Retinal detachment with a break at pars plicata associated with congenital malformation of the lens-zonule-ciliary body complex

Abstract

Retinal detachment with a break at the pars plicata associated with congenital malformation of lens-zonule-ciliary body complex is rare; most reports are of young Japanese male patients with atopic dermatitis. The present case report is the first to describe the condition in a Chinese patient with no atopic dermatitis or trauma history. A 22-year-old male presented with blurred vision in the left eye for 4 months. Fundus examination revealed shallow lower temporal retinal detachment. Further examination with scleral indentation under maximal pupil dilatation identified a break at the far periphery beyond the ora serrata and pars plana. Gonioscopy revealed a pars plicata break at the nonpigmented ciliary epithelium associated with congenital ciliary process hypoplasia and subtle lens defect at the same meridian. The retina was successfully reattached after segmental scleral buckling, cryopexy, and laser photocoagulation.

Keywords: malformation of the lens—zonule—ciliary body complex; pars plicata break; retinal detachment; scleral buckle.

Fig. 1

Shallow retinal detachment at the temporal lower quadrant from the 2 to 5 o’clock meridian (arrowheads) involving macula with no visible retinal break in the corresponding area.

Fig. 2

Optical coherence tomography of (A) the detached neurosensory retina at presentation and (B) the attached macula postoperatively.

Fig. 3

Ten days after performing the scleral buckling and cryopexy, the retina is well attached. A break (arrow) anterior to ora serrata is found at the temporal lower quadrant.

Fig. 4

Gonioscopy reveals a break at the temporal lower nonpigmented epithelium of pars plicata (arrow) with its edge pulled to the lens.

Fig. 5

Diffuse light with retroillumination during maximal pupil dilatation shows a subtle lens defect with segmental flattening adjacent to the pars plicata break.

Fig. 6

(A) Additional dense laser photocoagulation is applied directly onto the scleral buckle edge to confine the subretinal fluid after accidental head trauma. (B) The break sealed well with laser scar on sclera buckle after 3 years of follow-up.