Benzar Iryna M, Levytskyi Anatolii F, Pylypko Vlasii M
Bogomolets National Medical University, Kyiv, Ukraine.
Wiad Lek. 2020;73(6):1267-1271.
The aim is to determine the risk factors of sternal cleft and segmental facial hemangiomas association in children with PHACES syndrome.
Materials and methods: 32 inpatient children with segmental facial hemangiomas and 19 children with sternal cleft were investigated concerning the Metry criteria of PHACES syndrome.
Results: In 6 children PHACE syndrome was diagnosed. Patients with bilateral S3 hemangiomas (50%, 3/6) had airway involvement with respiratory disorders. Conservative treatment was propranolol monotherapy (66.7%, 4/6), or combination of prednisolone and propranolol (33.3%, 2/6). Duration of propranolol treatment in children with PHACES syndrome was on an average 24.25 ± 4.49 months exceeding the duration of propranolol therapy in children with isolated soft tissue lesions (p<0.05). Primary surgical treatment of sternal cleft performed in children aged 2 (n=3) and 4 (n=1) months. The later period of surgery associated with the localization of hemangioma in the surgery region. Primary repair of sternal cleft was completed successfully in all cases; partial resection of the thymus made closure easier.
Conclusions: Primary surgical correction of a sternal cleft performed in young children provides good results. Partial resection of the thymus prevents respiratory and cardiovascular complications. Preoperative propranolol treatment averts the hemorrhagic complications in children with hemangiomas in surgical region.
确定患有PHACES综合征的儿童出现胸骨裂与节段性面部血管瘤相关联的风险因素。
材料和方法：对32例患有节段性面部血管瘤的住院儿童以及19例患有胸骨裂的儿童，依据PHACES综合征的梅特里标准进行调查。
结果：6例儿童被诊断为PHACES综合征。患有双侧S3血管瘤的患者（50%，3/6）出现气道受累并伴有呼吸障碍。保守治疗为单用普萘洛尔（66.7%，4/6），或泼尼松龙与普萘洛尔联合使用（33.3%，2/6）。患有PHACES综合征的儿童普萘洛尔治疗平均时长为24.25±4.49个月，超过了患有孤立软组织病变儿童的普萘洛尔治疗时长（p<0.05）。胸骨裂的一期手术治疗在2个月大（n=3）和4个月大（n=1）的儿童中进行。手术后期与手术区域血管瘤的定位有关。所有病例均成功完成了胸骨裂的一期修复；部分切除胸腺使缝合更容易。
结论：对幼儿进行胸骨裂一期手术矫正效果良好。部分切除胸腺可预防呼吸和心血管并发症。术前普萘洛尔治疗可避免手术区域有血管瘤的儿童出现出血性并发症。
