|Year : 2021 | Volume
| Issue : 4 | Page : 747-751
Grapevines in the neck
Antariksh Vijan, Aashna Karbhari, Abhishek Mahajan
Department of Radiodiagnosis and Imaging, Tata Memorial Hospital, TMC and Homi Bhabha National Institute, Mumbai, Maharashtra, India
|Date of Submission||12-Sep-2021|
|Date of Decision||19-Oct-2021|
|Date of Acceptance||17-Nov-2021|
|Date of Web Publication||29-Dec-2021|
Department of Radiodiagnosis and Imaging, Tata Memorial Hospital, TMC and Homi Bhabha National Institute, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Vijan A, Karbhari A, Mahajan A. Grapevines in the neck. Cancer Res Stat Treat 2021;4:747-51
| Case History|| |
A 51-year-old woman without any comorbidities presented with a gradually increasing swelling in the left side of her neck for the past 6 years. Although this caused her occasional discomfort, there was no associated swallowing or speech dysfunction, pain, or fever. There was no family history of similar complaints.
On physical examination, the swelling corresponded to an ill-defined highly compressible, tubular, soft tissue prominence in the left cervical region at levels II, III, and IV inferiorly, extending into level VI, up to the suprasternal notch. The area was soft, non-tender, and non-pulsatile, without any bruit, thrill, or skin changes. The transillumination test was negative.
Additional examination of the head-and-neck region revealed sub-centimeter sized areas of bluish discoloration involving the lower lip, left buccal mucosa, and the oral tongue, which were compressible and spongy on palpation [Figure 1]a and [Figure 1]b.
|Figure: 1: (a and b) Clinical photographs demonstrating blue-tinged lesions involving the lip and tongue; (c) high-frequency gray scale ultrasound demonstrating heteroechoic, soft tissue lesion with anechoic vascular channels; (d) color Doppler image revealing venous flow at low scale settings; (e and f) axial T2- and T1-weighted magnetic resonance images, respectively, demonstrating T2 hyperintense lesion with isointense signal intensity on T1-weighted image|
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High-frequency ultrasonography of the neck [Figure 1]c and [Figure 1]d revealed a highly compressible heteroechoic lesion situated beneath the subcutaneous plane at the left levels II-IV, with anechoic tubular channels within, anteriorly reaching up to the suprasternal notch. Gray scale ultrasound showed evidence of stasis within few channels. Power and color Doppler examination (at low scale settings) revealed focal areas of monophasic slow flow. A spectral study showed the absence of arterial flow.
The rest of the neck appeared unremarkable on the ultrasound examination.
Contrast-enhanced magnetic resonance imaging (MRI) was then performed to better delineate the extent of involvement. T2/short tau inversion recovery (STIR)-weighted images [Figure 1]e, [Figure 1]f and [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f demonstrated multiple (at least 5) hyperintense ill-defined areas involving various cervico-facial subsites. In addition to the left cervical region lesion (1b-IV and V), lesions were found in the deep suprahyoid neck involving the left masticator and parapharyngeal spaces, left posterior triangle, and the oral cavity involving the lips, tongue, and left sublingual space. On dynamic contrast imaging [Figure 3], these showed delayed persistent enhancement with filling-in in the later phases. No phleboliths were noted.
|Figure 2: (a-d) Axial short tau inversion recovery (STIR) images demonstrating the ill-defined venous malformations with excellent soft tissue detail and anatomical relations. STIR hyperintense lesions are seen in the oral tongue, left para pharyngeal space, and left masticator space in images a and b. The left cervical lesion is seen extending from the submandibular space central compartment in images c and d, with mild extension into the para glottic space. No airway compromise is seen. Images e and f are coronal STIR images showing the extent of the lesion|
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|Figure 3: (a-e) Serial dynamic contrast enhanced magnetic resonance imaging demonstrating the paucity of arterial flow, with gradual slow filling-in, an enhancement characteristic to venous malformations|
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What is the diagnosis, and what should be done next? Once you have finalized your answer, turn to the next page to read on.
| Differential Diagnosis and Further Management|| |
The various differential diagnoses considered included [Table 1]:
|Table 1: Distinguishing features between few commonly encountered vascular anomalies of the head-and-neck region|
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- Venous malformation – They are compressible, ill-defined soft tissue lesions, often with bluish discoloration and low flow on Doppler imaging. On MRI, they show intense T2 hyperintensity, with filling-in during the venous phase and lack of significant arterial enhancement on dynamic contrast series
- Hemangiomas (”mass” like) - They may appear similar to venous malformations clinically. They have low flow on Doppler imaging. On MRI, they show intermediate signal on T1-weighted images and bright signal (”mass-forming”) on T2-weighted images, with few internal flow voids. They show vigorous enhancement after contrast administration
- Lymphatic low-flow malformations – These are multicystic, multi-spatial masses, with no skin color changes usually. They have no flow on Doppler imaging but high signal intensity on T2-weighted MRI, with fluid–fluid or debris levels within occasionally. They do not show post-contrast enhancement, except within the septae
- Arterio-venous malformations – These are a tangle of vessels without an obvious interspersed mass and may have a reddish hue, bruit, and thrill due to arterial supply. Arterial flow is present in the lesion on Doppler with arterialization of the venous structures. T1- and T2-weighted image sequences show serpiginous signal voids without a circumscribed mass, with distinct arterial and venous components on dynamic images
Thus, the features were consistent with multifocal, low-flow, venous malformations involving the head and neck. The basic metabolic workup and coagulation profile (prothrombin time, activated partial thromboplastin time, and international normalized ratio) for the patient were within normal limits. Given the lack of symptoms or significant cosmetic concern and as there was no evidence of coagulopathy, the decision was made to follow-up the patient for any clinical or symptomatic abnormality without any active intervention.
