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Idiopathic Orbital Hemorrhage Related to the Inferior Rectus Muscle
Idiopathic Orbital Hemorrhage Related to the Inferior Rectus Muscle A Rare Cause for Acute-Onset Diplopia and Unilateral Proptosis Guy J. Ben Simon, MD,1,2 Alan A. McNab, FRANZCO2 Purpose: To report 6 patients with spontaneous orbital hemorrhage in relation to the inferior rectus muscle. Design: Retrospective observational case series. Participants: Six patients with acute onset orbital pain, diplopia, and proptosis referred to the orbital clinic at the Royal Victorian Eye and Ear Hospital, January 1995 through December 2004. Methods: Review of clinical history, imaging studies, and follow-up. Main Outcome Measures: Resolution of proptosis and diplopia and imaging studies of the orbit, including computed tomography (CT), magnetic resonance imaging (MRI), or both. Results: Six patients (4 men, 2 women; mean age, 68 years) were referred with acute onset of unilateral proptosis and diplopia. None of the patients had a history of bleeding disorder. Visual acuity and intraocular pressure were normal; 3 patients showed marked limitation in upgaze on the affected side. Imaging studies of the orbit by CT, MRI, or both showed inferior orbital hemorrhage either within (4 patients) or adjacent to (2 patients) the inferior rectus muscle, possibly within the muscular sheath. Symptoms resolved completely in all but 1 patient, who had persistent diplopia in extreme upgaze. Follow-up CT, MRI, or both showed complete resolution in 3 patients and mild persistent inferior rectus muscle thickening in 2 patients. Conclusions: Acute onset proptosis and diplopia may be secondary to spontaneous orbital hemorrhage within the inferior rectus muscle or its sheath with no other orbital or systemic pathologic features. Symptoms resolved spontaneously over the course of days to weeks, and orbital imaging showed complete resolution or mild persistent enlargement of the muscle without significant symptoms. Ophthalmology 2005;112:1838 –1843 © 2005 by the American Academy of Ophthalmology.
Orbital hemorrhage in the absence of trauma is a rare condition that may occur spontaneously or as a result of a preexisting orbital disease such as tumor, vascular malformation, or inflammation. Case reports in the literature describe spontaneous orbital hemorrhage in the presence of arterial hypertension and systemic coagulopathies and after surgery.1–7 Spontaneous hemorrhage also may occur in defined anatomic space, such as subperiosteally (usually in the orbital roof),6 or within the capsule surrounding alloplastic orbital implants.8 It usually manifests as acute onset of orbital pain, proptosis, and diplopia,9 and in most cases subsides within weeks with no long-term sequelae. Surgery rarely is required, except in patients with vision-threatening Originally received: March 4, 2005. Accepted: April 13, 2005. Manuscript no. 2005-188. 1 Orbital, Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital, Melbourne, Australia. 2 Department of Ophthalmology, Royal Victorian Eye and Ear Hospital, Melbourne, Australia. Correspondence to Guy J. Ben Simon, MD, The Royal Victorian Eye and Ear Hospital, 32 Gisbourne Street, East Melbourne 3002, Victoria, Australia. E-mail: [email protected].
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© 2005 by the American Academy of Ophthalmology Published by Elsevier Inc.
hematoma, and is aimed at relieving increased intraorbital pressure and optic nerve compression. Orbital imaging either by computed tomography (CT) or magnetic resonance imaging (MRI) is required to diagnose the hemorrhage and to help rule out preexisting orbital disease such as tumor or vascular malformation. We previously reported 3 cases of spontaneous orbital hemorrhage confined to a rectus muscle.2 In all cases, hemorrhage resolved spontaneously, with no effect on visual acuity. There is a paucity of literature regarding this topic; to our knowledge, there are only 3 reports about spontaneous orbital hemorrhage with no underlying orbital or systemic disease. The purpose of the current study was to report an additional 6 cases of spontaneous orbital hemorrhage within the inferior rectus muscle or its sheath.
