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Severe macular pucker after retinal detachment surgery in an infant
  1. S Nishina,
  2. N Azuma
  1. Department of Ophthalmology, National Children's Hospital, Tokyo, Japan
  1. Correspondence to: Noriyuki Azuma, MD, Department of Ophthalmology, National Children's Hospital, 3-35-31 Taishido, Setagaya-ku, Tokyo, 154–8509, Japan; nazuma{at}

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The appearance and clinical course of rhegmatogenous retinal detachments in children are more complicated than those of adults, because the retinal detachments are generally associated with trauma or congenital anomalies.1, 2 In an infant, especially, an unexpected course may develop; however, the incidence of these detachments is so low that treatment and complications have scarcely been described in the literature. We report an infant who developed severe macular pucker after retinal detachment surgery.

Case report

A 2 week old male infant was referred with a diagnosis of bilateral congenital cataracts. The pregnancy and delivery had been uncomplicated. His mother also had had congenital cataracts. Both eyes of the infant had dense zonular cataracts. The patient had no associated ocular problems on slit lamp biomicroscopy or gonioscopy and no abnormalities on physical examination. He underwent bilateral lensectomy and anterior vitrectomy by limbal approach at 3 weeks of age; both fundi were unremarkable. Although his right eye underwent sufficient anterior vitrectomy, retropupillary membrane developed on the residual vitreous surface and the pupil closed 3 months postoperatively. During the second surgery, pupilloplasty was performed and we found a tear in the ciliary epithelium of the pars plana resulting from traction of the retropupillary membrane tissue, and also discovered a localised detachment of the retina and ciliary epithelium (Fig 1A). We performed cryopexy with encircling scleral buckle, vitrectomy, and fluid-air exchange, and the retina was reattached. One month later, macular pucker rapidly developed and enfolded the entire posterior retina (Fig 1B). The original tear was completely sealed, and signs of the recurrent rhegmatogenous retinal detachment with proliferative vitreoretinopathy, including vitreous haze and wrinkling of retina in the other location, were not identified. After additional vitrectomy to remove residual cortex and to perform membrane peeling, the retina was finally reattached with a residue of degeneration near the fovea (Fig 1C).

Figure 1

(A) Schema of retinal detachment in the right eye. Ciliary epithelial tear is observed between the 7 and 8 o'clock positions. (B) Macular pucker enfolds the entire posterior retina and covers the optic disc. (C) Postoperative fundus with residual retinal degeneration.

Haematoxylin and eosin and Azan stains and transmission electron microscopy of the surgically removed membrane showed pigmented cells, fibroblast-like cells, vessels, and collagen fibres (Fig 2A). The membrane was immunostained with antibodies for cytokeratin and α-smooth muscle actin (Fig 2B, C), while there was slight immunoreactivity for vimentin and no immunoreactivity for glial fibrillary acidic protein or desmin. The membrane also contained vessels, which was confirmed by positive immunoreactivity for Von Willebrand factor (Fig 2D).

Figure 2

(A) Light micrograph shows pigmented cells, fibroblast-like cells, and collagen (Azan, original magnification ×100). Positive immunostaining for cytokeratin (B) and α-smooth muscle actin (C) is seen prominently in the membrane. (D) Positive staining for Von Willebrand factor shows the vessels (peroxidase-antiperoxidase, original magnification ×100).


Macular pucker in this infant is significantly different from that in adults. The severe posterior retinal folds may be formed by strong contraction of the epiretinal membrane, a firm vitreoretinal juncture, and an extensible retina. A previous histopathological report on recurrent proliferation after vitrectomy in two children with tractional retinal detachments showed that retinal glial cells were the main reactive cells.3 However, in our case, the major component of the pucker was retinal pigment epithelium (RPE) cells, the same as in adults with rhegmatogenous retinal detachment.4, 5 Cytoplasmic actin within the membrane, rather residual vitreous cortex on the retinal surface, may be involved in forming strong contractile elements, which also may underlie idiopathic cases in children.6 Vessels in the pucker may have originated from the adherent retina, because no residual hyaloid vessels were identified during surgery.

Because excessive cryopexy tends to disperse and activate RPE cells, minimal cryopexy is recommended; laser may be preferable to treat rhegmatogenous retinal detachment in infants. Enzyme assisted vitrectomy to separate the firm vitreoretinal juncture may prevent the development of subsequent macular pucker. Frequent examinations and careful management are required even after successful reattachment surgery.