Characterization of Patient-Derived GNAQ Mutated Endothelial Cells from Capillary Malformations.

Capillary malformations (CM) (port-wine stains) are congenital skin lesions that are characterized by dilated capillaries and postcapillary venules. CMs are caused by altered functioning of the vascular endothelium. Somatic genetic mutations have predominantly been identified in the endothelial cells of CMs, providing an opportunity for the development of targeted therapies. However, there is currently limited in-depth mechanistic insight into the pathophysiology and a lack of preclinical research approaches. In a monocenter exploratory study of 17 adult patients with CMs, we found somatic sequence variants in the GNAQ (p.R183Q, p.R183G, or p.Q209R) or GNA11 (p.R183C) genes. We applied an endothelial-selective cell isolation protocol to culture primary endothelial cells from skin biopsies from these patients. We successfully expanded patient-derived cells in culture in 3 of the 17 cases while maintaining endothelial specificity as demonstrated by vascular endothelial-cadherin immunostainings. In addition, we tested the angiogenic capacity of endothelial cells from a patient with a GNAQ (p.R183G) sequence substitution. These proof-of-principle results reveal that primary cells isolated from CMs may represent a functional research model to investigate the role of endothelial somatic mutations in the etiology of CMs, but improved isolation and culture methodologies are urgently needed to advance the field.

Healthcare professionals' views on shared decision-making in plastic surgery in the Netherlands.

BACKGROUND: In shared decision-making (SDM), patients and healthcare professionals (HCPs) reach a joint clinical decision based on the best available evidence and the patient's preferences. SDM seems particularly valuable in plastic surgery, as often multiple treatment options are available. This cross-sectional online survey study aimed to assess HCPs' views and knowledge about SDM, identify facilitators and barriers of SDM, and determine specific requirements for SDM within plastic surgery. METHODS: Participants were HCPs working in plastic surgery in the Netherlands. Participant characteristics, SDM knowledge, perceived facilitators and barriers, and requirements were assessed using a custom-made online survey. Two researchers thematically analyzed qualitative data. RESULTS: We received 124 responses (with a response rate of 23%). Most respondents were attending plastic surgeons (79%), and 60% had more than 10 years of experience. Almost all respondents considered SDM important (91%), and most (78%) indicated that they applied SDM during consultations. However, only 15% of the HCPs showed a comprehensive understanding of the principle of SDM. Sufficient time, available sources of information (on treatment options and SDM), and decision support tools were identified as important requirements for SDM. CONCLUSIONS: Despite the positive attitudes toward SDM, there is a clear need for SDM training of HCPs, uniform sources of information and guidelines, and improved awareness and availability of decision support tools. National plastic surgery societies can play a crucial role in improving SDM-related knowledge, the availability of information and decision support tools, and the implementation of SDM in the field of plastic surgery.

Lymphatic differentiation and microvascular proliferation in benign vascular lesions of skin and soft tissue: Diagnostic features following the International Society for The Study of Vascular Anomalies Classification-A retrospective study.

Background: Discrepancies have been noted between the clinical and histologic diagnosis of vascular malformations. Objective: To evaluate the effectiveness of the International Society for Study of Vascular Anomalies (ISSVA) classification in diagnosing benign vascular anomalies based on clinical and (immuno) histologic parameters, focusing on lymphatic differentiation and vascular proliferation. Method: A retrospective study of 121 consecutive patients with benign skin and soft-tissue vascular anomalies located in the head and neck region (pyogenic granulomas and angioma senilis were excluded) by applying multiplex immunohistochemistry staining for lymph vessels (D2-40), endothelial blood vessels, and proliferating cells (Ki67). Clinical and histologic diagnosis was revised after the ISSVA classification. Results: Initially, 64 lesions were diagnosed as tumors and 57 as malformations. Revision diagnosis following the ISSVA classification revealed 27 tumors, 90 malformations (22.2% lymphatic), and 4 non-ISSVA. Immunostaining showed lymphatic differentiation in 24 (19.8%) of 121 cases, of which 20 were malformations. Proliferative activity (Ki67+) was found in 41 (33.8%) of 121 cases, of which 8 were arteriovenous malformations. Limitation: Quality and size of materials (biopsies vs resections) and clinical information. Conclusion: The diagnostic accuracy of combined histologic and clinical approaches for identifying vascular anomalies following the ISSVA classification can be substantially enhanced by incorporating additional immunostaining techniques to evaluate lymphatic differentiation and proliferative activity, particularly in identifying the occurrence of vascular malformations.

Microvascular proliferation in arteriovenous malformation of the hand worsens during pregnancy: a case report.

