scholarly journals Sporadic Multifocal Venous Malformations of the Head and Neck

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Michael V. Amato ◽  
Neha A. Patel ◽  
Shirley Hu ◽  
Harry Pantelides

Objective. To report a case of unusually widespread sporadic venous malformations of the head and neck associated with normal D-dimer levels and, due to the protean clinical manifestations and increased risk of coagulopathy of these lesions, to review their diagnosis and clinical management.Case Report. A 25-year-old man presented with a one-year history of intermittent right-sided neck swelling and tongue swelling. Physical exam revealed additional lesions present throughout the head and neck. There was no family history suggestive of heritable vascular malformations. Radiographic imaging demonstrated 15 lesions located in various tissue layers consistent with venous malformations. A coagulation screen showed a normal prothrombin time, activated partial thromboplastin time, international normalized ratio, D-dimer level, and fibrinogen level. It was determined that the patient was not at increased risk for intraoperative coagulopathy and preoperative heparin administration would not be necessary. The patient’s buccal and tongue lesions were subsequently excised with no complications. The patient also underwent sclerotherapy evaluation for his neck mass.Conclusion. This case describes a unique presentation of sporadic multifocal venous malformations. It also emphasizes the importance of prompt diagnosis and workup when multiple venous malformations are present to prevent morbidity during surgical excision secondary to intravascular coagulopathy.

2021 ◽  
Vol 48 (6) ◽  
pp. 622-629
Author(s):  
Yun Hyun Kim ◽  
Jeong Yeop Ryu ◽  
Joon Seok Lee ◽  
Seok Jong Lee ◽  
Jong Min Lee ◽  
...  

Background Venous malformations (VMs) are the most common type of vascular malformations. Intramuscular venous malformations (IMVMs) are lesions involving the muscles, excluding intramuscular hemangiomas. The purpose of this study was to compare clinical outcomes between patients with IMVMs who were treated with sclerotherapy and those who were treated with surgical excision.Methods Of 492 patients with VMs treated between July 2011 and August 2020 at a single medical center for vascular anomalies, 63 patients diagnosed with IMVM were retrospectively reviewed. Pain, movement limitations, swelling, and quality of life (QOL) were evaluated subjectively, while radiological outcomes were assessed by qualified radiologists at the center. Complication rates were also evaluated, and radiological and clinical examinations were used to determine which treatment group (sclerotherapy or surgical excision) exhibited greater improvement.Results Although there were no significant differences in pain (P=0.471), swelling (P=0.322), or the occurrence of complications (P=0.206) between the two treatment groups, the surgical treatment group exhibited significantly better outcomes with regard to movement limitations (P=0.010), QOL (P=0.013), and radiological outcomes (P=0.017). Moreover, both duplex ultrasonography and magnetic resonance imaging showed greater improvements in clinical outcomes in the surgical excision group than in the sclerotherapy group.Conclusions Although several studies have examined IMVM treatment methods, no clear guidelines for treatment selection have been developed. Based on the results of this study, surgical excision is strongly encouraged for the treatment of IMVMs.


2016 ◽  
Vol 29 (6) ◽  
pp. 440-446 ◽  
Author(s):  
Sagar Kansara ◽  
Diana Bell ◽  
Jason Johnson ◽  
Mark Zafereo

Inflammatory pseudotumor (IP) is an uncommon idiopathic lesion that often imitates malignancy clinically and radiologically. Inflammatory pseudotumors have been found to occur in various sites but rarely in the head and neck. The histopathology, imaging, and treatment of three unique cases of head and neck inflammatory pseudotumors are described in this case series. Patients in Cases 1 and 2 presented with right level II neck mass and left parotid tail mass, respectively. The patient in Case 3 presented with otalgia, jaw pain and trismus, and a left parapharyngeal space mass. The tumors in Cases 1 and 3 significantly decreased in size with tapered courses of oral corticosteroids. The tumor in Case 2 was surgically excised without disease recurrence. Malignancy must be ruled out with incisional or excisional biopsy. Treatment includes surgical excision, oral corticosteroids, or both. The literature shows that radiotherapy and small-molecule inhibitors may be promising alternatives.


2004 ◽  
Vol 10 (1) ◽  
pp. 37-46 ◽  
Author(s):  
A. Churojana ◽  
P. Chiewwit ◽  
A. Chuangsuwanich ◽  
C. Aojanepong ◽  
O. Chawalaparit ◽  
...  

