Extraocular Muscle Hypertrophy Research Articles

A Case of Orbital Infarction Syndrome in a Patient with Hemophagocytic Lymphohistiocytosis

Purpose: To report a case of orbital infarction syndrome in a patient with hemophagocytic lymphohistiocytosis (HLH).Case summary: A 70-year-old woman with diabetes mellitus and hypertension was referred to the Department of Ophthalmology for sudden-onset left upper eyelid ptosis after being diagnosed with HLH. After 3 days, the best corrected visual acuity in the right eye was 0.8, while there was no light perception in the left eye. In the left eye, the ocular motility examination showed limitations in all fields of gaze. On fundus examination, optic disc pallor, retinal hemorrhage, and narrowed retinal arteries were observed in the left eye. Fluorescein angiography showed no blood flow in the retinal arteries and veins in the left eye. On neck computed tomography angiography, the left distal internal carotid artery was narrowed. Orbit computed tomography showed exophthalmos and extraocular muscle hypertrophy in the left eye. Orbit magnetic resonance imaging confirmed optic nerve edema, enhancement of the optic nerve sheath, and high signal intensity of the intraocular fat in the left eye. Slit-lamp examination revealed ischemia of the anterior segment and ischemic necrosis of the eyelid in the left eye. The patient was diagnosed with orbital infarction syndrome.Conclusions: Several factors may lead to ischemia of the orbital tissues in patients with HLH, including coagulopathy, an increased incidence of blood clots, and inflammation in the orbit. It is necessary to consider the possibility of orbital infarction syndrome when HLH patients present with visual loss, ocular movement limitations, and anterior segment ischemia.

Local recurrence as extraocular muscle lymphoma after 6 years of chronic myositis: a case report

BackgroundExtraocular muscle is usually affected by thyroid disease or inflammatory pseudotumor, but seldom by neoplastic process. Primary malignant lymphoma involving isolated extraocular muscle is very rare, especially after 6 years of chronic myositis.Case presentationA middle-aged female presented with swelling of the lower lid of the right eye for 2 months. Magnetic resonance imaging showed significant enlargement of the right inferior rectus muscle belly. The patient first presented 6 years prior with upper eyelid swelling. A total of 5 surgical biopsies of the right eye were performed during 6 years with the following successive findings: inflammatory pseudotumor, chronic inflammation, inflammatory lesions, IgG4-related ophthalmic disease, and lastly, extraocular muscle extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma).ConclusionMALT lymphoma may have occurred as a result of chronic extraocular myositis. Malignancy should be considered in patients with recurrent painless extraocular muscle hypertrophy. Differential diagnosis can rule out thyroid-associated ophthalmopathy (TAO), whose symptoms are similar. Diagnosis confirmation by biopsy is warranted if necessary.

Tolosa-Hunt Syndrome with Multiple Orbital Myositis in a Patient with Crohn’s Disease

Purpose: We report a case of Tolosa-Hunt syndrome with multiple orbital myositis identified via orbital magnetic resonance imaging in a patient with Crohn's disease who developed right eye pain and binocular horizontal diplopia.Case summary: A 46-year-old woman visited our clinic with a 2-month history of right eye pain and migraine, as well as a 3-day history of acute horizontal diplopia. She had previously been diagnosed with Crohn's disease and was taking immunosuppressive drugs. In the eye movement test, esotropia and an abduction limitation of -3.0 in the right eye were observed on the Krimsky test. There were no specific findings in anterior segment and fundus examinations. Orbital magnetic resonance imaging showed multiple extraocular muscle enhancement in the right eye and multiple extraocular muscle hypertrophy in the left eye. The patient was diagnosed with binocular multiple orbital myositis and right Tolosa-Hunt syndrome; she was treated with high-dose steroids for 3 days followed by lower dose oral medications. During the first week of treatment, the right eye pain disappeared and the right eye abduction limitation showed slight improvement. After 3 months of treatment, the right eye abduction limitation and esotropia completely disappeared.Conclusions: Orbital myositis and Tolosa-Hunt syndrome are idiopathic, nonspecific chronic granulomatous diseases with painful ophthalmoplegia. We describe a rare case in which the two diseases appear together.