Arteriovenous malformations (AVMs) are rare congenital disorders characterized by episodes of disproportionate growth that can cause pain and severe bleeding, with microvascular proliferation (MVP) associated with these episodes. Hormonal influences can also worsen the symptoms in patients with AVM. Case presentation: This case report presents a female patient with congenital vascular malformations of the left hand since birth, whose symptoms worsened during puberty and pregnancy, ultimately leading to amputation of the left hand due to unbearable pain and loss of function. Pathologic analysis revealed substantial MVP activity within the tissues of the AVM, with an expression of receptors for estrogen, growth hormone, and follicle-stimulating hormone in the vessels of the AVM, including MVP areas. Resected materials not related to pregnancy revealed chronic inflammation and fibrosis but hardly any MVP. Discussion and conclusion: These findings suggest a role for MVP in the progressive growth of AVM during pregnancy, with a potential role for hormonal influences. The case highlights the relationship between AVM symptoms and size during pregnancy and the pathological findings of MVP areas within the AVM with hormone receptor expression on proliferating vessels in resected materials.

Gluteal Fasciocutaneous Flap Reconstruction After Salvage Surgery for Pelvic Sepsis.

BACKGROUND: Chronic pelvic sepsis mostly originates from complicated pelvic surgery and failed interventions. This is a challenging condition that often requires extensive salvage surgery consisting of complete debridement with source control and filling of the dead space with well-vascularized tissue. OBJECTIVE: This study aimed to describe the outcomes of gluteal fasciocutaneous flaps for the treatment of secondary pelvic sepsis. DESIGN: Retrospective single-center cohort study. SETTINGS: Tertiary referral center. PATIENTS: Patients who underwent salvage surgery for secondary pelvic sepsis between 2012 and 2020 using a gluteal flap were included in this study. MAIN OUTCOME MEASURES: Percentage of complete wound healing. RESULTS: In total, 27 patients were included, of whom 22 underwent index rectal resection for cancer and 21 had undergone (chemo)radiotherapy. A median of 3 (interquartile range, 1-5) surgical and 1 (interquartile range, 1-4) radiological interventions preceded salvage surgery during a median period of 62 (interquartile range, 20-124) months. Salvage surgery included partial sacrectomy in 20 patients. The gluteal flap consisted of a V-Y flap in 16 patients, superior gluteal artery perforator flap in 8 patients, and a gluteal turnover flap in 3 patients. Median hospital stay was 9 (interquartile range, 6-18) days. During a median follow-up of 18 (interquartile range, 6-34) months, wound complications occurred in 41%, with a reintervention rate of 30%. The median time to wound healing was 69 (interquartile range, 33-154) days, with a complete healing rate of 89% at the end of follow-up. LIMITATIONS: Retrospective design and heterogeneous patient population. CONCLUSIONS: In patients undergoing major salvage surgery for chronic pelvic sepsis, the use of gluteal fasciocutaneous flaps is a promising solution because of the high success rate, limited risks, and relatively simple technique. See Video Abstract at http://links.lww.com/DCR/C160 . RECONSTRUCCIN CON COLGAJO FASCIOCUTNEO GLTEO DESPUS DE UNA CIRUGA DE RESCATE POR SEPSIS PLVICA: ANTECEDENTES:La sepsis pélvica crónica esta causada principalmente por cirugías pélvicas complicadas e intervenciones fallidas. Esta es una condición desafiante que a menudo requiere una cirugía de rescate extensa que consiste en un desbridamiento completo controlando el orígen infeccioso y rellenando el espacio muerto con tejido bien vascularizado, como por ejemplo un colgajo de tejido autólogo. La pared abdominal (colgajo de recto abdominal) o la pierna (colgajo de gracilis) se utilizan principalmente como sitios donantes para esta indicación, mientras que los colgajos glúteos pueden ser alternativas atractivas.OBJETIVO:Describir los resultados de los colgajos fasciocutáneos glúteos en el tratamiento de la sepsis pélvica secundaria.DISEÑO:Estudio de cohortes retrospectivo en un solo centro.AJUSTES:Centro de referencia terciario.PACIENTES:Todos aquellos que se sometieron a cirugía de rescate por sepsis pélvica secundaria entre 2012 y 2020 utilizando un colgajo fasciocutáneo glúteo.PRINCIPALES MEDIDAS DE RESULTADO:El porcentaje de cicatrización completa de la herida.RESULTADOS:En total, se incluyeron 27 pacientes, de los cuales 22 fueron sometidos a resección rectal por cáncer indicada y 21 pacientes que habían recibido (quimio)radioterapia. Una mediana de tres (RIC 1-5) intervenciones quirúrgicas y una (RIC 1-4) intervenciones radiológicas precedieron a la cirugía de rescate durante una mediana de 62 (RIC 20-124) meses.La cirugía de rescate incluyó una resección parcial del sacro en 20 pacientes. El colgajo fasciocutáneo glúteo consistió en la confección de un colgajo en V-Y en 16 pacientes, un colgajo incluyendo la perforante de la arteria glútea superior en 8 y un colgajo de rotación de músculo glúteo en 3 pacientes.La mediana de estancia hospitalaria fue de nueve (RIC 6-18) días. Durante una mediana de seguimiento de 18 (IQR 6-34) meses, se produjeron complicaciones de la herida en el 41%, con una tasa de reintervención del 30%.La mediana de tiempo hasta la cicatrización de la herida fue de 69 (IQR 33-154) días con una tasa de cicatrización completa del 89 % al final del seguimiento cicatricial.LIMITACIONES:Diseño retrospectivo y población heterogénea de pacientes.CONCLUSIONES:En pacientes sometidos a cirugía mayor de rescate por sepsis pélvica crónica, el uso de colgajos fasciocutáneos glúteos es una solución prometedora debido a la alta tasa de éxito, los riesgos limitados y la técnica relativamente simple. Video Resumen en http://links.lww.com/DCR/C160 . (Traducción-Dr. Xavier Delgadillo ).

