Safety and efficacy of foam sclerotherapy for treatment of low-flow vascular malformations in children

Background: Venous malformations (VMs) are a variety of low flow vascular malformations, which are developmental error of morphogenesis of veins where veins are dysplastic lined by quiescent or normal endothelium. Although surgical extirpation is the standard method for the treatment of vascular malformations, this procedure often leads to significant loss of motor function, nerve damage, or massive bleeding in patients which may endanger the life. Therefore, sclerotherapy has now been accepted as a less invasive alternative and good results have been obtained. Objectives: This study was conducted to evaluate the clinical outcomes after Foam sclerotherapy with injection Ethanolamine Oleate (EO) for the treatment of VMs in head-neck region. Methods: This quasi-experimental study was conducted in the Department of Otolaryngology- Head & Neck Surgery, Bangabandhu Sheikh Mujib Medical University (BSMMU). Forty-three patients with venous malformation in head neck region that had the inclusion criteria were enrolled as a study sample. The patients were diagnosed mostly clinically & confirmed by demonstrating non-pulsatile blood flow and venous space using Duplex ultrasound. The sclerosing solution 5% Ethanolamine Oleate (EO) was used in this study. Sclerofoam was produced using the Tessari method in 4:1 air to liquid ratio, the foam had been used within 60- 90 seconds. Results of the study were categorized as excellent, good, fair and poor. Ethical clearance was obtained from the Institutional Review Board (IRB) of BSMMU. Results: Among 43 patients 34(79.1%) patients underwent single session and 9(20.9%) were two sessions. All the lesions were responded to EO. Response to sclerotherapy categorized as excellent were in two third 29 (67.4%) patients and 14 (32.6%) had good response. No sessions resulted in poor responses. No complications occurred following any procedures. All of the sessions were performed as a day case basis without anesthesia. Conclusions: Foam sclerotherapy with injection EO appears to be safe and effective for the treatment of VMs in the head and neck region and should be considered when treating these complex lesions. Bangladesh J Otorhinolaryngol; October 2020; 26(2): 79-85

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The use of foam sclerotherapy to treat low-flow vascular malformations of the head and neck

Journal of Surgical Case Reports ◽

10.1093/jscr/rju095 ◽

2014 ◽

Vol 2014 (9) ◽

pp. rju095-rju095 ◽

Cited By ~ 3

Author(s):

I. Balasundaram ◽

I. Al-Hadad ◽

K. Rehman ◽

I. McCafferty ◽

A. Monaghan

Keyword(s):

Head And Neck ◽

Vascular Malformations ◽

Low Flow ◽

Foam Sclerotherapy

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Spontaneously Resolved Macrocystic Lymphatic Malformations: Predictive Variables and Outcomes

Plastic Surgery ◽

10.1177/2292550317693815 ◽

2017 ◽

Vol 25 (1) ◽

pp. 27-31 ◽

Cited By ~ 1

Author(s):

Michael J. Phang ◽

Douglas J. Courtemanche ◽

Marija Bucevska ◽

Claudia Malic ◽

Jugpal S. Arneja

Keyword(s):

Respiratory Tract ◽

Median Time ◽

Vascular Malformations ◽

Neck Region ◽

Retrospective Chart Review ◽

Spontaneous Resolution ◽

Low Flow ◽

Upper Respiratory Tract ◽

Lymphatic Malformations ◽

Upper Respiratory

Introduction: Lymphatic malformations are benign, low-flow vascular malformations that typically present at or near birth. Due to morbidity associated with operative treatment, nonoperative treatment with injection of sclerosant has become the mainstay of therapy. Over the past 15 years, several patients at our centre with macrocystic (>2 cm cyst size) lymphatic malformations have seen their lesions resolve spontaneously while awaiting treatment. In this study, we review features of these patients that may contribute to spontaneous resolution. Method: A retrospective chart review was conducted from our Vascular Anomalies Clinic database (1999-2014) of all macrocystic lymphatic malformations; characteristics of patients with spontaneous resolution were reviewed. Results: Of 61 patients with macrocystic lymphatic malformations, 7 cases (11.5%; 4 females, 3 males) resolved spontaneously. Median age at malformation appearance was 2 years (range: 0-6.5 years), with median age at resolution of 4 years (range: 10 months-7 years). Median time from appearance to resolution was 24 months (range: 3-43 months), with a median follow-up time of 4 years (range: 1-15 years). All but 1 case was associated with local or upper respiratory tract infection antecedent to resolution. Six of the 7 lesions were located in the neck. Conclusion: Among the cases reviewed, there was a common theme of upper respiratory tract or local infection antecedent to spontaneous lesion resolution. Compared to the literature, our proportion of malformations presenting after birth and the proportion of malformations presenting in the neck region were higher than those of other series. Although side effects associated with treatment are generally mild and/or rare, risks related to sclerotherapy and the accompanying requirement for general anesthesia in pediatric populations nevertheless exist. As the median time from lesion appearance to resolution was 24 months, it may be reasonable to observe these malformations for up to 24 months before proceeding with treatment if the lesion does not impair function and disfigurement is not considerable, particularly if the lesion presents after birth and/or is located in the neck region.