Kimura Disease Presenting as Unilateral Proptosis

Purpose: Kimura disease is a rare chronic inflammatory disease that usually presents with painless subcutaneous nodules at the head and neck area, but rarely occurs around the eyes. We report unique case of Kimura disease involving the orbit and arm, which has not been reported in Korea.Case summary: A 16-year-old boy presented with exophthalmos and eyelid swelling of right eye over the last 2 years. Orbital magnetic resonance imaging (MRI) showed hypertrophy of extraocular muscles of right eye. He also complained of multiple painless masses of the left forearm that persisted during the same period. The MRI showed hypertrophy of extraocular muscles of right eye and multiple lymphadenopathy along the brachial artery of the left elbow. Excisional biopsy of the left arm mass and lymph nodes was performed and the lesions were diagnosed as Kimura disease involving the dermal nodule and lymph nodes. The patient was managed with oral corticosteroid and cyclosporine and showed remarkable improvement of proptosis and hypertrophy of extraocular muscles.Conclusions: We report a rare case of Kimura disease causing hypertrophy of extraocular muscles which was successfully treated with immunosuppressive agents.

Immunoglobulin G4-Related Orbital Disease with Bilateral Optic Perineuritis and Maxillary Nerves Involvement: A Case Report.

Immunoglobulin G4-related optic neuropathy caused by optic perineuritis is a rare complication of immunoglobulin G4-related disease (IgG4-RD). Herein, we report a 38-year-old Asian man with history of sinusitis who presented with painless blurred vision and proptosis for over 6 months. Examination with the Hertel exophthalmometer revealed 21.5 mm on both eyes. Magnetic resonance imaging revealed a doughnut sign encircling the right optic nerve, bilateral tram-track signs on both optic nerves, enlarged bilateral maxillary nerves with perineural spreading to the infraorbital nerves, hypertrophy of extraocular muscles, and pansinusitis. Visual evoked potentials displayed bilateral delayed P100 latency, indicating bilateral optic neuropathy. Biopsy with functional endoscopic sinus surgery demonstrated diffuse dense lymphoplasmacytic infiltrate and fibrosis. IgG4-positive plasma cells exceeded 50 cells per high-power field while the overall IgG4/IgG ratio was above 40%. Serological studies unveiled extremely high serum concentrations of IgG4 (2650 mg/dL), and the calculated serum IgG4/IgG ratio was 100%. These comprehensive features supported the diagnosis of IgG4-RD with bilateral optic perineuritis, branches of trigeminal nerve involvement, and pansinusitis. The visual acuity improved slightly following the initiation of treatment with corticosteroids, but it became worse again during the tapering course. Following another course of corticosteroids followed by subsequent immunosuppressant treatment with azathioprine, vision in both eyes ultimately improved during the 2-year follow-up period.

A rare case report of conjunctival fibrolipoma in an adolescent

Fibrolipomas are a fat-containing lesions that are characterised by the presence of adipose tissue and rich amounts of fibrous tissues. We present a case of a18-year-old female presented with complaints of swelling over outer part of left eye for 2 years . Magnetic resonance imaging (MRI) revealed T1 and T2 hyper-intense lesion in temporal aspect of left eyeball without any involvement of optic nerve or any hypertrophy of extra-ocular muscles. A careful dissection was done because of proximity of the lesion with lateral rectus and optic nerve, followed by excision. The diagnosis of fibrolipoma was made on histopathological examination. Fibrolipoma is a rare variant of the lipoma and only few cases have been reported in the orbit till date. The case we have reported is first of its kind in India.

Orbital diseases mimicking graves' orbitopathy: a long-standing challenge in differential diagnosis.