Clinical differences in sirolimus treatment with low target levels between children and adults with vascular malformations - A nationwide trial.

The clinical presentation of patients with slow-flow vascular malformations is very heterogeneous. High clinical burden and subsequent reduced health-related quality of life is something they have in common. There is an unmet medical need for these patients for whom regular treatments like surgery and embolization are either insufficient or technically impossible. Sirolimus has been reported to be effective and overall well-tolerated in most patients. However, the main limitation of sirolimus is the reported high toxicity, especially when target levels of 10-15 ng/mL are being used. We report the results of a phase IIB single-arm open-label clinical trial consisting of 68 (67 in the challenge phase and 68 in the rechallenge phase) evaluable patients (children n = 33 and adults n = 35) demonstrating that treatment with low sirolimus target levels (4-10 ng/mL) is effective in 79.1% of the patients. When sirolimus treatment was stopped, the majority of patients experienced a recurrence of symptoms, supporting prolonged or even lifelong treatment requirement. Adults experienced a higher baseline pain score compared with children, having an estimated marginal mean of 6.2 versus 4.1, p < 0.05; however, they showed a similar decrease to children. Furthermore, the pediatric population experienced less often a sirolimus-related grade I-IV adverse event (35.9% vs. 64.1%, p > 0.05) compared with adults. Additionally, response rates were higher in children compared with adults (93.8% vs. 65.7%, p < 0.05), and children responded faster (28 vs. 91 days, p < 0.05). These results suggest benefits of sirolimus in patients with slow-flow vascular malformations and support its initiation as young as possible.

Safety and effectiveness of surgical excision of medium, large, and giant congenital melanocytic nevi: A systematic review and meta-analysis.

BACKGROUND: Treatment indications of congenital melanocytic naevi (CMN) have shifted from the prevention of malignant transformation more towards the improvement of appearance and psychosocial health. Surgical excision is often preferred, but its safety and effectiveness remain unclear. OBJECTIVE: To assess the outcomes of surgical excision of medium-to-giant CMN. PRIMARY OUTCOME: safety (complications). SECONDARY OUTCOME: effectiveness (satisfaction and CMN core outcomes). METHODS: PubMed, EMBASE, and CENTRAL were searched for studies on the excision of medium-to-giant CMN and/or CMN requiring reconstruction or serial excision. Meta-analyses of safety per patient were conducted, and pooled outcomes of safety and effectiveness were presented in summary-of-findings tables. RESULTS: A total of 1444 studies were found, of which 22 were included, evaluating 643 eligible patients. Study quality varied, and reporting of baseline characteristics and outcomes was heterogeneous. Pooled proportions were overall 9.8% for major wound-related complications, 1.2% for minor wound-related complications, 1.2% for scar-related complications, and 4.3% for anatomical deformities. For large/giant CMN, complication rates were, respectively, 23.1%, 2.9%, 12.9%, and 2.4%; and for CMN with eyelid involvement, 0.5%, 3.3%, 0.4%, and 54.2%. Patients rated their satisfaction with the cosmetic outcome as 24.4% excellent, 71.0% good, and 4.6% poor/moderate. Physicians rated this as 18.3% excellent, 70.1% good, and 11.7% poor/moderate. Thirty-five other outcomes of effectiveness were summarized. However, many were rarely reported. CONCLUSIONS: Surgical excision of CMN appears to be safe and effective in many cases, depending on CMN size and location. Major wound-related complications and scar-related complications occurred more frequently with large/giant CMN, whereas anatomical deformities occurred with the majority of CMN with eyelid involvement.

