Presenting Characteristics for Symptomatic, as Compared to Asymptomatic (assumed), Orbital Cavernous Venous Malformations : Ophthalmic Plastic & Reconstructive Surgery

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Presenting Characteristics for Symptomatic, as Compared to Asymptomatic (assumed), Orbital Cavernous Venous Malformations

Vahdani, Kaveh M.D., F.R.C.Ophth.; Rose, Geoffrey E. D.Sc., F.R.C.Ophth.

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Ophthalmic Plastic and Reconstructive Surgery: November/December 2022 - Volume 38 - Issue 6 - p 546-550
doi: 10.1097/IOP.0000000000002195
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Previously and inappropriately termed cavernous hemangiomas (implying neoplasm)1, orbital cavernous venous malformations (OCVMs) are benign, well-circumscribed, slowly progressive vascular lesions that, in adults, are the commonest benign tumor and account for ~5% of all orbital masses.2,3 Although OCVMs may be asymptomatic and discovered incidentally during neuroimaging, patients with symptoms typically present in the 4th to 6th decades, more frequently in women, and with mass-related symptoms.3–8 The proportion of asymptomatic (and therefore presumed; so-called “incidentalomas”9) OCVMs remains uncertain, with estimates ranging from 13%–30%, and these are usually discovered on investigation for unrelated causes.5,8

We compare the presenting characteristics for symptomatic OCVMs and asymptomatic (presumed) lesions; the long-term follow-up of these malformations is not considered.

PATIENTS AND METHODS

Patients who presented between 1972 and 2020 with either a histologically confirmed or a presumptive imaging diagnosis of OCVM were identified from diagnostic and pathological databases. Details of gender, age at presentation, symptoms and their maximum duration, laterality, and orbital functions were recorded from a retrospective review of clinical notes that were recorded by an ophthalmologist. A presumptive diagnosis of OCVM was made based on the typical CT and/or MRI characteristics,10 these patients having had imaging for unrelated conditions, such as sinusitis, cerebrovascular disease, or tinnitus/vertigo.

Two-tailed Student t-tests (paired where appropriate) were applied for continuous variables and the chi-square or Fisher Exact test for discrete variables, with an α-risk of 0.05 being considered clinically significant. To provide suitably-sized groups when comparing LogMAR acuities for the asymptomatic and symptomatic patients, age was considered in quintiles. The relationship between age at presentation and degree of exophthalmos was investigated using a linear regression model, with Pearson’s correlation coefficient being used to quantify linearity.

The study was approved by the Institutional Review Board (MEH #783) and adhered to the tenets of the Declaration of Helsinki.

RESULTS

Four hundred and fourteen patients (258 female; 62%) presented at a mean age of 49.4 years (median 47; range 11–91 years), all with unilateral lesions; records were insufficient to assess the frequency of multiple, or multilobulated, OCVMs. Of the 414 patients, 322 (78%) were symptomatic OCVMs presenting at an average age of 47 years (median 46; range 11–90 years) and 92 were asymptomatic being discovered, on average, a decade later than symptomatic patients (mean 57.8 years, median 56, range 25–91; p < 0.001) (Fig. 1) (Table 1).

TABLE 1. - Clinical characteristics for 414 patients with symptomatic or asymptomatic (presumed) orbital cavernous venous malformations
Clinical characteristic All patients (414 patients) Symptomatic malformations (322 patients) Asymptomatic malformations (92 patients) Significance
Proportion of females 258/414 (62%) 195/322 (61%) 63/92 (69%) 0.18
Proportion of right orbits 211/414 (51%) 161/322 (50%) 50/92 (54%) 0.48
Proportion histologically-proven 299/414 (72%) 290/322 (90%) 9/92 (10%) <0.001
Age at presentation
Mean (median; range) 49.4 years (47; 11–91) 47.0 years (46; 11–90) 57.8 years (56; 25–91) <0.001
 11–20 years 5/414 (1%) 5/322 (2%) 0/92 <0.001
 21–30 years 25/414 (6%) 21/322 (7%) 4/92 (4%)
 31–40 years 100/414 (24%) 86/322 (27%) 14/92 (15%)
 41–50 years 109/414 (26%) 94/322 (29%) 15/92 (16%)
 51–60 years 84/414 (20%) 66/322 (21%) 18/92 (20%)
 61–70 years 41/414 (10%) 29/322 (9%) 12/92 (13%)
 71–80 years 36/414 (9%) 15/322 (5%) 21/92 (23%)
 >80 years 14/414 (3%) 6/322 (2%) 8/92 (9%)
Significance refers to comparison of symptomatic and asymptomatic groups.