Graves' orbitopathy (GO) is the most common cause of orbital tissue inflammation, accounting for ~ 60% of all orbital inflammatory conditions in the population aged 21-60years, and for ~ 40% in the population aged > 60year. GO is observed in 25-30% of patients with Graves' hyperthyroidism and more rarely in association with hypothyroid autoimmune thyroiditis. In addition, a small proportion of GO patients (1-2%) do not have a clinically overt thyroid dysfunction. Clinically, GO is characterized by proptosis, inflammation involving the eyelids and the conjunctiva, extraocular muscle hypertrophy, with consequent reduction of ocular motility and diplopia, and in the most severe cases, compression of the optic nerves at the orbital apex, with reduction of visual acuity. At CT scan or MRI, a muscle increase involving the superior, medial and inferior rectus is quite typical. In the most severe forms, compression of the optic nerves at the orbital apex can be observed. Euthyroid GO is usually an early sign of a full-blown Graves' disease; however, in some cases, the orbital disease can remain isolated. Moreover, euthyroid GO can rarely be unilateral, which makes the picture even more confusing. Under those circumstances, the diagnostic process becomes obviously quite difficult, having other conditions mimicking GO been excluded. A number of inflammatory conditions affecting orbital tissue can mimic GO, thereby requiring an accurate evaluation for a proper differential diagnosis. The majority of these conditions are immune mediated. Most of them are benign, but they can be rather aggressive and some can cause visual loss. The most common inflammatory condition affecting orbital tissues and mimicking GO is idiopathic orbital inflammation. Other, more rare, orbital diseases that should be considered in the differential diagnosis are infections, orbital manifestations of systemic diseases, primitive and secondary orbital neoplasms, and orbital vascular alterations. In most instances, when an orbitopathy occurs in the absence of hyperthyroidism, the diagnosis of the disease underlying the ocular symptoms and signs is based on exclusion of the other conditions. Here we review the conditions that can mimic GO and how to distinguish them from this obnoxious eye disease.

Induced hypertrophy of extraocular muscles after the administration of synthetic peptides into them

Induced hypertrophy of extraocular muscles after the administration of synthetic peptides into them

Magnetic resonance imaging in dissociated strabismus complex demonstrates generalized hypertrophy of rectus extraocular muscles

Dissociated strabismus complex (DSC) is an enigmatic form of strabismus that includes dissociated vertical deviation (DVD) and dissociated horizontal deviation (DHD). We employed magnetic resonance imaging (MRI) to evaluate the extraocular muscles in DSC. We studied 5 patients with DSC and mean age of 25years (range, 12-42years), and 15 age-matched, orthotropic control subjects. All patients had DVD; 4 also had DHD. We employed high-resolution, surface coil MRI with thin, 2mm slices and central target fixation. Volumes of the rectus and superior oblique muscles in the region 12mm posterior to 4mm anterior to the globe-optic nerve junction were measured in quasi-coronal planes in central gaze. Patients with DSC had no structural abnormalities of rectus muscles or rectus pulleys or the superior oblique muscle but exhibited modest, statistically significant increased volume of all rectus muscles ranging from 20% for medial rectus to 9% for lateral rectus (P<0.05). DSC includes various combinations of sursumduction, excycloduction, and abduction not conforming to Hering's law. We have found modest generalized enlargement of all rectus muscles. DSC is associated with generalized rectus extraocular muscle hypertrophy in the absence of other orbital abnormalities.

Magnetic resonance imaging (MRI) in dissociated strabismus complex (DSC) demonstrated generalized hypertrophy of extraocular muscles (EOMs)

Magnetic resonance imaging (MRI) in dissociated strabismus complex (DSC) demonstrated generalized hypertrophy of extraocular muscles (EOMs)

Clinical Features of Dry Eye in Thyroid-Associated Ophthalmopathy According to Disease Activity