#MadelungDeformity: Insights Into a Rare Congenital Difference Using Social Media.

BACKGROUND: Madelung deformity is a rare congenital hand difference with little known regarding the patient perspective. In this cross-sectional survey study, we harnessed the global reach of social media to understand the clinical spectrum of Madelung deformity and its impact on physical, mental, and social health. METHODS: A survey was developed based on a previously published protocol and multiple Patient-Reported Outcomes Measurement Information System (PROMIS) short forms. The survey was distributed on several Madelung deformity communities on Facebook and Instagram. T-scores were calculated, interpreted, and compared between patients who underwent surgery and those who did not. Correlations between scores were calculated using the Spearman rank correlation coefficient. RESULTS: Mean PROMIS scores for adults were as follows: pain intensity, 4.9 ± 2.8; pain interference, 57.6 ± 10.0; upper extremity, 35.2 ± 8.1; depression, 53.8 ± 11.1; anxiety, 55.4 ± 11.4; and ability to participate in social roles and activities, 42.5 ± 7.7. Mean scores for children were as follows: pain intensity, 5.0 ± 2.8; pain interference, 55.7 ± 11.3; upper extremity function, 24.6 ± 10.4; depressive symptoms, 57.7 ± 11.3; anxiety, 57.3 ± 11.9; and peer relationships, 42.2 ± 10.3. CONCLUSIONS: Madelung deformity has significant effects on patients' physical, mental, and social well-being, even after surgical treatment. Using social media, we were able to compensate for Madelung deformity's rarity by engaging an international audience, demonstrating the feasibility to conduct research through it, and providing a global perspective of the disease entity.

A core outcome domain set for clinical research on capillary malformations (the COSCAM project): an e-Delphi process and consensus meeting.

BACKGROUND: There is limited evidence on the best available treatment options for capillary malformations (CMs), mainly due to the absence of uniform outcome measures in trials on therapies. A core outcome set (COS) enables standard reporting of trial outcomes, which facilitates comparison of treatment results. OBJECTIVES: To develop a core outcome domain set (CDS), as part of a core outcome set (COS), for clinical research on CMs. METHODS: Sixty-seven potentially relevant outcome subdomains were recognized based on the literature, focus group sessions, and input from the COSCAM working group. These outcome subdomains were presented in an online Delphi study to CM experts (medical specialists and authors of relevant literature) and (parents of) patients with CM (international patient associations). During three e-Delphi study rounds, the participants repeatedly scored the importance of these outcome subdomains on a seven-point Likert scale. Participants could also propose other relevant outcome subdomains. Consensus was defined as ≥ 80% agreement as to the importance of an outcome subdomain among both stakeholder groups. The CDS was finalized during an online consensus meeting. RESULTS: In total 269 participants from 45 countries participated in the first e-Delphi study round. Of these, 106 were CM experts from 32 countries, made up predominantly of dermatologists (59%) and plastic surgeons (18%). Moreover, 163 (parents of) patients with CM from 28 countries participated, of whom 58% had Sturge-Weber syndrome. During the two subsequent e-Delphi study rounds, 189 and 148 participants participated, respectively. After the entire consensus process, consensus was reached on 11 outcome subdomains: colour/redness, thickness, noticeability, distortion of anatomical structures, glaucoma, overall health-related quality of life, emotional functioning, social functioning, tolerability of intervention, patient satisfaction with treatment results, and recurrence. CONCLUSIONS: We recommend the CDS to be used as a minimum reporting standard in all future trials of CM therapy. Our next step will be to select suitable outcome measurement instruments to score the core outcome subdomains. What is already known about this topic? Besides physical and functional sequelae, capillary malformations (CMs) often cause emotional and social burden. The lack of uniform outcome measures obstructs proper evaluation and comparison of treatment strategies. As a result, there is limited evidence on the best available treatment options. The development of a core outcome set (COS) may improve standardized reporting of trial outcomes. What does this study add? A core outcome domain set (CDS), as part of a COS, was developed for clinical research on CMs. International consensus was reached on the recommended core outcome subdomains to be measured in CM trials: colour/redness, thickness, noticeability, distortion of anatomical structures, glaucoma, overall health-related quality of life, emotional functioning, social functioning, tolerability of intervention, patient satisfaction with treatment results, and recurrence. This CDS enables the next step in the development of a COS, namely to reach consensus on the core outcome measurement instruments to score the core outcome subdomains. What are the clinical implications of this work? The obtained CDS will facilitate standardized reporting of treatment outcomes, thereby enabling proper comparison of treatment results. This comparison is likely to provide more reliable information for patients about the best available treatment options.

