Department of Ophthalmology, Beijing Anzhen Hospital, Capital Medical University, Beijing, 100029, China.
BMC Ophthalmol. 2022 May 2;22(1):201. doi: 10.1186/s12886-022-02422-x.
Congenital anomalies of the pupil are quite varied, including abnormal size, shape, color, response to stimulus, and function. We are here reporting an unusual case presented with the absence of pupillary opening with folds of iris tissue at the center. Only an extremely small pupil (diameter < 0.5 mm) could be observed during the operation.
A 15-year-old male patient visited our outpatient clinic due to vision difficulty in his right eye for more than ten years. The best-corrected visual acuity was 2.0 logMAR and 0 logMAR for the right and left eye, respectively. There were amblyopia, astigmatism and constant exotropia in his right eye. Ophthalmic examination of the right eye showed flat iris root, minimal iris pigmentation, and the pupil area was entirely covered by iris tissue. Lens status and fundus evaluation could not be commented. The left eye was found to be within normal limit. Based on ophthalmic examination, the admission diagnosis was given as acorea. Pupilloplasty was performed on the right eye due to the situation that the iris tissue blocked the visual axis, which led to visual impairment and stimulus deprivation amblyopia. However, an extremely small pupil at the center of his pupillary area was observed during the operation. The postoperative course was favorable, and a normal pupil was secured. Hospital discharge diagnosis was given as microcoria, and amblyopia treatment was followed.
We report a rare case of congenital pupillary abnormality. The further diagnosis was given as microcoria, which should be differentiated from acorea. For this kind of pupil disorder which blocks the visual axis, early diagnosis and treatment can help prevent the development of stimulus deprivation amblyopia.
瞳孔的先天性异常多种多样，包括大小、形状、颜色、对刺激的反应和功能异常。我们在此报告一例异常病例，其表现为瞳孔无张开，虹膜组织在中央形成褶皱。术中仅能观察到极小的瞳孔（直径<0.5mm）。
一名 15 岁男性患者因右眼视力障碍超过十年就诊于我院门诊。最佳矫正视力右眼为 2.0 logMAR，左眼为 0 logMAR。右眼有弱视、散光和恒定性外斜视。右眼眼科检查显示虹膜根部平坦，虹膜色素极少，瞳孔区域完全被虹膜组织覆盖。晶状体情况和眼底评估无法进行评价。左眼正常。根据眼科检查，入院诊断为虹膜缺损。由于虹膜组织阻塞视轴，导致视力损害和剥夺性弱视，对右眼行瞳孔成形术。然而，术中观察到瞳孔中央有极小的瞳孔。术后过程顺利，获得正常瞳孔。出院诊断为小瞳孔，并进行弱视治疗。
我们报告了一例罕见的先天性瞳孔异常病例。进一步诊断为小瞳孔，应与虹膜缺损相鉴别。对于这种阻塞视轴的瞳孔障碍，早期诊断和治疗有助于预防剥夺性弱视的发展。
