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Purtscher-Like Retinopathy Secondary to an Appendiceal Neuroendocrine Neoplasm Complicated by a Periappendiceal
Bartosz Skulimowski1, Slawomir Liberski1, Danuta Nikratowicz1
1Ophthalmology, Poznan University of Medical Sciences, Poznań, POL.
Abstract:
Purtscher-like retinopathy (PLR) is a rare retinal microvasculopathy that is induced by non-traumatic systemic illnesses. We describe the case of a woman in her 70s who was diagnosed with PLR, ultimately linked to an appendiceal neuroendocrine neoplasm (ANEN) complicated by a periappendiceal abscess. The patient presented with sudden, unilateral, painless visual impairment. Initial examination revealed Purtscher flecken (PF) and cotton-wool spots (CWS) in the right eye, with corresponding hyperreflectivity and swelling of the inner retinal layers on optical coherence tomography (OCT). Systemic evaluation revealed elevated D-dimer levels: 547 ng/mL fibrinogen equivalent units (FEU) (normal <500 ng/mL FEU), and subsequent imaging identified a periappendiceal infiltrate. Laparoscopic appendectomy confirmed the presence of a well-differentiated grade 3 ANEN. Postoperative follow-up demonstrated regression of retinal findings and partial visual improvement (of BCVA from 1.4 logMAR to 0.5 logMAR in the affected eye). However, she presented a scotoma in the visual field of the right eye. The follow-up OCT showed localized inner retinal atrophy corresponding to prior ischemic changes. To our knowledge, this is the first reported case linking PLR to ANEN or appendicitis. This case highlights the importance of systemic evaluation in patients with PLR, particularly when typical aetiologies are excluded.
Insights
Purtscher-like retinopathy (PLR), a rare vision impairment, was linked to an appendiceal neuroendocrine neoplasm (ANEN) with an abscess. This case highlights the need for systemic evaluation in PLR diagnosis.
Area of Science:
- Ophthalmology
- Oncology
- Gastroenterology
Background:
- Purtscher-like retinopathy (PLR) is a rare microvasculopathy of the retina, typically associated with systemic conditions.
- The etiology of PLR often involves non-traumatic systemic illnesses, but atypical causes require investigation.
Observation:
- A woman in her 70s presented with sudden, unilateral, painless visual impairment.
- Ophthalmic examination revealed Purtscher flecken and cotton-wool spots, with retinal swelling on OCT.
- Elevated D-dimer and imaging identified a periappendiceal infiltrate, leading to the diagnosis of appendiceal neuroendocrine neoplasm (ANEN) with an abscess.
Findings:
- Laparoscopic appendectomy confirmed a well-differentiated grade 3 ANEN.
- Postoperatively, retinal findings regressed, and visual acuity improved, though a visual field scotoma persisted.
- Follow-up OCT showed localized inner retinal atrophy due to prior ischemic changes.
Implications:
- This is the first reported case associating PLR with ANEN or appendicitis.
- The case underscores the importance of comprehensive systemic evaluation for PLR, especially when common causes are ruled out.
- Early diagnosis and management of underlying systemic conditions are crucial for visual outcomes in PLR.
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