McCormick Andrew A, Tarchichi Tony, Azbell Christopher, Grunwaldt Lorelei, Jabbour Noel
Department of Pediatrics Children's Hospital of Pittsburgh of UPMC, 4401 Penn Avenue, Faculty Pavilion, 3rd Floor, Pittsburgh, PA, USA, 15224.
Department of Otolaryngology--Head & Neck Surgery Children's Hospital of Pittsburgh of UPMC, 4401 Penn Avenue, Faculty Pavilion, 7th Floor, Pittsburgh, PA, USA, 15224.
Int J Pediatr Otorhinolaryngol. 2018 Oct;113:34-37. doi: 10.1016/j.ijporl.2018.07.019. Epub 2018 Jul 12.
A subglottic hemangioma (SGH) is a benign tumor of infancy that can cause severe obstruction of the airway. Infantile hemangiomas, in general, are the most common head and neck tumor in children, affecting 4-5% of the pediatric population. This retrospective cohort study characterizes subglottic infantile hemangiomas at a single vascular anomaly center over a 5-year period (2013-2017) during the era of propranolol treatment.
Queried the Vascular Anomaly Database at Children's Hospital of Pittsburgh for all infantile hemangioma(s) and then identified case of subglottic hemangiomas. Characterized key features of presentation, natural history and management for subglottic hemangiomas. A secondary differentiation focused on differences between subglottic hemangiomas associated with Beard Distribution (BD) vs not (NBD).
Analysis of 761 cases of infantile hemangiomas demonstrated only 13 patients with subglottic hemangiomas (1.7%). Of those 13 patients, only 4 patients (30%) had BD while 2 patients (15%) had other cutaneous hemangiomas and 7 patients (55%) had no cutaneous hemangiomas. Secondarily, a total of 31 case of beard distribution cutaneous hemangiomas with 11 patients having oropharyngeal involvement (35%) but only 4 patients with subglottic hemangiomas (13%). Interestingly, 2 of the 4 BD patients had treatment failure on propranolol and required second line treatment with steroids or surgical excision while only 1 of 9 NBD patients failed propranolol treatment. As well the same 2 BD patients which failed propranolol also had PHACES syndrome.
Subglottic hemangiomas are a rare presentation of infantile hemangiomas but with significant morbidity. While the classic teaching that a segmental beard distribution hemangioma raises concern for a subglottic hemangioma, this cohort indicates subglottic hemangiomas occur in a NBD presentation (1.3%), and demonstrated only an approximate 10% incidence rate with a beard distribution. But more importantly, this study raises the question that beard distribution in setting of PHACES syndrome may herald a more recalcitrant and complicated natural history for a subglottic hemangioma. This is of significant concern as risk for CVA in setting of PHACES is highest with use of steroid treatment. None of our patients had high risk extra or intra cranial vascular arterial anomalies and no CVA were noted.
声门下血管瘤（SGH）是一种婴儿期的良性肿瘤，可导致严重的气道梗阻。一般来说，婴儿血管瘤是儿童中最常见的头颈部肿瘤，影响4%-5%的儿童人群。这项回顾性队列研究描述了在普萘洛尔治疗时代，单一血管异常中心5年期间（2013 - 2017年）声门下婴儿血管瘤的特征。
查询匹兹堡儿童医院的血管异常数据库中的所有婴儿血管瘤病例，然后确定声门下血管瘤病例。描述声门下血管瘤的临床表现、自然病程和治疗的关键特征。二级分析重点关注与胡须分布（BD）相关的声门下血管瘤与无胡须分布（NBD）的声门下血管瘤之间的差异。
对761例婴儿血管瘤病例的分析显示，只有13例患者有声门下血管瘤（1.7%）。在这13例患者中，只有4例（30%）有BD，2例（15%）有其他皮肤血管瘤，7例（55%）没有皮肤血管瘤。其次，共有31例胡须分布的皮肤血管瘤患者，其中11例（35%）有口咽受累，但只有4例（13%）有声门下血管瘤。有趣的是，4例BD患者中有2例普萘洛尔治疗失败，需要二线治疗，使用类固醇或手术切除，而9例NBD患者中只有1例普萘洛尔治疗失败。同样，这2例普萘洛尔治疗失败的BD患者也患有PHACES综合征。
声门下血管瘤是婴儿血管瘤的一种罕见表现，但发病率较高。虽然传统观点认为节段性胡须分布血管瘤提示有声门下血管瘤，但该队列研究表明，声门下血管瘤以无胡须分布（NBD）表现为主（1.3%），有胡须分布的发病率仅约为10%。但更重要的是，这项研究提出了一个问题，即PHACES综合征背景下的胡须分布可能预示着声门下血管瘤的自然病程更顽固和复杂。这一点值得高度关注，因为在PHACES综合征背景下，使用类固醇治疗时发生脑血管意外（CVA）的风险最高。我们的患者中没有高风险的颅外或颅内血管动脉异常，也未观察到CVA。
