PARACENTRAL ACUTE MIDDLE MACULOPATHY WITH SICKLE CELL TRAIT

Abstract

Purpose: To report a case of paracentral acute middle maculopathy in a pediatric patient with sickle cell trait.

Methods: The patient was evaluated with a comprehensive ophthalmic examination, optical coherence tomography, optical coherence tomography angiography, and fluorescein angiography.

Results: Acute loss of vision occurred immediately after an uneventful scleral buckling procedure for retinal detachment in a 16-year-old female patient. Retinal imaging studies confirmed the occurrence of paracentral acute middle maculopathy, an ischemic condition characterized by infarction of the inner nuclear layer of the retina caused by hypoperfusion of the intermediate and deep capillary plexuses. Laboratory evaluation was only remarkable for sickle cell trait. Over a course of 3 months after the loss of vision, visual acuity improved from 20/1,000 to 20/20, and optical coherence tomography lesion resolved to a parafoveal area of inner and middle retinal layer thinning. Paracentral scotoma corresponding to the atrophic area persisted.

Conclusion: Dehydration because of the presurgical nil-per-os status and transient increase in intraocular pressure during the buckling surgery may have predisposed this patient with sickle cell trait to a sickling event that caused localized ischemia in the middle retina's end-capillaries. Avoiding long nil-per-os status, being mindful of transient intraocular pressure elevation during scleral buckling procedures, and presurgical hemoglobin electrophoresis in at-risk populations are recommended to prevent sickling attacks during retinal surgeries in individuals with sickle cell trait.

Fig. 1.

Wide-field pseudocolor fundus photographs of the left eye. A. Inferotemporal rhegmatogenous retinal detachment (arrowheads) with atrophic retinal holes within lattice degeneration area at presentation. The patient had extensive lattice degeneration with atrophic holes in all four quadrants of the peripheral retina (not shown in the photograph). B. Postoperation day 1 with no subretinal fluid, moderate to high encircling scleral buckle with a segmental tire, and cryotherapy effect. C. Buckle impression and cryotherapy at 14-month follow-up.

Fig. 2.

Optical coherence tomography scan through the foveal center of the left eye 1 day (A), 1 week (B), 6 weeks (C), 14 weeks (D), and 14 months (E) after scleral buckling procedure. Inner and outer plexiform and nuclear layers were hyperreflective (arrowheads), characteristic of paracentral acute middle maculopathy, at Day 1 and Week 1 after the surgery. Thinning of the inner and middle retina layers corresponding to the previous areas of hyperreflectivity are seen at the subsequent follow-up visits (C–E). F. Paracentral scotoma as documented by 10-2 Humphrey visual field test at 14-month follow-up.