February 2009




The coincidence of macular telangiectasia with macular holes

by Rich Daly EyeWorld Contributing Editor



Case series supports new imaging tool to confirm type 2 idiopathic macular telangiectasia cases

Example of a macular hole Source: William Trattler, M.D.

Ophthalmologists may underestimate the coincidence of macular telangiectasia with macular holes because only subtle ophthalmoscopic changes occur in early stages of macular telangiectasia, according to recent research.

Type 2 idiopathic macular telangiectasia (type 2 IMT) has been found by optical coherence tomography (OCT) to include a disruption within the foveal photoreceptor layer and intraretinal hyporeflective spaces. Despite this, full-thickness macular holes (FTMHs) and lamellar macular holes (LMHs) complicating type 2 IMT have been reported in only few patients.

To address this apparent disconnect, the authors of a report published online in the February 2008 issue of the journal Eye coordinated a case series from among multiple international sites to examine possible co-occurrence of the conditions. The report on the case series, led by Peter Charbel Issa, M.D., resident, department of ophthalmology, University of Bonn, Bonn, Germany, concluded that the altered foveal anatomy with progressive atrophic changes within the neurosensory retina in type 2 IMT may predispose patients to develop FTMH and LMH.

“In addition to the atrophic neurosensory changes in type 2 IMT, there could be mechanical stress on the fovea, resulting in disruption of the inner drape-like structure,” Dr. Issa and the others wrote. “FTMH or LMH would be a possible consequence.”

The case series of six patients with either FTMH or LMH and type 2 IMT were evaluated through OCT imaging, funduscopy, and fluorescein angiography. All of the eyes showed macular abnormalities typical for nonproliferative type 2 IMT except for one eye with a proliferative disease stage. Three patients had an FTMH, one presenting with bilateral FTMH, and three had an LMH on OCT. In all cases of FTMH, the macular holes did not have elevated margins. Surgery was performed in two patients with a FTMH without subsequent functional improvement. The results led the authors to conclude that physicians may underestimate the coincidence of macular telangiectasia with macular holes because only subtle ophthalmoscopic changes are present in early stages of macular telangiectasia.

“The Type 2 IMT should be considered in the differential diagnosis in patients presenting with macular holes,” wrote the authors.

They also noted that the association between the two conditions may reflect alternative pathogenetic mechanisms in the development of macular holes. This subgroup of macular holes could also be partially responsible for macular holes that developed without prior macular edema, vitreomacular, or tangential traction, they reported.

The authors noted that because fluorescein angiography is not performed as part of the diagnostic evaluation for macular holes, type 2 IMT may be missed as a causative factor. The authors suggested confocal blue-reflectance imaging “as a non-invasive technique to diagnose type 2 IMT.”

“Since this technique also documents the size of a macular hole, it is suggested to be included as a diagnostic tool, especially if the aetiology of the macular hole is questionable (i.e. no posterior vitreous detachment or epiretinal gliosis) or if there are signs for type 2 IMT in the opposite eye (as perifoveal greying of the retina or atrophic cystic changes in OCT imaging),” the authors wrote.

Another study author, Hendrik P.N. Scholl, M.D., consultant ophthalmologist, department of ophthalmology, University of Bonn, told EyeWorld that the awareness of type 2 IMT as a possible underlying pathogenetic trigger for macular holes should alert clinicians to look for this disease in patients with macular holes. And the conclusions could have impacts beyond patient diagnosis.

“Since the response to treatment and the functional outcome may differ from other macular hole entities, surgeons might be more reluctant to operate on these eyes,” he said.

Further understanding of the pathogenesis of macular holes in type 2 IMT will come from sequential imaging over time in more patients with type 2 IMT, said the study authors. They urged both sequential imaging over time as well as further investigation of the spontaneous course of LMHs in future natural history studies.

Dr. Scholl said he plans to investigate the pathophysiology of macular holes in patients with type 2 IMT compared to idiopathic macular holes using high resolution imaging devices such as the combined confocal scanning laser ophthalmoscope (cSLO) and spectral domain OCT.

Editors’ note: Dr. Scholl has no financial interests related to his comments.

Contact information

Scholl: +49-228-287-19075, Hendrik.Scholl@ukb.uni-bonn.de

The coincidence of macular telangiectasia with macular holes The coincidence of macular telangiectasia with macular holes
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