Alternating Proptosis A Case
Report of Acute Orbital Myositis Defined by the Computerized Tomographic Scan
James R. Keane, MD
27-year-old woman had a two-week of acute painful right proptosis with ptosis, medial conjunctival injection, and restriction of eye movements\p=m-\particularly abduction. One month later, a similar remitting painful left proptosis with complete limitation of abduction developed. Computerized tomographic x-ray \s=b\ A
course
showed marked contrast enhancement of both medial rectus muscles, documenting the presumptive diagnosis of acute orbital myositis without recourse to invasive diagnostic techniques. Myositis is a common component of idiopathic orbital inflammatory disease (orbital pseudotumor), but awareness of the rare patient with acute inflammation clinically localized to the extraocular muscles will decrease confusion with cranial nerve and cavernous sinus dis-
scanning
ease.
(Arch Neurol 34:642-643, 1977)
have been eliminated and betterdefined inflammatory diseases such as
sarcoidosis, Wegener's granuloma,
and polyarteritis nodosa considered, a number of cases of acute self-limited inflammation remain. These have been variously described as acute orbital myositis, tenonitis, sclerotenonitis, posterior scleritis, acute orbital pseudotumor, and acute thyroid ophthalmopathy when the orbit is in¬ volved; and as the superior orbital fissure syndrome, Collier's syndrome, the Tolosa-Hunt syndrome, and ophthalmoplegic migraine when retroorbital. No precise histologie or geographic division exists among the various idiopathic orbital inflammations, and the present tendency is to lump all varia¬ tions under the unfortunate term "or¬ bital pseudotumor."1 However, there are sufficient cases3·4 identical to the following patient, in which the extraocular muscles are the major site of transient inflammation, for the diag¬ nosis of acute orbital myositis (AOM) to be of practical clinical value, pending a more rational basis for clas¬ sification. -
conditions the orbit and cavernous a sinus diagnostic chal¬ lenge. In the absence of clues to pathogenesis, clinical descriptions are based sites of on visible or presumed involvement. After inflammatory tu¬ mor reactions and infectious causes
inflammatory Remitting involving present major
Accepted for publication May 5, 1977. From the Department of Neurology, Los Angeles County-University of Southern California Medical Center, Los Angeles. Reprint requests to Box 359, 1200 N State St, Los Angeles, CA 90033 (Dr Keane).
REPORT OF A CASE A 27-year-old previously healthy woman awoke with pain, redness, and mild swelling about the right eye. Her symp-
Downloaded From: http://archneur.jamanetwork.com/ by a University of Michigan User on 06/13/2015
progressed slightly over the next two days despite antibiotic therapy for pre¬ toms
sumed orbital cellulitis. Admission examination showed rightsided eye findings of 5 mm proptosis, mild upper-lid and conjunctiva] edema, and dilated conjunctival vessels, particularly at the medial canthus. Movements of the
right eye, especially abduction, were pain¬ fully limited. The rest of the results of the ocular examination were normal, as was her general physical examination. The ESR was consistently elevated at 50 mm/hr; otherwise, results of her blood studies were normal. Radiographs showed a right maxil¬ lary sinus soft tissue density; antral punc¬ ture of the maxillary sinus showed serous fluid without evidence of infection; and subsequent sinus radiographs were normal. Her eye involvement continued to progress for several days after admission and then slowly and completely resolved during the next two weeks. One month after the onset of her initial symptoms, she noted identical acute pain, redness, and swelling about her other eye. On readmission, the right eye appeared
Fig
1.—Left proptosis, mild ptosis, and limitation of abduction are evident attempted left gaze.
severe
on
of Bertelson's4 cases of sclerotenonitis had signs restricted to exophthalmos and limitation of eye movement, with one
patient experiencing yearly epi¬
sodes in one eye or the other for six years. Similarly, six episodes of
Fig 2.—A, Computerized tomographic scan before contrast injection, showing promi¬ nence of medial rectus muscles. B, Computerized tomographic scan in same plane after contrast injection showing marked asymmetric enhancement of medial recti. normal, and she had left-sided eye findings of 3 mm of proptosis, mild generalized conjunctival injection, particularly over the medial rectus muscle insertion, and mild upper-lid edema with slight ptosis. Mild
limitation of abduction with accompanying pain at the medial canthus progressed during the next several days to complete paralysis of abduction (Fig 1). The ESR was again elevated (45 mm/hr), but results of the VDRL, antinuclear anti¬ body titers, a latex fixation test, and thyroid function studies were normal. Ultrasound studies showed a mass at the medial aspect of each orbit, larger on the left side. Computerized tomographic (CT) x-ray scanning demonstrated asymmetric prominent medial rectus muscles, with dramatic enhancement along the entire muscle length after contrast injection
(Fig 2)
Her orbital signs and symptoms com¬ pletely resolved over a two-week period—a course identical to the initial right orbital
involvement. She has had no further eye problems in the six months since leaving the hospital.
