8.14 Hemangiomas, Vascular Malformations, and Lymphatic Malformations of the Head and Neck

2018 ◽  
Keyword(s):  
Head And Neck ◽  
Vascular Malformations ◽  
Lymphatic Malformations

Urgent and Emergent Embolization of Lesions of the Head and Neck in Children: Indications and Results

PEDIATRICS ◽  
1987 ◽  
Vol 80 (3) ◽  
pp. 386-394
Author(s):  
Patricia E. Burrows ◽  
Pierre L. Lasjaunias ◽  
Karel G. Ter Brugge ◽  
Olaf Flodmark
Keyword(s):  
Head And Neck ◽  
Vascular Malformations ◽  
High Flow ◽  
Vein Of Galen ◽  
Respiratory Obstruction ◽  
Facial Skeleton ◽  
Lymphatic Malformations ◽  
Proliferative Phase ◽  
Interventional Neuroradiologist ◽  
Cerebral Ischemic

Indications for and results and complications of embolization of lesions of the head and neck were analyzed retrospectively. The procedures were performed since 1980 on an emergent or urgent basis in 30 infants and children by an experienced interventional neuroradiologist in Bicetre, France. Indications for embolization included hemorrhage, occular occlusion, respiratory obstruction, CNS complications or potential complications, interference with nutrition, and functional impairment related to the effect of the lesion on the developing facial skeleton and teeth. The specific lesions included seven hemangiomas (palpebral, subglottic, and nasal) and 20 vascular malformations (maxillofacial), auricular, dural, cerebral [including three vein of Galen malformations] and spinomedullary). Embolization was efficacious in 28 of 30 patients. Hemangiomas (potentially involutive tumors) responded dramatically with arrest of the proliferative phase and shrinking of the mass. Combined hemovascular lymphatic malformations (hemolymphangiomas) of the tongue demonstrated a variable decrease in size. High-flow evolutive arteriovenous malformations involving the teeth and dura were controlled but required multiple embolizations. One infant with a vein of Galen arteriovenous malformation died. Three local complications occurred in two patients. No cerebral ischemic or femoral artery complications occurred.

scholarly journals Sirolimus: A Successful Medical Treatment for Head and Neck Lymphatic Malformations

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Steven Curry ◽  
Andrew Logeman ◽  
Dwight Jones
Keyword(s):  
Case Report ◽  
Medical Treatment ◽  
Head And Neck ◽  
Laser Therapy ◽  
Medical Therapy ◽  
Lymphatic System ◽  
Vascular Malformations ◽  
Mammalian Target Of Rapamycin ◽  
Female Infant ◽  
Lymphatic Malformations

Lymphatic malformations are abnormalities that arise in the developing lymphatic system, most frequently presenting in the head and neck. They are typically treated with sclerotherapy, laser therapy, or surgery for localized lesions. Sirolimus, an inhibitor of the mammalian target of rapamycin, is a relatively new medical therapy for the treatment of vascular malformations. This case report presents the improvements and complications seen in a female infant who was diagnosed with a large lymphatic malformation on prenatal ultrasound and has been treated with sirolimus during the first 9 months of life.

scholarly journals Lymphatic Malformation Responsive to Sirolimus in Keratinocytic Epidermal Nevus Syndrome with KRAS Mutation: A Case and Brief Literature Discussion

2021 ◽  
pp. 195-201
Author(s):  
Emily Sideris ◽  
Er Tsing Vivian Tng ◽  
Paul Chee
Keyword(s):  
Current Literature ◽  
Kras Mutation ◽  
Rare Case ◽  
Mtor Inhibitor ◽  
Vascular Malformations ◽  
Lymphatic Malformation ◽  
Literature Discussion ◽  
Lymphatic Malformations ◽  
Epidermal Nevus ◽  
Overgrowth Syndromes

We present a rare case of KRAS keratinocytic epidermal nevus syndrome with lymphatic malformation, responsive to treatment with sirolimus, an mTOR inhibitor. A brief review of the current literature regarding sirolimus use in vascular malformations, lymphatic malformations, regional overgrowth syndromes, and RASopathies is discussed.

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.

Management of Head and Neck Lymphatic Malformations

2012 ◽  
Vol 28 (06) ◽  
pp. 596-602 ◽  
Author(s):  
Karthik Balakrishnan ◽  
Jonathan Perkins
Keyword(s):  
Head And Neck ◽  
Lymphatic Malformations

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.

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