scholarly journals The Role of Interventional Radiologists in the Treatment of Congenital Lymphatic Malformations

2020 ◽  
Vol 37 (03) ◽  
pp. 285-294
Author(s):  
Julie Cronan ◽  
Anne E. Gill ◽  
Jay H. Shah ◽  
C. Matthew Hawkins
Keyword(s):  
Vascular Malformations ◽  
Pediatric Population ◽  
Mammalian Target Of Rapamycin ◽  
Disease Process ◽  
Low Flow ◽  
Complication Rates ◽  
Management Options ◽  
Lymphatic Malformations ◽  
Percutaneous Sclerotherapy

AbstractLymphatic malformations are low-flow vascular malformations that are typically apparent in the pediatric population and can cause significant functional limitations and effects on quality of life. While surgical resection has historically been the mainstay of therapy, percutaneous sclerotherapy has garnered increasing popularity due to its efficacy and low complication rates. The role of interventional radiology in the multidisciplinary management of these often complex malformations requires thorough understanding of the disease process. This article will review the pathophysiology, clinical presentation, imaging workup, and management options of lymphatic malformations. Special attention will be devoted to available sclerosants, the mammalian target of rapamycin inhibitor sirolimus, and complex lymphatic anomalies.

scholarly journals Spontaneously Resolved Macrocystic Lymphatic Malformations: Predictive Variables and Outcomes

2017 ◽  
Vol 25 (1) ◽  
pp. 27-31 ◽  
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.

Abstract 1122‐000135: Application of the Berenstein‐DeLeacy Grading Scale to Assess Treatment Outcome in Orbital Lymphatic Malformations

2021 ◽  
Vol 1 (S1) ◽  
Author(s):  
Reade De Leacy ◽  
Maximilian J Bazil ◽  
Neha Siddiqui ◽  
Stavros Matsoukas ◽  
Tomoyoshi Shigematsu ◽  
...  
Keyword(s):  
Granulation Tissue ◽  
Vascular Malformations ◽  
Low Flow ◽  
Complete Regression ◽  
Lymphatic Malformations ◽  
Interval Change ◽  
Radiologic Findings ◽  
Scale Scores ◽  
Complete Obliteration ◽  
Initial Imaging

Introduction : Lymphatic malformations (LMs) are low‐flow vascular malformations that arise as a result of erroneous vascular development during embryogenesis. Prior to the advent of the Berenstein‐De Leacy (BDL) scale, no reproducible grading system had been designed to compare sclerotherapy outcomes on the basis of radiologic findings. The soft‐tissue detail, absence of ionizing radiation, safety profile, and ubiquity of MR imaging made it an ideal technique on which the imaging‐based criteria was developed. The BDL scale ranges from 1–7 denoting complete obliteration to significant progression respectively. A “B” modifier is assigned for identification of granulation tissue in the treatment bed. We examine and validate the BDL scale on a cohort of 16 orbital LMs from our practice. Methods : Orbital LMs treated with sclerotherapy at our practice between 2000 and 2021 were assessed by an attending physician prior to initial and after final treatment to assign scale scores. The assigned scores represent changes in the orbit as defined by pre‐ and post‐septal spaces, above and below eyelids, and intra/extraconal spaces going to the coronal apex without the cavernous sinus. Results : The median age at initial imaging was 24 months (range: 1–445 months) and 108 months (range 12–528) at final imaging. The median imaging interval was 61 month. Males and females were represented in our cohort equally. Six cases presented with right orbital LMs (37.5%) and 10 presented on the left (62.5%). Six cases presented with macrocystic malformations (37.5%), five cases with microcystic (31.25%), and five cases with mixed (31.25%). 11 patients were treated with bleomycin and 5 patients were treated with bleomycin and doxycycline. BDL scale scores ranged from 2–7 with one case assigned the “B” modifier. Two cases were labelled as BDL7, or gross interval progression of the LM. Four cases were labelled as BDL6, or regression of the LM in one region with progression into a previously uninvolved/untreated area. Three cases were labelled as BDL5 with minimal or no gross interval change. One case was labelled as BDL4 and assigned the “B” modifier for partial regression with >50% estimated volume of residual malformation and granulation tissue in the treatment bed. Three cases were labelled as BDL3, or partial repression with <50% estimated volume of residual malformation. One case was labelled as BDL2 with near‐complete regression with trace residual of the lesion. No cases were labelled as BDL1, or complete regression of the lesion. Conclusions : The BDL scale was applied to a series of 16 orbital LMs to demonstrate its versatility in describing the treatment progression of this historically difficult‐to‐classify malformation. We hope visualization of BDL scores for orbital LMs will assist other interventionalists with incorporating this scale as a metric for treatment progression and outcomes.

scholarly journals Percutaneous sclerotherapy with gelified ethanol of low-flow vascular malformations of the head and neck region: preliminary results

2019 ◽  
Vol 25 (6) ◽  
pp. 459-464 ◽  
Author(s):  
Anna Maria Ierardi ◽  
◽  
Giacomo Colletti ◽  
Pierpaolo Biondetti ◽  
Margherita Dessy ◽  
...  
Keyword(s):  
Head And Neck ◽  
Vascular Malformations ◽  
Neck Region ◽  
Low Flow ◽  
Head And Neck Region ◽  
Preliminary Results ◽  
Percutaneous Sclerotherapy

scholarly journals Role of sclerotherapy in management of low flow vascular malformations

2021 ◽  
Vol 8 (4) ◽  
pp. 1234
Author(s):  
Manpreet Kaur ◽  
Parul Sachdeva ◽  
Rajan Syal ◽  
Savijot Singh
Keyword(s):  
Head And Neck ◽  
Vascular Malformations ◽  
Neck Region ◽  
Low Flow ◽  
Vascular Lesions ◽  
Surgical Removal ◽  
Complete Regression ◽  
Head And Neck Region ◽  
Life Threatening