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Bleomycin for orbital and peri-orbital veno-lymphatic malformations – A systematic review

Interventional Neuroradiology ◽

10.1177/1591019920972514 ◽

2020 ◽

pp. 159101992097251

Author(s):

Khunsa Faiz ◽

Stephanos Finitsis ◽

Janice Linton ◽

Jai Jai Shiva Shankar

Keyword(s):

Systematic Review ◽

Pulmonary Fibrosis ◽

Vascular Malformations ◽

Symptomatic Improvement ◽

Low Flow ◽

Cochrane Database ◽

Objective Reduction ◽

Retrospective Cohort Studies ◽

Subjective Reduction

Background Orbital and peri-orbital venolymphatic malformations (VLM) are low flow vascular malformations. Intralesional bleomycin is now commonly being used to treat such malformations. Objective The purpose of this systematic review is to synthesize evidence on the safety and efficacy of bleomycin/pingyangmycin sclerotherapy for the treatment of orbital and peri-orbital VLM. Methods We searched Medline, Embase, Scopus and Cochrane database for studies reporting outcomes of bleomycin/pingyangmycin sclerotherapy for orbital and peri-orbital VLM between 1974 to April 5th, 2019. Nine retrospective cohort studies enrolling 132 patients were included. Two reviewers independently screened and extracted data and assessed the risk of bias. Predefined outcome measures were subjective and objective reduction of the lesion and associated complications. Results Subjective reduction of the lesions was seen in 96.2% of the studies. Objective reduction of the lesion and symptomatic improvement were reported in 91.6 and 95% of the studies respectively. Non responders were 9.0%. Minor adverse events were reported in 18.1% of the studies. Major complications like pulmonary toxicity or pulmonary fibrosis was not encountered in any of the included studies. Quality of evidence was generally low. Conclusion Bleomycin/pingyangmycin sclerotherapy is very effective and relatively safe for the treatment of orbital and periorbital VLM and is not associated with any major side effects including pulmonary fibrosis. Limitations: The systematic review is limited mainly due to low quality of the included studies with retrospective design.

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Management of Low-Flow Vascular Malformations: Clinical Presentation, Classification, Patient Selection, Imaging and Treatment

CardioVascular and Interventional Radiology ◽

10.1007/s00270-015-1085-4 ◽

2015 ◽

Vol 38 (5) ◽

pp. 1082-1104 ◽

Cited By ~ 20

Author(s):

Ian McCafferty

Keyword(s):

Patient Selection ◽

Clinical Presentation ◽

Vascular Malformations ◽

Low Flow

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Transcriptome-wide Profiling of Cerebral Cavernous Malformations Patients Reveal Important Long noncoding RNA molecular signatures

Scientific Reports ◽

10.1038/s41598-019-54845-0 ◽

2019 ◽

Vol 9 (1) ◽

Cited By ~ 2

Author(s):

Santhilal Subhash ◽

Norman Kalmbach ◽

Florian Wegner ◽

Susanne Petri ◽

Torsten Glomb ◽

...

Keyword(s):

