背景:关于儿童正中弓状韧带综合征(MALS)的数据很少。据推测,MALS可引起慢性腹痛。目前尚不清楚患有这种疾病的儿童中有多少百分比是有症状的,以及哪些合并症与这种综合征有关。
方法:在这项回顾性研究中,我们回顾了在常规超声心动图中同时诊断为MALS的单中心连续患者的数据.症状负担,合并症,并研究了人体测量指标对MALS的影响。描述性统计和非参数检验用于描述研究结果并将变量与正态分布进行比较。
结果:在2013年至2020年之间,有82名儿童,55名女性(67%),平均年龄13.9±3.2岁,与MALS和完整的记录。狭窄区域的平均速度为2.6±0.4m/s。46例患者(57%)有腹痛。年龄,性别,体重,体重指数(BMI),多普勒速度对症状发生无统计学意义。相反,关节活动过度和体位不耐受症状的患者更有可能出现MALS引起的腹痛.在24名关节过度活动的患者中,18例患者出现腹痛(p=0.027)。38例MALS体位不耐受(OI)患者主诉腹痛,13例OI且无腹痛(p=<0.0001)。
结论:近一半的MALS患者有腹痛。年龄,性别,体重,狭窄程度对症状发生的影响无统计学意义。OI,特别是体位性心动过速综合征(POTS),和检查时关节过度活动预测MALS患者腹痛的倾向更高。
BACKGROUND: Data on median arcuate ligament syndrome (MALS) in children are scant. It is postulated that MALS can cause chronic abdominal pain. It is unclear what percentage of children with this condition are symptomatic and what comorbidities are associated with this syndrome.
METHODS: In this retrospective study, data on consecutive patients in a single center diagnosed coincidentally with MALS during routine echocardiogram were reviewed. Symptom burden, comorbidities, and the effect of anthropometric indices on MALS were investigated. Descriptive statistics and nonparametric tests were used to describe the findings and to compare variables with normal distribution.
RESULTS: Between 2013 and 2020, there were 82 children, 55 females (67%), mean age 13.9 ± 3.2 years, with MALS and complete record. Mean velocity across the stenotic area was 2.6 ± 0.4 m/s. Forty-six patients (57%) had abdominal pain. Age, gender, weight, body mass index (BMI), and Doppler velocity had no statistically significant influence on symptom occurrence. Conversely, patients with joint hypermobility and symptoms of orthostatic intolerance were more likely to have abdominal pain from MALS. Of 24 patients with joint hypermobility, 18 patients had abdominal pain (p=0.027). Thirty-eight patients with orthostatic intolerance (OI) with MALS complained of abdominal pain vs 13 patients with OI and no abdominal pain (p=<0.0001).
CONCLUSIONS: Nearly half of patients with MALS had abdominal pain. Age, gender, weight, and the degree of stenosis had no statistically significant influence on symptom occurrence. OI, specifically postural orthostatic tachycardia syndrome (POTS), and joint hypermobility on exam predicted a higher propensity for abdominal pain in patients with MALS.