Patients and Methods Medical record review of all patients referred with acute onset unilateral proptosis and diplopia at the Royal Victorian Eye and Ear Hospital was performed. All patients were referred between January 1995 and December 2004. Data regarding clinical signs and symptoms, comprehensive ISSN 0161-6420/05/$–see front matter doi:10.1016/j.ophtha.2005.04.019
Ben Simon and McNab 䡠 Idiopathic Orbital Hemorrhage Related to the Inferior Rectus Muscle Table 1. Demographics of 6 Patients with Spontaneous Orbital Hemorrhage within the Inferior Rectus Muscle or its Sheath Referred to the Royal Victorian Eye and Ear Hospital from January 1995 through December 2004
6 patients Signs and symptoms
Clinical examination
Imaging (CT/MRI) CT/MRI at follow-up
Clinical Feature
Value (Percentage/Standard Deviation/Range)
Male Female Age (yrs) Proptosis Pain/discomfort Diplopia Globe displacement Nausea/vomiting Visual acuity IOP (mmHg) Relative proptosis (mm)* Ocular motility limitation IR hemorrhage Inferior orbit hemorrhage† Complete resolution Residual mild thickening of IR
4 (67) 2 (33) 68 (⫾14; 39–77) 6 (100) 5 (83) 5 (83) 3 (50) 2 (33) 20/20 17 (⫾1.7; 15–18) 2.8 (⫾2.1; 1–5) 4 (67) 4 (67) 2 (33) 4 (67) 2 (33) 7.5 (⫾4.1; 1.2–12) 18.0 (1–84)
Time to resolution (wks) Follow-up (mos)
CT ⫽ computerized tomography; IOP ⫽ intraocular pressure; IR ⫽ inferior rectus; MRI ⫽ magnetic resonance imaging. *Relative proptosis in comparison with the other unaffected orbit. † Adjacent to or within the inferior rectus muscle sheath.
eye examination, and imaging studies (CT, MRI, or both) were collected and analyzed. All patients were diagnosed with spontaneous orbital hemorrhage within a rectus muscle or its sheath according to clinical symptoms, imaging findings (homogenous mass within a rectus muscle or its sheath, well circumscribed, hyperintense on MRI and hyperdense on CT, mild contrast enhancement, homogenous enhancement, fluid level possibly present), and spontaneous clinical and radiographic resolution. Blood tests and coagulation profiles were reported in only 2 patients. No surgical intervention was required and no underlying orbital or systemic disease that may have caused orbital hemorrhage was found.
Results Six patients (4 men, 2 women; mean age, 68 years; range, 39 –77 years) with acute onset of proptosis and diplopia with or without orbital discomfort were diagnosed with spontaneous orbital hemorrhage within the inferior rectus muscle or its sheath. The demographics of the study population are summarized in Table 1. No patient gave a history of trauma, straining, heavy lifting, or bleeding tendencies.
Case Reports Patient 1. A 69-year-old woman with a past medical history of controlled hypertension and hypercholesterolemia who was taking low-dose aspirin was referred with acute right proptosis, blurred vision, vertical diplopia, nausea, and vomiting. Clinical examination disclosed right proptosis of 1 mm relative to the left globe, normal visual acuity of 20/20, and normal intraocular pressure of 18 mmHg. Ocular motility was restricted in upgaze and downgaze (20° and 10°, respectively) but normal in adduction and abduction. Anterior segments and fundus examination results were normal. Computed visual field examination results were normal. Magnetic resonance imaging of the orbit revealed a well-defined mass lesion in the floor of the right orbit mea-
suring 2.5 ⫻ 2 cm, displacing the inferior rectus muscle medially and the optic nerve superiorly. Signal characteristics were homogenous hyperintensity compatible with a diagnosis of recent hemorrhage. Computed tomography of the orbit showed similar findings with a teardrop configuration of the lesion and an elongated tail toward the orbital apex. The lesion was hyperdense on CT scan, and no contrast enhancement was observed. Symptoms resolved spontaneously over the course of 6 weeks. Repeated CT of the orbit showed mild thickening of the posterior half of the right inferior rectus muscle. The patient was diagnosed with spontaneous inferior orbital hemorrhage related to the right inferior rectus muscle. Patient 2. A 77-year-old man had sudden onset of diplopia associated with redness and swelling of the left lower eyelid; diplopia improved over the course of days after the episode. Past medical and ophthalmic history was unremarkable, with no history of easy bruising, bleeding abnormality, or oral medications. Clinical examination disclosed left visual acuity of 20/30, left proptosis with lateral displacement of the globe, and mild limitation in upgaze to the left. Eyelid swelling resolved within several days. Imaging studies of the orbit, including CT and MRI, showed a well-demarcated inferomedial orbital floor lesion tapering to the orbital apex. The inferior rectus muscle belly was displaced laterally and appeared to be within the wall of the lesion, suggesting intrasheath hemorrhage. Magnetic resonance imaging showed low signal on T1 and T2 imaging with minimal enhancement of the rim of the lesion. The patient was diagnosed with spontaneous orbital hemorrhage related to the left inferior rectus muscle. Clinical signs and symptoms resolved gradually over the course of 9 days, and repeated MRI studies showed complete resolution of the orbital hemorrhage. Patient 3. A 39-year-old man was referred with right orbital pain and acute onset of proptosis accompanied by vomiting. Clinical examination disclosed right proptosis of 1 mm relative to the left globe, normal visual acuity of 20/20, intraocular pressure of 18 mmHg, and no ocular motility restriction. Computed tomography of the orbit revealed a mass in the right inferior orbit that smoothly enlarged the belly of the right inferior rectus in the posterior two thirds (Fig 1). Magnetic resonance imaging of the orbit disclosed
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Figure 1. Sagittal postcontrast computed tomography scan of a 39-year-old man with acute painful right proptosis and vomiting at presentation. A homogenous mass is shown in the inferior orbit with smooth enlargement of the posterior two thirds of the inferior rectus muscle.
