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杜安综合征合并 Rubinstein-Taybi 综合征Ⅱ型。

Duane syndrome associated with Rubinstein-Taybi syndrome type II.

机构信息

Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida.

Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida.

出版信息

J AAPOS. 2024 Oct;28(5):103990. doi: 10.1016/j.jaapos.2024.103990. Epub 2024 Sep 4.

Abstract

We report the case of an 8-month-old infant born at 33 weeks' gestation referred to our institution for evaluation of left eye abduction deficit and microcephaly. Ophthalmic examination revealed a left eye abduction deficit with palpebral fissure narrowing on adduction along with palpebral fissure widening on abduction, consistent with Duane syndrome. Genetic testing revealed a pathogenic EP300 mutation confirming Rubinstein-Taybi syndrome type II. The cooccurrence of Duane syndrome and Rubinstein-Taybi syndrome is rare, with only 3 cases in the literature, 2 with genetic confirmation. Potential involvement of the cranial nerves in Rubinstein-Taybi syndrome may explain its cooccurrence with Duane syndrome, which is seemingly more common in EP300-mediated disease.

摘要

我们报告了一例 8 个月大的婴儿,该婴儿在 33 周时出生,因左眼外展不足和小头畸形到我们机构就诊。眼科检查显示左眼外展不足,内收时睑裂变窄,外展时睑裂变宽,符合 Duane 综合征。基因检测显示 EP300 致病性突变,确诊为 Rubinstein-Taybi 综合征 II 型。Duane 综合征和 Rubinstein-Taybi 综合征同时发生的情况非常罕见,文献中仅有 3 例,其中 2 例有基因证实。Rubinstein-Taybi 综合征可能涉及颅神经,这可以解释其与 Duane 综合征同时发生的原因,而 Duane 综合征在 EP300 介导的疾病中更为常见。

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