从喘息声到症状缓解：巴勒斯坦地区儿童科默雷尔憩室和血管环的管理（2016–2024年）：一项单中心队列研究

《Frontiers in Pediatrics》：From stridor to symptom relief; management of pediatric Kommerell's diverticulum and vascular rings in Palestine [2016–2024]: a single center cohort study

KD合并血管环患儿临床特征及诊疗分析。本研究回顾2016-2024年巴勒斯坦Al-Makassed医院14例确诊KD合并血管环患儿的诊疗资料，发现中位诊断年龄8个月（2-15月），92.9%患儿因呼吸道症状（64.3%声门阻塞）及喂养困难（28.6%）就诊，经CTA确诊后13例（92.9%）行手术（包括结扎动脉韧带、胸腺切除等），1例保守观察。术后中位住院18天（7-25天），90.5%症状缓解，但1例术后死亡。研究强调CTA为确诊金标准，手术可显著改善症状，需扩大样本验证长期预后。

　　
Kommerell's diverticulum (KD) is a rare congenital vascular anomaly characterized by an aneurysmal dilation at the origin of an aberrant subclavian artery from the aortic arch. This condition often presents with complex respiratory and feeding complications in pediatric patients, but its diagnosis is frequently delayed due to overlapping symptoms with common childhood illnesses. A recent retrospective study conducted at Al-Makassed Hospital in Jerusalem (2016-2024) aimed to characterize the clinical spectrum, diagnostic challenges, and surgical outcomes of pediatric KD cases with associated vascular rings in Palestine. The findings highlight critical insights into early diagnosis, imaging modalities, and surgical strategies while emphasizing the need for larger, multi-center studies to address knowledge gaps.

The study enrolled 14 pediatric patients diagnosed with vascular rings accompanied by KD. Median age at diagnosis was 8 months, with symptoms typically emerging within the first two months of life. Over 90% of patients presented with respiratory distress (stridor in 64.3% and recurrent wheezing in 35.7%) or feeding difficulties (dysphagia/choking in 28.6%). A notable feature was the high rate of prior misdiagnosis (42.9%), with symptoms initially attributed to pneumonia or reactive airway disease. Diagnostic delays averaged 3.5 months, underscoring the challenges in differentiating KD from other pediatric conditions.

Imaging played a pivotal role in diagnosis. Barium swallow studies identified extrinsic esophageal compression in 87.5% of cases, while echocardiography detected anatomical abnormalities in 55.6% of patients. However, these modalities had limited sensitivity and specificity. Contrast-enhanced CT angiography (CTA) emerged as the gold standard, confirming vascular ring anatomy in 100% of cases where performed. This shift toward CTA reflects advancements in imaging technology, which now enable three-dimensional visualization of both vascular and airway structures, critical for preoperative planning.

Anatomical subtypes revealed a higher prevalence of right aortic arch with aberrant left subclavian artery (RAA/ALSA) in this cohort (57.1%), followed by double aortic arch (28.6%) and innominate artery compression (14.3%). Over half of the patients (57.1%) exhibited minor intracardiac anomalies such as atrial septal defects (ASD) or ventricular septal defects (VSD). These findings align with global patterns but emphasize the importance of comprehensive cardiac evaluation in pediatric KD cases.

Surgical intervention was required in 92.9% of patients, with operations tailored to anatomical subtypes. Thoracoscopic approaches dominated, particularly ligamentum arteriosum division for RAA/ALSA cases, while double aortic arch required arch division. Postoperative complications were common, including pneumothorax (30.8%), sepsis (15.4%), and chylothorax (7.7%). Median hospital stay was 18 days, with one late mortality attributed to cardiac arrest following complications. Notably, all surgically treated patients experienced partial or complete symptom resolution, with 53.8% becoming asymptomatic at last follow-up.

The study identified key diagnostic and therapeutic principles. First, CTA remains indispensable for confirming vascular ring anatomy and guiding surgical planning. Second, while barium swallow and bronchoscopy are accessible tools, their diagnostic yield is inferior to CTA. Third, surgical outcomes were strongly correlated with timely intervention; patients diagnosed earlier than 12 months showed better recovery trajectories. Fourth, the decision to reimplant the aberrant subclavian artery or perform aortopexy was influenced by the presence of dynamic airway collapse and vertebral artery dominance, though the latter was underdiagnosed due to limited imaging protocols.

Limitations of the study include its single-center design and small sample size, which precluded formal subgroup analyses. The absence of standardized diverticulum size measurements also restricted conclusions about anatomical thresholds for intervention. Additionally, long-term follow-up data were incomplete, particularly regarding growth trajectories and quality of life. These gaps highlight the need for multi-center registries with standardized protocols for imaging, surgical approaches, and outcome assessment.

The study's implications extend beyond the pediatric population. It reinforces the clinical relevance of vascular rings in children, emphasizing that symptoms like stridor and feeding difficulties should prompt urgent evaluation. For healthcare providers in the Arab region, the findings underscore the necessity of integrating advanced imaging (CTA) into diagnostic algorithms and establishing protocols for multidisciplinary management involving cardiology, surgery, and radiology.

Future research should focus on three areas: (1) developing predictive models for symptom onset based on anatomical subtypes and associated anomalies, (2) optimizing imaging protocols to reduce reliance on invasive bronchoscopy, and (3) exploring endovascular techniques as alternatives to open surgery in select cases. Larger cohorts with longer follow-up periods will be critical to validate these strategies and refine guidelines for KD management in pediatric populations.

In conclusion, this study advances understanding of pediatric KD with vascular rings by documenting the high frequency of missed diagnoses, the superiority of CTA in diagnosis, and the effectiveness of anatomically tailored surgery. While postoperative complications remain a challenge, the majority of patients achieved significant symptom relief. The findings advocate for proactive screening in symptomatic infants and the integration of CTA into routine diagnostic pathways. Collaborative efforts across institutions in the Arab region are essential to build a robust database for evidence-based practice and reduce diagnostic delays in future cases.