an enlargement of the right inferior rectus muscle with an area of high signal, characteristic of recent hemorrhage contained within the muscle (Fig 2). No contrast enhancement was noted on the postgadolinium studies. Diagnosis was consistent with spontaneous intramuscular hematoma. Repeated imaging studies 2 months later showed complete resolution of the intramuscular hematoma. Three additional patients (2 men, 1 woman; mean age, 73
years) had acute onset of painful proptosis and double vision. In all patients, CT or MRI studies, or both, confirmed the diagnosis of orbital hemorrhage confined to the inferior rectus muscle or its sheath and no contrast enhancement was noted on imaging studies. A complete blood count and determination of the international normalized ratio were performed in 2 patients and revealed normal results. Symptoms resolved in all but 1 patient, who had persistent
Figure 2. Coronal magnetic resonance imaging scans of a 39-year-old man with acute painful right proptosis and vomiting. A, T1-weighted image showing a hyperintense homogenous mass (asterisk) within the inferior rectus muscle (white arrow). B, T1-weighted image after gadolinium fat saturation showing homogenous contrast enhancement of the lesion (asterisk), characteristic of a recent clot with methemoglobin.
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Ben Simon and McNab 䡠 Idiopathic Orbital Hemorrhage Related to the Inferior Rectus Muscle
Figure 3. A, A 69-year-old man with sudden onset of left orbital pain, double vision, and proptosis. Clinical examination revealed normal visual acuity of 20/20, mild limitation in upgaze on the left, and left relative proptosis of 5 mm with conjunctival chemosis. B, Coronal computed tomography (CT) scan of the orbit showing left inferior rectus muscle enlargement consistent with intramuscular hemorrhage (black arrow). Note that the inferior rectus muscle cannot be distinguished (asterisk denotes the back of the right globe). C, Axial CT scan of the orbits revealing the anteroposterior margins of the hemorrhage and a fluid level within the clot (black arrow).
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Ophthalmology Volume 112, Number 10, October 2005 diplopia on extreme upgaze only. Figure 3 shows that patient in the acute phase of the hemorrhage.
Discussion We describe 6 patients with spontaneous orbital hemorrhage confined to the inferior rectus muscle or its sheath. All had acute painful proptosis and diplopia at presentation. Computed tomography, MRI, or both demonstrated a well-defined hematoma, sometimes with a fluid level, within the belly of the inferior rectus muscle or its sheath and displacing the muscle belly to one side. In all patients, visual acuity was preserved. None of the patients had a history of bleeding disorder. No patient had a history of trauma or sudden rise in venous pressure that can occur with straining or heavy lifting. Orbital hemorrhage, proptosis, and motility restriction resolved spontaneously, with only 2 patients showing residual asymptomatic mild rectus muscle enlargement, presumably secondary to fibrosis. In 1994, we reported 3 patients with spontaneous orbital hemorrhage within a rectus muscle.2 All 3 patients had sudden onset of unilateral painful proptosis, diplopia, and an enlarged rectus muscle as shown by CT or MRI; hemorrhage resolved spontaneously in all patients. Similarly, no history of bleeding disorder was found, and visual acuity was preserved in 2 patients; 1 patient had counting fingers acuity secondary to a vascular event 50 years before presentation. That report differed from the current series in that the muscles involved were the lateral, medial, and inferior rectus. Residual thickening was noted in just 1 patient. In both reports, no surgical intervention was needed. It is important to distinguish our patients from previous reports of spontaneous orbital hemorrhage into the subperiosteal space,10 –13 which were associated with a sudden rise in cranial venous pressure or systemic coagulopathies. The cause of the hemorrhage in these patients is obscure; most of the patients were older adults without significant medical problems. We cannot postulate any specific anomaly that may be