Telangiectasias, recurrent epistaxis and a strong family history-a case of Osler- Weber-Rendu Syndrome in Pakistan.

Osler-Weber-Rendu syndrome or Hereditary Haemorrhagic Telangiectasia (HHT) is a rare condition, with very few reported cases, especially in Pakistan. As healthcare workers, we encounter multiple cases of recurrent epistaxis in the emergency as well as outpatient departments. However, patients are usually treated symptomatically without a thorough workup. HHT should be considered among the differentials for recurrent epistaxis, as a clinical diagnosis can be made with detailed family history and physical examination. Here is the case of a 58-year-old male who presented to the Gastroenterology OPD, Combined Military Hospital, Lahore, in November 2021, with complaints of generalised weakness and blood in stools. He had a history of recurrent epistaxis and telangiectasias, and further inquiry revealed a strong family history of similar symptoms. He was diagnosed as a case of Osler-Weber- Rendu Syndrome. Informed consent was taken from the patient prior to the writing of the manuscript.

A comparative study between cryo-laser cryo-sclerotherapy and sclerotherapy in the treatment of telangiectasia and reticular veins: A randomized controlled trial.

OBJECTIVE: Telangiectasias, characterized by dilated venules, are frequently observed in the lower extremities. Sclerotherapy stands out as the predominant treatment of these vascular lesions. The integration of laser therapy with a mild sclerosing agent, serving as an osmotic sclerosant, presents an enhanced cosmetic treatment approach, aiming to optimize outcomes and minimize potential adverse effects. This study sought to evaluate the feasibility, efficacy, and safety of cryo-laser and cryo-sclerotherapy (CLaCS) and compare it with injection sclerotherapy for the treatment of telangiectasia and reticular veins. METHODS: In this randomized controlled trial, individuals expressing concerns about telangiectasia and reticular veins were recruited for aesthetic treatment. The enrolled patients were prospectively randomized according to the chosen treatment technique. Group A included patients undergoing CLaCS with 70% dextrose, focusing on a single area measuring 20 cm by 20 cm. Group B included patients receiving polidocanol injection sclerotherapy for a single area of the same dimensions. RESULTS: Group A comprised 195 patients and group B comprised 197 patients. The rates of complete lesion elimination after the first, second, and third treatment sessions were 64.6%, 86.2%, and 100% in group A and 50.3%, 74.1%, and 85.3% in group B, respectively. Group A exhibited a significantly higher complete elimination rate compared with group B at the conclusion of the study (P < .001). Furthermore, group A demonstrated a statistically significant lower incidence of postprocedural pigmentation and other complications compared with group B (P < .001). These findings underscore the enhanced efficacy and safety profile associated with the CLaCS technique using 70% dextrose compared with injection sclerotherapy with polidocanol. CONCLUSIONS: CLaCS, combining cryo-laser and cryo-sclerotherapy, demonstrated superior efficacy and safety compared with traditional polidocanol sclerotherapy for treating telangiectasia and reticular veins.

The Association of Telangiectasias with Other Peripheral Vascular Lesions of Systemic Sclerosis.

