Department of Ophthalmology and Visual Science, College of Medicine, Daejeon St. Mary's Hospital, The Catholic University of Korea, Seoul, Korea.
Department of Ophthalmology, Chungnam National University Hospital, Korea.
Medicine (Baltimore). 2022 Jun 17;101(24):e29468. doi: 10.1097/MD.0000000000029468.
A few cases of intercalary staphyloma have been reported in patients with Marfan syndrome, but we believe that this is the first case of intercalary staphyloma in Marfan syndrome developing after strabismus surgery.
A 9-year-old girl diagnosed with Marfan syndrome visited a strabismus clinic for treatment of esotropia. Both eyes were aphakic and had 60 prism diopter esotropia at distance and 55 prism diopter esotropia at near. There were no corneal, conjunctival, or scleral abnormalities. Six millimeters of recession was performed on both medial rectus muscles via an inferonasal fornix approach under general anesthesia. 5 days after surgery, a dark gray protruding lesion was observed on the upper nasal side of the left eye.
Intraocular ultrasonography showed no bleeding, retinal detachment, or other abnormal findings. Computed tomography showed a conical protrusion of the scleral wall which was diagnosed as intercalary staphyloma.
To reduce risk of progression of the staphyloma in the left eye and to reduce risk of development of a new staphyloma, intraocular pressure lowering eye drops were administered.
We just observed it without any intervention except the intraocular pressures lowering eye drops. It remained stable for 12 months.
Clinicians need to be alert to the possibility of this serious complication in Marfan syndrome patients after minor surgical trauma, which can occur during uneventful strabismus surgery.
已有少数马凡综合征患者出现夹层葡萄肿的病例报告，但我们认为这是首例马凡综合征患者在斜视手术后发生夹层葡萄肿。
一名 9 岁女孩被诊断为马凡综合征，因斜视到斜视诊所就诊。双眼无晶状体，远距斜视 60 棱镜度，近距斜视 55 棱镜度。无角膜、结膜或巩膜异常。全身麻醉下，通过鼻下穹隆入路对双侧内直肌各行 6mm 后退术。术后 5 天，左眼鼻上方出现暗灰色突出病变。
眼部超声检查未见出血、视网膜脱离或其他异常。计算机断层扫描显示巩膜壁呈圆锥形突出，诊断为夹层葡萄肿。
为降低左眼葡萄肿进展和新发葡萄肿的风险，给予降眼压滴眼液。
除降眼压滴眼液外，我们未进行其他干预，病情稳定 12 个月。
在斜视手术顺利进行的情况下，轻微手术创伤后，马凡综合征患者也可能发生这种严重并发症，临床医生需要对此保持警惕。