associated with this rare clinical presentation, but the clinical and imaging findings are sufficient characteristics to allow a confident diagnosis and conservative treatment. Our case series differs from other cases of spontaneous orbital hemorrhage in that our patients did not have any orbital or systemic disease and did not undergo surgery or general anesthesia. Previous reports described orbital hemorrhage associated with surgery or occurring as a complication of general anesthesia.10,11,13–15 One report describes spontaneous bilateral orbital hemorrhage in a newborn that subsided with no further sequelae.16 The vasoocclusive process in sickle cell anemia has been implicated with orbital subperiosteal hematomas. Other rare causes include orbital reconstruction with implant8,17 and systemic diseases such as septicemia,18 leukemia,19 bleeding diathesis or systemic coagulopathies12,20 –23; it has even been reported after vigorous paroxysmal coughing and other causes of a sudden rise in cranial venous pressure.24 In the largest series of orbital hemorrhage reported in the literature, Sullivan and Wright3 described 115 cases of
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nontraumatic orbital hemorrhage. An associated orbital vascular malformation was present in 90% of their patients, whereas in only 6 patients (5%) no underlying cause was observed. Most patients showed spontaneous complete or partial resolution, with only 8 (7%) requiring surgery for optic nerve compression. Final visual acuity was reduced in 23 patients (20%). No patient in this series was described as having a hemorrhage within an extraocular muscle. Orbital imaging, either by CT or MRI scan, is helpful in diagnosing and localizing a hemorrhage and is important to rule out any coexisting orbital pathologic features. An MRI scan may help to determine the age of the hemorrhage.2 A fresh clot containing deoxyhemoglobin appears hypointense on T1 and T2 imaging; as it changes to methemoglobin, the signal becomes hyperintense from the periphery to the center of the hemorrhage, eventually producing a homogenous high signal.2 The intensity lessens as the methemoglobin breaks down to hemosiderin and ferritin. In our patients, the hemorrhage was always well defined with no spread into adjacent orbital fat, suggesting confinement of the hemorrhage by the muscle sheath. In most instances, the hemorrhage is larger anteriorly with an almost perpendicular anterior face and tapers posteriorly. In some patients, a fluid level can be identified within the hematoma (Fig 3). The differential diagnosis of an enlarged extraocular muscle includes inflammatory and neoplastic processes, but these are usually associated with a more gradual onset and additional orbital or systemic features.25,26 The main groups of diseases include idiopathic orbital inflammation and myositis, thyroid-related orbitopathy, and orbital lymphoma. The sudden onset, spontaneous resolution, and CT and MRI findings help to distinguish acute hemorrhage from orbital myositis. Limitations of the current study stem from its retrospective design. No formal testing to exclude bleeding diathesis was performed except in 2 patients. Visual field testing was performed in only 1 patient; therefore, optic nerve function could be estimated based solely on normal visual acuity. We describe 6 patients with spontaneous orbital hemorrhage to the inferior rectus muscle or its sheath with acute onset proptosis, pain, and diplopia at presentation. Symptoms subsided within days to weeks from the initial presentation. Repeated CT or MRI scans, or both, showed complete resolution of the hemorrhage in 4 patients, and in 2 patients, mild asymptomatic thickening of the muscle was noted. This case series highlights another well-defined entity of spontaneous hemorrhage within the inferior rectus muscle or its sheath.
References 1. Hajji Z, Cherqi J, Berraho A. Spontaneous orbital hematoma in an adult. A case report [in French]. J Fr Ophtalmol 2004; 27:267–70. 2. Hakin KN, McNab AA, Sullivan TJ. Spontaneous hemorrhage within the rectus muscle. Ophthalmology 1994;101:1631– 4. 3. Sullivan TJ, Wright JE. Non-traumatic orbital haemorrhage. Clin Experiment Ophthalmol 2000;28:26 –31.