Purpose: Systemic sclerosis (SSc) is a relatively rare collagenosis manifested as microvasculopathy, excessive cutaneous and visceral fibrosis in a background of autoimmune alteration. Autoimmune vasculopathy in SSc occurs early and begins with endothelial cell activation followed by blood vessel intimal proliferation in a context of defective angiogenesis. The alteration of peripheral micro and macrocirculation in SSc is evident through vascular lesions, such as Raynaud's phenomenon, telangiectasias, acrocyanosis, digital ulcers, gangrene, peripheral pulse deficiency. Our paper details the results of the study on the association between telangiectasias and other types of immune-mediated peripheral vascular lesions that can be identified in SSc. The presence of these peripheral vascular lesions can provide information about the magnitude of the peripheral vasculopathy. Patients and Methods: A total of 37 patients diagnosed with SSc, recruited from a university clinic in Bucharest between February 2019 and March 2020, were enrolled in an observational study. We evaluated the presence of telangiectasias, as a stigma of autoimmune microvasculopathy, and their association with other immune-mediated peripheral vascular lesions that may be present in SSc. Results: The presence of telangiectasias was identified in the absence, but especially in the presence of acrocyanosis and digital ulcerations, and patients with peripheral pulse deficiency almost always had telangiectasias. Less than a quarter of the patients with digital ulcers progressed unfavorably to gangrene, and only one required amputation, telangiectasias being present not only in the patient with amputation but in all patients with gangrene. Conclusion: We appreciate that telangiectasias may be the clinical expression of peripheral vasculopathy characteristic of SSc, they can often be present in association with other peripheral vascular lesions and may represent a valuable indicator for the gangrene risk of digital ulcerations in SSc.

Postoperative Pain Following Coblation of Sinonasal Hereditary Hemorrhagic Telangiectasias.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a rare, autosomal dominant disease and epistaxis is the most common symptom. This can be treated conservatively but severe cases may require operative interventions. Endoscopic endonasal coblation of HHT lesions has been used successfully but postoperative pain management has not been well described. OBJECTIVES: This study aimed to assess levels of postoperative pain and opioid use among patients with HHT who underwent coblation of sinonasal lesions. METHODS: This is a longitudinal, prospective cohort study of adult patients undergoing endoscopic endonasal coblation for treatment of HHT lesions with or without bevacizumab injection between November 2019 and March 2020 at a single academic university hospital. Patients were given preoperative questionnaires and contacted via telephone 48 hours after surgery. If they reported using opioids for pain control, they were called every 2 days until they no longer used these medications. RESULTS: Fourteen cases, including 13 unique patients, were included in this study. Opioids were ordered on discharge in 4 cases and the average morphine milligram equivalent prescribed on discharge was 41. The median pain score on postoperative day (POD) 2 was 4 of 10. Twelve patients reported using acetaminophen and 4 were using opioid pain medications. Of those using opioid pain medications, only 1 patient was using opioid pain medication by POD 4 and denied any use after POD 10. CONCLUSION: This study is the first to analyze postoperative pain management and opioid prescribing patterns in HHT patients undergoing endonasal coblation of telangiectasias. Postoperative pain was mild to moderate and most patients stopped using opioid medications by POD 4, although the majority of patients solely used acetaminophen. Future studies with increased sample size will be useful to further identify predictors of need for analgesics postoperatively and other non-opioid adjuncts for pain control.

Managing High Cardiac Output Failure in a Patient With Hereditary Hemorrhagic Telangiectasias During Pregnancy.

Hemorrhagic telangiectasias is a rare genetic vascular disorder that may complicate pregnancy. We report a case of a pregnant hemorrhagic telangiectasias patient with innumerable hepatic arteriovenous malformations that developed high output cardiac failure necessitating delivery. Postpartum, the patient was treated with bevacizumab that resulted in clinical improvement.

MINI-INVASIVE TREATMENT METHODS OF SPIDER VEINS: SCLEROTHERAPY AND RADIOFREQUENCY THERMOCOAGULATION.

OBJECTIVE: The aim: This study was conducted to compare the results of spider vein: sclerotherapy or radiofrequency thermocoagulation. PATIENTS AND METHODS: Materials and methods: The study included 52 patients with spider veins, who were randomized into two treatment groups: sclerotherapy or radiofrequency thermocoagulation. Treatment outcomes were assessed using: a self-assessed questionnaire, CIVIQ 20 questionnaire, computer evaluation of images, registration relapses complications, negative manifestations, and intensity of the pain syndrome. RESULTS: Results: Both methods showed a statistically significant difference in the quality of life indicators before and one month after treatment (p<0.001 for both groups). Radiofrequency thermocoagulation showed a greater impact on the patient's quality of life (p = 0.003). The average length of spider veins in the treatment area decreased the most with radiofrequency thermocoagulation (by 92.1%), slightly less after sclerotherapy (by 73.4%) (p < 0,01). CONCLUSION: Conclusions: Both treatments have shown good results for spider veins and were reasonably safe with few negative manifestations. Radiofrequency coagulation better eliminates small veins, less than 0.3 mm.

