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一例伴有双侧黄斑裂孔的2型黄斑毛细血管扩张症:影像学特征及手术治疗

A case of macular telangiectasia type 2 with bilateral macular holes: Imaging features and surgical management.

作者信息

Usami Yuta, Wahyuningsih Eka Rahmawati, Nakano Kana, Kuwayama Satoshi, Ogura Shuntaro, Kimura Masayo, Kato Aki, Hirano Yoshio, Ogura Yuichiro, Yasukawa Tsutomu

机构信息

Department of Ophthalmology and Visual Science, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.

Department of Ophthalmology, Gamagori Municipal Hospital, Gamagori, Japan.

出版信息

Medicine (Baltimore). 2025 Mar 21;104(12):e41847. doi: 10.1097/MD.0000000000041847.

Abstract

RATIONALE

A macular hole (MH) secondary to macular telangiectasia (MacTel) type 2 is generally considered a poor surgical candidate when internal limiting membrane (ILM) peeling is performed. Due to the scarcity of studies on MH in MacTel type 2 patients, the number of published studies exploring the surgical outcomes of these patients is limited.

PATIENT CONCERNS

In this case report, we report a case with MacTel type 2 with bilateral MHs and had a good visual prognosis after surgical treatments.

DIAGNOSES

A 66-year-old woman was referred to our hospital for macular abnormalities in the right eye. The best-corrected visual acuity (BCVA) was 20/32 in the right eye and 20/16 in the left eye at the initial examination. Ophthalmic examination revealed decreased retinal transparency temporal to the fovea in both eyes, cystoid spaces at the macula of the right eye by optical coherence tomography, telangiectasia temporal to the fovea, and fluorescent leakage temporal to the fovea by fluorescein angiography in both eyes and the patient was diagnosed with MacTel type 2.

INTERVENTIONS

Since there was no tendency toward vision loss, the patient was followed up without any treatment. Four years later, the BCVA of the right eye had decreased to 20/50, and outer retinal layer atrophy at the macula had progressed, so the patient underwent cataract surgery, pars plana vitrectomy (PPV), and fluid-air exchange. Seven months after the initial surgery, an MH was formed in the right eye. Second surgery including PPV, ILM peeling, and sulfur hexafluoride gas tamponade were performed.

OUTCOMES

Postoperatively, the MH was closed, and the BCVA improved to 20/32 at 1 year, and 20/20 at 12 years after the surgery, respectively. Twelve years after the initial visit, an MH appeared in the left eye, and the BCVA decreased to 20/50, so cataract surgery, PPV with inverted ILM-flap technique, and sulfur hexafluoride gas tamponade were performed in the left eye. Postoperatively, the MH was closed with no recurrence for longer than 2 years after surgery, and the BCVA improved to 20/16. Microperimetry performed after the surgery showed decreased retinal sensitivity consistent with areas of retinal atrophy in both eyes.

LESSONS

We experienced a case of bilateral MHs associated with MacTel type 2. The right eye underwent PPV with ILM peeling and the left eye underwent PPV with an inverted ILM flap technique, both eyes had good visual prognosis. Imaging modalities and microperimetry can provide valuable information on the associated anatomical and functional changes.

摘要

原理

继发于2型黄斑毛细血管扩张症(MacTel)的黄斑裂孔(MH)在进行内界膜(ILM)剥除术时通常被认为是手术效果不佳的候选病例。由于关于2型MacTel患者黄斑裂孔的研究较少,探索这些患者手术结果的已发表研究数量有限。

患者情况

在本病例报告中,我们报告了一例患有双侧黄斑裂孔的2型MacTel患者,经手术治疗后视力预后良好。

诊断

一名66岁女性因右眼黄斑异常转诊至我院。初诊时右眼最佳矫正视力(BCVA)为20/32,左眼为20/16。眼科检查发现双眼黄斑中心凹颞侧视网膜透明度降低,右眼黄斑区经光学相干断层扫描显示有囊样间隙,双眼黄斑中心凹颞侧有毛细血管扩张,荧光素血管造影显示双眼黄斑中心凹颞侧有荧光渗漏,该患者被诊断为2型MacTel。

干预措施

由于没有视力下降的趋势,患者未接受任何治疗进行随访。四年后,右眼BCVA降至20/50,黄斑区外层视网膜萎缩进展,因此患者接受了白内障手术、玻璃体切割术(PPV)和液气交换。初次手术后七个月,右眼形成了一个黄斑裂孔。进行了包括PPV、ILM剥除和六氟化硫气体填塞的二次手术。

结果

术后,黄斑裂孔闭合,术后1年BCVA提高到20/32,术后12年提高到20/20。初诊12年后,左眼出现黄斑裂孔,BCVA降至20/50,因此左眼进行了白内障手术、采用反向ILM瓣技术的PPV和六氟化硫气体填塞。术后,黄斑裂孔闭合,术后2年以上无复发,BCVA提高到20/16。术后进行的微视野检查显示双眼视网膜敏感度降低,与视网膜萎缩区域一致。

经验教训

我们遇到了一例与2型MacTel相关的双侧黄斑裂孔病例。右眼进行了带ILM剥除的PPV,左眼进行了带反向ILM瓣技术的PPV,双眼视力预后良好。影像学检查和微视野检查可以提供有关相关解剖和功能变化的有价值信息。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d8b/11936658/7229c3eb683a/medi-104-e41847-g001.jpg

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