Child Kidney Dis.  2025 Feb;29(1):32-38. 10.3339/ckd.25.003.

Incidentally detected bilateral sub-internal limiting membrane hemorrhages in typical hemolytic uremic syndrome: a case report

• Kwon HJ
• Song JY

• KMID: 2565479
• DOI: http://doi.org/10.3339/ckd.25.003

Abstract

We present an incidentally detected case of bilateral sub-internal limiting membrane (ILM) hemorrhage secondary to typical hemolytic uremic syndrome (HUS) along with a literature review. A 4-year-old girl presented to our emergency department with bloody diarrhea, abdominal pain, fever, and oliguria. HUS was suspected based on clinical symptoms and laboratory findings suggestive of hemolytic anemia, thrombocytopenia, and acute kidney injury. Supportive management, including intermittent dialysis, was provided during admission. However, the patient exhibited central nervous system involvement and presented with a seizure. Despite the absence of visual symptoms, a fundus examination revealed unexpected bilateral sub-ILM hemorrhages and a cotton wool spot in the right eye. Blood dyscrasia improved with repeated hemodialysis and red blood cell transfusions. The sub-ILM hemorrhage gradually resolved, as observed on consecutive fundus photographs. Multimodal retinal imaging revealed healthy capillary structures without evidence of macular ischemia or fibrosis. Typical HUS can be accompanied by a sub-ILM hemorrhage without preceding symptoms. The absence of visual symptoms, despite significant retinal abnormalities, emphasizes the importance of immediate and routine fundus examinations in patients with typical HUS. This proactive approach improves early detection of ocular involvement, enabling prompt intervention to manage potential visual complications, and ultimately improving patient outcomes.

Keyword

Typical hemolytic uremic syndrome; Case reports; Pediatrics; Sub-internal limiting membrane hemorrhage

Figure

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  Fig. 1. Bilateral sub-internal limiting membrane (ILM) hemorrhages in typical hemolytic uremic syndrome at the initial ophthalmic examination. (A, B) Fundus photography. (C, D) Cross-sectional horizontal and (E, F) vertical optical coherence tomography (OCT) B-scan images at the initial ophthalmic examination. In both eyes, hourglass-patterned preretinal hemorrhages surround the fovea with distinct and horizontal fluid-clot levels. A small cotton wool spot is on the right macula at the superonasal side of the fovea. In the OCT images, these preretinal hemorrhages corresponding to hyper-signal intensity beneath the detached ILM were diagnosed as sub-ILM hemorrhages. The right visual acuity is 20/50, and the left is 20/25.

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  Fig. 2. Progressive resolution of sub-internal limiting membrane (ILM) hemorrhages and multimodal retinal imaging of typical hemolytic uremic syndrome. At 2, 6, and 10 weeks after the initial ophthalmic examination: (A-C) right fundus photographs and (D-F) left fundus photographs. Sub-ILM hemorrhages were gradually absorbed. (G) At week 7, fluorescein angiography reveals absence of nonperfusion or neovascularization in the right eye. The focal hypofluorescence region is a blockade of background fluorescence because of the less-absorbed sub-ILM hemorrhage. At week 10, (H) normal superficial capillary plexus on optical coherence tomography (OCT) angiography and (I, J) normal macular structures on OCT are observed. The patient’s vision improved to 20/16. (K, L) The left eye has no complications, such as nonperfusion, neovascularization, or capillary dropout. (M, N) Focal sub-ILM hemorrhage remnants and mild foveal elevation are observed in the left macula; however, the left visual acuity was restored to 20/16.

Reference

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