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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 2  |  Page : 242-244

Giant venous malformation of the tongue with macroglossia: A hidden menace


1 Department of Dermatology, Venereology and Leprosy, Dr. Rajendra Prasad Medical College, Kangra, Himachal Pradesh, India
2 Department of Medicine, SN Medical College and Hospital, Jodhpur, Rajasthan, India
3 Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
4 Department of Radiodiagnosis, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Date of Submission22-Jun-2020
Date of Decision26-Sep-2020
Date of Acceptance03-Feb-2021
Date of Web Publication26-Aug-2021

Correspondence Address:
Mudita Gupta
Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CDR.CDR_98_20

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  Abstract 


Venous malformations (VMs) are congenital slow flow vascular malformations that are usually present at birth. They grow proportionate with the age and do not regress with time differentiating them from hemangiomas. VM presents as bluish discoloration of skin/mucosa or as soft subcutaneous masses. It may affect any organ, including viscera-like gastrointestinal tract or brain. VM is generally slowly progressive and asymptomatic as they grow slowly with age. We report the case of asymptomatic giant VM over the tongue for which the patient presented due to herpetic stomatitis.

Keywords: Asymptomatic, macroglossia, venous malformations


How to cite this article:
Sharma RK, Makharia A, Gupta M, Thakur S. Giant venous malformation of the tongue with macroglossia: A hidden menace. Clin Dermatol Rev 2021;5:242-4

How to cite this URL:
Sharma RK, Makharia A, Gupta M, Thakur S. Giant venous malformation of the tongue with macroglossia: A hidden menace. Clin Dermatol Rev [serial online] 2021 [cited 2021 Dec 1];5:242-4. Available from: https://www.cdriadvlkn.org/text.asp?2021/5/2/242/324587




  Introduction Top


Vascular malformation is a generalized term used to describe a group of lesions, present at birth, formed by an anomaly of angiovascular or lymphovascular structures that occur in approximately 1% of births.[1] Venous malformations (VMs) are slow flow vascular malformations, which are usually present at birth. VM may involve skin, mucosa, or any viscera. As VMs are slowly progressive and compressible, our patient is a 21-year-old male in spite of having giant VM of the tongue was least concerned about the abnormality and presented to us for painful ulcerative lesions due to viral stomatitis.


  Case Report Top


A 21-year-old male presented in the dermatology outpatient department with painful ulcerative lesions over the tongue for the past 5 days. He also complained of progressive asymptomatic swelling of the tongue since birth which started as a small bluish red swelling in the anterior tongue and gradually increased in size till the age of 14–15 years when he noticed maximum growth. As per the history, there was no difficulty in mastication, swallowing, or respiration. On examination, he was looking well without any respiratory discomfort but had difficulty in phonation of some words. On protruding the tongue, there was a giant bluish red bi-lobed swelling of the size about 10 cm × 7 cm and 9 cm × 6 cm over the anterior aspect of the tongue along the longitudinal axis of the tongue with a part of normal tongue visible at the posterior aspect of swelling [Figure 1]. There were multiple reddish and white vesicular lesions over the surface of swelling along with multiple erosions having polycyclic margins. The lesion was soft in consistency, easily compressible, nontender (except over the erosions), nonpulsatile, and not fixed to underlying structures. Routine investigations including complete hemogram and biochemistry were within the normal limits. Tzanck smear from the erosions showed acantholytic cells and multinucleated giant cells. Doppler ultrasonography showed heterogeneous hypoechoic shadows suggesting vascular malformation [Figure 2]. There was a monophasic slow flow with the presence of phlebolith indicating VM. Computed tomography (CT) scan showed a mass arising from the anterior aspect of the tongue measuring 10 cm × 7.5 cm with filling in the venous phase with phlebolith [Figure 3]. Hence, a final diagnosis of giant VM was made. There was no involvement of the bone or muscle. He was treated with oral acyclovir tablet 400 mg thrice a day for 7 days, topical benzocaine and chlorhexidine mouth wash. The patient received sclerotherapy after healing of ulcerative lesions. Sclerotherapy was done by inserting CT-guided 21 G needle and 2.5 ml of absolute ethanol was injected. There was slight reduction in size of the lesion excision was advised but the patient refused.
Figure 1: Giant bilobed venous malformation

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Figure 2: Ultrasound showing hypoechoic echoes with phlebolith

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Figure 3: Computed tomography scan showing mass arising from anterior aspect of tongue