Noncoding Rna ◽

Vascular Malformations ◽

Cerebrovascular Disorders ◽

Gene Set Enrichment Analysis ◽

Differentially Expressed ◽

Low Flow ◽

Pcr Analysis ◽

Cerebral Cavernous Malformations ◽

Cavernous Malformations ◽

Non Coding Rnas

AbstractCerebral cavernous malformations (CCMs) are low-flow vascular malformations in the brain associated with recurrent hemorrhage and seizures. The current treatment of CCMs relies solely on surgical intervention. Henceforth, alternative non-invasive therapies are urgently needed to help prevent subsequent hemorrhagic episodes. Long non-coding RNAs (lncRNAs) belong to the class of non-coding RNAs and are known to regulate gene transcription and involved in chromatin remodeling via various mechanism. Despite accumulating evidence demonstrating the role of lncRNAs in cerebrovascular disorders, their identification in CCMs pathology remains unknown. The objective of the current study was to identify lncRNAs associated with CCMs pathogenesis using patient cohorts having 10 CCM patients and 4 controls from brain. Executing next generation sequencing, we performed whole transcriptome sequencing (RNA-seq) analysis and identified 1,967 lncRNAs and 4,928 protein coding genes (PCGs) to be differentially expressed in CCMs patients. Among these, we selected top 6 differentially expressed lncRNAs each having significant correlative expression with more than 100 differentially expressed PCGs. The differential expression status of the top lncRNAs, SMIM25 and LBX2-AS1 in CCMs was further confirmed by qRT-PCR analysis. Additionally, gene set enrichment analysis of correlated PCGs revealed critical pathways related to vascular signaling and important biological processes relevant to CCMs pathophysiology. Here, by transcriptome-wide approach we demonstrate that lncRNAs are prevalent in CCMs disease and are likely to play critical roles in regulating important signaling pathways involved in the disease progression. We believe, that detailed future investigations on this set of identified lncRNAs can provide useful insights into the biology and, ultimately, contribute in preventing this debilitating disease.

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Pilot study in patients with congenital low-flow vascular malformation treated with low dose sirolimus

10.21203/rs.3.rs-132412/v1 ◽

2020 ◽

Author(s):

Veroniek Harbers ◽

Gerard Rongen ◽

van der Carine Vleuten ◽

Bas Verhoeven ◽

de Peter Laat ◽

...

Keyword(s):

Pilot Study ◽

Adverse Events ◽

Vascular Malformation ◽

Low Dose ◽

Liver Toxicity ◽

Vascular Malformations ◽

Low Flow ◽

Bone Marrow Toxicity ◽

Serious Adverse Events ◽

Grade Iii

Abstract Background Patients with congenital low-flow vascular malformations (capillary (CM), lymphatic (LM), venous (VM) or combined) may have an impaired quality of life (QoL), due to their symptoms, which include pain, swelling, bleeding, thrombosis, and functional impairment. Unfortunately, current treatment methods are challenging and not always successful. Previous studies have shown that the mTOR-inhibitor sirolimus is an effective treatment for these patients. Target levels of 10–15 ng/ml were well tolerated; however, grade three adverse events were observed (ranged 20–40%). Methods A pilot study was performed using a Challenge–Dechallenge–Rechallenge (CDR) design to determine the pharmacodynamics of low target levels of sirolimus (target levels 4–10 ng/ml) in respect of efficacy and adverse events in patients with disabling low-flow vascular malformations without treatment alternatives. The patients received sirolimus over a three-to-six-month period (Challenge), followed by the withdrawal of sirolimus (Dechallenge). If the complaints returned, sirolimus was reintroduced during a twelve month period (Rechallenge). Efficacy was determined on pain (end point of the pilot study) and other symptoms related to the vascular malformation; and adverse events were determined in all phases of the study. Results An improvement in symptoms was seen in 92% (n = 11/12) of patients during the Challenge phase. In the Rechallenge phase, a positive response rate of 78% was found (n = 7/9). These response rates are comparable to those found in the literature despite low target levels of sirolimus. However, less serious adverse events were observed with low dose sirolimus, especially bone marrow toxicity and grade III liver toxicity. Conclusions This pilot using low dose sirolimus showed high efficacy in patients with therapy resistant and disabling low-flow malformation, with a lower incidence of serious adverse events (especially bone marrow toxicity and grade III liver toxicity). This is extremely relevant to patients with low-flow vascular malformation, as current clinical protocols tend to advise lifelong treatment. Trial registration The pilot study was part of a phase III study. Trial registration: EudraCT number: 2016-002157-38 and ClinicalTrials.gov Identifier: NCT03987152, registered 06/14/2019 - Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT03987152?term=sirolimus&cond=Vascular+Malformations&cntry=NL&draw=2&rank=1

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Angiomatosis in the neck and mediastinum: an example of low-flow vascular malformations

European Radiology ◽

10.1007/s00330-002-1555-3 ◽

2003 ◽

Vol 13 (5) ◽

pp. 981-985 ◽

Cited By ~ 1

Author(s):

Sifrid Hendrickx ◽

Robert Hermans ◽

Guy Wilms ◽

Raf Sciot

Keyword(s):

Vascular Malformations ◽

Low Flow

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