Neurological signs, symptoms and MRI abnormalities in patients with congenital melanocytic naevi and evaluation of routine MRI-screening: systematic review and meta-analysis.

BACKGROUND: A congenital melanocytic naevus (CMN) is a rare skin condition that can be associated with abnormalities of the central nervous system (CNS). These anomalies can sometimes cause severe complications, and rarely death. Adequate information about aetiology and management is therefore crucial. To identify how to monitor patients with CMN, we aimed to estimate the prevalence of neurological involvement in patients with CMN and to summarize what specific neurological signs and symptoms and MRI abnormalities are reported in the medical literature. In addition, we summarized and evaluated the recommendations regarding MRI-screening reported in the medical literature. METHODS: This review was registered in PROSPERO and reported according to the MOOSE checklist. A search was conducted in EMBASE (Ovid), PubMed, and the Cochrane Library. We included studies with 10 or more patients with CMN, reporting on neurological signs and symptoms or CNS MRI. Study selection, data extraction and methodological quality assessment were performed by two independent reviewers. A meta-analysis was used to assess the prevalence of neurological signs and symptoms. RESULTS: Out of 1287 studies, fourteen studies were eligible for inclusion of which eight were included in the meta-analysis. Neurological signs and symptoms prevalence was 7.04% (CI 95% 4.47-10.93%) in the meta-analysis group and 6.26% (95% CI 3.85-10%) in a subgroup of patients with a CMN > 6 cm, evaluated in seven studies. Neurodevelopmental delay and seizures were the most frequently reported signs and symptoms. CNS melanocytosis and hydrocephalus were the most frequently reported MRI abnormalities. It was not possible to estimate the increased risk of neurological involvement in patients with CMN due to low quality of evidence and clinical heterogeneity. CONCLUSION: Standardization in CMN studies and a multi-centre prospective study are needed to evaluate neurological involvement. Based on current literature, it is not possible to make strong recommendations on routine MRI-screening. For now, every clinical centre should decide on its own policy and weigh the advantages and disadvantages of routine MRI.

The long-term progression of macrodactyly.

BACKGROUND: Macrodactyly is a rare congenital disorder of overgrowth affecting the digits of the upper or lower extremity. Mostly, patients are surgically treated during childhood to reduce the digit or to stop growth. There are no standardized guidelines for the treatment and follow-up of macrodactyly. Consequently, follow-up may not be regularly scheduled into adulthood. METHODS: A retrospective, descriptive analysis of patients with the long-term progression of macrodactyly who presented at our tertiary referral hospital between July 2018 and March 2020 was performed. All patients from our local macrodactyly database were screened for progression of macrodactyly since adulthood; this resulted in four patients. The aim of these case series is to highlight the clinical features and disease course at long-term follow-up. RESULTS: All patients were surgically treated during childhood and showed progression of tissue overgrowth during adult life. All patients developed severe secondary degenerative bone changes in macrodactyly affected digits, such as ankyloses of joints, new bone formation, and bony spurs. Subsequently, tissue overgrowth and degenerative bone changes led to functional problems. CONCLUSION: Patients with macrodactyly may experience growth during adult life, which may progress to deforming changes. Consequently, patients should be informed about the possible growth, and the progressive growth should be monitored.

Clinical characteristics associated with pain in patients with peripheral vascular malformations.

OBJECTIVE: Vascular malformations (VM) can negatively impact the patient's quality of life (QoL). Pain is a common problem in these patients. The aim of this study was to investigate risk factors associated with pain and to assess how pain affects QoL. METHODS: This prospective cross-sectional study was conducted in a tertiary vascular anomaly expertise center. Between June and December 2020, all patients from our local database (334 adults and 189 children) with peripheral VMs were invited to complete the Outcome Measures for VAscular MAlformations questionnaire to evaluate the presence, frequency, and intensity of pain. Additionally, patients were asked to complete several Patient-Reported Outcome Measurement Information System scales to evaluate their QoL. Risk factors associated with pain were identified in bivariate analysis and multivariable logistic regression. QoL domains were compared between patients who experienced pain and patients who did not. RESULTS: A total of 164 patients completed the questionnaire about pain and 133 patients completed all QoL questionnaires. Approximately one-half of the patients (52%) reported pain in the past four weeks and 57% of these patients reported pain daily or several times a week. Female sex (P = .009), lesions located in the upper extremity (P < .001) or lower extremity (P < .001), and intramuscular/intraosseous lesions (P = .004) were independently associated with the presence of pain. The following QoL domains were diminished in patients who experienced pain in comparison with patients who did not: pain interference (P < .001), physical functioning (P < .001), and social participation (P < .001) in adults, and pain interference (P = .001), mobility (P = .001), and anxiety (P = .024) in children. CONCLUSIONS: Pain is a frequently reported complaint in patients with VMs and is present in approximately one-half of the patients. Patients with lesions located in the upper or lower extremity, intramuscular/intraosseous lesions, and female patients are more likely to experience pain. The presence of pain negatively impacted patients' QoL. Although VM are a benign condition and expectative management is frequently applied, our study shows that pain is a serious concern and needs to be actively assessed. Pain is a sign of various etiologies and should be examined to properly treat the pain.

