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.

Keywords: capillary malformation; encephalotrigeminal angiomatosis; mental health; port wine stain; sturge-weber syndrome.

Figure 1. November 9, 1988. Preoperative images.

A= Left profile, B= Frontal, C= Right profile

Figure 2. October 23, 1989. Postoperative procedure no. 1 images showing the raised deltopectoral flap with crusting and scaling at the donor insertion site.

A= Left profile, B= Frontal, C= Right profile, D= Extended frontal, E= Posterior

Figure 3. February 26, 1990. Postoperative procedures no. 2 and 3 images showing the deltopectoral flap covering the defect of the hemangioma excision site, with self-inflicted excoriation to the nose.

A= Left profile, B= Frontal, C= Right profile

Figure 4. April 23, 1990. Postoperative procedure no. 4 images showing local wound breakdown over the scalp and forehead areas.

A= Left profile, B= Frontal, C= Right profile

Figure 5. August 15, 1990. Postoperative procedures no. 5 and 6 images showing reconstitution of the frontal hairline and flap degeneration following failed tissue expander course.

A= Left profile, B= Frontal, C= Right profile

Figure 6. January 4, 1991. Images showing parietal and temporal scalp flap degeneration.

A= Left profile, B= Frontal, C= Right profile

Figure 7. August 12, 1991. Postoperative procedure no. 7 images showing worsening flap degeneration secondary to repeated self-inflicted trauma.

A= Left profile, B= Frontal, C= Right profile

Figure 8. November 16, 1992. Postoperative procedure no. 8 images showing complete loss of the skin graft on the scalp and forehead, and partial loss in the right eyebrow and nasolabial fold following self-removal.

A= Left profile, B= Frontal, C= Right profile

Figure 9. January 15, 1993. Images showing increased granulation tissue of the exposed scalp after debridement and cleaning with Dakin's solution.

A= Left profile, B= Frontal, C= Right profile

Figure 10. April 24, 1995. Final outcome.

A= Left profile, B= Frontal, C= Partial right profile, D= Full right profile