Surgical Management of Facial Port-Wine Stain in Sturge Weber Syndrome

Abstract

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.

Symmetry and Aesthetics in Dentistry

“…Unilateral facial port-wine stain is another congenital asymmetry, but without major macroscopic, structural malformations. Treatment is nevertheless mandatory to restore facial aesthetics [115].…”

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“…It is caused by a somatic mutation (a change in DNA that occurs after conception at the level of precursors in the affected area) [2]. Sturge Weber Syndrome is characterized by the classic triad of a facial port-wine stain, leptomeningeal angiomatosis, and glaucoma [3]. Over the years, several authors and patholophysiologists have defined this syndrome as being distributed followinh-g the territory of the trigeminal nerve with a close relationship with this nerve, it was only after 2014 that a work team led by waelchli proposed that the distribution of this syndrome followed the territories of blood vessels, embrynic blood cells from the face and not from the trigeminal Image TDM Image MRI nerve.…”

Epileptic seizures as an initial symptom for Sturge‑Weber syndrome type III: A report of two cases

“…However, the risk of bleeding and the development of complications such as subdural hematoma, albeit rare, necessitate careful monitoring. The decision for surgical intervention, particularly hemispherectomy, is carefully considered in cases with medically refractory seizures and unilateral brain involvement, acknowledging the risks and potential outcomes, especially in bilaterally affected patients ( 30 , 31 ). Furthermore, the recent addition of cannabidiol (CBD) as a treatment option represents an important development in the management of refractory seizures in SWS ( 32 ).…”