Evaluating the Quality, Readability, and Activity of Online Information on Brain Arteriovenous Malformations.

Introduction Brain arteriovenous malformations (AVMs) are vascular deformities created by improper connections between arteries and veins, most commonly in the brain and spinal cord. The management is complex and patient-dependent; further understanding of patient education activities is imperative. Internet access has become more ubiquitous, allowing patients to utilize a large database of medical information online. Using Google Trends (GT) (Google LLC, Mountain View, CA, USA), one can see the public interest in a particular topic over time. Further, when presented with numerous search results, patients may not be able to identify the highest-yielding resources, making objective measures of information quality and readability imperative. Methods A GT analysis was conducted for "hereditary hemorrhagic telangiectasia," "cerebral aneurysm," and "arteriovenous malformation". These relative search volumes (RSV) were compared with the 2017 to 2019 annual USA AVM diagnosis quantity for correlation. These RSVs were also compared with the 2017 to 2019 annual USA deaths due to cerebral hemorrhagic conditions. One search was conducted for "brain arteriovenous malformation". Since most users looking for health information online use only the first page of sources, the quality and readability analyses were limited to the first page of results on Google search. Five quality tools and six readability formulas were used. Results Pearson's correlation coefficients showed positive correlations between USA AVM RSVs and annual AVM deaths per capita from 2017 to 2019 (R2=0.932). The AVM annual diagnosis quantity and AVM RSVs showed a strong positive correlation as well (R2=0.998). Hereditary hemorrhagic telangiectasia and cerebral aneurysms had strong positive correlations between their RSVs and their corresponding annual diagnoses in the 2017 to 2019 time period (R2=0.982, R2=0.709). One-way ANOVA, for USA's 2004 to 2021 AVM RSVs and 2004 to 2019 deaths per capita, displayed no month-specific statistically significant repeating pattern (all p>0.483). The DISCERN tool had four websites that qualified as "poor" and five as "good." The average score for the tool was "good." The Journal of the American Medical Association (JAMA) benchmark scores were very low on average, as four websites achieved zero points. There was a wide variance in the currency, relevance, authority, accuracy, and purpose (CRAAP) scores, indicating an inconsistent level of webpage reliability across results. The patient education materials assessment tool (PEMAT) understandability (86.6%) showed much higher scores than the PEMAT actionability (54.6%). No readability score averaged at or below the American Medical Association (AMA)-recommended sixth-grade reading level. Conclusion These GT correlations may be due to patients and families with new diagnoses researching those same conditions online. The seasonality results reflect that no prior research has detected seasonality for AVM diagnosis or presentation. The quality study showed a wide variance in website ethics, treatment information quality, website/author qualifications, and actionable next steps regarding AVMs. Overall, this study showed that patients are routinely attempting to access information regarding these intracranial conditions, but the information available, specifically regarding AVMs, is not routinely reliable and the reading level required to understand them is too high.

Hereditary Hemorrhagic Telangiectasia With Multiple Ear, Nose, and Throat (ENT) Manifestations: A Case Report.

Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a rare autosomal dominant multisystem disorder. It is a mucocutaneous and fibrovascular dysplasia, the diagnosis of which is based on the fulfillment of the four Curaçao criteria: 1) recurrent epistaxis; 2) dermatovascular mucosal telangiectasias at characteristic sites: skin of the face, ears, fingertips, lips, tongue, and oral and nasal cavity; 3) arteriovenous malformations (AVMs) of visceral organs and central nervous system; and 4) family history: diagnosis of HHT in a first-degree relative. We describe a case of a 76-year-old patient who presented to our department with clinical manifestations of HHT in the skin (face, fingertips), lips, hard palate, tongue, ears, and nasal cavities. Individual and family history was obtained, as well as clinical laboratory examination, pan-endoscopy of the ear, nose, and throat (ENT) systems, and treatment of active foci of bleeding from the above areas. The otolaryngologist may be the first doctor to suspect Rendu-Osler-Weber syndrome and the one responsible for treating patients with HHT since recurrent epistaxis is the most frequent (90-96% of patients) and the earlier manifestation of the disease and the main reason for the arrival of these patients in the Emergency Department. The purpose of this study is to present a clinical case of Rendu-Osler-Weber syndrome with multiple ENT manifestations, as well as a review of the literature on their management and treatment.

Low concentration of sodium tetradecyl sulfate and hypertonic glucose solution for the treatment of telangiectasia: A prospective randomized clinical trial.

OBJECTIVES: This study aimed to compare telangiectasias disappearance after sclerotherapy with hypertonic glucose (HG) and different concentrations of sodium tetradecyl sulfate (STS). METHODS: Women aged 18-70 years with telangiectasias were included. The primary efficacy endpoint was telangiectasia disappearance. The clearing of vessels was assessed using a six-point scale (from 0 to 5). RESULTS: A total of 116 women completed an 8-week follow-up: 31, 27, 25, and 33 were in the HG 75%, STS 0.05%, STS 0.1%, and STS 0.15% groups, respectively. The median score of vein disappearance was significantly lower in the STS 0.05% (3, 0.25-4), STS 0.1% (3, 1.25-4), and STS 0.15% (4, 2-4) groups than in the HG group (4, 3-5) after 56 days, p = .00002. CONCLUSION: Sclerotherapy of telangiectasias with 75% HG showed significantly better results than low concentrations of STS. TRIAL REGISTRATION: ClinicalTrials.gov NCT04132323.

Case report: Novel leg vein treatment CLaCS guided by augmented reality 11 years follow-up.

Telangiectasias is most prevalent on the lower limbs and has been estimated to manifest in 40%-90% of the population. Treatments for telangiectasias include sclerotherapy, laser therapy, intense pulsed light treatment, microphlebectomy, and thermocoagulation. Cryo-Laser & Cryo-Sclerotherapy (CLaCS) effectively combines thermal and injection sclerotherapy. In this treatment, unwanted veins are targeted by a transdermal laser and immediately receive injection sclerotherapy. During the whole procedure, an air-cooling device (Cryo) blows onto the surrounding skin and tissue to prevent skin burn. Here, we present a case report of a challenging telangiectasias treated with ClaCS.

Lasers' Q-switched treatment in skin and subcutaneous lesions - review.

The numerous medical fields like dermatology, ophthalmology and surgery widely use laser therapy including Q-switched lasers. This review aims to provide information on the application and effectiveness of Q-switched lasers in dermal and vascular lesions. Q-switched lasers play a crucial part in the athlete's foot treatment and onychomycosis both in mono- and polytherapy. Laser therapy remains the gold standard for tattoo removal. Additionally, laser therapy shows high effectiveness in melasma, telangiectasias and photoaging therapy. The ability to adjust precise laser parameters like length or beam energy provides tight control of the treated area, significantly reducing the risk of adverse effects.

Evaluation of the Pinhole Method Using Carbon Dioxide Laser on Facial Telangiectasia.