Therapeutic Strategies for Untreated Capillary Malformations of the Head and Neck Region: A Systematic Review and Meta-Analyses.

BACKGROUND: Capillary malformations of the head and neck region often cause psychological and physical burden. As the effectiveness of modern laser and light therapies is still suboptimal, patients often seek different therapeutic strategies. Other recognized, but not routinely proposed therapies include cosmetic camouflage, surgery, and medical tattooing. Information on therapeutic outcomes is currently lacking for patients to adequately participate in the treatment decision-making process. OBJECTIVE: The objective of this systematic review was to review the effectiveness and safety of recognized therapies for untreated capillary malformations of the head and neck: laser and light treatment modalities, photodynamic therapy, cosmetic camouflage, medical tattooing, and surgery. METHODS: PubMed, Embase, and the Cochrane Central Register of Controlled Trials were searched up to 16 December, 2020 for observational and experimental studies examining recognized therapies for untreated capillary malformations of the head and neck. Two reviewers independently evaluated the risk of bias of included studies. Predefined treatment and safety outcomes of pooled data were scored using the Grading of Recommendations Assessment, Development and Evaluation (GRADE). RESULTS: We included 48 observational and three randomized studies (totaling 3068 patients), evaluating nine different therapies. No studies on surgery or cosmetic camouflage matched our inclusion criteria. The pooled proportion of patients reaching a ≥75% clearance was 43% (95% confidence interval 24-64%; I2 = 55%) for the pulsed dye laser after three to eight treatment sessions (GRADE score: very low). Other therapies were less effective. Hyperpigmentation was most frequently described after the pulsed dye laser (incidences up to 40%). Pain was most common after photodynamic therapy, yet the intensity was unreported. Substantial heterogeneity among studies as to patient characteristics and outcomes limited pooling and data comparisons. CONCLUSIONS: The pulsed dye laser seems preferable for treatment-naive capillary malformations of the head and neck region, yet demonstrates greater hyperpigmentation rates compared with other therapies. Our results are, however, based on low-quality evidence. Future studies using uniform outcome measures and validated metrics are warranted for study comparability. Based on this systematic review, clinicians and patients should be aware of the limited evidence about the available options when making (shared) treatment decisions for capillary malformations. TRIAL REGISTRATION: Review registration number PROSPERO database: CRD42020199445.

Variation in Treatment for Trapeziometacarpal Arthrosis.

BACKGROUND: Treatment recommendations for trapeziometacarpal (TMC) arthrosis are highly variable from surgeon to surgeon. This study addressed the influence of viewing radiographs on a decision to offer surgery for TMC arthrosis. METHODS: In an online survey, 92 hand surgeons viewed clinical scenarios and were asked if they would offer surgery to 30 patients with TMC arthrosis. Forty-two observers were randomly assigned to review clinical information alone and 50 to review clinical information as well as radiographs. The degree of limitation of daily activities, time since diagnosis, prior treatment, pain with grind, crepitation with grind, and metacarpal adduction with metacarpophalangeal hyperextension were randomized for each patient scenario to determine the influence of these factors on offers of surgery. A cross-classified binary logistic multilevel regression analysis identified factors associated with surgeon offer of surgery. RESULTS: Surgeons were more likely to offer surgery when they viewed radiographs (42% vs. 32%, P = 0.01). Other factors associated variation in offer of surgery included greater limitation of daily activities, symptoms for a year, prior splint or injection, deformity of the metacarpophalangeal joint. Factors not associated included limb dominance, prominence of the TMC joint, crepitation with the grind test, and pinch and grip strength. CONCLUSION: Surgeons that view radiographs are more likely to offer surgery to people with TMC arthrosis. urgeons are also more likely to offer surgery when people do not adapt with time and nonoperative treatment. Given the notable influence of surgeon bias, and the potential for surgeon and patient impatience with the adaptation process, methods for increasing patient participation in the decision-making process merit additional attention and study.

Recurrence Rates Following Reconstruction Strategies After Wide Excision of Hidradenitis Suppurativa: A Systematic Review and Meta-analysis.