Purpose: To evaluate the clinical features of dry eye in thyroid-associated ophthalmopathy (TAO) according to disease activity and analyze the related factors. Methods: This study included 157 patients diagnosed with TAO and dry eye between March 2009 and March 2015. According to the clinical activity score (CAS), TAO patients were divided into inactive (CAS < 3) and active (CAS ≥ 3) groups. Clinical features included age, sex, visual acuity, proptosis, palpebral fissure width, orbital computed tomography (CT) findings, thyroid hormones, and ocular surface parameters including tear film break-up time (TFBUT), Schirmer tests, keratoepitheliopathy scores, and ocular surface disease index (OSDI) were obtained and compared between the groups. In addition, correlations between clinical features and ocular surface parameters were analyzed in both groups. Results: In the inactive and active TAO groups, CAS was 1.24 ± 0.69 and 4.23 ± 1.13, respectively (p = 0.001). Thyrotropin-binding inhibitory immunoglobulin was significantly higher in the active TAO group than in the inactive TAO group (p = 0.048). On orbital CT, extraocular muscle hypertrophy was more common in the active TAO group than the inactive TAO group (p = 0.020). No significant difference was found in age, sex, visual acuity, free T4, and thyroid-stimulating hormone between the two groups. During analysis of the tear film and ocular surface parameters, TFBUT (p = 0.006) was shorter and OSDI score (p = 0.028) was higher in the active TAO group than the inactive TAO group. TFBUT was negatively correlated with proptosis (r = -0.432, p = 0.001; r = -0.308, p = 0.032) and palpebral fissure width (r = -0.367 p = 0.012; r = -0.312, p = 0.031) in both groups. OSDI was positively correlated with proptosis in the active TAO group (r = 0.301, p = 0.033). Conclusions: TFBUT was shorter and OSDI score higher in dry eye patients with active TAO than in patients with inactive TAO. The TFBUT was negatively correlated with proptosis and palpebral fissure width in both groups. J Korean Ophthalmol Soc 2016;57(7):1037-1043

Extraocular muscle hypertrophy in myotonia congenita

Myotonia congenita (MC) is a rare disorder of skeletal muscle caused by mutations in the CLCN1 gene,(1,2) which encodes the chloride ion channel found in the t-tubule of skeletal muscle. MC is characterized by impaired relaxation of voluntary muscle after sudden contraction that diminishes with muscle activity, known as the "warm-up effect." Individuals with MC can develop muscular hypertrophy despite little physical activity. Esotropia and reduced saccadic velocities have been reported in the dominant form of MC. We report two cases in which orbital magnetic resonance imaging (MRI) imaging showed extraocular muscle hypertrophy.

Prise en charge chirurgicale des diplopies dysthyroïdiennes

Forty percent of thyroid-related orbitopathies are associated with strabismus. In the healing phase of disease, fibrous hypertrophy of extraocular muscles may result in restrictive myopathy. We report our experience of surgical management in these cases. We present a retrospective case series conducted on 42 patients, operated between September 1998 and April 2003. All patients underwent complete oculoplastic and orthoptic examinations. Muscular recession was performed in all cases, with general anesthesia in cases of clinical muscular restriction; adjustable recession with topical anesthesia was performed in cases of moderate muscular restriction. Postoperative alignment and elimination of diplopia in the functional position of the gaze were evaluated. The study included 42 patients (28 women, 14 men) with a mean age of 51 years. The mean follow-up was 48 months (range, 10 months to 6 years). Twenty-six patients presented vertical diplopia, 11 horizontal diplopia, and five mixed diplopia with a vertical and a horizontal component. These patients were operated on twice, beginning with vertical surgery. In cases of general anesthesia, muscular recession was adapted to an intraoperative forced duction test and a muscular elongation test. We obtained 94% good results for vertical diplopia and 81% for horizontal diplopia. Incomplete results were under correction. Adjustable muscular recession was adapted to intraoperative evaluation with patient participation. All patients had good results. Successful surgical treatment depends on precise identification of the muscles that are restricting motility and producing misalignment. We insist on the importance of an intraoperative forced duction test and a muscular elongation test in case of clinical muscular restriction. Adjustable surgery is useful in cases of moderate muscular fibrosis.

