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Journal of Personalized Medicine Jun 2023Port-wine stains (PWS) are congenital low-flow vascular malformations of the skin. PWS tend to become thicker and darker with time. Laser therapy is the gold standard... (Review)
Review
BACKGROUND
Port-wine stains (PWS) are congenital low-flow vascular malformations of the skin. PWS tend to become thicker and darker with time. Laser therapy is the gold standard and the first-line therapy for treating PWS. However, some resistant PWS, or PWS that have tissue hypertrophy, do not respond to this therapy. Our aim is to evaluate the role of surgery in the treatment of PWS birthmarks.
METHODS
A literature search was performed in PubMed, Scopus, Web of Science (WOS) and Google Scholar for all papers dealing with surgery for port-wine stains, from January 2010 to December 2020 using the search strings: (capillary vascular malformation OR port-wine stains OR Sturge Weber Syndrome OR sws OR pws) AND (surgical OR surgery).
RESULTS
Ten articles were identified and used for analysis. They were almost all case series with a short follow up period and lacked an objective-systematic score of evaluation.
CONCLUSIONS
Delay in treatment of port wine stains may result in soft tissue and bone hypertrophy or nodules with disfiguring or destructive characteristics. The correction of PWS-related facial asymmetry often requires bone surgery followed by soft tissue corrections to achieve a more harmonious, predictable result.
PubMed: 37511671
DOI: 10.3390/jpm13071058 -
Life (Basel, Switzerland) Aug 2022Vascular malformations are frequent in the head and neck region, affecting the nervous system. The wide range of therapeutic approaches demand the correct anatomical,... (Review)
Review
Vascular malformations are frequent in the head and neck region, affecting the nervous system. The wide range of therapeutic approaches demand the correct anatomical, morphological, and functional characterization of these lesions supported by imaging. Using a systematic search protocol in PubMed, Google Scholar, Ebsco, Redalyc, and SciELO, the authors extracted clinical studies, review articles, book chapters, and case reports that provided information about vascular cerebral malformations, in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 385,614 articles were grouped; using the inclusion and exclusion criteria, three of the authors independently selected 51 articles about five vascular cerebral malformations: venous malformation, brain capillary telangiectasia, brain cavernous angiomas, arteriovenous malformation, and leptomeningeal angiomatosis as part of Sturge-Weber syndrome. We described the next topics-"definition", "etiology", "pathophysiology", and "treatment"-with a focus on the relationship with the imaging approach. We concluded that the correct anatomical, morphological, and functional characterization of cerebral vascular malformations by means of various imaging studies is highly relevant in determining the therapeutic approach, and that new lines of therapeutic approaches continue to depend on the imaging evaluation of these lesions.
PubMed: 36013378
DOI: 10.3390/life12081199 -
Neurosurgical Focus May 2022Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder presenting mostly with a facial port-wine stain and leptomeningeal angiomatosis. More than 85% of the... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder presenting mostly with a facial port-wine stain and leptomeningeal angiomatosis. More than 85% of the patients are affected by epilepsy by the age of 2 years. Seizure and symptom control is the focus of SWS treatment, since no causal therapy exists yet. For pharmacologically intractable epilepsy, surgery is a treatment option. The aim of this systematic review and meta-analysis was to provide an overview of the literature regarding lesionectomy in SWS with a focus on seizure outcome, complications, and motor and cognitive development.
METHODS
The PubMed and Embase databases were searched using a systematic search strategy to identify studies on SWS from their inception until 2021. Two independent researchers assessed the studies for inclusion and quality. Outcome measures were seizure outcome, postoperative complications, and motor and cognitive development. Thereafter, a systematic review was conducted, and a meta-analysis was performed for all included cohort studies. Risk of bias was assessed using the Newcastle-Ottawa Scale. Forest plots have been generated for all outcomes; risk ratio was used for pooled outcomes. A p value < 0.05 was considered as statistically significant.
RESULTS
After removal of duplicates, the authors screened 439 articles, of which 9 articles with 150 patients were included. Our case and 5 case reports and 4 retrospective cohort studies were included for systematic review. The latter 4 studies qualified for the meta-analysis. In these 4 articles, 144 patients received surgical treatment: 81 (56%) underwent focal lesionectomy and 63 (44%) hemispherectomy. Pooled outcome analysis for postoperative favorable seizure outcome showed a nonsignificant difference between lesionectomy and hemispherectomy (69.2% vs 87.3%; RR 0.73, 95% CI 0.50-1.08; t = -2.56, p = 0.08). Lesionectomy showed a significantly lower rate for developmental delay and postoperative hemiparesis in comparison with hemispherectomy (29.8% vs 76.3%; RR 0.41, 95% CI 0.28-0.59; z = -4.77, p < 0.0001 and 18.1% vs 100%; RR 0.11, 95% CI 0.06-0.21; z = -6.58, p < 0.0001, respectively).
CONCLUSIONS
Based on the limited literature available, lesionectomy leads to a nonsignificant lower seizure control rate, while postoperative developmental or motor deficits are significantly lower compared with hemispherectomy. Therefore, focal lesionectomy remains a valid alternative to hemispherectomy in SWS with a clearly localized epileptogenic area; however, individual case-based decisions in a specialized multidisciplinary team are of paramount importance.
Topics: Child, Preschool; Epilepsy; Hemispherectomy; Humans; Retrospective Studies; Seizures; Sturge-Weber Syndrome
PubMed: 35535828
DOI: 10.3171/2022.2.FOCUS21788 -
Epilepsy & Behavior : E&B Dec 2021This meta-analysis aimed to determine the main factors influencing surgical outcomes in children <3 years old with refractory epilepsy. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
This meta-analysis aimed to determine the main factors influencing surgical outcomes in children <3 years old with refractory epilepsy.
METHODS
The PubMed and Cochrane database were systematically searched for epilepsy surgery outcomes from December 1, 1991, to March 30, 2021, using the following search terms: "Epilepsy surgery OR Seizure operation" AND "under three years" OR "first three years" OR "early childhood" OR "infancy OR infants." Seizure onset, duration of epilepsy, magnetic resonance imaging findings, age at the time of surgery, surgical methods, resection extent, and pathological findings were considered potential moderators of differences in seizure outcomes. The fixed-effects models, combined effect sizes, and 95% confidence intervals (CI) were used to calculate the influence of potential factors on seizure outcomes.
RESULTS
Thirty two studies (559 cases) were included in the meta-analysis. The significant factors that correlated with a lower seizure control rate were frontal lobectomy (odds ratio [OR]: 0.33, 95% CI: 0.12-0.91; p = 0.03) and malformation of cortical development (MCD) (OR, 0.38; 95% CI: 0.24-0.62; p < 0.01). A higher seizure control rate was observed in children with tumors (92.86%) and Sturge-Weber syndrome (SWS, 91.43%). Frontal lobe epilepsy induced by MCD was related to the worst postoperative efficacy (OR, 0.26; 95% CI: 0.13-0.53; p < 0.01).
SIGNIFICANCE
The results of our meta-analyses revealed that pathology and surgical location play critical roles in the outcome of epilepsy surgery in children <3 years old. Clarification of the etiology of epilepsy before surgery is critical for better postoperative outcomes.
Topics: Child; Child, Preschool; Drug Resistant Epilepsy; Epilepsy, Frontal Lobe; Humans; Infant; Magnetic Resonance Imaging; Seizures; Treatment Outcome
PubMed: 34731717
DOI: 10.1016/j.yebeh.2021.108369