Abstract：
BACKGROUND: In January 2021, we found one case of Axenfeld-Rieger syndrome combined with pigment dispersion syndrome (PDS), and this patient additionally manifested a symptom of ectropion uveae. The co-existence of both 2 syndromes is very rare and has not been reported in any literature yet.
METHODS: A 34-year-old female truck driver presented to our institution with a dimness of vision in her right eye. The patient had obvious posterior embryotoxons at bitamporal, and peripheral anterior synechia could be visualized by the slit lamp. The dispersion of pigment granules was observed behind the cornea. The pupil was slightly shifted upwards the nose, with 360° ectropion uveae. Gonioscopy revealed pigment accumulation on the trabecular meshwork. The patient underwent cataract surgery on her right eye, during which, flaky pigmentation around the posterior capsule was observed. These signs were consistent with Axenfeld-Rieger syndrome and PDS.
CONCLUSIONS: We report a rare case of Axenfeld-Rieger syndrome with PDS and uveal eversion. Although the patient did not present with glaucoma, follow-up should be noted. Besides, the correlation between these 2 syndromes needs to be demonstrated by more cases or further evidence.
METHODS: A 34-year-old female truck driver presented to our institution with a dimness of vision in her right eye. The patient had obvious posterior embryotoxons at bitamporal, and peripheral anterior synechia could be visualized by the slit lamp. The dispersion of pigment granules was observed behind the cornea. The pupil was slightly shifted upwards the nose, with 360° ectropion uveae. Gonioscopy revealed pigment accumulation on the trabecular meshwork. The patient underwent cataract surgery on her right eye, during which, flaky pigmentation around the posterior capsule was observed. These signs were consistent with Axenfeld-Rieger syndrome and PDS.
CONCLUSIONS: We report a rare case of Axenfeld-Rieger syndrome with PDS and uveal eversion. Although the patient did not present with glaucoma, follow-up should be noted. Besides, the correlation between these 2 syndromes needs to be demonstrated by more cases or further evidence.
摘要：
背景：2021年1月，我们发现一例Axenfeld-Rieger综合征合并色素分散综合征（PDS），该患者还表现出外翻葡萄膜的症状。两种综合征的共存非常罕见，尚未在任何文献中报道。
方法：一位34岁的女性卡车司机向我们的机构展示了她的右眼视力模糊。患者有明显的后胚胎毒素，裂隙灯可以看到周围的前粘连。在角膜后面观察到颜料颗粒的分散。瞳孔稍微向上移动了鼻子，360°外翻葡萄膜。房角镜检查显示小梁网上的色素积累。病人的右眼接受了白内障手术，在此期间，在后囊周围观察到片状色素沉着。这些体征与Axenfeld-Rieger综合征和PDS一致。
结论：我们报告了一例罕见的Axenfeld-Rieger综合征伴PDS和葡萄膜外翻。尽管患者没有出现青光眼，应该注意后续行动。此外,这两种综合征之间的相关性需要更多的病例或进一步的证据来证明.
方法：一位34岁的女性卡车司机向我们的机构展示了她的右眼视力模糊。
结论：我们报告了一例罕见的Axenfeld-Rieger综合征伴PDS和葡萄膜外翻。
