Raol Nikhila, Caruso Paul, Hartnick Christopher J
Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA.
Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts, USA.
Ann Otol Rhinol Laryngol. 2015 Apr;124(4):261-5. doi: 10.1177/0003489414554943. Epub 2014 Oct 15.
This study aimed to describe syndromes where preoperative imaging may be essential in determining the course of the carotid arteries before velopharyngeal insufficiency (VPI) surgery and therefore affect surgical planning.
Records of children who underwent surgery for VPI between May 1, 2012, and October 30, 2013, at a tertiary care pediatric otolaryngology center were reviewed. Data collected included age at operation, preoperative and postoperative nasometry values, presence of underlying genetic disorders, and imaging findings.
Twenty-five patients underwent 33 surgeries for VPI, with 1 posterior pharyngeal wall augmentation, 6 Furlow palatoplasties, 11 sphincter pharyngoplasties, and 15 pharyngeal flaps. Medialized carotid vasculature was identified in 5 of 25 patients (20%). Seven syndromic patients were identified, with 3 patients having 22q11.2 deletion syndrome. All patients (100%) with 22q11.2 deletion syndrome had medialized carotid arteries. Of the 4 additional syndromic children, 2 (50%) had medial internal carotid arteries, with 1 patient diagnosed with neurofibromatosis 1 and another with Prader-Willi syndrome. Both patients underwent Furlow palatoplasty.
Whereas 22q11.2 deletion syndrome is recognized as an entity where a medial carotid is often present, other syndromic children may also demonstrate vascular variability. Although a larger study is needed to confirm an increased prevalence in these patients, clinicians may need a higher degree of suspicion while performing the physical examination. If examination findings are suggestive or the disease process may lend itself to this abnormality, vascular imaging can be considered as part of presurgical planning to help guide surgical technique and prevent complications.
本研究旨在描述一些综合征,在这些综合征中,术前影像学检查对于确定腭咽闭合不全(VPI)手术前颈动脉的走行可能至关重要,从而影响手术规划。
回顾了2012年5月1日至2013年10月30日在一家三级儿科耳鼻喉科中心接受VPI手术的儿童记录。收集的数据包括手术年龄、术前和术后鼻音测量值、潜在遗传疾病的存在情况以及影像学检查结果。
25例患者接受了33次VPI手术,其中1例进行了咽后壁增厚术,6例进行了Furlow腭裂修复术,11例进行了括约肌咽成形术,15例进行了咽瓣手术。25例患者中有5例(20%)发现颈动脉血管内移。确定了7例综合征患者,其中3例患有22q11.2缺失综合征。所有(100%)患有22q11.2缺失综合征的患者都有颈动脉内移。在另外4例综合征儿童中,2例(50%)有颈内动脉内移,其中1例被诊断为神经纤维瘤病1型,另1例被诊断为普拉德-威利综合征。这两名患者均接受了Furlow腭裂修复术。
虽然22q11.2缺失综合征被认为是一种常伴有颈动脉内移的疾病,但其他综合征儿童也可能表现出血管变异。尽管需要更大规模的研究来证实这些患者中血管变异的患病率增加,但临床医生在进行体格检查时可能需要更高的怀疑度。如果检查结果提示或疾病过程可能导致这种异常,血管成像可被视为术前规划的一部分,以帮助指导手术技术并预防并发症。