Ainuz Bar Y, Wolfe Erin M, Wolfe S Anthony
Plastic and Reconstructive Surgery, Florida International University, Herbert Wertheim College of Medicine, Miami, USA.
Plastic and Reconstructive Surgery, University of Miami Miller School of Medicine, Miami, USA.
Cureus. 2021 Jan 11;13(1):e12637. doi: 10.7759/cureus.12637.
Sturge Weber Syndrome is characterized by the classic triad of a facial port-wine stain, leptomeningeal angiomatosis, and glaucoma. The resultant facial vascular anomaly can lead to soft tissue and bone irregularities, causing psychosocial distress and mental health morbidity. When severe, patients can opt for multi-staged surgical intervention by reconstructive surgeons to restore normal symmetry and improve the aesthetic appearance of the face. This study reports a case of surgical correction for severe facial vascular malformation resulting in poor outcomes due to the associated mental comorbidities seen in Sturge Weber Syndrome. A 37-year-old male with previously diagnosed Sturge Weber Syndrome presented to the outpatient craniofacial clinic for surgical evaluation of a large facial tuberous hemangioma. The patient underwent multiple operations for facial reconstruction including a staged full-thickness skin graft, facial recontouring, and extracranial correction of vertical orbital dystopia. The case was complicated by the patient's poorly controlled seizure disorder and psychosocial illness, resulting in self-mutilation of the repair and poor follow-up. Over the span of 10 years, the patient's mental illness caused him to fail numerous attempts at facial restoration and ultimately led to a poor final result. The psychosocial distress seen in patients with Sturge Weber Syndrome can adversely affect surgical outcomes. Physicians should be mindful of the possible complications that can arise in these patients and have the clinical means to address them.
斯特奇-韦伯综合征的特征是典型的三联征，即面部葡萄酒色斑、软脑膜血管瘤病和青光眼。由此产生的面部血管异常可导致软组织和骨骼不规则，引起心理社会困扰和心理健康问题。病情严重时，患者可选择由整形外科医生进行多阶段手术干预，以恢复正常对称性并改善面部美观。本研究报告了一例因斯特奇-韦伯综合征相关精神共病导致严重面部血管畸形手术矫正效果不佳的病例。一名先前被诊断为斯特奇-韦伯综合征的37岁男性到门诊颅面诊所就诊，接受对面部巨大结节性血管瘤的手术评估。该患者接受了多次面部重建手术，包括分期全厚皮片移植、面部轮廓重塑和眶垂直移位的颅外矫正。该病例因患者癫痫发作控制不佳和心理社会疾病而复杂化，导致修复部位自残和随访不佳。在10年的时间里，患者的精神疾病导致他多次面部修复尝试失败，最终导致最终结果不佳。斯特奇-韦伯综合征患者出现的心理社会困扰可能会对手术结果产生不利影响。医生应注意这些患者可能出现的并发症，并具备临床应对手段。
