Literature DB >> 33585124

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

Bar Y Ainuz1, Erin Marshall2, S Anthony Wolfe3.   

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.
Copyright © 2021, Ainuz et al.

Entities:  

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

Year:  2021        PMID: 33585124      PMCID: PMC7872872          DOI: 10.7759/cureus.12637

Source DB:  PubMed          Journal:  Cureus        ISSN: 2168-8184


  18 in total

Review 1.  Pathophysiology of Sturge-Weber syndrome.

Authors:  Anne M Comi
Journal:  J Child Neurol       Date:  2003-08       Impact factor: 1.987

2.  Surgical and laser treatment of Sturge-Weber syndrome.

Authors:  Maria Giuseppina Onesti; Paolo Fioramonti; Sara Carella; Graziano Spinelli; Nicolò Scuderi
Journal:  Aesthetic Plast Surg       Date:  2009-03-19       Impact factor: 2.326

3.  Reconstruction of the Large Face and Scalp Defects of a Patient with Sturge-Weber Syndrome.

Authors:  Fawei Xu; Ying Hu; Hua Li; Li Chen; Xing He
Journal:  Facial Plast Surg       Date:  2016-06-01       Impact factor: 1.446

Review 4.  Sturge-Weber syndrome.

Authors:  R Nabbout; C Juhász
Journal:  Handb Clin Neurol       Date:  2013

5.  Surgical treatment of dermatomal capillary malformations in the adult face.

Authors:  Yoojeong Kim; Suk Joon Oh; Junsang Lee; Jihoon Yang; Sung Hoon Koh; Sung Won Jung
Journal:  Arch Plast Surg       Date:  2012-03-14

6.  Active Mental Illnesses Adversely Affect Surgical Outcomes.

Authors:  David S Lee; Laura Marsh; Mauro A Garcia-Altieri; Louisa W Chiu; Samir S Awad
Journal:  Am Surg       Date:  2016-12-01       Impact factor: 0.688

Review 7.  Capillary Malformations (Portwine Stains) of the Head and Neck: Natural History, Investigations, Laser, and Surgical Management.

Authors:  Jeong Woo Lee; Ho Yun Chung
Journal:  Otolaryngol Clin North Am       Date:  2018-02       Impact factor: 3.346

8.  Sturge-Weber syndrome: soft-tissue and skeletal overgrowth.

Authors:  Arin K Greene; Sarah F Taber; Karen L Ball; Bonnie L Padwa; John B Mulliken
Journal:  J Craniofac Surg       Date:  2009-03       Impact factor: 1.046

9.  Surgical treatment of head and neck port-wine stains by means of a staged zonal approach.

Authors:  Eric W Cerrati; Teresa M O; David Binetter; Hoyun Chung; Milton Waner
Journal:  Plast Reconstr Surg       Date:  2014-11       Impact factor: 4.730

10.  The measurable cost of complications for outpatient cosmetic surgery in patients with mental health diagnoses.

Authors:  Sunishka M Wimalawansa; Justin P Fox; R Michael Johnson
Journal:  Aesthet Surg J       Date:  2014-02       Impact factor: 4.283

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