Van Laeken Jana, Leysen Laura, Keer Karel Van
Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium.
Oogartsen Associatie Maasmechelen, Maasmechelen, Belgium.
J Curr Ophthalmol. 2023 Dec 21;35(2):203-206. doi: 10.4103/joco.joco_291_22. eCollection 2023 Apr-Jun.
To report a rare case of microspherophakia and lens subluxation in a young patient with Klinefelter syndrome who underwent cataract surgery, emphasizing the importance of a thorough general health assessment and anamnesis prior to cataract surgery.
The case concerns a 52-year-old male referred for phacoemulsification under general anesthesia. A review of literature was performed.
Preoperative assessment revealed a corticonuclear cataract in both eyes, with dislocation of the crystalline lens in the left eye in an area of zonular dehiscence. Upon careful examination, both eyes showed a microspherophakic lens with an increased lens thickness and the lens equator being visible over 360° in the left eye. The patient denied any trauma or medical conditions. His medical health record revealed the coexistence of Klinefelter syndrome (47, XXY). The association between Klinefelter syndrome and microspherophakia has only once been reported in the literature. Intraoperatively, a more cautious approach was withheld and a capsular tension ring was used. Postoperative outcome was successful with good visual outcome and no interoperative complications.
This case report highlights the importance of proper preoperative assessment before cataract surgery, especially in unusual cases such as early-onset cataract and/or lens subluxation. In addition, it stresses the importance of a systemic and/or genetic evaluation in patients with microspherophakia and an ophthalmological examination in patients with Klinefelter syndrome.
报告一例患有克兰费尔特综合征的年轻患者发生小晶状体及晶状体半脱位并接受白内障手术的罕见病例，强调白内障手术前进行全面的全身健康评估和病史询问的重要性。
该病例为一名52岁男性，因需在全身麻醉下进行超声乳化白内障吸除术前来就诊。并进行了文献回顾。
术前评估显示双眼为皮质核性白内障，左眼晶状体在 zonular 裂开区域脱位。仔细检查发现，双眼晶状体均为小晶状体，晶状体厚度增加，左眼晶状体赤道在360°范围内可见。患者否认有任何外伤或疾病史。其病历显示同时患有克兰费尔特综合征（47, XXY）。克兰费尔特综合征与小晶状体之间有关联的情况在文献中仅被报道过一次。术中采用了更为谨慎的方法并使用了囊袋张力环。术后结果成功，视力良好，无术中并发症。
本病例报告强调了白内障手术前进行适当术前评估的重要性，尤其是在诸如早发性白内障和/或晶状体半脱位等特殊病例中。此外，它强调了对小晶状体患者进行系统和/或基因评估以及对克兰费尔特综合征患者进行眼科检查的重要性。