F1
FIG 1.:
Proportion presenting in each decade of life with either symptomatic or asymptomatic (presumed) orbital cavernous venous malformations (OCVMs).

Patients with symptomatic OCVMs had an average symptom duration of 3.3 years (median 1.5; range 1 week to 33 years), with the commonest symptoms being altered vision (184/322; 57%), proptosis (177/322; 55%), orbital ache (69/322; 21%), and diplopia (41/322; 13%) (Table 2). A subjective change in color vision was reported by 22% (56/260) and visual obscurations—gaze-evoked and/or postural—in 11% (26/238) of patients (Table 2). Where documented, the incidence of ipsilateral premature presbyopia or hyperopic shift was 30% (75/248) in symptomatic patients and 3% (3/90) with asymptomatic OCVMs (p < 0.001) (Table 3).

TABLE 2. - Presenting symptoms for 322 patients with ‘symptomatic’ orbital cavernous venous malformations
Presenting symptom Proportion with symptom
“Change in eyesight” 184/322 (57%)
“Change in colours” 56/260 * (22%)
“Fading out of vision” (obscurations) 26/238 * (11%)
 Gaze-evoked 19/238 * (8%)
 Postural 5/238 * (2%)
 Gaze-evoked and postural 2/238 * (1%)
Proptosis; subjective globe dystopia 177/322 (55%); 4/322 (1%)
Orbital ache; headache 69/322 (21%); 14/322 (4%)
Diplopia 41/322 (13%)
Subjectively palpable lump 27/322 (8%)
Variable periorbital swelling 27/322 (8%)
Watering eye; intermittent red eye 14/322 (4%); 10/322 (3%)
Blepharoptosis 6/322 (2%)
“Gritty” or “dry” eyes; photophobia 4/322 (1%); 2/322 (0.6%)
Periorbital sensory change 2/322 (0.6%)
Abnormal pupil 2/322 (0.6%)
Hemorrhage 2/322 (0.6%)
(No symptoms) (92 additional patients)
Mean duration of longest symptom 3.3 years (median 1.5 years; range 1 week–30 years)
*Not recorded for some patients.

TABLE 3. - Visual function at presentation for 414 patients with orbital cavernous venous malformations
Clinical characteristic All patients (414 patients) Symptomatic malformations (322 patients) Asymptomatic malformations (92 patients) Significance
Mean LogMAR acuity * (median; range)
 Whole group 0.33 (0.2; 0–4) 0.39 (0.2; 0–4) 0.1 (0; 0–0.8) <0.001
 Aged <21years (5 patients) 0 (0; 0–0) 0 (0; 0–0)
 Aged 21–40 years (125 patients) 0.37 (0.2; 0–4) 0.42 (0.2; 0–4) 0.06 (0; 0–0.5) 0.039
 Aged 41–60 years (193 patients) 0.31 (0; 0–4) 0.37 (0.2; 0–4) 0.05 (0; 0–0.6) 0.013
 Aged 61–80 years (77 patients) 0.33 (0.2; 0–3) 0.46 (0.2; 0–3) 0.15 (0; 0–0.8) 0.021
 Aged >80 years (14 patients) 0.26 (0.2; 0–4) 0.33 (0.25; 0–0.8) 0.2 (0.2; 0–0.5) 0.36
Proportion with LogMAR acuity≤0.5 74/413 (18%) 68/321 (21%) 6/92 (7%) 0.001
Ishihara test—proportion of plates
 >75% (≥13/16 plates correct) 251/331 (76%) 176/248 (71%) 75/83 (90%)
 26%–75% (5–12/16 plates correct) 33/331 (10%) 26/248 (10%) 7/83 (8%) <0.001
 <26% (≤4/16 plates correct) 47/331 (14%) 46/248 (19%) 1/83 (1%)
 (Not recorded or color-blind) 83/414 (20%) 74/322 (23%) 9/92 (10%) 0.005
Ishihara test—speed of reading
 Fast or fairly fast 184/260 (69%) 120/190 (63%) 64/70 (87%)
 Fairly slow or slow 36/260 (14%) 30/190 (16%) 6/70 (9%) <0.001
 None read 43/260 (17%) 40/190 (21%) 3/70 (4%)
 (Not recorded or color-blind) 154/414 (37%) 132/322 (41%) 22/92 (24%) <0.001
Premature presbyopia or hyperopic shift 78/338 (23%) 75/248 (30%) 3/90 (3%) <0.001
Relative afferent pupillary defect
 None 285/409 (69%) 199/317 (62%) 86/92 (93%)
 Mild 44/409 (11%) 41/317 (13%) 3/92 (3.5%)
 Moderate 19/409 (5%) 19/317 (6%) <0.0001
 Marked 12/409 (3%) 12/317 (4%)
 Positive, but ungraded 49/409 (12%) 46/317 (14%) 3/92 (3.5%)
 (Not recorded) 5/414 (1%) 5/322 (2%) 0/92 0.59
*For conversion to LogMAR scale: value 2.0 assigned to “counting fingers”, 3.0 to hand movements” and 4.0 to “light perception”.
Not recorded for some patients.
Significance refers to comparison of symptomatic and asymptomatic groups.

