Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 9  |  Issue : 3  |  Page : 267-270

Venolymphatic malformation


Department of Oral Medicine and Radiology, Faculty of Dentistry, Meenakshi Academy of Higher Education and Research, Meenakshi Ammal Dental College, Chennai, Tamil Nadu, India

Date of Submission17-Dec-2021
Date of Decision10-Feb-2022
Date of Acceptance14-Feb-2022
Date of Web Publication21-Aug-2022

Correspondence Address:
N Nagammai
Department of Oral Medicine and Radiology, Faculty of Dentistry, Meenakshi Academy of Higher Education and Research, Meenakshi Ammal Dental College, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_131_21

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  Abstract 


Vascular lesions are the most common congenital and neonatal abnormalities. About 60% of anomalies occur in the head-and-neck region due to its complex vascular anatomy. There is confusion in the descriptive terminology for the vascular entity and eponyms. Thus, appropriate classification based on clinical examination and imaging is essential for proper diagnosis and treatment. Thus, the aim of this article is to report a case of venolymphatic malformation with comprehensive knowledge about classifications, pathogenesis, clinical presentation, and its management.

Keywords: Low flow lesion, lymphatic malformation, sclerotherapy, vascular lesions and venous malformation


How to cite this article:
Gopal K S, Nagammai N, Srinivasan S. Venolymphatic malformation. Indian J Vasc Endovasc Surg 2022;9:267-70

How to cite this URL:
Gopal K S, Nagammai N, Srinivasan S. Venolymphatic malformation. Indian J Vasc Endovasc Surg [serial online] 2022 [cited 2022 Sep 27];9:267-70. Available from: https://www.indjvascsurg.org/text.asp?2022/9/3/267/354068




  Introduction Top


Vascular anomalies (VAs) describe the various pathologies involving the vascular structures of the human body VAs were originally first classified by Mulliken and Glowacki in 1982 based on biological behavior clinical features.[1] However, later, the International Society for the Study of VAs classification system is widely accepted and utilized to categorize VAs into two basic types: (1) vascular neoplasms and (2) Vascular malformations. The malformations were further categorized into four subtypes depending on their anatomy of the vessels. Type I simple vascular malformations consist of malformation of a single type of vascular channel, and are named according to the vessel involved type (e.g., capillary malformation and venous malformation). Type II combined vascular malformations consist of two or more distinct vascular malformations within a single lesion (e.g., capillary-venous malformation and venolymphantic malformations).[2]

Vascular malformations arise due to an error in individual or combination morphogenesis of any arterial, venous, or lymphatic vascular networks. These VAs are present from birth, grow proportionally with the growth of the body, and do not exhibit any tendency to involute spontaneously. Puberty, Trauma, and pregnancy can also cause accelerated growth. The rate of occurrence is 1.5% and mostly two-third of malformation occurring are predominantly venous, and they are evenly distributed according to sex and race.[3]

Vascular malformations are subdivided into high-flow and slow-flow lesions. Venous malformation, lymphatic malformation, and venolymphatic malformations are considered to be slow-flow lesions. These venolympahtic malformations (VLMs) are the combination of dysplastic venous and lymphatic structures and 50% of them occur in the region of head and neck. Imaging modalities, such as Doppler ultrasound, magnetic resonance imaging (MRI), Contrast-enhanced computerized tomography (CT), phlebography, and nuclear imaging studies, distinguish vascular lesions.[4] Various treatment modalities including conservative management, debulking surgery, application of sclerotherapy, and lasers can be employed. This article discusses about a case of venolymphatic malformation and with a detailed review on the possible etiopathogenesis, clinical features, and its various imaging modalities.


  Case Report Top


A23-year-old female presented with swelling in the lower half of the face and difficulty in swallowing food since childhood. Her mother first noticed the swelling at 6 months of age. This was initially small but grew proportionately as she grew and involved the entire lower half of the face and tongue. She also noted an increase in size of the swelling with crying. At 2 years, she presented with difficulty in breathing and swallowing solid foods and was diagnosed to have a lymphangioma. She underwent her first debulking surgery at the age of 2 years, followed by a Lefort I and bilateral sagittal split osteotomy with glossectomy at the age of 14 years. The size reduced after surgery and has since gradually increased to the present size.