Effect of steroid pulse therapy with and without orbital radiotherapy on Graves’ ophthalmopathy

PurposeTo report the effect of high-dose intravenous corticosteroid pulse therapy with and without orbital radiotherapy on Graves’ ophthalmopathy. DesignNonrandomized clinical trial. MethodsWe selected 39 Japanese patients (age range, 22–64 years; mean, 48 years; 31 women, 8 men) who had active Graves’ ophthalmopathy among 195 consecutive patients. In the first 20 patients, high-dose intravenous methylprednisolone pulse therapy (1 g per day for 3 successive days, repeated 3 times within 3 weeks) followed by 24-Gy orbital radiotherapy was performed. In the other 19 of the 39 patients, high-dose intravenous methylprednisolone pulse therapy without orbital radiotherapy was performed. Coronal computed tomography (CT) of the orbit and exophthalmometry were performed before the corticosteroid pulse therapy, and 1 and 6 months after the corticosteroid pulse therapy. The maximum coronal section area of the most hypertrophic rectus muscle in each eye was measured based on orbital CT imaging. ResultsClinical findings at study entry were not significantly different between the two groups. No significant difference was found in the maximum coronal section area of the most hypertrophic rectus muscle and the results of exophthalmometry measurements in both groups before the therapy. Extraocular muscle hypertrophy was significantly reduced 1 month and 6 months after the therapy (P < .01) in both groups. However, no beneficial therapeutic effect on proptosis was observed in either group at 1 month and 6 months after the therapy. No significant difference in the therapeutic effect on extraocular muscle hypertrophy and proptosis was found between the two groups. ConclusionsOrbital irradiation after corticosteroid pulse therapy had no beneficial therapeutic effects on rectus muscle hypertrophy or proptosis of active Graves’ ophthalmopathy during the 6-month follow-up period.

Effect of High-dose Intravenous Steroid Pulse Therapy Followed by 3-Month Oral Steroid Therapy for Graves' Ophthalmopathy

Purpose: To evaluate the effect of high-dose intravenous steroid pulse therapy followed by 3-month oral steroid therapy for Graves' ophthalmopathy. Methods: We selected 41 Japanese patients (age range, 21–76 years; mean = 49 years) who had active Graves' ophthalmopathy among 205 consecutive patients examined at Sapporo Medical University Hospital between 1997 and 1999. In a prospective study, we investigated the effect on the 41 patients of high-dose intravenous methylprednisolone pulse therapy (1 g/day × 3 days × 3 times) followed by 3-month oral prednisone therapy. Coronal computed tomography (CT) of the orbit, exophthalmometry and monocular fixation field measured by Goldmann perimetry were carried out before the steroid pulse therapy, and 1 and 6 months after the steroid pulse therapy. The maximum coronal section area of the rectus muscle in each eye was measured using an orbital CT image. Results: Extraocular muscle hypertrophy was significantly reduced 1 and 6 months after the pulse therapy (paired t-test, P < .01), and was not significantly different between 1 and 6 months after the pulse therapy. Proptosis was not significantly reduced by the pulse therapy. Monocular fixation fields were measured in 34 patients with diplopia, and limitation of eye movements was improved in 15 patients (44%) by the pulse therapy. In the other patients, improvement of the limitation was not detectable by the test of the monocular fixation field. Conclusions: The treatment in this study is effective for extraocular muscle hypertrophy, and relapse was minimum within 6 months. However, this treatment has limited effect on limitation of eye movements and less effect on proptosis.

Increased retinal blood flow in patients with Graves' disease: influence of thyroid function and ophthalmopathy.