The purpose of this study is to determine the effectiveness of embolization of each type of vascular malformation. Thirty three patients with a diagnosis of vascular malformations in head and neck regions who were treated by embolization at Siriraj Hospital, Thailand, between 1997–2002 were reviewed retrospectively. There were 19 arterio venous malformations (AVMs), two arteriolar-capillary types, ten venous malformations, one veno-lymphatic malformation and one mixed capillary and venous types. The goal of treatment is to restore function and to prevent bleeding complications with particularly good cosmetic results. The technique and therapeutic agents depended on the types and flow characteristics of each malformation. Transarterial embolization with N-butyl cyanoacrylate were used in AVMs, unless no arterial route approach, then direct puncture was tried. This was carried out in five patients with AVMs. Polyvinyl alcohol was used in two capillary lesions. All venous malformations were treated by absolute alcohol injection, percutaneously. All had good outcome without serious complications. Two patients had further plastic surgery, one was AVM of eyelid and the other was facial venolymphatic malformation. Embolization is considered to be the primary treatment by eradication of those abnormal vessels directly at the target, whereas delayed plastic surgery may be needed later.


2020 ◽  
Vol 03 (01) ◽  
pp. e10-e15
Author(s):  
Yuki Sato ◽  
Takao Hamamoto ◽  
Takashi Ishino ◽  
Tsutomu Ueda ◽  
Masay Takumida ◽  
...  

AbstractVenous malformations commonly occur in the head and neck regions, in the mucous membrane of the nasal and oral cavities. Venous malformations are found in the extremities in 40% of cases, in the trunk in 20% of cases, and in the cervicofacial area in 40% of cases. However, they are rarely encountered in the parapharyngeal space. We report our experience of surgical treatment of a patient with this rare tumor. The patient was a 21-year-old man who presented with a left mandibular swelling and was admitted to a nearby hospital. He was referred to our hospital for examination and treatment. Contrast-enhanced computed tomography (CT) revealed a bulky tumor with some calcification in the parapharyngeal space; on contrast-enhanced CT, the tumor showed some enhanced effects in the arterial and venous phases. Additional enhancement magnetic resonance imaging (MRI) showed the lesion as low signal intensity on T1-weighted images and as high signal intensity on T2-weighted images, and contrast-enhanced MRI showed some enhanced effects of the lesion. Based on these findings, we suspected venous malformation and performed surgical excision. Histopathology confirmed the venous malformation, and the lesion was diagnosed as a cavernous angioma. Vascular malformations in the head and neck regions should be precisely diagnosed so that appropriate multimodality treatment can be undertaken.


Children ◽  
2021 ◽  
Vol 8 (4) ◽  
pp. 312
Author(s):  
Johanna Aronniemi ◽  
Satu Långström ◽  
Katariina A. Mattila ◽  
Anne Mäkipernaa ◽  
Päivi Salminen ◽  
...  

Introduction: Venous malformations (VMs) are congenital low-flow lesions with a wide spectrum of clinical manifestations. An increasing number of studies link VMs to coagulation abnormalities, especially to elevated D-dimer and decreased fibrinogen. This condition, termed localized intravascular coagulopathy (LIC), may pose a risk for hemostatic complications. However, detailed data on the laboratory variables for coagulation and fibrinolytic activity in VM patients are limited. We addressed this question by systematically analyzing the coagulation parameters in pediatric VM patients. Methods: We included 62 patients (median age 11.9 years) with detailed laboratory tests for coagulation and fibrinolytic activity at a clinically steady phase. We assessed clinical and imaging features of VMs and their correlations with coagulation and fibrinolysis variables using patient records and MRI. Results: D-dimer was elevated in 39% and FXIII decreased in 20% of the patients, as a sign of LIC. Elevated D-dimer and decreased FXIII were associated with large size, deep location, and diffuse and multifocal VMs. FVIII was elevated in 17% of the patients and was associated with small VM size, superficial and confined location, discrete morphology, and less pain. Surprisingly, antithrombin was elevated in 55% of the patients but without associations with clinical or other laboratory variables. Conclusions: LIC was common in pediatric patients with VMs. Our results provide a basis for when evaluating the risks of hemostatic complications in children with VMs. Further research is warranted to explore the mechanisms behind coagulation disturbances and their relation to clinical complications.