Facial telangiectasias are small, dilated blood vessels frequently located on the face. They are cosmetically disfiguring and require an effective solution. We aimed to investigate the effect of the pinhole method using a carbon dioxide (CO2) laser to treat facial telangiectasias. This study included 155 facial telangiectasia lesions in 72 patients who visited the Kangnam Sacred Heart Hospital, Hallym University. Treatment efficacy and improvement were evaluated by quantitative measurements performed by two trained evaluators who assessed the percentage of residual lesion length using the same tape measure. Lesions were evaluated before laser therapy and 1, 3, and 6 months after the first treatment. Based on the initial lesion length (100%), the average percentages of the residual length at 1, 3, and 6 months were 48.26% (p < 0.01), 4.25% (p < 0.01), and 1.41% (p < 0.01), respectively. Complications were evaluated using the Patient and Observer Scar Assessment Scale (POSAS). The average POSAS scores improved from 46.09 at the first visit to 23.42 (p < 0.01), and 15.24 (p < 0.01) at the 3- and 6-month follow-up. No recurrence was noted at the 6-month follow-up. CO2 laser treatment using the pinhole method to treat facial telangiectasias is a safe, inexpensive, and effective treatment that provides patients with excellent aesthetic satisfaction.

Quantitative analysis validation for sclerotherapy treatment of lower limb telangiectasias.

BACKGROUND: The evaluation of sclerotherapy efficacy for lower limb telangiectasias, which is the standard treatment for such condition, is commonly assisted by scores based on before and after pictures. This method is marked by its subjectivity, which impairs the precision of studies on the subject, making it unfeasible to evaluate and compare different interventions. We hypothesize that a quantitative method for evaluating the effectiveness of sclerotherapy for lower limb telangiectasias may present more reproducible results. Reliable measurement methods and new technologies may become part of the clinical practice in the near future. METHODS: Before and after treatment photographs were analyzed using a quantitative method and compared with a validated qualitative method based on improvement scores. Reliability analysis of the methods was performed, applying the intraclass correlation coefficient (ICC) and kappa coefficient with quadratic weights (Fleiss Cohen), for analysis of inter-examiner and intra-examiner agreement in both evaluation methods. Convergent validity was evaluated by applying the Spearman test. To assess the applicability of the quantitative scale, the Mann-Whitney test was used. RESULTS: A better agreement between examiners is shown for the quantitative scale, with a mean kappa of .3986 (.251-.511) for qualitative analysis and a mean kappa of .788 (.655-.918) for quantitative analysis (P < .001 for all examiners). Convergent validity was achieved by correlation coefficients of .572 to .905 (P < .001). The quantitative scale results obtained between the specialists with different degrees of experience did not show statistical difference (seniors: 0.71 [-0.48/1.00] × juniors: 0.73 [-0.34/1.00]; P = .221). CONCLUSIONS: Convergent validity between both analyses has been achieved, but quantitative analysis has been shown to be more reliable and can be applied by professionals of any degree of experience. The validation of quantitative analysis is a major milestone for the development of new technology and automated, reliable, applications.

Efficacy, tolerance and acceptability of pulsed dye laser on facial and neckline telangiectasias in systemic scleroderma: a prospective open-label monocentric study in 21 patients.

Facial and neckline telangiectasias have an underestimated yet important impact on quality of life of patients with systemic scleroderma (SSc). This monocentric, prospective, open-label, intra-patient comparative study was conducted in 21 consecutive patients with SSc. Patients underwent 4 sessions of PDL 8 weeks apart. A final quadruple assessment was performed by several raters 2 months after the last session, based on the following criteria: change in telangiectasia number; subjective improvement score (LINKERT scale); impact on the quality of life (QoL; SKINDEX score); visual analog pain scale; adverse effects (AEs), including treatment discontinuation for PDL-induced purpura and patient satisfaction. The mean telangiectasia number decreased by 5 (32%) at the end of the protocol. Eighteen patients (85.7%) reported an improvement or a strong improvement, versus 73.81% for the expert committee. Immediate session pain (mean = 3.4/10) was slightly less than overall pain (mean = 4.6/10). Ten patients (47%) experienced at least one AE (oozing/crusts, edema, epidermal blistering), including PDL-induced purpura in 3 patients (14%). AEs were mostly transient (<1 week) and mild (CTCAE grade 1). All QoL parameters improved after treatment, and 85% of patients were satisfied.