On extraoral examination, there was a diffuse swelling noted on the entire lower third of the face on either side [Figure 1]. It extends 1 cm below from ala-tragus line of the right side involving the parasymphyseal region of the right side, symphysis crossing the midline, involving the parasymphyseal region of the left side, and to the 1 cm below ala-tragus line of the left side. Skin over the region was stretched and normal in color compared to the adjacent skin. There was a well-healed surgical scar on the anterior cervical region of the neck. The swelling was nontender with a variable consistency.
Figure 1: Profile picture

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On intraoral examination of the tongue revealed that the dorsal, ventral, and lateral borders were pebbly with multiple red pitpoint papules on the anterior twothird of the tongue and with a large dome-shaped papule on the mid-dorsal aspect of the tongue. All tongue movements such as protrusion, retraction, elevation, depression, and lateral movements were restricted [Figure 2]. On palpation, the nodular areas were nontender and firm. The hard palate had an erythematous spot measuring 0.5 mm approximately in the palatal region of 15.
Figure 2: Dorsal surface of the tongue

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Duplex ultrasound revealed a soft-tissue lesion seen along the mandible with multiple cystic spaces, slightly compressible, vascularity seen only on the periphery [Figure 3]. Contrast-enhanced computer tomography revealed a large transpatial multilobulated cystic lesion with loculations. Phleboliths were seen in the submental region, premandibular plane, right premassetric plane, and submandibular region extending into the parapharyngeal space with feeders from facial vein suggestive of a venolymphatic malformation [Figure 4]. Thus, correlating the patient history, past medical, and clinical findings, the provisional diagnosis was made as recurrent venolymphatic malformation of the lower half of the face.
Figure 3: Duplex ultrasound cystic spaces

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Figure 4: Contrast-enhanced computed tomography showing phleboliths

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She underwent debulking of the malformation and the histopathological analysis revealed numerous dilated vascular channels lined by a single layer of endothelium, dilated lymphatics in the subepithelial layer, and scattered lymphocytes consistent with a venolymphatic malformation of the lower third of the face.


  Discussion Top


Venous malformations are the most common congenital vascular malformation with a higher incidence >50%, lymphatic malformations are less common, and combinations of venous and lymphatic malformations have a lower incidence. VLM was previously termed lymphangiohemangiomas or hemangiolymphangiomas. These name change occurred in 2015.[5]

Vascular malformations occur in vessels that are morphologically and histologically related to veins. Possible pathogenesis may be due to germline mutation in TEK (chromosome 9p) gene which encodes the endothelial cell tyrosine kinase receptor TIE2.[6] Congenital, trauma, infections, hormonal, puberty, and pregnancy may cause vascular malformation to enlarge and become symptomatic.[7] The clinical features can be categorized into superficial or deep based on the anatomical location. The appearance of most superficial ones is purple, whereas the deeper intramuscular ones may appear as ill-defined swelling with normal overlying skin. These lesions are soft on palpation, nonpulsatile, and get easily enlarged with Valsalva maneuver and emptied on by local application of pressure.[8] Due to its sluggish blood flow, stasis may lead to the formation of phlebothrombosis which presents clinically as recurrent pain and tenderness. They can permeate across the various tissue planes such as fat, muscle, and bone. It can be completely intraosseous and the mandible is the most common bone involved, although maxillary, nasal, and frontal lesions have also been reported in the literature.[9] Duplex ultrasound reveals phleboliths with posterior acoustic shadowing in 48% and the flow could be identified as monophasic.[10] On cross-sectional imaging modality, the lesions have a characteristic involvement with spread across different tissue planes.[11] Contrast-enhanced computer tomography is necessary for demonstrating the various components within the lesions. It readily identifies small calcified phleboliths termed ” shining pearl sign.”[12] Computed tomography also demonstrates bone involvement and secondary remodeling of the lesion. In MRI, lesions appear to hypointense in T1 and hyperintense in T2 W with phlebolith identified as low signal void.

Lymphatic malformations constitute about 74% of all vascular malformation, and the most common site of occurrence is the posterior triangle and submandibular spaces.[13] The main etiopathogenesis of lympahtic malformations is believed presumably due to the failure of embryonic lymphatic systems to adequately separate from or to connect to the venous system due to mutation in the vascular endothelial growth receptor 3 and as well as TIE2/TEK.[14] The lesions are painless, nonpulsatile masses with rubbery consistency covered by normal-appearing skin. Secondary complications such as infection, bleeding, obstruction of the airway, disturbances of speech, and abnormal facial growth can occur. In some uncommon cases even skeletal distortion such as hypertrophy can be seen.[8] Imaging modality such as ultrasound and MRI is the method of choice. Ultrasound reveals well-defined hypoechoic areas with internal loculations. On Doppler ultrasound, no vascularity is exhibited. On contrast-enhanced MRI, lymphatic malformation does not enhance except in the region of septation, where the lymphatic component may be in the form of a mixed malformation.[15]

In our case, the swelling was present congenitally with wide involvement of fascial muscle and bone. The color appeared normal because of the deeper location of the lesion. Doppler ultrasound revealed a cystic lesion with very scanty flow and phlebolith with posterior acoustic shadowing. The presence of phlebolith served as the main cause of pain in the patient.

