Vannessa Leung BMed MD Michael Wei MBBS Timothy V Roberts MMed FRANZCO
Metastasis to the extraocular muscles (EOM) is rare. The existing literature comprises only of isolated case reports therefore a summative description of these lesions is lacking. This study presents a case of bilateral rectus muscle metastasis from malignant melanoma. Furthermore a literature review and pooled data analysis is undertaken with 43 articles encompassing 77 patients and 101 eyes. Mean age was 53 years, 54% were male, 66% had unilateral involvement and 34% had bilateral involvement. The primary malignancies were melanoma (n = 17, 22%), breast (n = 15, 15%) and carcinoid (n = 11, 14%). A single muscle was involved in 56 eyes (67%) and multiple muscles in 27 eyes (33%). Lateral rectus was most commonly affected (n = 44, 53%). Presenting symptoms included restricted eye movements (n = 48, 62%), proptosis (n = 45, 58%), diplopia (n = 27, 35%) and pain (n = 18, 23%). Scattered case reports have previously made it difficult to characterise this phenomenon thus a pooled data analysis is presented.
Amun Sachdev, Nigel Timothy O’Connor, Suresh Reddy Sagili
Orbital involvement in chronic lymphocytic leukemia (CLL) is rare with very few published cases. We describe a case of unilateral isolated extraocular muscle enlargement in a patient with CLL. An incisional biopsy was performed from the left medial rectus muscle and histology revealed a lymphocytic infiltrate suggestive of CLL. Complete resolution of signs and symptoms was subsequently achieved with chemotherapy. We would suggest that in patients presenting with atypical clinical features, it is important to consider nonthyroid-related causes of extraocular muscle enlargement and a muscle biopsy should be considered to exclude neoplastic causes.
Giovanni H. Greaves, Kym Livingston, Grant T. Liu, Kenneth S. Shindler, Nicholas J. Volpe, Maxwell Pistilli, Sonul Mehta & Madhura A. Tamhankar
Neoplastic infiltration of the extraocular muscle (EOM) is a rare condition which can pose a diagnostic dilemma due to its rarity and overlapping ultrasonographic features with orbital myositis. The ultrasonographic features of neoplastic enlargement of EOM have not been systematically studied and previously have been described in only a few case reports. Orbital ultrasonography, in conjunction with the pattern of ocular misalignment, was assessed for its potential role in identifying patients with neoplastic EOM enlargement. Retrospective chart review of patients with neoplasm and myositis. The clinical features of 8 patients with neoplastic infiltration of the EOM were compared to 15 patients with myositis. In the neoplastic group the width of the EOM was (10.5 mm) almost twice the normal width of the muscle with myositis (p < 0.001). All the muscles in the neoplastic category were low to medium reflective. Paretic deviation was seen in 4/8(50%), purely restrictive in 2/8 (25%) and combined pattern in 2/8 (25%) were noted. In the myositis group the average EOM enlargement was 5.8 mm and all muscles showed low reflectivity. Although ultrasonographic features overlapped between the 2 groups paretic deviations were more common in the neoplastic group versus the myositis group (50% versus 7%). Neoplastic muscle enlargement tends to be larger with paretic deviations of ocular motility seen clinically. These findings in a patient with EOM enlargement should raise the suspicion of neoplasm as the etiology and further work up should be considered.
Tom Kornhauser, Ahed Imtirat, Jaime Levy, Erez Tsumi
PurposeTo describe a simple and novel technique of obtaining an extraocular muscle (EOM) biopsy.
MethodsOur sutureless method involves obtaining a full-thickness 3-mm EOM specimen using a disposable punch biopsy.
ResultsThe procedure was performed successfully, with no worsening of eye movement, muscle weakness, or cosmetic defects postoperatively. The acquired full-thickness specimen was sufficient for analysis.
ConclusionsThis technique to obtain an EOM biopsy allows a straightforward sutureless method, providing an adequate sample for tissue scrutiny.
F Shafi, P Mathewson, P Mehta and H S Ahluwalia
Extraocular muscle enlargement (EOME) is most commonly associated with thyroid eye disease, but there are other causes. We report our outcomes of investigating and managing non-thyroid-related EOME (NTR-EOME).
Retrospective consecutive case series. Sixteen patients identified by clinical features and orbital imaging. Patient demographics, radiological features, and adjuvant tests including biopsy and final diagnosis were recorded.
Mean age at presentation 59.3 years (range 24–89 years). Mean follow-up 3.2 years (range 3 months to 5.5 years). Superior rectus (SR) was most commonly involved muscle (8/16 cases) followed by lateral rectus (4/16). Of the 16 cases, 14 were associated with underlying systemic neoplasia (5 lymphoma, 5 metastatic carcinoma, and 4 presumed paraneoplastic syndrome). All SR enlargement was associated with underlying neoplasia. All patients underwent orbital imaging followed by systemic imaging based on clinical index of suspicion (14/16 patients (13 full body CT (FBCT), 1 mammography)). Positive systemic radiological findings were detected in 12/14 cases. Of the remaining 2 patients, 1 underwent full body positron emission tomography-computed tomography (FBPET-CT), which detected thyroid carcinoma, and the second patient underwent FBCT for staging following orbital biopsy showing lymphoma. Four patients (25%) died within 3 years of follow-up due to disseminated systemic malignancy.
All cases of NTR-EOME should be viewed with a high level of clinical suspicion for systemic neoplasia, especially when the SR is involved. FBCT can help to identify a primary systemic cause. FBPET-CT is best reserved for cases negative on FBCT or for staging and monitoring systemic disease. NTR-EOME can be associated with significant mortality (25%), hence warrants prompt and thorough systemic investigation.
Modi, Yasha M.D.; Erickson, Benjamin M.D.; Ranadive, Isha B.A.; Pasol, Joshua M.D.; Wester, Sara M.D.; Cavuoto, Kara M.D.
A 6-month-old boy presented with a unilateral motility deficit of the right eye in all fields of gaze. Neuroimaging revealed unilateral enlargement of the medial, lateral, and inferior rectus muscles with sparing of the tendons. An evaluation for thyroid eye disease, idiopathic orbital inflammation, myositis, inflammatory and neoplastic infiltration of the muscle, vascular anomalies, and metastatic neuroblastoma was unrevealing. Biopsy of the muscle revealed normal architecture with an absence of inflammation, infiltration, or fibrosis. A review of the literature reveals the exceptionally rare nature of this finding. While the authors cannot rule out an atypical case of congenital euthyroid eye disease, this constellation of findings is not consistent with thyroid eye disease and may represent previously described cases of idiopathic enlargement of the extraocular muscles.