Ben Simon and McNab 䡠 Idiopathic Orbital Hemorrhage Related to the Inferior Rectus Muscle 4. Krohel GB, Wright JE. Orbital hemorrhage. Am J Ophthalmol 1979;88:254 – 8. 5. Law FW. Spontaneous orbital haemorrhage. Br J Ophthalmol 1971;55:556 – 8. 6. Atalla ML, McNab AA, Sullivan TJ, Sloan B. Nontraumatic subperiosteal orbital hemorrhage. Ophthalmology 2001;108:183–9. 7. Zenobii M, Galzio RJ, Lucantoni D, et al. Spontaneous intraorbital hemorrhage cause by cavernous angioma of the orbit. J Neurosurg Sci 1984;28:37– 40. 8. Gilhotra JS, McNab AA, McKelvie P, O’Donnell BA. Late orbital haemorrhage around alloplastic orbital floor implants: a case series and a review. Clin Experiment Ophthalmol 2002;30:352–5. 9. Turpin-Fenoll L, Carneado-Ruiz J, Pampliega-Perez A, et al. Binocular diplopy as the presentation of a hematoma in the rectus inferior [in Spanish]. Rev Neurol 2004;38:1152–5. 10. Doan AP, Lee SK, Chaloupka J, et al. Subperiosteal hematoma of the orbit following an aneurysm coiling procedure. Am J Ophthalmol 2004;138:680 –2. 11. Griffeth MT, Dailey RA, Ofner S. Bilateral spontaneous subperiosteal hematoma of the orbits: a case report [letter]. Arch Ophthalmol 1997;115:679 – 80. 12. Moorthy RS, Yung CW, Nunery WR, et al. Spontaneous orbital subperiosteal hematomas in patients with liver disease. Ophthal Plast Reconstr Surg 1992;8:150 –2. 13. Nakai K, Doi E, Kuriyama T, Tanaka Y. Spontaneous subperiosteal hematoma of the orbit. Surg Neurol 1983;20:100 –2. 14. Peden MC, Bhatti MT. Spontaneous orbital hemorrhage complicating cardiac surgery. Cardiovasc Intervent Radiol 2004; 27:294 – 6.
15. Gunning KE, Collett BJ. Spontaneous retrobulbar haemorrhage following anaesthesia. Anaesthesia 1987;42:875– 6. 16. Ezzadin EM, Liu D, Al-Rashed W, Jacquemin C. Bilateral orbital hemorrhage in a newborn. Am J Ophthalmol 2000;129: 531–3. 17. Custer PL, Lind A, Trinkaus KM. Complications of supramid orbital implants. Ophthal Plast Reconstr Surg 2003;19:62–7. 18. Singh AJ. Spontaneous orbital haemorrhage in purpura fulminans secondary to meningococcal septicemia. Eye 2002;16: 190 –3. 19. Jinnai K, Hayashi Y. Hemorrhage in the oculomotor nerve as a complication of leukemia. Neuropathology 2001;21:241– 4. 20. Scholl HP, Thiel HJ, Schlote T. Orbital hemorrhage as a sequela of heparin-induced thrombocytopenia [in German]. Klin Monatsbl Augenheilkd 1999;215:197–200. 21. Chorich LJ, Derick RJ, Chambers RB, et al. Hemorrhagic ocular complications associated with the use of systemic thrombolytic agents. Ophthalmology 1998;105:428 –31. 22. Chang WJ, Nowinski TS, Repke CS, Buerger DG. Spontaneous orbital hemorrhage in pregnant women treated with subcutaneous heparin. Am J Ophthalmol 1996;122:907– 8. 23. Sires BS, Goodrich SD, Holds JB. Spontaneous orbital hemorrhage in a child with autoimmune hepatitis. Am J Ophthalmol 1993;15:384 –5. 24. Rieger G. A rare cause of complete “spectacle hematoma.” Ger J Ophthalmol 1996;5:415– 6. 25. Patrinely JR, Osborn AG, Anderson RL, Whiting AS. Computed tomographic features of nonthyroid extraocular muscle enlargement. Ophthalmology 1989;96:1038 – 47. 26. Rothfus WE, Curtin HD. Extraocular muscle enlargement: a CT review. Radiology 1984;151:677– 81.
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Orbital hemorrhage in the absence of trauma is a rare condition that may occur spontaneously or as a result of a preexisting orbital disease such as tumor, vascular malformation, or inflammation. Case reports in the literature describe spontaneous orbital hemorrhage in the presence of arterial hypertension and systemic coagulopathies and after surgery.1–7 Spontaneous hemorrhage also may occur in defined anatomic space, such as subperiosteally (usually in the orbital roof),6 or within the capsule surrounding alloplastic orbital implants.8 It usually manifests as acute onset of orbital pain, proptosis, and diplopia,9 and in most cases subsides within weeks with no long-term sequelae. Surgery rarely is required, except in patients with vision-threatening Originally received: March 4, 2005. Accepted: April 13, 2005. Manuscript no. 2005-188. 1 Orbital, Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital, Melbourne, Australia. 2 Department of Ophthalmology, Royal Victorian Eye and Ear Hospital, Melbourne, Australia. Correspondence to Guy J. Ben Simon, MD, The Royal Victorian Eye and Ear Hospital, 32 Gisbourne Street, East Melbourne 3002, Victoria, Australia. E-mail: [email protected].
