Massachusetts Eye and Ear Infirmary, Harvard School of Medicine,
Boston, Massachusetts; †Wilmer Eye Institute, Johns Hopkins School of
Medicine, Baltimore, Maryland, U.S.A.; and ‡King Khaled Eye Specialist
Hospital, Riyadh, Saudi Arabia
Accepted for publication June 21, 2011.
Supported in part by an unrestricted grant to the Wilmer Ophthalmological
Institute from Research to Prevent Blindness Inc., New York, New York, U.S.A.
The authors have no financial or conflict of interest to disclose.
Address correspondence and reprint requests Dr. Timothy J. McCulley,
M.D., The Wilmer Eye Institute, Johns Hopkins School of Medicine, 600
North Wolfe Street, Wilmer 110, Baltimore, MD 21287, U.S.A. E-mail:
tmccull5@jhmi.edu
DOI: 10.1097/IOP.0b013e31822ddf85
CT scan. A, Coronal view demonstrates homogeneous right
supero-medial orbital mass. Adjacent ethmoid and frontal
sinuses are opacified. B, Sagittal view shows superior right
orbital mass with indentation of the superior rectus/levator
complex and indentation of the globe. The frontal sinus mucosa
is only partially thickened.
On orbital exploration, via an eyelid crease incision, a
copious amount of noncoagulated blood in the subperiosteal
space was evacuated. No evidence of neoplasm, pus, or other
sign of infection was found. No organisms grew on culture. A
drain placed at the time of surgery was removed on postopera-
tive day 4 without further consequence.
The diagnosis of chronic myelogenous leukemia was
confirmed on bone marrow biopsy, with positive BCR-Abl flu-
orescent in situ hybridization and cytogenetic testing revealing
the 9;22 translocation (Philadelphia chromosome). The patient
underwent leukapheresis for concern of leukostasis causative of
the hemorrhage. He was subsequently initiated on hydroxyurea
and dasatinib with good response.
The proptosis and eyelid position had resolved by the end
of the second postoperative week. Visual acuity was 20/20 OU,
there was no afferent pupillary defect, extraocular motility was
full, and he was orthophoric in all fields of gaze.
DISCUSSION
Approximately half of patients with CML are asymp-
tomatic at the time of diagnosis, being identified on routine lab

Ophthal Plast Reconstr Surg, Vol. 28, No. 4, 2012 e79

Case Reports
screening. White blood cell count is typically elevated greater
than 100,000/l. Systemic symptoms may include fatigue,
weight loss, and abdominal fullness. Up to a fifth of patients
report abnormal bleeding episodes, usually secondary to plate-
let dysfunction.5 However, in many cases, platelet count may
be normal, as in our patient. Bleeding due to CML often mani-
fests as excessive bruising or hemorrhage from the nasal or oral
mucosa. We are unaware of previous reports of patients present-
ing with spontaneous orbital hemorrhage.
The etiology of the hemorrhage in our patient is not
entirely clear. The platelet count was normal, raising the possi-
bility of platelet dysfunction. The elevated international normal-
ized ratio and prothrombin time also likely contributed. Another
likely contributing factor is leukostasis. This is most commonly
seen in patients with acute myeloid or chronic myeloid leuke-
mia with an elevated blast cell count resulting in plugging of
the microvasculature. Local hypoxemia and cytokine release
can result in endothelial damage and subsequent hemorrhage.
The most common symptoms are respiratory or neurologi-
cal distress, and untreated, the 1-week mortality is 20–40%.6
Leukostasis has not been implicated in orbital hemorrhage pre-
viously and may have contributed to our patient’s hemorrhage.
Treatment was directed toward leukostasis with intravenous
hydration, leukapheresis, and hydroxyurea.
Our patient developed hemorrhage in the supero-medial
aspect of the orbit, which is a common location for nontrau-
matic subperiosteal orbital hemorrhages. In a review article by
Atalla et al.,4 25 of 29 previously published patients had bleeds
adjacent to the orbital roof. They suggested that the roof is most
commonly affected due to the anatomy of the periosteum. The
periorbita has firm attachments at the orbital fissures, optic
canal, orbital margins, suture lines, and other orbital foramina.
In part, this relatively high occurrence rate may relate to the
simple fact that this portion of the orbit has the largest area of
uninterrupted periorbita. Although the exact reason for the prev-
alence of hematomas in the superior orbit is not known, our case
falls in line with previous reports.
Various causes of and associations with nontraumatic
subperiosteal orbital hemorrhages have been reported. Valsalva
maneuvers and other sudden increases in cranial venous pres-
sure, including vomiting, strangulation, and child birth, have
been reported.2,4 Clotting abnormalities from liver dysfunction
and scurvy have been associated with nontraumatic subperi-
osteal orbital hemorrhages.3,4 Other cases have no identifiable
associations.