course
and
findings
litis, inflammatory tumor reaction, acute orbital pseudotumor syndromes, and perhaps acute thyroid ophthalmopathy. Remitting cavernous sinus
syndromes share similar features of pain, a self-limited course with rare residual signs, frequent elevation of the ESR, and probable steroid respon¬ siveness, but are distinguished by a pattern of discrete cranial
COMMENT
The
able but usually severe in relation to the mild proptosis. Progression ceases within one week, and the eye returns to normal during the next two to eight weeks. The patient is afebrile and has a normal WBC count, syphilis serology, thyroid function studies, and screening tests for vasculitis. Fre¬ quent malaise and elevation of the ESR indicate systemic disease but rarely can any definite connective tissue disease be diagnosed.5 Steroid therapy frequently appears to hasten resolution. The differential diagnosis of the initial episode includes bacterial cellu-
involvement and absence of in the
present case are very similar to that of
small group of patients with orbital inflammation best characterized as acute orbital -myositis.34 An abrupt a
onset of periorbital pain increasing on eye motion is rapidly followed by mild proptosis, with conjunctival and upper lid edema and the appearance of
dilated vessels overlying the hori¬ zontal muscle insertions. The limita¬ tion of eye movements is highly vari-
cant
proptosis.
nerve
signifi¬
"Chronic orbital myositis" is an even less well-defined condition, with minimal inflammation and a pro¬ tracted course, but reliable differen¬ tiation from thyroid ophthalmopathy is probably impossible.1L'e Cases reported as acute sclerotenonitis, tenonitis, and posterior scleritis include patients identical to present one, as well as many with prominent posterior globe inflammation.4 78 Six
Downloaded From: http://archneur.jamanetwork.com/ by a University of Michigan User on 06/13/2015
alternating painful ophthalmoplegia and proptosis occurred during a sevenyear period in a patient considered to have ophthalmoplegic migraine.9 Although the evaluation of prop¬ tosis has been revolutionized through the use of ultrasonic and CT scanning devices,10 the precise diagnosis of orbital inflammatory conditions re¬ mains a challenge.11 The CT scan in the present case dramatically con¬ firmed the clinical impression of
medial rectus inflammation, demon¬ strated continuing abnormality in the clinically recovered opposite medial rectus muscle, and obviated further evaluation of what could have been mistaken for a sixth-nerve paresis.
Stewart Winthrop, MD, and George A. Curry, MD, assisted with the clinical evaluation. John Ratcliffe, MD, provided the German transla¬ tion.
References 1. Jakobiec FA, Jones IS: Orbital inflammain Duane TD (ed): Clinical Ophthalmology New York, Harper & Row Publishers Inc, 1976, vol 2, pp 1-75. 2. Blodi FC, Gass JDM: Inflammatory pseudotumor of the orbit. Trans Am Acad Ophthalmol Otolaryngol 71:303-323, 1967. 3. Esslen E, Papst W: Die Beduetung der Elektromyographic f\l=u"\rdie Analyse von Motilitatsstorungen der Augen. Basel, Switzerland, S Karger, 1961, pp 62-70. 4. Bertelson TI: Acute sclerotenonitis and ocular myositis complicated by papillitis, retinal detachment and glaucoma. Acta Ophthalmol 38:136-152, 1960. 5. Brenner EH, Shock JP: Proptosis secondary to systemic lupus erythematosus. Arch Ophthalmol 91:81-82, 1974. 6. Wolter JR, Hoy HE, Schmidt DM: Chronic orbital myositis. Am J Ophthalmol 62:292-298, 1966. 7. Duke-Elder S, MacFaul PA: The Ocular Adnexa. System of Ophthalmology, St Louis, CV Mosby Co, 1974, vol 13, part 2, pp 878-880. 8. Leira H: Sclerotenonitis and orbital myositis. Mod Probl Ophthalmol 14:669-674, 1975. 9. Hedges TR: Alternating exophthalmos with painful ophthalmoplegia. Arch Ophthalmol 74:625-627, 1965. 10. Dallow RL, Momose KJ, Weber Al, et al: Comparison of ultrasonography, computerized
tion,
tomography (EMI scan), and radiographic techniques in evaluation of exophthalmos. Trans Am Acad Ophthalmol Otolaryngol 81:305-322,
1976. 11. Enzmann D, Donaldson SS, Marshall WH, et al: Computed tomography in orbital pseudotumor (idiopathic orbital inflammation). Radiology 120:597-601, 1976.
