Spontaneous closure of macular holes secondary to posterior uveitis: case series and a literature review
© Bonnin et al.; licensee Springer. 2013
Received: 31 August 2012
Accepted: 25 January 2013
Published: 11 February 2013
The occurrence of a macular hole due to posterior uveitis is infrequently reported. We report the evolution of three cases of macular holes secondary to posterior segment inflammation. A complete inflammatory and infectious assessment found one case of toxocariasis, one of sarcoidosis, and one of syphilis. After medical etiological treatment, macular hole closure was rapidly obtained in all the cases and confirmed by spectral domain optic coherence tomography, with visual acuity improvement. Fibrous scarring developed in two cases, and foveal photoreceptor complex normalization was observed in the sarcoidosis case. These observations demonstrate that macular holes secondary to posterior uveitis frequently resolve without surgical intervention and so could be underdiagnosed if the patient is not evaluated at the time of onset before spontaneous hole closure.
Ocular inflammatory diseases can cause sight loss by macular impairment. Cystoid macular edema is the principal sight-threatening complication  in uveitis patients. Among possible macular inflammatory alterations, Nussenblatt describes macular hole occurrence as a very rare complication , being more often idiopathic or senile, postoperative or due to an epiretinal membrane, but rarely to posterior segment inflammation.
Here we report three cases of macular hole secondary to posterior uveitis that spontaneously resolved after etiological treatment, and present a review of relevant literature. Written informed consent was obtained from all patients for publication of this report.
Case no. 1
Case no. 2
Case no. 3
Intraocular inflammatory diseases have long been described as a major cause of blindness . In all kinds of uveitis, bilateral legal blindness develops in 4% of patients, unilateral blindness in 14%, bilateral visual impairment in 6%, unilateral impairment in 11%, and unilateral impairment with blindness of the other eye in 4% to 5%.
The occurrence of a macular hole in a context of uveitis is an unusual, little-described complication.
In 1986, Nussenblatt  studying macular alterations in uveitic patients described this evolution as ‘uncommon’. Later, in a retrospective study of 582 patients with uveitis , no macular hole was described. Neither prevalence nor incidence of macular holes linked to posterior segment inflammation can be established from the literature, cases or short series being too scant.
On the other hand, in a histopathological study of 17 lamellar and 18 full-thickness macular holes , mild to moderate choroiditis was present in six eyes in the macular area. One of these cases was suspected of being an incidental finding because the choriocapillaris, Bruch's membrane, and the retinal pigment epithelium (RPE) were intact. This study gives us an approximate figure for the proportion of macular holes linked to posterior uveitis in all macular holes. However, there is a selection bias: the eyes of this study were obtained from enucleations or post-mortem, so the results do not reflect the general epidemiology of the disease.
Review of the literature of macular holes in a context of intraocular inflammation
Number of cases
Idiopathic intermediate uveitis
Idiopathic posterior uveitis
Bilateral idiopathic anterior uveitis of unknown etiology
Immune recovery uveitis in AIDS patients with CMV retinitis
Idiopathic macular holes can be explained by a triple constraint : (1) an antero-posterior traction, often effected by the posterior vitreous detachment; (2) a tangential traction especially when there is an epiretinal membrane or a lateral shifting of vitreoretinal adherence; and (3) an intraretinal constraint that weakens the inner retinal surface by inducing cystic changes and contributes to the macular hole (Figure 7A). A parallel can be done in circumstances of posterior segment inflammation: the inflammation often induces changes or liquefaction of the vitreous body and contributes to the posterior vitreous detachment which realizes the first constraint. The development of an epiretinal membrane is classical in uveitis , responsible of a tangential traction, as seen in the cases 1 and 2. Finally, chorioretinitis foci or cystoid macular edema cause the third constraint, fragilizing the retinal tissue (Figure 7B). This explains why treating the inflammation can relieve these constraints or tractions and produce spontaneous healing of the macular hole. The report of Halkiadakis et al.  on a spontaneous macular hole closure after peribulbar steroid injection in the case of a HLA B27 uveitis demonstrates this well.
The relatively old references on the low prevalence of macular hole in uveitic patients could be explained by the fact that OCT is a recent tool. Our cases also show the importance of the SD-OCT in uveitis to confirm cystoid macular edema, the principal sight-threatening effect in uveitis . OCT can also be a diagnostic criterion in some etiologies such as intermediate uveitis or a poor-prognosis marker as in Birdshot retinochoroidopathy, indicating a specific care requirement. OCT is necessary during follow-up after resolution of active inflammation to seek the cause of any unexplained sight loss and may reveal a closed macular hole filled by fibrosis.
SD-OCT is useful for the preoperative evaluation of the inflammatory secondary epiretinal membrane to evaluate the benefits of the procedure: in our first case, in which an epiretinal membrane developed, there was a partial posterior vitreous detachment, and so there is a persistent risk of macular hole recurrence in a such damaged and weakened retina, with very small hope of visual improvement.
The occurrence of a macular hole linked to posterior uveitis is an uncommon but possibly underestimated cause of sight loss. Etiological treatment seems to lead to macular hole closure in most such cases. SD-OCT is very useful for assessing the evolution of these cases and should be performed whenever there is a posterior involvement or unexplained sight loss in uveitis patients.
This paper was presented at the SFO (Société Française d'Ophtalmologie) 2011 Congress. We are grateful to ATT Medical Society for proofreading the manuscript.
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