Background: Low flow vascular malformations are most common in the head and neck region. Only symptomatic malformations require treatment. Sclerotherapy followed by surgery was considered the gold standard treatment but in the head and neck region, it may produce cosmetic and physiological defects. In the present study, multiple injections of sclerotherapy with 3% sodium tetradecyl sulphate was used for the treatment of low flow vascular malformations.Methods: Twenty cases of low flow vascular malformations of the oral cavity who presented in the outpatient department of ESIC Model Hospital, Ludhiana from 2014-2016 were selected for the study. Only significantly sized (>4 cm) and easily accessible lesions were included. Staged sequential sclerotherapy with 3% STS under strict fluoroscopy control was used as the sole treatment.Results: A total of 20 patients were taken of which, 25% required three sessions, 65% five to six sessions each and 10% required eight sessions each. All patients showed good results with complete regression and no mucosal ulceration.Conclusions: Staged sequential sclerotherapy with 3% STS should be the treatment of choice in low flow vascular lesions involving mucosal and cutaneous structures of head and neck region especially anterior two-thirds of tongue, palate, gingiva, buccal mucosa and lips. Surgical removal may affect critical neurovascular structures and cause cosmetic deformity. So the removal is advisable in life-threatening conditions, lesions requiring general anaesthesia and single sitting removal.

Role of sclerotherapy in vascular malformations

Phlebologie ◽  
2017 ◽  
Vol 46 (02) ◽  
pp. 87-91
Author(s):  
R. Mattassi
Keyword(s):  
Arteriovenous Malformations ◽  
Vascular Malformations ◽  
Complete Information ◽  
Great Variability ◽  
The Body ◽  
Diagnostic Process ◽  
Lymphatic Malformations ◽  
Sclerosing Agent ◽  
Selection Of

SummaryCongenital vascular malformations (CVM) are a heterogeneous group of vessels defects that include venous, lymphatic, arteriovenous and combined anomalies. CVM may be located in every part of the body with great variability. A precise diagnostic process is required to get complete information about the anomaly prior to decide treatment between different options: surgery, sclerotherapy, embolization and laser. Sclerotherapy has a role in each type of CVM. However, correct indication, selection of the best sclerosing agent, and knowledge of technical skills in CVM are required for a good result. In venous malformations, ethanol is the most effective sclerosing agent but experience in management is required. Lymphatic malformations, mainly in the macrocystic form, can be treated easily with different substances. The nidus of arteriovenous malformations can be occluded by alcohol sclerosis. However, because of the high hemodynamic of AVM, treatment may be difficult; a good sclerosing technique is necessary to avoid complications and to get good results, which are always possible.

scholarly journals A Step-by-step Sonographic Approach to Vascular Anomalies in the Pediatric Population: A Pictorial Essay

2021 ◽  
Author(s):  
Anushka Mittal ◽  
Rama Anand ◽  
Richa Gauba ◽  
Subhasis Roy Choudhury ◽  
Pooja Abbey
Keyword(s):  
Vascular Malformations ◽  
Pediatric Population ◽  
Vascular Supply ◽  
Clinical Findings ◽  
Vascular Anomalies ◽  
Doppler Imaging ◽  
Low Flow ◽  
Vascular Tumors ◽  
Pictorial Essay ◽  
Soft Tissue Masses

AbstractVascular anomalies are a common cause of soft-tissue masses in children and often referred for ultrasonographic (USG) evaluation. They are broadly classified as vascular tumors (hemangiomas, hemangioendotheliomas, and angiosarcomas) or vascular malformations (venous malformations, lymphatic malformations, and arteriovenous malformations). Findings on USG and Doppler imaging can be used to categorize vascular anomalies into high- or low-flow lesions, which forms the basis for further workup, diagnosis, and management. On careful evaluation of various sonographic features, in conjunction with clinical findings, an accurate clinicoradiological diagnosis can be made in most cases. Further imaging with magnetic resonance (MR) imaging or computed tomography (CT) helps in delineation of lesion extent, whereas MR or CT angiography is useful to map the vascular supply of high-flow lesions. We have illustrated and discussed a step-by-step approach to diagnose vascular anomalies using ultrasound and Doppler imaging.

Vascular birthmarks

VASA ◽  
2008 ◽  
Vol 37 (1) ◽  
pp. 5-17 ◽  
Author(s):  
Willenberg ◽  
Baumgartner
Keyword(s):  
Vascular Malformations ◽  
Treatment Strategies ◽  
Multidisciplinary Care ◽  
High Flow ◽  
Low Flow ◽  
Management Options ◽  
Benign Neglect ◽  
Current Availability ◽  
History Of ◽  
Vascular Birthmarks

Vascular birthmarks can be classified into hemangioma and vascular malformations. Hemangioma are frequent tumours of early infancy demonstrating endothelial hyperplasia, a history of rapid neonatal growth and slow involution during later childhood. Treatment of hemangioma is dependent of stage and type of the lesion. Given the current availability of drugs, lasers, and other techniques to treat hemangioma safely, philosophy of "benign neglect" should not be considered anymore. Vascular malformations show a normal endothelial turnover, being present at birth and growing commensurately with the child. Exact diagnosis by employing modern diagnostic means, which are able to differentiate low-flow from high flow lesions is important for further therapeutic management. Beside conservative treatment strategies, use of laser, sclerotherapy, interventional embolization and surgical treatment are possible management options. Patients should receive multidisciplinary care in qualified vascular centres.

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