Incidence and clinical profile of Marcus Gunn jaw-winking phenomenon in congenital ptosis at a tertiary eye hospital in western Uttar Pradesh, India

Suraj K. Chaurasiya, M. S. Alam, P. Agarwal, L. Chauhan
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Abstract

The objectives of this study were to report the hospital-based incidence and a rare case series of Marcus Gunn jaw-winking phenomenon (MGJWP) in patients with congenital ptosis This was a retrospective non-interventional case series. The medical records of all patients diagnosed with MGJWP over the past 3.5 years were retrieved from the electronic medical record. Patients with documented evidence of the presence of ptosis with MGJWP were included and analyzed for visual acuity, the presence of squint and amblyopia, and the presence of other aberrant regenerations. A total of 198 patients were diagnosed with congenital ptosis, out of which 23 (11.6%) patients had MGJWP along with congenital ptosis and 175 (84.4%) patients have congenital ptosis without MGJWP. There were 12 males and 11 females. The mean age at presentation was 10.35 ± 8.4 years (range from 2 to 28 years). All the patients presented unilateral MGJWP. The right eye was involved in seven patients (30.4%) and the left eye in 16 patients (69.5%). Most of the patients had observed the onset of MGJWP by parents since birth. Visual acuity was measured by Snellen’s distance acuity chart in 15 patients. Eight patients were preverbal, and their vision was assessed by the fixation pattern, which was central, steady, and maintained. Nine patients (39.1%) were emmetropic, seven patients (30.4%) had astigmatism, and seven patients were amblyopic in the affected eye. Strabismus was noted in nine patients (39.1%) in this study. Monocular elevation deficiency was noted in five patients, three patients have exotropia, and one had hypotropia. All the patients had a mild form of MGJWP with no intervention required in any of the cases. The hospital-based incidence of MGJWP in congenital ptosis is 11.6%. Most of the patients had observed the onset of MGJWP by their parents since birth.
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印度北方邦西部一家三级眼科医院先天性上睑下垂患者Marcus Gunn眨眼现象的发生率和临床分析
本研究的目的是报道先天性上睑下垂患者Marcus Gunn眨眼现象(MGJWP)在医院的发生率和罕见病例系列。从电子病历中检索过去3.5年中所有诊断为MGJWP的患者的病历。研究纳入了有文献证据证明存在上睑下垂的MGJWP患者,并对其视力、斜视和弱视的存在以及其他异常再生的存在进行了分析。198例诊断为先天性上睑下垂,其中23例(11.6%)合并先天性上睑下垂,175例(84.4%)合并先天性上睑下垂。其中男性12人,女性11人。平均发病年龄为10.35±8.4岁(2 ~ 28岁)。所有患者均表现为单侧MGJWP。右眼受累7例(30.4%),左眼受累16例(69.5%)。大多数患者自出生时即由父母观察到MGJWP的发病。采用Snellen距离视力表测定15例患者的视力。8例患者为语言前患者,他们的视力通过固定模式进行评估,固定模式为中心,稳定和维持。远视9例(39.1%),散光7例(30.4%),弱视7例(30.4%)。本研究中有9例(39.1%)患者出现斜视。5例患者单眼抬高不足,3例患者外斜视,1例患者低视。所有患者均为轻度MGJWP,不需要任何干预。先天性上睑下垂中MGJWP的医院发生率为11.6%。大多数患者自出生起即由父母观察到MGJWP的发病。
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