Ocular manifestations and long-term complications of rhabdomyosarcoma in children,Eye

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Ocular manifestations and long-term complications of rhabdomyosarcoma in children
Eye ( IF 2.8 ) Pub Date : 2024-06-21 , DOI: 10.1038/s41433-024-03175-1
Sybille Graef ₁ , Dan DeAngelis ₁ , Abha A Gupta ₂ , Michael J Wan ₁

Affiliation

Background/objectives

The purpose of the study was to describe the ocular manifestations of rhabdomyosarcoma in a large cohort of children.

Subject/methods

This was a retrospective observational cohort study. The medical records of all pediatric patients with head and neck rhabdomyosarcoma diagnosed between 1997 and 2021 at a tertiary-care pediatric hospital were analyzed. The main outcome measures were the incidence and prognostic role of ocular findings at presentation and long-term ocular complications.

Results

There were 77 children with head and neck rhabdomyosarcoma in the study cohort with 38 patients showing ocular manifestations at presentation. Median age at diagnosis was 6.0 years, the median follow-up was 5.7 years and 54.5% were male. At last follow-up, 70.1% had no evidence of progression, 26.0% were deceased, and 2.6% were on palliative treatment. Orbital signs were common (44.2%). The most common ocular findings were proptosis (18.2%), restriction of extraocular motility (28.6%), strabismus/diplopia (22.1%) and ptosis (16.9%). The most common long-term complications were bony hypoplasia/facial asymmetry (40.3%) and keratopathy/dry eye (31.2%). Poor visual acuity (≤20/200) was noted in 13 (16.9%) patients with 5 (6.5%) patients requiring an exenteration. Survival was 100% in primary orbital RMS (p = 0.02), whereas any or a combination of cranial nerve palsies carried a poor prognosis (42% survival, p = 0.008).

Conclusions

In our cohort, half of children with rhabdomyosarcoma had ocular manifestations at presentation with about one-third showing orbital tumor involvement. Cranial nerve involvement carried a significantly worse prognosis for survival.

中文翻译：

儿童横纹肌肉瘤的眼部表现和远期并发症

背景/目标

该研究的目的是描述一大群儿童横纹肌肉瘤的眼部表现。

主题/方法

这是一项回顾性观察队列研究。分析了 1997 年至 2021 年间在三级儿科医院诊断的所有头颈部横纹肌肉瘤儿科患者的病历。主要结局指标是就诊时眼部表现和远期眼部并发症的发生率和预后作用。

结果

研究队列中有 77 名头颈部横纹肌肉瘤儿童，其中 38 名患者在就诊时表现出眼部表现。诊断时的中位年龄为 6.0 岁，中位随访为 5.7 岁，54.5% 为男性。在最后一次随访中，70.1% 的患者没有进展的证据，26.0% 的患者死亡，2.6% 的患者正在接受姑息治疗。眼眶体征很常见（44.2%）。最常见的眼部发现是眼球突出（18.2%）、眼外运动受限（28.6%）、斜视/复视（22.1%）和上睑下垂（16.9%）。最常见的长期并发症是骨发育不全/面部不对称（40.3%）和角膜病变/干眼症（31.2%）。13 例（16.9%）患者视力差（≤20/200），其中 5 例（6.5%）患者需要切除。原发性眼眶 RMS 的生存率为 100% （p = 0.02），而任何或颅神经麻痹的组合预后不良（42% 生存率，p = 0.008）。