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Orbital Hemorrhage and Eyelid Ecchymosis in Acute Orbital Myositis
Orbital Hemorrhage and Eyelid Ecchymosis in Acute Orbital Myositis David M. Reifler, M.D., Douglas Leder, D.O., and Todd Rexford, B.S.
We examined two patients with acute orbital myositis associated with orbital hemorrhage and eyelid ecchymosis. Both patients were young women (aged 22 and 30 years) who had painful proptosis, diplopia, and computed tomographic evidence of single extraocular muscle involvement with spillover of inflammatory edema into the adjacent orbital fat. Patient 1 showed contralateral preseptal eyelid inflammation and did not suffer an orbital hemorrhage until after an episode of vomiting. In Patient 2, the diagnosis of occult orbital varix was initially considered but an orbital exploration and a biopsy specimen showed no vascular anomaly. Both patients were treated successfully with high-dose systemic corticosteroids. Some cases of idiopathic orbital inflammation may be related to preexisting vascular anomalies or orbital phlebitis. ORBIT AL MYOSITIS is a subtype of the orbital pseudotumor (nonspecific orbital inflammatory) syndrome in which one or more of the extraocular muscles are primarily infiltrated by an inflammatory process.P Initial features of acute orbital myositis may include pain, diplopia, proptosis, blepharoptosis, conjunctival chemosis, and injection over the involved extraocular muscles. We studied two cases of acute orbital myositis associated with orbital hemorrhage and eyelid ecchymosis.
Accepted for publication Nov. 29, 1988. From the Departments of Ophthalmology, Michigan State University College of Human Medicine (Grand Rapids Area Medical Education Center), Blodgett Memorial Medical Center, and Butterworth Hospital (Dr. Reifler and Mr. Rexford) and Metropolitan Hospital (Dr. Leder), Grand Rapids, Michigan. Reprint requests to David M. Reifler, M.D., 1000 E. Paris Ave. S.E., Suite 221, Grand Rapids, MI 49506.
©AMERICAN JOURNAL OF OPHTHALMOLOGY
107:111-113,
Case Reports Case 1 A 22-year-old woman was referred for management of "right orbital cellulitis." She had a one-week history of a left upper eyelid "stye," but had developed contralateral painful orbital swelling and diplopia. The patient was afebrile and the white blood cell count was 10,100/mm3 with a normal differential. The patient was hospitalized and started on intravenous antibiotics. At the time of initial consultation there was 3 mm of proptosis, limited abduction, and chemosis of the right eye, as well as vasocongestion overlying the insertion of the right lateral rectus muscle. On palpation, the edematous left upper eyelid had a firm, rubbery consistency. A clinical diagnosis of orbital pseudotumor was favored. Computed tomographic scans showed enlargement of the right lateral rectus muscle and an infiltrating mass in the left upper eyelid (Fig. 1). That evening, after a brief episode of emesis, the patient experienced a right orbital hemorrhage with bulbar and upper eyelid involvement (Fig. 2). A regimen of systemic high-dose prednisone was begun, and the pathologic orbital findings resolved as the corticosteroids were tapered and discontinued. Case 2 A 30-year-old woman developed acute painful left proptosis of 5.5 mm, with lateral displacement of the left eye. The ductions of the left eye were limited and the patient had diplopia in all fields of gaze. Subcutaneous ecchymosis was present in the medial aspect of the left upper eyelid and more superficial, darker ecchymosis was present in the medial left lower eyelid (Fig. 3). The patient was afebrile and the white blood cell count was 12,OOO/mm3 with a
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Fig. 3 (Reifler, Leder, and Rexford). Case 2. Acute orbital myositis in a 3D-year-old woman with proptosis and ecchymoses of the left lower and upper eyelids.
Fig. 1 (Reifler, Leder, and Rexford). Case 1. Computed tomographic scan showing right lateral rectus muscle enlargement and left upper eyelid infiltration.
normal differential. The patient was hospitalized and begun on intravenous ceftriaxone. Computed tomography showed infiltration in the left medial orbit involving the insertion of the medial rectus muscle. A round, radiodense lesion was identified within the area of the inflammation, suggesting possible phlebolith formation (Fig. 4). The patient showed mild progression of the proptosis and a left orbital exploration and biopsy was performed the day after admission. No venous abnormalities or phleboliths were identified at the time of surgery. A biopsy specimen of the orbital fat showed scattered inflammatory cells consistent with orbital pseudotumor. A regimen of oral prednisone was begun, and the inflammation
Fig. 2 (Reifler, Leder, and Rexford). Case 1. Acute orbital myositis with spontaneous right orbital hemorrhage extending anteriorly into the bulbar conjunctiva and right upper eyelid. Left upper eyelid erythema and a subcutaneous mass are also present.
and proptosis resolved completely without residual motility disturbance. The prednisone was tapered and subsequently discontinued over six weeks.
