Sir,

Paediatric macular holes are rare and typically are secondary to trauma. We report a case where a child developed an idiopathic, macular hole.

Case report

An 8-year-old girl presented with blurred vision in her left eye. She had a history of amblyopia treatment at the age of 6 (resulting acuity right 6/6, left 6/9). Her birth, medical, drug, and family history were unremarkable. There was no history of trauma.

Acuity was right 6/6 and left 6/12. Ocular examination was normal except for the appearance of the left fundus consistent with a macular hole (Figure 1). There was no evidence of posterior vitreous detachment (PVD), uveitis, trauma, fibrous membrane, macular oedema, or optic nerve pit. Optical coherence tomography (OCT) showed a hole in the outer retinal layers, but with an intact inner retinal layer (no oedema or detachment) (Figure 2). By Gass definition this is a stage I macular hole (or impending macular hole).

Figure 1
figure 1

Left eye macular hole in a child, with no history of trauma and no other ocular pathology.

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Figure 2
figure 2

OCT of the left fovea showing a hole of outer retinal layers, but intact inner retinal layer (Stage I macular hole, when defined according to Gass).

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The OCT does not reveal any tangential traction, suggesting that the responsible traction could be anterio-posterior. It was hoped that relief of such traction would occur spontaneously with a PVD as is often the case in adults. With further observation, vision deteriorated to 6/24. Surgery was considered, as acuity was worsening, amblyopia was probably developing, and spontaneous regression via PVD in a child was theoretically less likely than that which often occurs in adults. Her macular hole was treated by pars plana vitrectomy, internal limiting membrane peel, and 10% gas (C3F8) followed by 2 weeks of posturing face down.

Vision improved to acuity 6/12 six months following vitrectomy. Fundoscopy showed mild retinal pigment epithelial change and closure of the macular hole (confirmed by OCT—Figure 3). Three years later, vision remains good.

Figure 3
figure 3

Six months following surgery, OCT of the left eye shows return towards normality.

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Comment

Macular holes in children are rare. All the documented cases report trauma as the underlying cause, except for a case by Nakano et al,1 where the macular hole was associated with a shallow detachment attached to a peri-papillary membrane (potentially a Bergmeister papilla remnant).

This is the first published case of a paediatric, macular hole that appears truly idiopathic, given the absence of trauma and other ocular anomalies.