In affected orbits, the mean LogMAR for symptomatic OCVMs was 0.39 (median 0.2; range 0–4), this being significantly worse than that for asymptomatic lesions (LogMAR 0.1; p < 0.001), and the symptomatic patients also had worse Ishihara color testing (only 71% “normal” tests vs. 90% with incidentalomas; p < 0.001) (Table 3). An ipsilateral relative afferent pupillary defect was detected in 38% (118/317) of symptomatic OCVMs and 7% (6/92) of incidentalomas (p < 0.001). Patients with symptomatic OCVMs had more relative exophthalmos (mean 3.9 mm vs. 1.5 mm; p < 0.001); the degree of proptosis at presentation was, however, unrelated to age (r = 0.024; p = 0.67) (Fig. 2), to gender (mean male 4.2 mm vs. female 3.7 mm; p = 0.14), or to assumed female menopausal state (females <40 years had mean 3.4 mm, compared with a mean of 4 mm for females >50 years; p = 0.17). Compared with incidentalomas, symptomatic OCVMs also had higher rates of nonaxial displacement (30% vs. 7%; p < 0.00001), more objectively-palpable masses (17% vs. 2%; p = 0.0001), and a different prevalence of impaired ocular motility (29% vs. 10%; p = 0.0024) (Table 4). The incidence of fundal abnormalities was significantly less in asymptomatic patients (26% abnormal) as compared to symptomatic (63%) (p < 0.0001). Where fundal abnormality was present, however, the proportions with disc swelling, disc pallor or choroidal folds were similar in the 2 groups, although other anomalies (such as age-related macular changes) were more common in the asymptomatic group (Table 4).

TABLE 4. - Presenting ophthalmic signs for 414 patients with orbital cavernous venous malformations
Clinical characteristic All patients (414 patients) Symptomatic malformations (322 patients) Asymptomatic malformations (92 patients) Significance
Mean exophthalmos for affected eye (median; range) 19.7 mm (19.5; 12–32) 20 mm (20; 12–32) 18.6 mm (18; 12–29) 0.001
Relative proptosis (mm)
 Mean (median; range) 3.3 (3; -2 to 15) 3.9 (4; -2 to 15) 1.5 (1; -1 to 6) <0.001
 Men (156 patients) 3.7 (3; -1 to 15) 4.2 (4; 0 to 15) 1.5 (1; -1 to 6) <0.001
 Women (258 patients) 3.1 (3; -2 to 12) 3.7 (4; -2 to 12) 1.4 (1; -1 to 5) <0.001
 Women >50 years (117 patients) 3.0 (3; -1 to 10) 4.0 (4; 0 to 10) 1.1 (1; -1 to 5) <0.001
 Proportion relative proptosis ≥ 1mm 338/414 (82%) 274/322 (85%) 64/92 (70%) 0.0012
Non-axial globe displacement 101/414 (24%) 95/322 (30%) 6/92 (7%) <0.00001
Palpable orbital mass 57/414 (14%) 55/322 (17%) 2/92 (2%) 0.0001
Reduction in ocular motility
 None 294/388 (76%) 212/297 (71%) 82/91 (90%)
 Mild 63/388 (16%) 55/297 (19%) 8/91 (9%)
 Moderate 28/388 (7%) 27/297 (9%) 1/91 (1%) 0.0024
 Severe 3/388 (0.8%) 3/297 (1%) 0/91
 (Uncertain) 26/414 (6%) 25/322 (8%) 1/92 (1%) 0.015
Fundus examination
 Abnormal fundus examination 224/407 (55%) 201/317 (63%) 23/90 (26%) <0.0001
  Disc swelling 92/224 (41%) 82/201 (41%) 10/23 (44%) 0.83
  Choroidal folds 83/224 (37%) 77/201 (38%) 6/23 (26%) 0.36
  Disc pallor 62/224 (28%) 58/201 (29%) 4/23 (17%) 0.33
  Other anomalies 22/224 (10%) 16/201 (8%) 6/23 (26%) 0.015
  (Uncertain) 7/414 (2%) 5/322 (2%) 2/92 (2%) 0.65
Significance refers to comparison of symptomatic and asymptomatic groups.