Discussion Trauma is the most common cause of orbital hemorrhage and eyelid ecchymosis." Other causes of spontaneous orbital hemorrhage and
Fig. 4 (Reifler. Leder, and Rexford). Case 2. Computed tomographic scan showing proptosis and spillover of left medial rectus muscle inflammation into the surrounding orbital fat. Focal radiodense area adjacent to infiltrated left medial rectus tendon correlated with pseudoencapsulated orbital hemorrhage found at surgical exploration, but a phlebolith was not found.
Vol. 107, No.2
Acute Orbital Myositis
eyelid ecchymosis include orbital vascular anomaly, lymphangioma, hypertension, various bleeding disorders, metastatic neuroblastoma, and Valsalva maneuver. 4-6 In both of our cases, acute proptosis preceded the hemorrhage, although emesis also occurred in Case 1. Eyelid ecchymosis was noted by Krohel and Wright' in eight of 17 cases of spontaneous orbital hemorrhage. Eleven of their 17 patients had an underlying vascular malformation. In posterior orbital hemorrhage, eyelid ecchymosis may not appear until the proptosis gradually resolves and blood tracks forward into the anterior orbit. 7 In our patients, eyelid ecchymoses occurred concurrently with proptosis in one case and within 24 hours of documented proptosis in the other case. Perhaps the anterior involvement of the extraocular muscle tendons and the overlying conjunctiva allowed hemorrhage to appear in the eyelids relatively early after the development of acute proptosis. The cause of orbital hemorrhage in our patients may have been a combination of inflammatory changes in the orbital circulation and mechanical stresses produced by sudden proptosis. Using orbital venography, Kennerdell" noted that orbital pseudotumor is occasionally associated with venous abnormalities that are characteristic of inflammation. While orbital vascular anomalies have been demonstrated by orbital venography, 4,7 this technique may fail to demonstrate small or even moderate sized lesions." and its use has been supplanted by computed orbital tomography. Idiopathic orbital inflammation and orbital vascular anomaly may share certain clinical features including sudden proptosis, edema, and hemorrhage. In some cases, occult orbital vascular anomalies may be associated with phlebitis, resulting in alterations of vascular permeability and secondary edema. In other cases an orbital varix may be readily identified by computed tomography or magnetic reso-
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nance imaging. We propose that some cases diagnosed as "idiopathic orbital inflammation" may be related to preexisting vascular anomalies or orbital phlebitis. Spontaneous orbital hemorrhage may be seen in a variety of primary orbital and systemic diseases. The presence of eyelid ecchymosis may provide evidence for the presence of deeper orbital hemorrhage and aid in the differential diagnosis. We believe that acute orbital myositis should be included in the differential diagnosis of conditions that may be associated with spontaneous orbital hemorrhage and eyelid ecchymosis. References 1. Weinstein, G. S., Dresner, S. c.. Slamovits, T. L., and Kennerdell, J. S.: Acute and subacute orbital myositis. Am. J. Ophthalmol. 96:209, 1983. 2, Kennerdell, J. S., and Dresner, S. c.. The nonspecific orbital inflammatory syndromes. Surv. Ophthalmol. 29:93, 1984, 3. Reifler, D, M., and Hornblass, A.: The orbit, An introduction to orbital anatomy, patient examination, and orbital diseases, In Rhode, S. J" and Ginsberg, S. P, (eds.): Ophthalmic Technology, A Guide for the Eye Care Assistant. New York, Raven Press, 1987, p. 81. 4, Krohel, G. B., and Wright, J. E.: Orbital hemorrhage. Am. J. Ophthalmol. 88:254, 1979. 5. Dryden, R. M" Wule, A, E" and Day, D.: Eyelid ecchymosis and proptosis in lymphangioma, Am, J. Ophthalmol. 100:486,1985. 6, Albert, D. M., Rubenstein, R. A., and Scheie, H. G.: Tumor metastasis to the eye. Part II. Clinical study in infants and children. Am. J, Ophthalmol. 63:727, 1967, 7. Wright, J. E.: Orbital vascular anomalies. Trans, Am, Acad. Ophthalmol. Otolaryngol. 78:606, 1974. 8. Kennerdell, J. S,: Orbital diagnosis, In Smith, B, c., Della Rocca, R. C.; Nesi, F. A., and Lisman, R, D. (eds.): Ophthalmic Plastic and Reconstructive Surgery. St. Louis, C. V. Mosby Co" 1987, vol. 2, p. 1008,
We examined two patients with acute orbital myositis associated with orbital hemorrhage and eyelid ecchymosis. Both patients were young women (aged 22 and 30 years) who had painful proptosis, diplopia, and computed tomographic evidence of single extraocular muscle involvement with spillover of inflammatory edema into the adjacent orbital fat. Patient 1 showed contralateral preseptal eyelid inflammation and did not suffer an orbital hemorrhage until after an episode of vomiting. In Patient 2, the diagnosis of occult orbital varix was initially considered but an orbital exploration and a biopsy specimen showed no vascular anomaly. Both patients were treated successfully with high-dose systemic corticosteroids. Some cases of idiopathic orbital inflammation may be related to preexisting vascular anomalies or orbital phlebitis. ORBIT AL MYOSITIS is a subtype of the orbital pseudotumor (nonspecific orbital inflammatory) syndrome in which one or more of the extraocular muscles are primarily infiltrated by an inflammatory process.P Initial features of acute orbital myositis may include pain, diplopia, proptosis, blepharoptosis, conjunctival chemosis, and injection over the involved extraocular muscles. We studied two cases of acute orbital myositis associated with orbital hemorrhage and eyelid ecchymosis.
