Fundus photographs showing extensive retinal hemorrhages over the posterior poles of both eyes. A, The right eye has a dome-shaped cavity centered over the macula. The cavity is filled with fluid blood and surrounded by a raised ridge outlined by a shiny retinal reflex resembling a perimacular fold (arrow). B, The left eye has superficial flame-shaped hemorrhages and a premacular hemorrhage. No perimacular fold is present.
Fundus photographs showing persistent premacular blood 6 weeks later (A) and showing the eye after Nd:YAG laser membranotomy to drain blood into the vitreous cavity (B). Note that the appearance of the perimacular fold (arrows) remained unchanged.
Bhatnagar A, Wilkinson LB, Tyagi AK, Willshaw HE. Subinternal Limiting Membrane Hemorrhage With Perimacular Fold in Leukemia. Arch Ophthalmol. 2009;127(11):1547-1553. doi:10.1001/archophthalmol.2009.284
We report a case that demonstrates the appearance of perimacular folds associated with extensive retinal hemorrhages occurring in the absence of trauma in a patient with acute myeloid leukemia. We discuss the clinical features, relevant literature, and suggested mechanisms for perimacular folds in such a situation.
A 14-year-old boy with acute myeloid leukemia visited the eye department with blurred vision of recent onset. Visual acuity was 6/48 OD and 6/18 OS. Examination revealed bilateral extensive retinal hemorrhages over the posterior pole involving all retinal layers (Figure 1). The patient's right eye had a dome-shaped cavity centered over the macula. This cavity was filled with fluid blood and surrounded by a raised ridge outlined by a shiny retinal reflex resembling a perimacular fold (Figure 1A). The patient had no history of head injury and no signs of intracranial hemorrhage. Hematological investigations showed a markedly reduced platelet count (18 × 103/μL; to convert to ×109 per liter, multiply by 1.0) and hemoglobin level (6.7 g/dL; to convert to grams per liter, multiply by 10.0); the prothrombin time was 13 seconds (reference range for this age group, 9-13 seconds) and the activated partial thromboplastin time was 32 seconds (reference range, 26-35 seconds).
Six weeks later, the premacular hemorrhage in the right eye persisted (Figure 2A). Nd:YAG laser membranotomy drained the blood into the vitreous cavity and his visual acuity improved to 6/6 (Figure 2B).
Traumatic retinoschisis with perimacular fold has been described following abusive head injury, particularly when characterized by the repetitive acceleration and deceleration forces seen in shaken baby syndrome.1,2 A similar ophthalmoscopic appearance was reported twice in fatal crush head injury of childhood3,4 and in adult Terson syndrome.5 In shaken baby syndrome, violent shaking is believed to generate shearing forces at the vitreoretinal interface, resulting in traction on the retina that causes traumatic retinoschisis and creates perimacular retinal folds.1 Retinoschisis may involve only separation of the internal limiting membrane (ILM) or a more sight-threatening split in deeper retinal layers. Elevated retinal blood vessels over the cavity aid in differentiation.
We believe the reduced platelet count in our patient caused extravasation of blood from retinal vessels, resulting in rapid accumulation of blood in the sub-ILM space in the right eye. This raised the ILM from the inner retina and created a perimacular fold at the edge where the ILM remained attached.
Retinal hemorrhages at any level are often seen in leukemia with thrombocytopenia.6 It is unlikely that the appearance of a perimacular fold is unique to this setting. It may be seen in eyes with rapid accumulation of large premacular sub-ILM hemorrhage due to any cause and may be more common than reported in the literature. Published photographs of a large premacular hemorrhage seen in a patient with lymphoma7 and in Valsalva retinopathy8 show what appears to be a perimacular retinal fold. The fact that this has not been commented on in these reports perhaps reflects the lack of familiarity with this sign among ophthalmologists not used to seeing patients with shaken baby syndrome.
This case highlights the facts that perimacular folds may be seen with large sub-ILM hemorrhage in a nonabusive setting and that hematological diseases should be excluded in all cases. However, the term retinoschisis resulting in perimacular fold may be reserved for traumatic settings where shearing forces at the vitreoretinal interface cause perimacular folds.
Correspondence: Ms Wilkinson, Eye Department, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, West Midlands B4 6NH, England (firstname.lastname@example.org).
Financial Disclosure: None reported.