Department of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, Wis.
Department of Anesthesiology and Pediatrics, Medical College of Wisconsin, Milwaukee, Wis.
J Thorac Cardiovasc Surg. 2016 Sep;152(3):709-17. doi: 10.1016/j.jtcvs.2016.03.082. Epub 2016 Apr 14.
PHACE syndrome is characterized by infantile hemangioma and developmental abnormalities of the brain, arteries of head and neck, and aortic arch.
We retrospectively reviewed The PHACE Syndrome International Clinical Registry to identify children with PHACE who had operative repair of aortic arch obstruction at Children's Hospital of Wisconsin.
Seven patients (median 11 months, range 1 week-6 years) with PHACE required aortic arch reconstruction from 1996 to 2015. All needed complex surgical approaches (4 conduit grafts, 2 patch aortoplasties, 1 subclavian flap) to relieve the obstruction because of long-segment transverse and proximal descending aortic arch dysplasia that included multiple areas of stricture with adjacent aneurysmal dilatation. Aberrant origin of a subclavian artery was found in 6 of 7. The 3 children who had surgery after age 1 showed significant progression of the arch obstruction and/or adjacent aneurysmal segment dilatation after their initial infant evaluation. No deaths or perioperative complications occurred despite associated cerebrovascular arterial dysplasia in 5 of 7. Recurrent arch obstruction developed in 3 of 7 at an intermediate follow-up interval of 6.2 years (2 had interposition graft replacement at 8 and 11 years due to somatic growth; 1 had repeat patch aortoplasty 11 months after initial repair secondary to recurrent stenosis).
Extensive aortic arch reconstruction is commonly required in children with PHACE syndrome and coarctation due to the bizarre nature of the obstruction. Complete preoperative imaging is needed to fully characterize the aortic and cerebrovascular arterial anomalies. Recurrent obstruction is common given the non-native tissue techniques needed to relieve the arch anomaly.
PHACE 综合征的特征是婴儿血管瘤和头颈部动脉及主动脉弓的发育异常。
我们回顾性地审查了 PHACE 综合征国际临床登记处的数据，以确定在威斯康星州儿童医院接受主动脉弓阻塞手术修复的 PHACE 患儿。
1996 年至 2015 年期间，7 例（中位数 11 个月，范围 1 周-6 岁）PHACE 患儿需要主动脉弓重建。所有患者均因长节段的主动脉弓横部和降部发育不良，包括多个狭窄区域和相邻的动脉瘤样扩张，需要复杂的外科方法（4 个管道移植物、2 个补片主动脉成形术、1 个锁骨下瓣）来解除阻塞。7 例中有 6 例存在异常起源的锁骨下动脉。3 例在 1 岁后接受手术的患儿，在最初婴儿评估后的随访中，弓部阻塞和/或相邻动脉瘤样扩张明显进展。尽管 5 例患儿存在脑血管动脉发育不良，但无死亡或围手术期并发症。7 例中有 3 例在中期随访 6.2 年后出现复发性弓部阻塞（2 例因身体生长在 8 岁和 11 岁时进行了间置移植物置换；1 例在初次修复后 11 个月因再次狭窄进行了补片主动脉成形术）。
由于阻塞的奇异性质，PHACE 综合征和缩窄患者通常需要广泛的主动脉弓重建。需要进行完整的术前影像学检查以充分描述主动脉和脑血管动脉异常。鉴于需要采用非原生组织技术来缓解弓部异常，复发性阻塞很常见。