The treatment options for congenital vascular malformations are sclerotherapy using a sclerosing agent such as sodium tetradecyl sulphate 2–3 ml or polidocanol 3 ml or ethanol – 0.15 ml/kg,[16] Embolotherapy using an angiographic catheter and also using neodymium-doped yttrium–aluminum garnet (Nd: Yag) laser[17] and surgical excision are recommended. Endovascular sclerotherapy is the most accepted treatment of choice, especially for lesions with diffuse involvement, but in our case, there were severe cosmetic impairment, disabling pain, and also involvement of the bone. Thus, debulking was preferred over sclerotherapy.


  Conclusion Top


Vascular malformations are a disorder of aberrant vasculogenesis or lymphangiogenesis or angiogenesis. They are of clinical importance for clinicians and academicians due to their common occurrence in the head-and-neck region. Imaging plays an important role in their diagnosis. Ultrasound, contrast-enhanced computer tomography, and MRI have emerged as necessary to differentiate venous from lymphatic lesions.

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 initials 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.
Steiner JE, Drolet BA. Classification of vascular anomalies: An update. Semin Intervent Radiol 2017;34:225-32.  Back to cited text no. 1
    
2.
Merrow AC, Gupta A, Patel MN, Adams DM. 2014 revised classification of vascular lesions from the International Society for the Study of vascular anomalies: Radiologic-pathologic update. Radiographics 2016;36:1494-516.  Back to cited text no. 2
    
3.
Nair SC. Vascular anomalies of the head and neck region. J Maxillofac Oral Surg 2018;17:1-12.  Back to cited text no. 3
    
4.
Jackson IT, Carreño R, Potparic Z, Hussain K. Hemangiomas, vascular malformations, and lymphovenous malformations: Classification and methods of treatment. Plast Reconstr Surg 1993;91:1216-30.  Back to cited text no. 4
    
5.
Flors L, Leiva-Salinas C, Maged IM, Norton PT, Matsumoto AH, Angle JF, et al. MR imaging of soft-tissue vascular malformations: Diagnosis, classification, and therapy follow-up. Radiographics 2011;31:1321-40.  Back to cited text no. 5
    
6.
Griauzde J, Srinivasan A. Imaging of vascular lesions of the head and neck. Radiol Clin North Am 2015;53:197-213.  Back to cited text no. 6
    
7.
Syed NM. Vascular lesions of head and neck: A literature review. Indian J Dent Sci 2016;8:176.  Back to cited text no. 7
  [Full text]  
8.
Ethunandan M, Mellor TK. Haemangiomas and vascular malformations of the maxillofacial region – A review. Br J Oral Maxillofac Surg 2006;44:263-72.  Back to cited text no. 8
    
9.
Cohen MM Jr. Vasculogenesis, angiogenesis, hemangiomas, and vascular malformations. Am J Med Genet 2002;108:265-74.  Back to cited text no. 9
    
10.
Donnelly LF, Adams DM, Bisset GS 3rd. Vascular malformations and hemangiomas: A practical approach in a multidisciplinary clinic. AJR Am J Roentgenol 2000;174:597-608.  Back to cited text no. 10
    
11.
Bhat V, Salins PC, Bhat V. Imaging spectrum of hemangioma and vascular malformations of the head and neck in children and adolescents. J Clin Imaging Sci 2014;4:31.  Back to cited text no. 11
    
12.
Fishman SJ, Mulliken JB. Hemangiomas and vascular malformations of infancy and childhood. Pediatr Clin North Am 1993;40:1177-200.  Back to cited text no. 12
    
13.
Redondo P. Vascular malformations (I). Concept, classification, pathogenesis and clinical features. Actas Dermosifiliogr 2007;98:141-58.  Back to cited text no. 13
    
14.
Richter GT, Friedman AB. Hemangiomas and vascular malformations: Current theory and management. Int J Pediatr 2012;2012:645678.  Back to cited text no. 14
    
15.
Elias G, McMillan K, Monaghan A. Vascular lesions of the head and oral cavity – Diagnosis and management. Dent Update 2016;43:859-60, 862-4, 866.  Back to cited text no. 15
    
16.
Choi BE, Kim Y, Leem DH, Baek JA, Ko SO. Utility of sodium tetradecyl sulfate sclerotherapy from benign oral vascular lesion. Maxillofac Plast Reconstr Surg 2016;38:44.  Back to cited text no. 16
    
17.
Burkey MD BB, Garrett G. Use of the laser in the oral cavity. Otolaryngol Clin North Am 1996;29:949-61.  Back to cited text no. 17
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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