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© 2005 by the American Academy of Ophthalmology Published by Elsevier Inc.
hematoma, and is aimed at relieving increased intraorbital pressure and optic nerve compression. Orbital imaging either by computed tomography (CT) or magnetic resonance imaging (MRI) is required to diagnose the hemorrhage and to help rule out preexisting orbital disease such as tumor or vascular malformation. We previously reported 3 cases of spontaneous orbital hemorrhage confined to a rectus muscle.2 In all cases, hemorrhage resolved spontaneously, with no effect on visual acuity. There is a paucity of literature regarding this topic; to our knowledge, there are only 3 reports about spontaneous orbital hemorrhage with no underlying orbital or systemic disease. The purpose of the current study was to report an additional 6 cases of spontaneous orbital hemorrhage within the inferior rectus muscle or its sheath.
Patients and Methods Medical record review of all patients referred with acute onset unilateral proptosis and diplopia at the Royal Victorian Eye and Ear Hospital was performed. All patients were referred between January 1995 and December 2004. Data regarding clinical signs and symptoms, comprehensive ISSN 0161-6420/05/$–see front matter doi:10.1016/j.ophtha.2005.04.019
Ben Simon and McNab 䡠 Idiopathic Orbital Hemorrhage Related to the Inferior Rectus Muscle Table 1. Demographics of 6 Patients with Spontaneous Orbital Hemorrhage within the Inferior Rectus Muscle or its Sheath Referred to the Royal Victorian Eye and Ear Hospital from January 1995 through December 2004
6 patients Signs and symptoms
Clinical examination
Imaging (CT/MRI) CT/MRI at follow-up
Clinical Feature
Value (Percentage/Standard Deviation/Range)
Male Female Age (yrs) Proptosis Pain/discomfort Diplopia Globe displacement Nausea/vomiting Visual acuity IOP (mmHg) Relative proptosis (mm)* Ocular motility limitation IR hemorrhage Inferior orbit hemorrhage† Complete resolution Residual mild thickening of IR
4 (67) 2 (33) 68 (⫾14; 39–77) 6 (100) 5 (83) 5 (83) 3 (50) 2 (33) 20/20 17 (⫾1.7; 15–18) 2.8 (⫾2.1; 1–5) 4 (67) 4 (67) 2 (33) 4 (67) 2 (33) 7.5 (⫾4.1; 1.2–12) 18.0 (1–84)
Time to resolution (wks) Follow-up (mos)
CT ⫽ computerized tomography; IOP ⫽ intraocular pressure; IR ⫽ inferior rectus; MRI ⫽ magnetic resonance imaging. *Relative proptosis in comparison with the other unaffected orbit. † Adjacent to or within the inferior rectus muscle sheath.
eye examination, and imaging studies (CT, MRI, or both) were collected and analyzed. All patients were diagnosed with spontaneous orbital hemorrhage within a rectus muscle or its sheath according to clinical symptoms, imaging findings (homogenous mass within a rectus muscle or its sheath, well circumscribed, hyperintense on MRI and hyperdense on CT, mild contrast enhancement, homogenous enhancement, fluid level possibly present), and spontaneous clinical and radiographic resolution. Blood tests and coagulation profiles were reported in only 2 patients. No surgical intervention was required and no underlying orbital or systemic disease that may have caused orbital hemorrhage was found.
Results Six patients (4 men, 2 women; mean age, 68 years; range, 39 –77 years) with acute onset of proptosis and diplopia with or without orbital discomfort were diagnosed with spontaneous orbital hemorrhage within the inferior rectus muscle or its sheath. The demographics of the study population are summarized in Table 1. No patient gave a history of trauma, straining, heavy lifting, or bleeding tendencies.
