目标:VACTERL关联或综合征包括椎体,肛门直肠,心脏,气管食管,肾,和肢体缺陷。诊断依赖于这些结构异常中至少三种的存在。本研究报告1例VACTERL综合征(VS)患者,旨在帮助牙医认识到一般方面,系统性变化,与这种情况有关的口腔护理。
方法:一名14岁女性患者,医学诊断为VS。该研究评估了系统改变的存在,药物使用,行为偏差,认知发展,和口头方面。患者表现出心血管改变,包括室间隔缺损(C),肛门闭锁(A),多指(考虑肢体改变-L),和脊柱侧凸(椎体异常的可能指征-V)。在口内检查中,发现包括牙齿拥挤,几颗牙齿的釉质矿化不足,深而隐秘的上颚,全身性牙龈炎,出血,牙龈增生,III类错牙合,和正确的单边交叉咬伤。
结论:诊断为VACTERL综合征的患者会出现明显的系统损害。研究对象表现出口腔健康受损,牙科管理中的挑战,神经精神运动发育的延迟,尽管这些并不是该综合征固有的。至关重要的是预防口腔疾病并为这些患者提供早期牙科护理,以避免由于全身损伤而需要复杂和侵入性的牙科治疗。
OBJECTIVE: The VACTERL association or Syndrome consists of Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal, and Limb defects. The diagnosis relies on the presence of at least three of these structural abnormalities. This study reports a single case of a patient with VACTERL Syndrome (VS), aiming to assist dentists in recognizing the general aspects, systemic changes, and oral care related to this condition.
METHODS: A 14-year-old female patient medically diagnosed with VS. The study evaluated the presence of systemic alterations, medication use, behavioral deviations, cognitive development, and oral aspects. The patient exhibited cardiovascular alterations including a ventricular septal defect (C), anal atresia (A), polydactyly (considered limb alteration-L), and scoliosis (a possible indication of vertebral anomalies-V). In the intraoral examination, findings included tooth crowding, enamel hypomineralization in several teeth, a deep and atretic palate, generalized gingivitis, bleeding, gingival hyperplasia, Class III malocclusion, and a right unilateral crossbite.
CONCLUSIONS: Patients diagnosed with VACTERL syndrome experience significant systemic impairments. The research subject presented compromised oral health, challenges in dental management, delays in neuropsychomotor development, though these are not inherent to the syndrome. It is crucial to prevent oral diseases and provide early dental care for these patients to avoid the need for complex and invasive dental treatments due to systemic impairments.