Bilateral Brown's syndrome with Parry-Romberg syndrome: frozen gaze and fading face.

IF 0.7 Q4 OPHTHALMOLOGY
Vaishali Tomar, Purva Verma, Shivani Kumari, Padma Chorol, Neha Garg, Subhash Dadeya
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Abstract

Purpose: We report a rare case of bilateral Brown's syndrome associated with Parry-Romberg Syndrome (PRS), highlighting this rare syndromic association and its possible shared pathophysiology. Methods: An 8-year-old female presented with longstanding right-eye inward deviation and decreased vision in the right eye. Ocular examination revealed bilateral limitation of elevation in adduction, consistent with Brown's syndrome. Systemic findings included progressive right-sided facial atrophy, preauricular skin anomalies, and dental abnormalities. Imaging and laboratory investigations confirmed PRS. Surgical management and ongoing follow-up outcomes were documented. Results: Forced duction testing demonstrated bilateral superior oblique tightness. Imaging revealed generalized extraocular muscle atrophy with relative fibrosis of the superior obliques. The patient underwent right eye superior oblique tenotomy and 5 mm medial rectus recession under general anesthesia. Postoperatively, ocular alignment improved significantly, with residual 5 prism diopters of hypotropia in primary gaze. Multidisciplinary evaluation confirmed PRS, and amblyopia therapy was initiated with ongoing follow up for progressive facial changes. Conclusion: This is a rare reported case of bilateral Brown's syndrome associated with PRS. This case broadens the ophthalmic spectrum of PRS and suggests a potential link between progressive hemifacial atrophy and restrictive strabismus patterns. Comprehensive ophthalmic and systemic evaluation is essential in such complex presentations.

双侧布朗综合征伴Parry-Romberg综合征:目光呆滞和面部褪色。
目的:我们报告一例罕见的双侧布朗综合征合并Parry-Romberg综合征(PRS),强调这种罕见的综合征关联及其可能的共同病理生理。方法:1例8岁女性右眼长期内偏,右眼视力下降。眼部检查显示双侧内收抬高受限,符合布朗综合征。全身表现包括进行性右侧面部萎缩、耳前皮肤异常和牙齿异常。影像学和实验室检查证实了PRS。记录了手术处理和持续的随访结果。结果:强制诱导试验显示双侧上斜紧绷。影像学显示广泛性眼外肌萎缩伴上斜肌相对纤维化。患者在全麻下行右眼上斜肌腱切开术及内侧直肌收缩5mm。术后眼球排列明显改善,原凝视剩余5个棱镜屈光度。多学科评估证实了PRS,并开始了弱视治疗,并持续随访进行性面部变化。结论:这是一例罕见的双侧布朗氏综合征合并PRS的病例。该病例拓宽了PRS的眼部频谱,提示进行性面瘫和限制性斜视模式之间的潜在联系。在这种复杂的表现中,全面的眼科和系统评估是必不可少的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Strabismus
Strabismus OPHTHALMOLOGY-
CiteScore
1.60
自引率
11.10%
发文量
30
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