Case Reports Patient 1. A 69-year-old woman with a past medical history of controlled hypertension and hypercholesterolemia who was taking low-dose aspirin was referred with acute right proptosis, blurred vision, vertical diplopia, nausea, and vomiting. Clinical examination disclosed right proptosis of 1 mm relative to the left globe, normal visual acuity of 20/20, and normal intraocular pressure of 18 mmHg. Ocular motility was restricted in upgaze and downgaze (20° and 10°, respectively) but normal in adduction and abduction. Anterior segments and fundus examination results were normal. Computed visual field examination results were normal. Magnetic resonance imaging of the orbit revealed a well-defined mass lesion in the floor of the right orbit mea-
suring 2.5 ⫻ 2 cm, displacing the inferior rectus muscle medially and the optic nerve superiorly. Signal characteristics were homogenous hyperintensity compatible with a diagnosis of recent hemorrhage. Computed tomography of the orbit showed similar findings with a teardrop configuration of the lesion and an elongated tail toward the orbital apex. The lesion was hyperdense on CT scan, and no contrast enhancement was observed. Symptoms resolved spontaneously over the course of 6 weeks. Repeated CT of the orbit showed mild thickening of the posterior half of the right inferior rectus muscle. The patient was diagnosed with spontaneous inferior orbital hemorrhage related to the right inferior rectus muscle. Patient 2. A 77-year-old man had sudden onset of diplopia associated with redness and swelling of the left lower eyelid; diplopia improved over the course of days after the episode. Past medical and ophthalmic history was unremarkable, with no history of easy bruising, bleeding abnormality, or oral medications. Clinical examination disclosed left visual acuity of 20/30, left proptosis with lateral displacement of the globe, and mild limitation in upgaze to the left. Eyelid swelling resolved within several days. Imaging studies of the orbit, including CT and MRI, showed a well-demarcated inferomedial orbital floor lesion tapering to the orbital apex. The inferior rectus muscle belly was displaced laterally and appeared to be within the wall of the lesion, suggesting intrasheath hemorrhage. Magnetic resonance imaging showed low signal on T1 and T2 imaging with minimal enhancement of the rim of the lesion. The patient was diagnosed with spontaneous orbital hemorrhage related to the left inferior rectus muscle. Clinical signs and symptoms resolved gradually over the course of 9 days, and repeated MRI studies showed complete resolution of the orbital hemorrhage. Patient 3. A 39-year-old man was referred with right orbital pain and acute onset of proptosis accompanied by vomiting. Clinical examination disclosed right proptosis of 1 mm relative to the left globe, normal visual acuity of 20/20, intraocular pressure of 18 mmHg, and no ocular motility restriction. Computed tomography of the orbit revealed a mass in the right inferior orbit that smoothly enlarged the belly of the right inferior rectus in the posterior two thirds (Fig 1). Magnetic resonance imaging of the orbit disclosed
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Figure 1. Sagittal postcontrast computed tomography scan of a 39-year-old man with acute painful right proptosis and vomiting at presentation. A homogenous mass is shown in the inferior orbit with smooth enlargement of the posterior two thirds of the inferior rectus muscle.
an enlargement of the right inferior rectus muscle with an area of high signal, characteristic of recent hemorrhage contained within the muscle (Fig 2). No contrast enhancement was noted on the postgadolinium studies. Diagnosis was consistent with spontaneous intramuscular hematoma. Repeated imaging studies 2 months later showed complete resolution of the intramuscular hematoma. Three additional patients (2 men, 1 woman; mean age, 73
years) had acute onset of painful proptosis and double vision. In all patients, CT or MRI studies, or both, confirmed the diagnosis of orbital hemorrhage confined to the inferior rectus muscle or its sheath and no contrast enhancement was noted on imaging studies. A complete blood count and determination of the international normalized ratio were performed in 2 patients and revealed normal results. Symptoms resolved in all but 1 patient, who had persistent
Figure 2. Coronal magnetic resonance imaging scans of a 39-year-old man with acute painful right proptosis and vomiting. A, T1-weighted image showing a hyperintense homogenous mass (asterisk) within the inferior rectus muscle (white arrow). B, T1-weighted image after gadolinium fat saturation showing homogenous contrast enhancement of the lesion (asterisk), characteristic of a recent clot with methemoglobin.
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Figure 3. A, A 69-year-old man with sudden onset of left orbital pain, double vision, and proptosis. Clinical examination revealed normal visual acuity of 20/20, mild limitation in upgaze on the left, and left relative proptosis of 5 mm with conjunctival chemosis. B, Coronal computed tomography (CT) scan of the orbit showing left inferior rectus muscle enlargement consistent with intramuscular hemorrhage (black arrow). Note that the inferior rectus muscle cannot be distinguished (asterisk denotes the back of the right globe). C, Axial CT scan of the orbits revealing the anteroposterior margins of the hemorrhage and a fluid level within the clot (black arrow).
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Ophthalmology Volume 112, Number 10, October 2005 diplopia on extreme upgaze only. Figure 3 shows that patient in the acute phase of the hemorrhage.