Report of Acute Orbital Myositis Defined by the Computerized Tomographic Scan
James R. Keane, MD
27-year-old woman had a two-week of acute painful right proptosis with ptosis, medial conjunctival injection, and restriction of eye movements\p=m-\particularly abduction. One month later, a similar remitting painful left proptosis with complete limitation of abduction developed. Computerized tomographic x-ray \s=b\ A
course
showed marked contrast enhancement of both medial rectus muscles, documenting the presumptive diagnosis of acute orbital myositis without recourse to invasive diagnostic techniques. Myositis is a common component of idiopathic orbital inflammatory disease (orbital pseudotumor), but awareness of the rare patient with acute inflammation clinically localized to the extraocular muscles will decrease confusion with cranial nerve and cavernous sinus dis-
scanning
ease.
(Arch Neurol 34:642-643, 1977)
have been eliminated and betterdefined inflammatory diseases such as
sarcoidosis, Wegener's granuloma,
and polyarteritis nodosa considered, a number of cases of acute self-limited inflammation remain. These have been variously described as acute orbital myositis, tenonitis, sclerotenonitis, posterior scleritis, acute orbital pseudotumor, and acute thyroid ophthalmopathy when the orbit is in¬ volved; and as the superior orbital fissure syndrome, Collier's syndrome, the Tolosa-Hunt syndrome, and ophthalmoplegic migraine when retroorbital. No precise histologie or geographic division exists among the various idiopathic orbital inflammations, and the present tendency is to lump all varia¬ tions under the unfortunate term "or¬ bital pseudotumor."1 However, there are sufficient cases3·4 identical to the following patient, in which the extraocular muscles are the major site of transient inflammation, for the diag¬ nosis of acute orbital myositis (AOM) to be of practical clinical value, pending a more rational basis for clas¬ sification. -
conditions the orbit and cavernous a sinus diagnostic chal¬ lenge. In the absence of clues to pathogenesis, clinical descriptions are based sites of on visible or presumed involvement. After inflammatory tu¬ mor reactions and infectious causes
inflammatory Remitting involving present major
Accepted for publication May 5, 1977. From the Department of Neurology, Los Angeles County-University of Southern California Medical Center, Los Angeles. Reprint requests to Box 359, 1200 N State St, Los Angeles, CA 90033 (Dr Keane).
REPORT OF A CASE A 27-year-old previously healthy woman awoke with pain, redness, and mild swelling about the right eye. Her symp-
Downloaded From: http://archneur.jamanetwork.com/ by a University of Michigan User on 06/13/2015
progressed slightly over the next two days despite antibiotic therapy for pre¬ toms
sumed orbital cellulitis. Admission examination showed rightsided eye findings of 5 mm proptosis, mild upper-lid and conjunctiva] edema, and dilated conjunctival vessels, particularly at the medial canthus. Movements of the
right eye, especially abduction, were pain¬ fully limited. The rest of the results of the ocular examination were normal, as was her general physical examination. The ESR was consistently elevated at 50 mm/hr; otherwise, results of her blood studies were normal. Radiographs showed a right maxil¬ lary sinus soft tissue density; antral punc¬ ture of the maxillary sinus showed serous fluid without evidence of infection; and subsequent sinus radiographs were normal. Her eye involvement continued to progress for several days after admission and then slowly and completely resolved during the next two weeks. One month after the onset of her initial symptoms, she noted identical acute pain, redness, and swelling about her other eye. On readmission, the right eye appeared
Fig
1.—Left proptosis, mild ptosis, and limitation of abduction are evident attempted left gaze.
severe
on
of Bertelson's4 cases of sclerotenonitis had signs restricted to exophthalmos and limitation of eye movement, with one
patient experiencing yearly epi¬
sodes in one eye or the other for six years. Similarly, six episodes of
Fig 2.—A, Computerized tomographic scan before contrast injection, showing promi¬ nence of medial rectus muscles. B, Computerized tomographic scan in same plane after contrast injection showing marked asymmetric enhancement of medial recti. normal, and she had left-sided eye findings of 3 mm of proptosis, mild generalized conjunctival injection, particularly over the medial rectus muscle insertion, and mild upper-lid edema with slight ptosis. Mild
limitation of abduction with accompanying pain at the medial canthus progressed during the next several days to complete paralysis of abduction (Fig 1). The ESR was again elevated (45 mm/hr), but results of the VDRL, antinuclear anti¬ body titers, a latex fixation test, and thyroid function studies were normal. Ultrasound studies showed a mass at the medial aspect of each orbit, larger on the left side. Computerized tomographic (CT) x-ray scanning demonstrated asymmetric prominent medial rectus muscles, with dramatic enhancement along the entire muscle length after contrast injection
(Fig 2)
Her orbital signs and symptoms com¬ pletely resolved over a two-week period—a course identical to the initial right orbital
involvement. She has had no further eye problems in the six months since leaving the hospital.