BACKGROUND: Wide excision (WE) is generally considered to be the most common treatment for recurrent hidradenitis suppurativa. When performed, excision is followed by decisions regarding best options for management of the surgical defect. Different reconstructive strategies (RSs) have been used, with varying rates of recurrence. OBJECTIVE: To provide an up-to-date systematic review of the complete literature for different RS after WE and their recurrence rates. METHODS: A systematic literature search of the complete available literature and a meta-analysis of proportions were performed on the included studies. RESULTS: Of a total of 1,813 retrieved articles, 79 were included in the analysis. Most were retrospective analyses, with only one randomized controlled trial (RCT) and 7 prospective analyses. The RS described were divided into primary closure (PC), secondary intention healing (SIH), skin graft (SG), and fasciocutaneous flaps (FCF). The average estimated recurrence for PC was 22.0% (95% confidence interval [CI], 8.0%-40.0%), for SIH 11.0% (95% CI, 5.0%-20.0%), for SG 2.0% (95% CI, 0.0%-5.0%), and for FCF 2.0% (95% CI, 1.0%-5.0%) (p < .001). Hidradenitis suppurativa below the umbilicus was significantly associated with overall recurrence (p = .006). Quality of evidence was poor, and the reporting of results was mostly heterogeneous. CONCLUSION: After WE, PC has the highest recurrence rates, whereas SG and FCF have the lowest rates. There is a need for more RCTs and guidelines, to be able to report uniformly on treatment outcomes.

Surgical Treatment of Peripheral Vascular Malformations: A Systematic Review and Meta-Analysis.

BACKGROUND: Surgical treatment of peripheral vascular malformations is widely performed as primary and secondary treatments. Excellent results have been reported; however, it is thought that complications are likely to occur because of damage to adjacent structures. This systematic review aimed to elucidate the indications and outcomes of surgical treatment of vascular malformations. METHODS: PubMed, EMBASE, and the Cochrane Central Register of Controlled Trials were searched for studies reporting outcomes of surgery in at least 15 patients with a single type of peripheral soft-tissue vascular malformation. The authors extracted data on patient and lesion characteristics, treatment characteristics, and outcomes (including complications). Meta-analysis was conducted on recurrence and complication rates. RESULTS: A total of 3042 articles were found, of which 24 were included: nine studies on arteriovenous malformations, seven on venous malformations, and eight on lymphatic malformations, totaling 980 patients. Meta-analyses showed pooled proportions for recurrences of 11 percent in arteriovenous malformations, 5 percent in venous malformations, and 9 percent in lymphatic malformations. Pooled proportions of major complications were 9 percent for arteriovenous malformations, 3 percent for venous malformations, and 1 percent for lymphatic malformations. The authors found a 5 percent pooled recurrence proportion in total resections, compared with 28 percent in subtotal resections. The pooled odds ratio for recurrence in total and subtotal resections showed a significant lower recurrence rate after total resection (odds ratio, 0.14, p = 0.02). CONCLUSIONS: Surgical treatment of vascular malformations appears to be effective and safe in many cases. However, it seems that surgery is performed predominantly in small lesions, and subtotal resection has a higher risk of recurrence than total resection.

A Multicenter Analysis of Wide Excision and Reconstruction for Severe Anogenital Hidradenitis Suppurativa.

BACKGROUND: An appropriate reconstruction strategy after wide excision for severe cases of anogenital hidradenitis suppurativa (aHS) is important to optimize outcomes, but there is no consensus on which reconstruction strategy should be preferred. OBJECTIVE: Evaluate which reconstruction strategy after wide excision in patients with severe aHS is associated with the best outcomes in terms of recurrence rate, complications and patient-reported outcomes on range of motion, pain, appearance, sexual health and satisfaction. METHODS: Multicenter retrospective analysis between 2009 and 2019 of wide excision and reconstruction by primary closure, secondary intention healing, split-thickness skin grafts or fasciocutaneous flaps (FCF). The recurrence rate was the primary endpoint of multivariable logistic regressions to determine variables with an independent effect on recurrence. RESULTS: A total of 93 patients were included. The overall recurrence rate was 62% after a median follow-up of 43 months, without statistical significance between reconstruction strategies (P = 0.737). The number of interventions during follow-up was an independent risk factor for recurrence (odds ratio, 2.55; confidence interval, 1.24-5.25; P = 0.011). Complications (37%) were more severe after FCF (P = 0.007). The mean score regarding patient-reported outcomes was 14.9 ± 2.8, of 24, with best appearance after FCF (P = 0.042). CONCLUSIONS: These long-term follow-up data on severe aHS demonstrate a high recurrence rate after wide excision and reconstruction. Patients should be informed that treatment consists of long-term medicinal and surgical interventions with high recurrence rates, and surgeons may choose their own preferred reconstruction methods. Furthermore, more attention should be paid to the sexual health of patients with aHS.