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  Discussion Top


Vascular anomalies are the most common developmental disorders, characterized by structural irregularities of the vasculature which occur during the various stages of embryogenesis. Early dysmorphogenesis during fetal life leads to extratruncular and late to truncular vascular disorders. Extratruncular forms retain their mesodermal features and hence have proliferative capacity. Depending on the vessel involved, they may be capillary, lymphatic, venous, or arteriovenous malformations (AVMs). Conventionally, congenital vascular malformation (CVM) was divided into slow-flow vascular malformations (i.e., capillary, venous, and lymphatic malformations) or fast-flow vascular malformations (i.e., arterial malformations, arteriovenous fistulas, and AVMs).[2] Post capillary ectasia leads to VMs and are associated with slow flow. VM is the most common type of and comprise up to two third of CVM, with a prevalence of 1%.[3] The lesions are always present at birth but may not become clinically noticeable until later in childhood as they grow with the child. There is no sex or racial predilection.[4] VMs are found as commonly on the head and neck as extremity. Tongue is the most common site involved. VM may also appear on palate, gingiva, and buccal mucosa. They are usually sporadic but may be occasionally familial.[3] Superficial VMs manifest as a bluish discoloration of the skin while deeper ones present as soft ill-defined subcutaneous mass with normal skin. Superficial VMs are soft, compressible, and tend to increase in volume with an increase in venous pressure (e.g., Valsalva maneuver, straining, and exercise).[5] They are usually asymptomatic. VM on the head and neck may be esthetically unacceptable. Intraoral VM if giant can cause macroglossia, obstruction of airway, speech disturbance, masticatory defects, distortion of teeth, problem in oral hygiene and VM on tongue due to recurrent trauma may bleed or erode.

Sluggish flow and stasis because of structural abnormalities leads to phlebothrombosis and later may present with phlebolith which presents as recurrent pain. Other complications include bleeding and localized intravascular coagulation. VMs can occasionally be completely intraosseous, commonly seen in the mandible although maxillary, nasal, and frontal lesions have also been reported.[6]

VM needs to be differentiated from AVM and hemangiomas of the tongue. AVM can be differentiated by the absence of palpable thrill with associated compressibility and pulsations and radiological features which shows a biphasic flow. Hemangiomas have the phase of growth and involution while VM never involutes and progressively increase in size with age.

The pathogenesis of VM is due to mutation in angiopoietin receptor (TEK). These mutations lead to loss of function of TIE2 receptor (tyrosine kinase receptor).[7] The presence of progesterone receptor are responsible for sudden increase in size of VM during puberty.

Histopathologically, the vascular channels of VMs are irregular, nonfunctional hemodynamically, have narrow lumens lined with flattened endothelial cells and lack smooth muscle cells. The basement membranes are thin and there is no expression of vascular endothelial growth factor or basic fibroblast growth factor.[4]

VMs may be associated with various syndromes such as Turner syndrome, blue rubber bleb nevus syndrome, and Maffucci syndrome. Patients with Turner syndrome may have VMs of the intestine and feet. In blue rubber bleb nevus syndrome may have cutaneous and gastrointestinal VMs with risk of severe gastrointestinal hemorrhage. Maffucci syndrome is a rare condition, characterized by VMs usually on the extremities and dyschondroplasia resulting in enchondromas.[4]

Complete blood count including coagulation profile should be done if there is any evidence of in case of bleeding VM. Noninvasive modalities like Doppler ultrasonography show hemodynamic features and help to rule out AVM and is safe and first diagnostic procedure used to diagnose VM. Arteriography is of no use. Injection venography helps in visualizing VM. CT and magnetic resonance imaging scans to determine the extent of lesion and differentiation from other malformations.

All VM's need not to be treated, only the problematic and those with severe symptoms require treatment. Treatment includes compression, surgical resection, and obliteration of the channel lumens by percutaneous sclerotherapy depending upon the site and size of VM. Sclerosing agents, for example, ethanol through direct vessel wall contact, cause endothelial damage, inflammation and fibrosis that obliterate the vascular channels.[8] Absolute ethanol is a cheap, easily available, potent sclerosing agent. Toxicity of ethanol depends on the dosage. Usually, a dose of >1 ml may cause respiratory depression, cardiac arrythmias, hypoglycemia, etc., Acute blistering and transient paralysis are the common complications. Good response has been reported in up to 60% patients, but multiple sessions are required. Our patient was lost to follow-up.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kapadia SR, Thakore VM, Patel HM. Vascular malformations: An update on classification, clinical features, and management principles. Indian J Vasc Endovasc Surg 2017;4:152-62.  Back to cited text no. 1
  [Full text]  
2.
Cohen MM Jr. Vascular update: Morphogenesis, tumors, malformations, and molecular dimensions. Am J Med Genet A 2006;140:2013-38.  Back to cited text no. 2
    
3.
Behravesh S, Yakes W, Gupta N, Naidu S, Chong BW, Khademhosseini A, et al. Venous malformations: Clinical diagnosis and treatment. Cardiovasc Diagn Ther 2016;6:557-69.  Back to cited text no. 3
    
4.
Goldsmith PC. Dermatoses resulting from disorders of the veins and arteries. In: Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D, editors. Rook's Textbook of Dermatology. 9th ed. West Sussex: Wiley Blackwell Publishers; 2010. p. 103.21-3.  Back to cited text no. 4
    
5.
Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, et al. Vascular anomalies classification: Recommendations from the international society for the study of vascular anomalies. Pediatrics 2015;136:e203-14.  Back to cited text no. 5
    
6.
Syed NM. Vascular lesions of head and neck: A literature review. Indian J Dent Sci 2016;8:176-82.  Back to cited text no. 6
  [Full text]  
7.
Brouillard P, Vikkula M. Vascular malformations: Localized defects in vascular morphogenesis. Clin Genet 2003;63:340-51.  Back to cited text no. 7
    
8.
Lee BB, Do YS, Byun HS, Choo IW, Kim DI, Huh SH. Advanced management of venous malformation with ethanol sclerotherapy: Mid-term results. J Vasc Surg 2003;37:533-8.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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