course
and
findings
litis, inflammatory tumor reaction, acute orbital pseudotumor syndromes, and perhaps acute thyroid ophthalmopathy. Remitting cavernous sinus
syndromes share similar features of pain, a self-limited course with rare residual signs, frequent elevation of the ESR, and probable steroid respon¬ siveness, but are distinguished by a pattern of discrete cranial
COMMENT
The
able but usually severe in relation to the mild proptosis. Progression ceases within one week, and the eye returns to normal during the next two to eight weeks. The patient is afebrile and has a normal WBC count, syphilis serology, thyroid function studies, and screening tests for vasculitis. Fre¬ quent malaise and elevation of the ESR indicate systemic disease but rarely can any definite connective tissue disease be diagnosed.5 Steroid therapy frequently appears to hasten resolution. The differential diagnosis of the initial episode includes bacterial cellu-
involvement and absence of in the
present case are very similar to that of
small group of patients with orbital inflammation best characterized as acute orbital -myositis.34 An abrupt a
onset of periorbital pain increasing on eye motion is rapidly followed by mild proptosis, with conjunctival and upper lid edema and the appearance of
dilated vessels overlying the hori¬ zontal muscle insertions. The limita¬ tion of eye movements is highly vari-
cant
proptosis.
nerve
signifi¬
"Chronic orbital myositis" is an even less well-defined condition, with minimal inflammation and a pro¬ tracted course, but reliable differen¬ tiation from thyroid ophthalmopathy is probably impossible.1L'e Cases reported as acute sclerotenonitis, tenonitis, and posterior scleritis include patients identical to present one, as well as many with prominent posterior globe inflammation.4 78 Six
Downloaded From: http://archneur.jamanetwork.com/ by a University of Michigan User on 06/13/2015
alternating painful ophthalmoplegia and proptosis occurred during a sevenyear period in a patient considered to have ophthalmoplegic migraine.9 Although the evaluation of prop¬ tosis has been revolutionized through the use of ultrasonic and CT scanning devices,10 the precise diagnosis of orbital inflammatory conditions re¬ mains a challenge.11 The CT scan in the present case dramatically con¬ firmed the clinical impression of
medial rectus inflammation, demon¬ strated continuing abnormality in the clinically recovered opposite medial rectus muscle, and obviated further evaluation of what could have been mistaken for a sixth-nerve paresis.
Stewart Winthrop, MD, and George A. Curry, MD, assisted with the clinical evaluation. John Ratcliffe, MD, provided the German transla¬ tion.
References 1. Jakobiec FA, Jones IS: Orbital inflammain Duane TD (ed): Clinical Ophthalmology New York, Harper & Row Publishers Inc, 1976, vol 2, pp 1-75. 2. Blodi FC, Gass JDM: Inflammatory pseudotumor of the orbit. Trans Am Acad Ophthalmol Otolaryngol 71:303-323, 1967. 3. Esslen E, Papst W: Die Beduetung der Elektromyographic f\l=u"\rdie Analyse von Motilitatsstorungen der Augen. Basel, Switzerland, S Karger, 1961, pp 62-70. 4. Bertelson TI: Acute sclerotenonitis and ocular myositis complicated by papillitis, retinal detachment and glaucoma. Acta Ophthalmol 38:136-152, 1960. 5. Brenner EH, Shock JP: Proptosis secondary to systemic lupus erythematosus. Arch Ophthalmol 91:81-82, 1974. 6. Wolter JR, Hoy HE, Schmidt DM: Chronic orbital myositis. Am J Ophthalmol 62:292-298, 1966. 7. Duke-Elder S, MacFaul PA: The Ocular Adnexa. System of Ophthalmology, St Louis, CV Mosby Co, 1974, vol 13, part 2, pp 878-880. 8. Leira H: Sclerotenonitis and orbital myositis. Mod Probl Ophthalmol 14:669-674, 1975. 9. Hedges TR: Alternating exophthalmos with painful ophthalmoplegia. Arch Ophthalmol 74:625-627, 1965. 10. Dallow RL, Momose KJ, Weber Al, et al: Comparison of ultrasonography, computerized
tion,
tomography (EMI scan), and radiographic techniques in evaluation of exophthalmos. Trans Am Acad Ophthalmol Otolaryngol 81:305-322,
1976. 11. Enzmann D, Donaldson SS, Marshall WH, et al: Computed tomography in orbital pseudotumor (idiopathic orbital inflammation). Radiology 120:597-601, 1976.