F2
FIG 2.:
Degree of relative exophthalmos related to the age at presentation for 414 patients with symptomatic or asymptomatic (presumed) orbital cavernous venous malformations (OCVMs). “r” refers to the linear correlation coefficient for each group (non-significant in each case).

DISCUSSION

This large series compares the presentation of patients with symptomatic OCVMs and presumed asymptomatic lesions (incidentalomas9): as with other series, there was a female predilection (about two-thirds), and most patients presented in the 4th to 6th decades, with a peak incidence in the 5th. 5,7,8,11 As most OCVMs are intraconal, the commonest symptom is, unsurprisingly, a slowly-progressive axial proptosis reported in over 70%.11 Multiple OCVMs lesions have been reported in several series,4,12 but only 3 patients from a historic Moorfields series4 were identifiable in this retrospective review. Paradoxically, there have been no cases of bilateral or multiple OCVMs identified since 1990, which possibly reflects, in recent decades, the earlier presentation of patients and the markedly increased use of imaging for other conditions.

Although the size of some OCVMs appears stationary, they can show a wide variation in growth pattern;4–11 one reported estimate for the growth rate of progressive lesions is ~0.2 ml per year.10 Most OCVMs remain stable or decrease in size in postmenopausal women (with reduced serum estrogen or progesterone)13 and this, together with the female predominance, suggests that hormonal factors may influence the growth of OCVMs. About a half of our symptomatic patients had noted exophthalmos (Table 2) and 85% had measurably asymmetric exophthalmometry (Table 4); 70% of asymptomatic patients also had mild (mean 1.5 mm) relative proptosis, although significantly less than that in symptomatic patients (3.9 mm; p < 0.001) (Table 4). In the symptomatic group there was no correlation between age at presentation and degree of proptosis, nor any difference between assumed premenopausal (mean 3.4 mm) or postmenopausal women (4 mm; p = 0.17).

Visual impairment is reported in half of patients with OCVMs due to globe indentation or compressive optic neuropathy.11 In the current study, relative afferent pupillary defects, impaired color perception or fields, choroidal folds, disc swelling, or optic atrophy were present in both symptomatic and asymptomatic patients (Table 4). With symptomatic OCVMs we found ipsilateral premature presbyopia or hyperopic shift in about one-third, signs of compressive optic neuropathy in over a third, and 11% who had gaze- or postural-evoked amaurosis; this transient amaurosis is an uncommon symptom, occurs with intraconal or extraconal lesions, and is probably due to optic nerve ischemia.14 None of the asymptomatic patients gave a retrospective history of transient visual obscuration.

Presumed asymptomatic OCVMs (“incidentalomas”) accounted for 22% of our patients: compared to symptomatic patients, the initial presentation was about a decade later (average age 57.8 vs. 47 years; p < 0.001). While this age difference is likely to reflect a higher incidence of imaging for nonophthalmic symptoms (such as stroke, hearing loss, or vertigo) in the elderly, it might also be the case that the long-standing facial appearance remains unchanged where an OCVM is nonprogressive, and such lesions will therefore tend to remain “silent” unless discovered on imaging. Our 92 patients with incidentalomas had significantly better visual functions, less globe displacement (axial or nonaxial), and less motility impairment (Tables 3 and 4). Other studies report asymptomatic OCVMs to have similar presenting ages to those in our series (51 years5; 54 years6), almost all having normal visual functions (30/315 and 11/116), there being none with disc swelling, choroidal folds, or globe indentation,5,6 and only 2/11 having proptosis.6 In contrast to these reports, a quarter of our patients with incidentalomas had recorded fundal abnormalities and, of these patients, ~60% had disc swelling or pallor and a quarter had choroidal folds (Table 4).

In summary, patients with symptomatic OCVMs may present with various degrees of reduced visual function, either due to compression of the posterior globe (with induced hyperopia) or the optic nerve. Incidental (asymptomatic) lesions, accounting for more than one-fifth of all CVMs, are usually detected a decade later than symptomatic CVMs, and a minority have impaired ophthalmic functions.

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