Abstract：
BACKGROUND: Infantile hemangiomas (IHs) can be part of PHACE (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies) syndrome when they are segmental, extensive, and located on the face or neck. The initial assessment is codified and well known, but there are no recommendations for the follow-up of these patients. The aim of this study was to assess the long-term prevalence of different associated abnormalities.
METHODS: Patients with a history of large segmental IHs of the face or neck. diagnosed between 2011 and 2016 were included in the study. Each patient underwent an ophthalmological, dental, ENT (ear, nose, and throat), dermatological, neuro-pediatric, and radiological assessment at inclusion. Eight patients including five with PHACE syndrome were prospectively evaluated.
RESULTS: After a mean follow-up of 8.5 years, three patients presented with an angiomatous aspect of the oral mucosa, two with hearing loss, and two with otoscopic abnormalities. No patients developed ophthalmological abnormalities. The neurological examination was altered in three cases. Brain magnetic resonance imaging follow-up was unchanged in three out four patients and revealed atrophy of the cerebellar vermis in 1 patient. Neurodevelopmental disorders were found in five of the patients and learning difficulties were observed in five patients. The S1 location appears to be associated with a higher risk of neurodevelopmental disorders and cerebellar malformations, while the S3 location was associated with more progressive complications, including neurovascular, cardiovascular, and ENT abnormalities.
CONCLUSIONS: Our study reported late complications in patients with a large segmental IH of the face or neck, whether associated with PHACE syndrome or not, and we proposed an algorithm to optimize the long-term follow-up.
METHODS: Patients with a history of large segmental IHs of the face or neck. diagnosed between 2011 and 2016 were included in the study. Each patient underwent an ophthalmological, dental, ENT (ear, nose, and throat), dermatological, neuro-pediatric, and radiological assessment at inclusion. Eight patients including five with PHACE syndrome were prospectively evaluated.
RESULTS: After a mean follow-up of 8.5 years, three patients presented with an angiomatous aspect of the oral mucosa, two with hearing loss, and two with otoscopic abnormalities. No patients developed ophthalmological abnormalities. The neurological examination was altered in three cases. Brain magnetic resonance imaging follow-up was unchanged in three out four patients and revealed atrophy of the cerebellar vermis in 1 patient. Neurodevelopmental disorders were found in five of the patients and learning difficulties were observed in five patients. The S1 location appears to be associated with a higher risk of neurodevelopmental disorders and cerebellar malformations, while the S3 location was associated with more progressive complications, including neurovascular, cardiovascular, and ENT abnormalities.
CONCLUSIONS: Our study reported late complications in patients with a large segmental IH of the face or neck, whether associated with PHACE syndrome or not, and we proposed an algorithm to optimize the long-term follow-up.
摘要：
背景：婴儿血管瘤（IHs）可以是PHACE（后颅窝异常，血管瘤,动脉异常,心脏异常,眼睛异常)节段性时的综合征，广泛的，并位于面部或颈部。初步评估是成文的，众所周知的，但没有对这些患者进行随访的建议。这项研究的目的是评估不同相关异常的长期患病率。
方法：有面部或颈部大段IH病史的患者。2011年至2016年间诊断的病例纳入研究.每个病人都接受了眼科检查，牙科,ENT(耳朵,鼻子,和喉咙)，皮肤病学,神经儿科,和纳入时的放射评估。前瞻性评估了8例患者，其中5例患有PHACE综合征。
结果：经过平均8.5年的随访，三名患者表现为口腔粘膜的血管瘤样，两个有听力损失，还有两个有耳镜异常。没有患者出现眼科异常。3例神经系统检查改变。在四分之三的患者中，大脑磁共振成像随访没有变化，并且在1例患者中发现了小脑部的萎缩。其中五名患者发现神经发育障碍，五名患者观察到学习困难。S1位置似乎与神经发育障碍和小脑畸形的高风险有关。虽然S3位置与更多进行性并发症相关，包括神经血管,心血管,和ENT异常。
结论：我们的研究报告了面部或颈部大段IH患者的晚期并发症，无论是否与PHACE综合征相关，并提出了一种优化长期跟踪的算法。
方法：有面部或颈部大段IH病史的患者。2011年至2016年间诊断的病例纳入研究.每个病人都接受了眼科检查，牙科,ENT(耳朵,鼻子,和喉咙)，皮肤病学,神经儿科,和纳入时的放射评估。前瞻性评估了8例患者，其中5例患有PHACE综合征。
结果：经过平均8.5年的随访，三名患者表现为口腔粘膜的血管瘤样，两个有听力损失，还有两个有耳镜异常。没有患者出现眼科异常。3例神经系统检查改变。在四分之三的患者中，大脑磁共振成像随访没有变化，并且在1例患者中发现了小脑部的萎缩。其中五名患者发现神经发育障碍，五名患者观察到学习困难。S1位置似乎与神经发育障碍和小脑畸形的高风险有关。虽然S3位置与更多进行性并发症相关，包括神经血管,心血管,和ENT异常。
结论：我们的研究报告了面部或颈部大段IH患者的晚期并发症，无论是否与PHACE综合征相关，并提出了一种优化长期跟踪的算法。
