Yamaguchi Yusei, Ohira Shinya, Yui Ryousuke, Sonobe Satoshi, Wada Kota
Otolaryngology, Toho University, Tokyo, JPN.
Otolaryngology, The Jikei University School of Medicine, Tokyo, JPN.
Cureus. 2025 Jun 26;17(6):e86809. doi: 10.7759/cureus.86809. eCollection 2025 Jun.
IgG4-related disease (IgG4-RD) is a chronic inflammatory condition characterized by elevated serum IgG4 levels, infiltration of IgG4-positive plasma cells, and fibrosis in various organs. We report the case of a 76-year-old man who presented with left-sided proptosis. Computed tomography revealed a mass lesion in the left orbit. An initial biopsy via a transnasal approach under local anesthesia was inconclusive. Although endoscopic sinus surgery was performed under general anesthesia, a definitive diagnosis could not be obtained. The lesion continued to enlarge, and subsequent ophthalmologic examinations revealed progressive optic nerve compression. Therefore, tumor resection was performed again under general anesthesia using the endoscopic Denker's approach. The tumor was successfully resected without complications. Histopathological findings led to a diagnosis of probable IgG4-related ophthalmic disease (IgG4-ROD). Following surgery, the residual lesion enlarged again; however, a three-day course of steroid pulse therapy resulted in reduction of the lesion and improvement of optic nerve compression. The patient has remained relapse-free. While 81% of IgG4-ROD cases involve the lacrimal gland, other orbital structures such as the pterygopalatine fossa, trigeminal nerve branches, extraocular muscles, orbital fat, eyelids, and nasolacrimal duct can also be affected. In cases without lacrimal gland involvement, the optimal approach for obtaining diagnostic biopsy specimens should be considered individually. Although there is no consensus on the required volume of tissue for diagnosis, we believe that aggressive resection of the central lesion is necessary for accurate diagnosis. The endoscopic Denker's approach facilitates wide exposure and resection of far lateral maxillary sinus lesions, enabling both decompression and definitive diagnosis, which can lead to appropriate subsequent treatment.
IgG4相关疾病（IgG4-RD）是一种慢性炎症性疾病，其特征为血清IgG4水平升高、IgG4阳性浆细胞浸润以及各器官纤维化。我们报告一例76岁男性患者，其表现为左侧眼球突出。计算机断层扫描显示左侧眼眶有一肿块病变。在局部麻醉下经鼻进行的初次活检结果不明确。尽管在全身麻醉下进行了内镜鼻窦手术，但仍无法获得明确诊断。病变持续增大，随后的眼科检查显示视神经受压逐渐加重。因此，再次在全身麻醉下采用内镜Denker入路进行肿瘤切除。肿瘤成功切除，无并发症发生。组织病理学检查结果提示可能为IgG4相关眼病（IgG4-ROD）。手术后，残留病变再次增大；然而，为期三天的类固醇脉冲疗法使病变缩小，视神经压迫症状改善。患者至今未复发。虽然81%的IgG4-ROD病例累及泪腺，但翼腭窝、三叉神经分支、眼外肌、眶脂肪、眼睑和鼻泪管等其他眼眶结构也可能受累。在不累及泪腺的病例中，应个体化考虑获取诊断性活检标本的最佳方法。尽管对于诊断所需的组织量尚无共识，但我们认为积极切除中央病变对于准确诊断是必要的。内镜Denker入路有助于广泛暴露和切除上颌窦外侧远部病变，既能实现减压又能进行明确诊断，从而为后续适当治疗提供依据。
