Alahmad Molham, Alkheder Ahmad, Alassaf Arige
Department of Otorhinolaryngology, Al Mouwasat University Hospital, Damascus University, Damascus, Syria.
Faculty of Medicine, Damascus University, Damascus, Syria.
Ear Nose Throat J. 2025 Jun 25:1455613251352732. doi: 10.1177/01455613251352732.
Isolated Horner syndrome (HS) in young children warrants thorough investigation for occult malignancy, despite its rarity as a presenting sign. This report details a unique case of a 30 month-old male presenting with sudden-onset, isolated left HS as the sole manifestation of rhabdomyosarcoma (RMS) in parapharyngeal space. Contrast-enhanced magnetic resonance imaging (MRI) revealed a 2.5 × 3.5 × 5 cm lesion within the left poststyloid parapharyngeal space, displacing the carotid artery anteriorly, and the internal jugular vein posteriorly. Gross total resection via a transcervical approach was performed; intraoperatively, the tumor involved the cervical sympathetic trunk, necessitating its sacrifice, resulting in persistent postoperative HS. Histopathological and immunohistochemical analysis (positive CK, PLAP, CD99) confirmed a diagnosis of embryonal RMS. The child received adjuvant chemoradiotherapy. One-year follow-up with positron emission tomography - computed tomography (PET-CT) showed no evidence of recurrence. This case underscores several critical points: (1) Isolated HS in young children necessitates urgent neuroimaging to exclude malignancy, even in the absence of palpable masses or neurological deficits; (2) The parapharyngeal space, though an exceptionally rare primary site for RMS (constituting a minor subset of head and neck cases), carries significant implications due to its proximity to the sympathetic chain, readily causing HS via mass effect; (3) MRI is pivotal for diagnosis and surgical planning in this anatomically complex region; (4) Multimodal therapy is essential for local control in embryonal RMS; (5) Sacrifice of the sympathetic trunk during resection likely leads to permanent HS, a factor for preoperative counseling. Vigilant long-term follow-up remains crucial.
尽管小儿孤立性霍纳综合征（HS）作为首发症状较为罕见，但仍需对隐匿性恶性肿瘤进行全面检查。本报告详细介绍了一例独特病例，一名30个月大的男性患儿，突然出现孤立性左侧HS，这是咽旁间隙横纹肌肉瘤（RMS）的唯一表现。对比增强磁共振成像（MRI）显示左侧茎突后咽旁间隙有一个2.5×3.5×5 cm的病变，将颈动脉向前推移，颈内静脉向后推移。通过经颈入路进行了根治性切除；术中，肿瘤累及颈交感干，必须将其切除，导致术后持续性HS。组织病理学和免疫组织化学分析（CK、PLAP、CD99阳性）确诊为胚胎型RMS。该患儿接受了辅助放化疗。正电子发射断层扫描-计算机断层扫描（PET-CT）一年随访显示无复发迹象。该病例强调了几个关键点：（1）小儿孤立性HS即使没有可触及的肿块或神经功能缺损，也需要紧急进行神经影像学检查以排除恶性肿瘤；（2）咽旁间隙虽然是RMS极为罕见的原发部位（占头颈部病例的一小部分），但由于其靠近交感神经链，通过占位效应很容易导致HS，因此具有重要意义；（3）MRI对于这个解剖结构复杂区域的诊断和手术规划至关重要；（4）多模式治疗对于胚胎型RMS的局部控制至关重要；（5）切除过程中交感干的牺牲可能导致永久性HS，这是术前咨询的一个因素。长期密切随访仍然至关重要。