Graves' ophthalmopathy (GO), resulting from the inflammation of retro-orbital tissue, is one of the major complications of Graves' disease (GD). We investigated the clinical usefulness of the measurement of retinal blood flow (RBF) in the evaluation of GO and its activity. RBF was quantitated by pulsed Doppler mode at just below the branch of central retinal artery, from which the resistance index (RI) was calculated. Forty-seven euthyroid GD patients and 70 gender- and age-matched normal controls were measured for RI to investigate the effect of GO on RBF. To investigate the effect of hyperthyroidism, 20 GD patients were measured for RI changes during antithyroid drug (ATD) therapy. Furthermore, 17 GD patients with clinically overt GO were measured for RI changes during treatment with glucocorticoid plus retro-orbital radiation. RI and exophthalmos showed a significant positive correlation in 47 treated euthyroid GD patients without clinically overt GO (r=0.307, P<0.05), but not in 70 age- and sex-matched normal subjects (r=0.185, P=0.161). Furthermore, RI, but not exophthalmos, significantly correlated with serum TSH receptor antibodies, an indicator for the disease activity of GO. ATD therapy significantly reduced RI in GD patients from 0.719+/-0.041 in the hyperthyroid state to 0.661+/-0.051 in the euthyroid state, but not to the levels observed in normal subjects having the similar exophthalmos (0.640+/-0.049). The fractional reduction of RI during ATD therapy significantly correlated with those of pulse pressure and ultrasonographic distensibility in carotid artery, but not with those of serum vascular injury markers. In 17 GD patients with clinically overt GO, all four patients having adipose tissue enlargement but not extraocular muscle hypertrophy (inactive GO) showed RI within the mean +/- 1 s.d. for treated GD patients without GO. In the other 13 GD patients having extraocular muscle hypertrophy (active GO), four and eight patients showed RI outside mean +/- 2 s.d. and mean +/- 1 s.d. respectively. Treatment with glucocorticoid plus radiation moved RI in 8 out of 10 patients toward the mean values of GD patients without GO, in spite of little improvement of exophthalmos. It was suggested that GD patients showed altered retinal hemodynamics, possibly resulting either from the cardiovascular effect of hyperthyroidism or from retro-orbital inflammation, particularly in extraocular muscle.

Serum levels of soluble Fas in patients with Graves' ophthalmopathy

AIMTo assess levels of soluble Fas (sFas) in the sera of patients with Graves' ophthalmopathy.METHODSThe subjects in this study were 43 patients with Graves' ophthalmopathy and 11 normal subjects. Serum...

Autoantibodies to IGF-1 binding sites in thyroid associated ophthalmopathy.

Graves' disease is an autoimmune disorder but the nature of the association between hyperthyroidism and ophthalmopathy is not yet understood. Serum autoantibodies to orbital tissues have previously been identified and the cross-reactivity with orbital and thyroid antigens has been implicated in the development of thyroid-associated ophthalmopathy (TAO). The ophthalmopathy of Graves' disease is remarkable for the hypertrophy of extraocular muscles and proliferation of fibroblasts within the orbit; features which suggest a possible involvement of growth factors. The present study was therefore undertaken to investigate the interaction of IgGs extracted from the sera of patients with Graves' disease, with or without overt ophthalmopathy, with respect to IGF-1 receptor binding sites on fibroblasts from human orbital tissue. IGF-1 binding sites were demonstrated on human orbital fibroblast monolayers grown from eye muscle explants. These cells exhibited a population of high affinity IGF-1 binding sites (Kd, 0.5nM SEM +/- 0.05). IgG prepared from sera taken from patients with Graves' disease (n = 23) significantly inhibited [125I]IGF-1 binding to orbital fibroblasts when compared to IgGs prepared from normal volunteers (n = 13, p < 0.002). It was found that 12 of 23 (52%) patients' IgG samples gave rise to significant levels of inhibition of [125I]IGF-1 binding to orbital fibroblasts. The IgG preparations did not bind directly to IGF-1. This study demonstrates that IgG prepared from patients with Graves' disease with or without overt ophthalmopathy interact with IGF-1 binding sites on orbital fibroblasts whereas IgG from normal subjects had no significant effect.(ABSTRACT TRUNCATED AT 250 WORDS)