| Final Diagnosis|| |
Overall, the clinico-radiological features represented multifocal venous malformations.
| Discussion|| |
The International Society for the Study of Vascular Anomalies classifies vascular anomalies as true vaso-proliferative neoplasms (such as hemangiomas) and vascular malformations (errors of vascular morphogenesis). Venous malformations are a part of the spectrum of vascular malformations and comprise aberrant ectatic vascular channels that show slow venous flow. They are congenital, grow with age, and do not spontaneously regress.
They may show an increase in size in response to hormonal changes, such as pregnancy or puberty, or trauma, and may have an otherwise occult history of presentation. Most common areas of involvement include the head and neck (40%), followed by the extremities (40%) and trunk (20%).
Venous malformations characteristically are compressible, non-pulsatile soft tissue lesions without any thrill or bruit, and many times are blue-tinged. There is considerable variation in their size and number, with few showing multi-spatial distribution crossing various tissue planes. Venous malformations tend to enlarge with the Valsalva maneuver and collapse on the application of pressure. Phleboliths may occasionally be palpable as tiny stony nodules within the lesion. Deeper lesions may produce discomfort, and additional features of mass effect such as dysphonia, dysphagia, stridor, or airway compression may be present in larger lesions, depending on their location.
Owing to the inherent stasis in these slow flow lesions, venous malformations have a high tendency to undergo intralesional thrombosis. Such localized intravascular coagulopathy results in elevated D-dimer levels and calls for preoperative coagulation profile assessment, followed by an apt institution of anticoagulation to avoid systemic sequelae. Rarely, the localized coagulopathy may be accompanied by disseminated intravascular coagulopathy.
A clinical diagnosis of superficial venous malformations may be made confidently, with imaging as an adjunct to support the diagnosis. Multifocality and a deeper location should prompt further workup and use of imaging to characterize and map the extent of involvement.
High-frequency ultrasound with color Doppler evaluation is generally the modality of choice for initial evaluation. A high-frequency linear array transducer (5–10 MHz) is preferred for optimal resolution.
In gray scale evaluation, superficial venous malformations are instantly compressible and demonstrate heterogeneous echotexture (in up to 80% of cases). Similar to our case, tubular anechoic structures indicative of vascular channels are seen in almost 50% of the cases. Calcific foci representing phleboliths may be appreciated on ultrasound in up to 16% of the cases, although computed tomography (CT) scanning remains the most sensitive modality for their detection. Occasionally, isoechoic soft tissue thickening may be the only gray scale feature.
Color Doppler and spectral examinations demonstrate low-velocity venous flow within the lesion. Power Doppler may be particularly useful to detect tiny low-flow foci. It is imperative to use appropriate Doppler settings to detect even the lowest perceptible flow within the lesion.
MRI is the modality of choice for accurately mapping out the anatomic extent and relations as well as classifying the vascular lesion based on flow dynamics. The suggested MRI protocol for suspected vascular malformations is described in [Table 2].
|Table 2: Suggested magnetic resonance imaging protocol in evaluation of suspected vascular malformation|
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Venous malformations are typically ill-defined or geographic lesions that are intensely hyperintense on T2-weighted images/STIR, with a predominantly isointense signal on T1-weighted images. The striking and consistent T2 hyperintensity of venous malformations makes a T2 fat-suppressed sequence such as STIR ideal for their identification. Occasional T1 hyperintensity may be present, which represents intralesional hemorrhage.
Punctate T2*/gradient echo hypointense foci represent scattered phleboliths, and when present, are confirmatory of venous malformations. Multiphase, gadolinium-enhanced dynamic imaging reveals slow, gradual enhancement without significant enhancement on arterial phases. The absence or paucity of arterial flow with delayed filling-in is a classic characteristic of venous malformations. CT plays a limited role in the evaluation of these venous malformations owing to the poor soft tissue resolution and may only serve as a tool to detect phleboliths.
The management is largely dependent on the fluid dynamics of the lesion. It is important to differentiate venous malformations from other low-flow lesions such as lymphatic malformations and hemangiomas. Once the diagnosis of a venous malformation is confirmed on imaging, the size and location of the lesion along with the presenting features dictate further therapy.
A wait-and-watch approach is usually adopted for small, painless lesions. However, larger symptomatic lesions call for intervention. Minor and superficial venous malformations of the head and neck can be treated with surgical excision, which entails careful removal of the skin and mucosa to prevent a recurrence, or neodymium-doped yttrium aluminum garnet laser treatments.
More extensive and multi-spatial malformations require a multimodal evaluation and are better suited to percutaneous sclerotherapy.
| Conclusion|| |
Venous malformations are the most common form of vascular malformation, with a high propensity for involvement of the various head-and-neck spaces. High-frequency ultrasound with Doppler evaluation is the initial modality of choice to demonstrate the low-flow nature. A dynamic contrast-enhanced MRI is useful for accurate anatomic delineation and further confirmation of flow dynamics. Multimodal management is usually adopted taking into account the size, location, and symptoms with evaluation for coagulopathy in multifocal cases.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published, due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]