One confounder in this case is the opacified adjacent
sinuses. Although this most likely represented contiguous
hemorrhage, we cannot exclude the possibility of infection.
Infectious sinusitis has been implicated as causative of orbital
hemorrhage.7 We therefore cannot entirely exclude the possi-
bility that the orbital hemorrhage related to sinus disease and
occurred independently of the CML. Another possibility is that
the 2 in combination led to the bleed.
In summary, we describe a 30-year-old man with CML
identified on evaluation following a nontraumatic orbital
hemorrhage. Leukemia should be considered when managing
patients presenting with nontraumatic orbital hemorrhage.
REFERENCES
1. Wolter JR. Subperiosteal hematomas of the orbit in young males: a
serious complication of trauma or surgery in the eye region. Trans
Am Ophthalmol Soc 1979;77:104–20.
2. Katz B, Carmody R. Subperiosteal orbital hematoma induced by
the valsalva maneuver. Am J Ophthalmol 1985;100:617–8.
3. Sloan B, Kulwin DR, Kersten RC. Scurvy causing bilateral orbital
hemorrhage. Arch Ophthalmol 1999;117:842–3.
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Ophthal Plast Reconstr Surg, Vol. 28, No. 4, 2012
4. Atalla ML, McNab AA, Sullivan TJ, Sloan B. Nontraumatic sub-
periosteal orbital hemorrhage. Ophthalmology 2001;108:183–9.
5. Savage DG, Szydlo RM, Goldman JM. Clinical features at diagno-
sis in 430 patients with chronic myeloid leukaemia seen at a referral
centre over a 16-year period. Br J Haematol 1997;96:111–6.
6. Porcu P, Cripe LD, Ng EW, et al. Hyperleukocytic leukemias and
leukostasis: a review of pathophysiology, clinical presentation and
management. Leuk Lymphoma 2000;39:1–18.
7. Harris GJ, Kay MC, Nilles JJ. Orbital hematoma secondary to fron-
tal sinusitis. Ophthalmology 1978;85:1229–34.
A Unique Case of Foreign-Body
Associated Orbital Myositis
Seongmu Lee, M.D.*, Debra J. Shetlar, M.D.*†,
and Michael T. Yen, M.D.*
Abstract: A 50-year-old woman presented with a 2-week
history of diplopia and right-sided orbital pain with eye
movement. Examination revealed an edematous, ptotic right
upper eyelid with conjunctival hyperemia, proptosis, and
significant limitation to upward and downward ductions
on the right. MRI was significant for a homogeneously
enhancing lesion within the superior rectus muscle. A
laboratory evaluation seeking an infectious, inflammatory,
or autoimmune process was nonrevealing. A diagnosis of
orbital myositis was made, and the patient experienced
significant improvement with oral corticosteroids. The
patient’s symptoms, however, recurred after attempts at a
slow taper of the corticosteroids. An orbital biopsy of the
lesion revealed fibroadipose tissue containing irregularly
shaped yellow-white deposits birefringent under polarized
light, suggestive of silica crystals. The patient denied
any history of trauma or prior surgery. An intraorbital
triamcinolone injection to the superior orbit allowed
resolution of symptoms and a successful taper off systemic
corticosteroids.
O
rbital myositis describes an inflammatory process involv-
ing primarily the extraocular muscles and is categorized
within the broader category of idiopathic orbital inflammation.
Various inflammatory, infectious, vascular, and neoplastic pro-
cesses may manifest in a similar fashion, with variable sever-
ity, time frame, laterality, and muscle involvement, obfuscating
prompt diagnosis. Although rapid response to corticosteroids is
characteristic, the disease course may be refractory, progressive,
or recurrent despite therapy, and adverse effects may be intoler-
able, making long-term management challenging.1,2 We report a
case of orbital myositis, associated with refractile foreign bod-
ies on biopsy, that was successfully treated with a single intraor-
bital triamcinolone injection.
*Cullen Eye Institute, Department of Ophthalmology, Baylor College of
Medicine; †Michael E. DeBakey Veterans Affairs Medical Center, Houston,
Texas, U.S.A.
Accepted for publication June 21, 2011.
Financial Support: Supported in part by an unrestricted educational grant
from Research to Prevent Blindness, Inc. (New York, NY, U.S.A.).
The authors have no financial interest in any of the materials or tech­
niques described herein.
Address correspondence and reprint requests to Dr. Michael T. Yen,
Cullen Eye Institute, Department of Ophthalmology, Baylor College of
Medicine, 6565 Fannin NC-205, Houston, TX 77030, U.S.A. E-mail:
MYen@bcm.edu
DOI: 10.1097/IOP.0b013e31822ddf9b