Treatment Outcome Measurement Instruments for Port Wine Stains: A Systematic Review of Their Measurement Properties.

BACKGROUND: A plethora of outcome measurement instruments (OMIs) are being used in port wine stain (PWS) studies. It is currently unclear how valid, responsive, and reliable these are. OBJECTIVES: The aim of this systematic review was to appraise the content validity and other measurement properties of OMIs for PWS treatment to identify the most appropriate instruments and future research priorities. METHODS: This study was performed using the updated Consensus-Based Standards for the Selection of Health Measurement Instruments (COSMIN) methodology and adhered to PRISMA guidelines. Comprehensive searches in Medline and Embase were performed. Studies in which an OMI for PWS patients was developed or its measurement properties were evaluated were included. Two investigators independently extracted data and assessed the quality of included studies and instruments to perform qualitative synthesis of the evidence. RESULTS: In total, 1,034 articles were screened, and 77 full-text articles were reviewed. A total of 8 studies were included that reported on 6 physician-reported OMIs of clinical improvement and 6 parent- or patient-reported OMIs of life impact, of which 3 for health-related quality of life and 1 for perceived stigmatization. Overall, the quality of OMI development was inadequate (63%) or doubtful (37%). Each instrument has undergone a very limited evaluation in PWS patients. No content validity studies were performed. The quality of evidence for content validity was very low (78%), low (15%), or moderate (7%), with sufficient comprehensibility, mostly sufficient comprehensiveness, and mixed relevance. No studies on responsiveness, minimal important change, and cross-cultural validity were retrieved. There was moderate- to very low-quality evidence for sufficient inter-rater reliability for some clinical PWS OMIs. Internal consistency and measurement error were indeterminate in all studies. CONCLUSIONS: There was insufficient evidence to properly guide outcome selection. Additional assessment of the measurement properties of OMIs is needed, preferentially guided by a core domain set tailored to PWS.

Clinical outcome measures and scoring systems used in prospective studies of port wine stains: A systematic review.

BACKGROUND: Valid and reliable outcome measures are needed to determine and compare treatment results of port wine stain (PWS) studies. Besides, uniformity in outcome measures is crucial to enable inter-study comparisons and meta-analyses. This study aimed to assess the heterogeneity in reported PWS outcome measures by mapping the (clinical) outcome measures currently used in prospective PWS studies. METHODS: OVID MEDLINE, OVID Embase, and CENTRAL were searched for prospective PWS studies published from 2005 to May 2020. Interventional studies with a clinical efficacy assessment were included. Two reviewers independently evaluated methodological quality using a modified Downs and Black checklist. RESULTS: In total, 85 studies comprising 3,310 patients were included in which 94 clinician/observer-reported clinical efficacy assessments had been performed using 46 different scoring systems. Eighty-one- studies employed a global assessment of PWS appearance/improvement, of which -82% was expressed as percentage improvement and categorized in 26 different scoring systems. A wide variety of other global and multi-item scoring systems was identified. As a result of outcome heterogeneity and insufficient data reporting, only 44% of studies could be directly compared. A minority of studies included patient-reported or objective outcomes. Thirteen studies of good quality were found. CONCLUSION: Clinical PWS outcomes are highly heterogeneous, which hampers study comparisons and meta-analyses. Consensus-based development of a core outcome-set would benefit future research and clinical practice, especially considering the lack of high-quality trials.

Recurrence Rates Following Excision of Hidradenitis Suppurativa: A Systematic Review and Meta-analysis.

BACKGROUND: Surgery is considered to be the best treatment for recurrent hidradenitis suppurativa (HS). Varying recurrence rates have been reported in the literature. OBJECTIVE: To provide an up-to-date systematic review of the complete literature for different excision strategies and their recurrence rates in HS. METHODS: A systematic literature search of the complete available literature and a meta-analysis of proportions were performed on the included studies. RESULTS: Of a total of 1,593 retrieved articles, 125 were included in the analysis. Most of these studies were retrospective with 8 prospective analyses and one randomized controlled trial (RCT). The techniques described were divided into partial excision (PE) and wide excision (WE), described in 33 and 97 included studies, respectively. The average estimated recurrences were 26.0% (95% confidence interval [CI], 16.0%-37.0%) for PE and 5.0% (95% CI, 3.0%-9.0%) for WE (p < .01). Female sex (p = .016) and HS caudal of the umbilicus (p = .001) were significantly associated with the overall recurrence rate. Quality of evidence was poor, and the reporting of results was mostly heterogeneous. CONCLUSION: This systematic review showed higher recurrence rates when it was not intended to resect affected HS tissue with a radical margin. There is a need for more RCT's and uniformly reported treatment outcomes.