Discussion We describe 6 patients with spontaneous orbital hemorrhage confined to the inferior rectus muscle or its sheath. All had acute painful proptosis and diplopia at presentation. Computed tomography, MRI, or both demonstrated a well-defined hematoma, sometimes with a fluid level, within the belly of the inferior rectus muscle or its sheath and displacing the muscle belly to one side. In all patients, visual acuity was preserved. None of the patients had a history of bleeding disorder. No patient had a history of trauma or sudden rise in venous pressure that can occur with straining or heavy lifting. Orbital hemorrhage, proptosis, and motility restriction resolved spontaneously, with only 2 patients showing residual asymptomatic mild rectus muscle enlargement, presumably secondary to fibrosis. In 1994, we reported 3 patients with spontaneous orbital hemorrhage within a rectus muscle.2 All 3 patients had sudden onset of unilateral painful proptosis, diplopia, and an enlarged rectus muscle as shown by CT or MRI; hemorrhage resolved spontaneously in all patients. Similarly, no history of bleeding disorder was found, and visual acuity was preserved in 2 patients; 1 patient had counting fingers acuity secondary to a vascular event 50 years before presentation. That report differed from the current series in that the muscles involved were the lateral, medial, and inferior rectus. Residual thickening was noted in just 1 patient. In both reports, no surgical intervention was needed. It is important to distinguish our patients from previous reports of spontaneous orbital hemorrhage into the subperiosteal space,10 –13 which were associated with a sudden rise in cranial venous pressure or systemic coagulopathies. The cause of the hemorrhage in these patients is obscure; most of the patients were older adults without significant medical problems. We cannot postulate any specific anomaly that may be associated with this rare clinical presentation, but the clinical and imaging findings are sufficient characteristics to allow a confident diagnosis and conservative treatment. Our case series differs from other cases of spontaneous orbital hemorrhage in that our patients did not have any orbital or systemic disease and did not undergo surgery or general anesthesia. Previous reports described orbital hemorrhage associated with surgery or occurring as a complication of general anesthesia.10,11,13–15 One report describes spontaneous bilateral orbital hemorrhage in a newborn that subsided with no further sequelae.16 The vasoocclusive process in sickle cell anemia has been implicated with orbital subperiosteal hematomas. Other rare causes include orbital reconstruction with implant8,17 and systemic diseases such as septicemia,18 leukemia,19 bleeding diathesis or systemic coagulopathies12,20 –23; it has even been reported after vigorous paroxysmal coughing and other causes of a sudden rise in cranial venous pressure.24 In the largest series of orbital hemorrhage reported in the literature, Sullivan and Wright3 described 115 cases of
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nontraumatic orbital hemorrhage. An associated orbital vascular malformation was present in 90% of their patients, whereas in only 6 patients (5%) no underlying cause was observed. Most patients showed spontaneous complete or partial resolution, with only 8 (7%) requiring surgery for optic nerve compression. Final visual acuity was reduced in 23 patients (20%). No patient in this series was described as having a hemorrhage within an extraocular muscle. Orbital imaging, either by CT or MRI scan, is helpful in diagnosing and localizing a hemorrhage and is important to rule out any coexisting orbital pathologic features. An MRI scan may help to determine the age of the hemorrhage.2 A fresh clot containing deoxyhemoglobin appears hypointense on T1 and T2 imaging; as it changes to methemoglobin, the signal becomes hyperintense from the periphery to the center of the hemorrhage, eventually producing a homogenous high signal.2 The intensity lessens as the methemoglobin breaks down to hemosiderin and ferritin. In our patients, the hemorrhage was always well defined with no spread into adjacent orbital fat, suggesting confinement of the hemorrhage by the muscle sheath. In most instances, the hemorrhage is larger anteriorly with an almost perpendicular anterior face and tapers posteriorly. In some patients, a fluid level can be identified within the hematoma (Fig 3). The differential diagnosis of an enlarged extraocular muscle includes inflammatory and neoplastic processes, but these are usually associated with a more gradual onset and additional orbital or systemic features.25,26 The main groups of diseases include idiopathic orbital inflammation and myositis, thyroid-related orbitopathy, and orbital lymphoma. The sudden onset, spontaneous resolution, and CT and MRI findings help to distinguish acute hemorrhage from orbital myositis. Limitations of the current study stem from its retrospective design. No formal testing to exclude bleeding diathesis was performed except in 2 patients. Visual field testing was performed in only 1 patient; therefore, optic nerve function could be estimated based solely on normal visual acuity. We describe 6 patients with spontaneous orbital hemorrhage to the inferior rectus muscle or its sheath with acute onset proptosis, pain, and diplopia at presentation. Symptoms subsided within days to weeks from the initial presentation. Repeated CT or MRI scans, or both, showed complete resolution of the hemorrhage in 4 patients, and in 2 patients, mild asymptomatic thickening of the muscle was noted. This case series highlights another well-defined entity of spontaneous hemorrhage within the inferior rectus muscle or its sheath.
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