Accepted for publication Nov. 29, 1988. From the Departments of Ophthalmology, Michigan State University College of Human Medicine (Grand Rapids Area Medical Education Center), Blodgett Memorial Medical Center, and Butterworth Hospital (Dr. Reifler and Mr. Rexford) and Metropolitan Hospital (Dr. Leder), Grand Rapids, Michigan. Reprint requests to David M. Reifler, M.D., 1000 E. Paris Ave. S.E., Suite 221, Grand Rapids, MI 49506.
©AMERICAN JOURNAL OF OPHTHALMOLOGY
107:111-113,
Case Reports Case 1 A 22-year-old woman was referred for management of "right orbital cellulitis." She had a one-week history of a left upper eyelid "stye," but had developed contralateral painful orbital swelling and diplopia. The patient was afebrile and the white blood cell count was 10,100/mm3 with a normal differential. The patient was hospitalized and started on intravenous antibiotics. At the time of initial consultation there was 3 mm of proptosis, limited abduction, and chemosis of the right eye, as well as vasocongestion overlying the insertion of the right lateral rectus muscle. On palpation, the edematous left upper eyelid had a firm, rubbery consistency. A clinical diagnosis of orbital pseudotumor was favored. Computed tomographic scans showed enlargement of the right lateral rectus muscle and an infiltrating mass in the left upper eyelid (Fig. 1). That evening, after a brief episode of emesis, the patient experienced a right orbital hemorrhage with bulbar and upper eyelid involvement (Fig. 2). A regimen of systemic high-dose prednisone was begun, and the pathologic orbital findings resolved as the corticosteroids were tapered and discontinued. Case 2 A 30-year-old woman developed acute painful left proptosis of 5.5 mm, with lateral displacement of the left eye. The ductions of the left eye were limited and the patient had diplopia in all fields of gaze. Subcutaneous ecchymosis was present in the medial aspect of the left upper eyelid and more superficial, darker ecchymosis was present in the medial left lower eyelid (Fig. 3). The patient was afebrile and the white blood cell count was 12,OOO/mm3 with a
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1989
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Fig. 3 (Reifler, Leder, and Rexford). Case 2. Acute orbital myositis in a 3D-year-old woman with proptosis and ecchymoses of the left lower and upper eyelids.
Fig. 1 (Reifler, Leder, and Rexford). Case 1. Computed tomographic scan showing right lateral rectus muscle enlargement and left upper eyelid infiltration.
normal differential. The patient was hospitalized and begun on intravenous ceftriaxone. Computed tomography showed infiltration in the left medial orbit involving the insertion of the medial rectus muscle. A round, radiodense lesion was identified within the area of the inflammation, suggesting possible phlebolith formation (Fig. 4). The patient showed mild progression of the proptosis and a left orbital exploration and biopsy was performed the day after admission. No venous abnormalities or phleboliths were identified at the time of surgery. A biopsy specimen of the orbital fat showed scattered inflammatory cells consistent with orbital pseudotumor. A regimen of oral prednisone was begun, and the inflammation
Fig. 2 (Reifler, Leder, and Rexford). Case 1. Acute orbital myositis with spontaneous right orbital hemorrhage extending anteriorly into the bulbar conjunctiva and right upper eyelid. Left upper eyelid erythema and a subcutaneous mass are also present.
and proptosis resolved completely without residual motility disturbance. The prednisone was tapered and subsequently discontinued over six weeks.