2013 ◽  
Vol 3 ◽  
pp. 13 ◽  
Author(s):  
Venkateswara Rao Chava ◽  
Ashwini Naveen Shankar ◽  
Naveen Shankar Vemanna ◽  
Sudheer Kumar Cholleti

Vascular malformations are congenital lesions that are present at birth and do not regress. However, they often present later in life. They are subdivided into two categories: (1) slow- or low-flow and (2) fast- or high-flow malformations. Low-flow malformations contain combinations of capillary, venous, and lymphatic components. Venous malformations can occur anywhere in the body, but are most frequently seen in the head and neck (40%). These lesions present in a variety of ways, from a vague blue patch to a soft blue mass, which may be single isolated or may occur in multiple areas. Treatment depends on the type of lesion, the location, degree of involvement, and the clinical symptoms. Here we are report the imaging and histopathologic findings in a patient with multiple venous malformations affecting the left side of the face and trunk.


2009 ◽  
Vol 24 (1) ◽  
pp. 38-42 ◽  
Author(s):  
S Wiegand ◽  
B Eivazi ◽  
R Karger ◽  
B Al Kadah ◽  
A M Sesterhenn ◽  
...  

Objectives The surgical treatment of congenital vascular malformations always bears the risk of severe bleeding. The aim of this study was to investigate the prevalence of haemostatic abnormalities in patients with congenital vascular malformations of the head and neck which could additionally complicate the complex surgery of vascular malformations. Methods Thirteen patients with congenital vascular malformations of the head and neck, who underwent scheduled surgery, were reviewed regarding their haemostatic tests, including prothrombin time, activated partial thromboplastin time, fibrinogen level, platelet count, platelet function analyzer-epinephrine (PFA-Epi) and -adenosine diphosphate (ADP) (PFA-100®), von Willebrand factor (vWF) and D-dimers. Results In six of 13 patients an impaired primary haemostasis was detected by prolonged PFA-Epi and one of these patients also had a slightly prolonged PFA-ADP. Additionally, two of the patients showed mild coagulation factor deficiency. One patient had a mild vWF deficiency that was not detected by PFA-100®. Conclusion Prior to surgery of congenital vascular malformations of the head and neck, a detailed coagulation analysis is needed in order to identify those patients at increased risk of bleeding. This proceeding is supported by the fact that a relevant portion of the patients with congenital vascular malformations of the head and neck show an impaired primary haemostasis.


2020 ◽  
pp. 026835552094621
Author(s):  
Yu-Yu Han ◽  
Li-Ming Sun ◽  
Si-Ming Yuan

Venous malformation is one of the slow-flow vascular malformations. Dysfunction of coagulation often occurs in most venous malformations, especially the diffuse and multifocal lesions, referred to as localized intravascular coagulopathy. It is characterized by the elevation of D-dimers and fibrin degradation products, low levels of fibrinogen, FV, FVIII, FXIII, and antithrombin III, and sometimes minor-to-moderate thrombocytopenia. Here we reviewed the clinical manifestations, pathogenesis, diagnosis, and treatment of localized intravascular coagulopathy in venous malformations.


2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Angela Musella ◽  
Innocenza Palaia ◽  
Lavinia Domenici ◽  
Assunta Casorelli ◽  
Angela Martoccia ◽  
...  

Type 1 neurofibromatosis (NF1) is a dominantly inherited neurologic disorder that affects primarily the skin, bones, and peripheral nervous system. It may be associated with a variety of clinical manifestations including cafe-au-lait spots, skinfold freckling, Lisch nodules, and visceral neurofibromas. Individuals affected by NF1 harbor an increased risk for both benign and malignant tumors. Malignant transformation is usually observed in the form of neurosarcoma. Rarely, NF1 affects the genital tract, and isolated vulvar localization is extremely rare. Here is reported a rare case of a solitary neurosarcoma of the vulva in a 43-year-old woman affected by NF1 syndrome treated with surgical excision. The purpose of this case is to underline the possibility of association between NF1 and genital tract sarcoma and to suggest an accurate evaluation of rapid growth vulvar mass in this setting.


2014 ◽  
Vol 151 (1_suppl) ◽  
pp. P63-P64
Author(s):  
Tara L. Rosenberg ◽  
Theodore D. Klug ◽  
Amanda B. Tullos ◽  
Gresham T. Richter

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