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继发于阑尾神经内分泌肿瘤并伴有阑尾周围脓肿的类Purtscher样视网膜病变。

Purtscher-Like Retinopathy Secondary to an Appendiceal Neuroendocrine Neoplasm Complicated by a Periappendiceal Abscess.

作者信息

Skulimowski Bartosz, Liberski Slawomir, Nikratowicz Danuta, Gotz-Wieckowska Anna

机构信息

Ophthalmology, Poznan University of Medical Sciences, Poznań, POL.

出版信息

Cureus. 2025 Jun 11;17(6):e85752. doi: 10.7759/cureus.85752. eCollection 2025 Jun.

DOI:10.7759/cureus.85752
PMID:40656371
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12247013/
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.

摘要

类Purtscher视网膜病变(PLR)是一种由非创伤性全身性疾病引起的罕见视网膜微血管病变。我们描述了一名70多岁女性的病例,她被诊断为PLR,最终发现与阑尾神经内分泌肿瘤(ANEN)合并阑尾周围脓肿有关。患者出现突发、单侧、无痛性视力障碍。初步检查发现右眼有Purtscher斑(PF)和棉絮斑(CWS),光学相干断层扫描(OCT)显示视网膜内层相应的高反射性和肿胀。全身评估显示D-二聚体水平升高:547 ng/mL纤维蛋白原当量单位(FEU)(正常<500 ng/mL FEU),随后的影像学检查发现阑尾周围有浸润。腹腔镜阑尾切除术证实存在高分化3级ANEN。术后随访显示视网膜病变消退,视力部分改善(患眼最佳矫正视力从1.4 logMAR提高到0.5 logMAR)。然而,她右眼视野出现了暗点。随访OCT显示与先前缺血性改变相对应的局限性视网膜内层萎缩。据我们所知,这是首例将PLR与ANEN或阑尾炎相关联的报道病例。该病例强调了对PLR患者进行全身评估的重要性,尤其是在排除典型病因时。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/abfa60c43b01/cureus-0017-00000085752-i13.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/7133bf54ef8a/cureus-0017-00000085752-i08.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/abfa60c43b01/cureus-0017-00000085752-i13.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/df5cce7c586a/cureus-0017-00000085752-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/aa7724710cfc/cureus-0017-00000085752-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/d3b191941f28/cureus-0017-00000085752-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/3c3009288f05/cureus-0017-00000085752-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/3e1aaa1eaaa8/cureus-0017-00000085752-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/f5768c4f942f/cureus-0017-00000085752-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/724f47b27d0b/cureus-0017-00000085752-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/7133bf54ef8a/cureus-0017-00000085752-i08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/7034105509fe/cureus-0017-00000085752-i09.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/50f848f98162/cureus-0017-00000085752-i10.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/c7afeb5c8d44/cureus-0017-00000085752-i11.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/58362b6c8b6f/cureus-0017-00000085752-i12.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad43/12247013/abfa60c43b01/cureus-0017-00000085752-i13.jpg

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