Discussion Trauma is the most common cause of orbital hemorrhage and eyelid ecchymosis." Other causes of spontaneous orbital hemorrhage and
Fig. 4 (Reifler. Leder, and Rexford). Case 2. Computed tomographic scan showing proptosis and spillover of left medial rectus muscle inflammation into the surrounding orbital fat. Focal radiodense area adjacent to infiltrated left medial rectus tendon correlated with pseudoencapsulated orbital hemorrhage found at surgical exploration, but a phlebolith was not found.
Vol. 107, No.2
Acute Orbital Myositis
eyelid ecchymosis include orbital vascular anomaly, lymphangioma, hypertension, various bleeding disorders, metastatic neuroblastoma, and Valsalva maneuver. 4-6 In both of our cases, acute proptosis preceded the hemorrhage, although emesis also occurred in Case 1. Eyelid ecchymosis was noted by Krohel and Wright' in eight of 17 cases of spontaneous orbital hemorrhage. Eleven of their 17 patients had an underlying vascular malformation. In posterior orbital hemorrhage, eyelid ecchymosis may not appear until the proptosis gradually resolves and blood tracks forward into the anterior orbit. 7 In our patients, eyelid ecchymoses occurred concurrently with proptosis in one case and within 24 hours of documented proptosis in the other case. Perhaps the anterior involvement of the extraocular muscle tendons and the overlying conjunctiva allowed hemorrhage to appear in the eyelids relatively early after the development of acute proptosis. The cause of orbital hemorrhage in our patients may have been a combination of inflammatory changes in the orbital circulation and mechanical stresses produced by sudden proptosis. Using orbital venography, Kennerdell" noted that orbital pseudotumor is occasionally associated with venous abnormalities that are characteristic of inflammation. While orbital vascular anomalies have been demonstrated by orbital venography, 4,7 this technique may fail to demonstrate small or even moderate sized lesions." and its use has been supplanted by computed orbital tomography. Idiopathic orbital inflammation and orbital vascular anomaly may share certain clinical features including sudden proptosis, edema, and hemorrhage. In some cases, occult orbital vascular anomalies may be associated with phlebitis, resulting in alterations of vascular permeability and secondary edema. In other cases an orbital varix may be readily identified by computed tomography or magnetic reso-
113
nance imaging. We propose that some cases diagnosed as "idiopathic orbital inflammation" may be related to preexisting vascular anomalies or orbital phlebitis. Spontaneous orbital hemorrhage may be seen in a variety of primary orbital and systemic diseases. The presence of eyelid ecchymosis may provide evidence for the presence of deeper orbital hemorrhage and aid in the differential diagnosis. We believe that acute orbital myositis should be included in the differential diagnosis of conditions that may be associated with spontaneous orbital hemorrhage and eyelid ecchymosis. References 1. Weinstein, G. S., Dresner, S. c.. Slamovits, T. L., and Kennerdell, J. S.: Acute and subacute orbital myositis. Am. J. Ophthalmol. 96:209, 1983. 2, Kennerdell, J. S., and Dresner, S. c.. The nonspecific orbital inflammatory syndromes. Surv. Ophthalmol. 29:93, 1984, 3. Reifler, D, M., and Hornblass, A.: The orbit, An introduction to orbital anatomy, patient examination, and orbital diseases, In Rhode, S. J" and Ginsberg, S. P, (eds.): Ophthalmic Technology, A Guide for the Eye Care Assistant. New York, Raven Press, 1987, p. 81. 4, Krohel, G. B., and Wright, J. E.: Orbital hemorrhage. Am. J. Ophthalmol. 88:254, 1979. 5. Dryden, R. M" Wule, A, E" and Day, D.: Eyelid ecchymosis and proptosis in lymphangioma, Am, J. Ophthalmol. 100:486,1985. 6, Albert, D. M., Rubenstein, R. A., and Scheie, H. G.: Tumor metastasis to the eye. Part II. Clinical study in infants and children. Am. J, Ophthalmol. 63:727, 1967, 7. Wright, J. E.: Orbital vascular anomalies. Trans, Am, Acad. Ophthalmol. Otolaryngol. 78:606, 1974. 8. Kennerdell, J. S,: Orbital diagnosis, In Smith, B, c., Della Rocca, R. C.; Nesi, F. A., and Lisman, R, D. (eds.): Ophthalmic Plastic and Reconstructive Surgery. St. Louis, C. V. Mosby Co" 1987, vol. 2, p. 1008,