1. Vascular Head and Neck lesions: Imaging and Management Essentials
Sean Woolen, MD, Ashok Srinivasan, MD
Department of Radiology
University of Michigan Health System, Ann Arbor

2. Disclosures
No relevant financial disclosures

3. Classification and terminology Role of imaging Management
Purpose
Classification and terminology
Role of imaging
Management

4. Mulliken and Glowacki Classification
(based on endothelial cell turnover)
Arteriovenous M.
Venous M.
Lymphatic M.
Capillary M.
Paraganglioma
Hemangioma
Nasopharyngeal angiofibroma
Vascular malformations
NORMAL
endothelial cell turnover
Vascular tumors
HIGH
endothelial cell turnover

5. New ISSVA Terminology Cavernous hemangioma Liver hemangioendothelioma
Infantile hemangioma
Port wine stain
Capillary malformation
Hepatic/vertebral/orbital hemangiomas
Venous malformation
Lymphangioma
Cystic hygroma
Lymphatic malformation

6. Role of Imaging Anatomic localization Assessing extent
Often no specific features

7. Vascular Malformations
Birth 4 years
15 years
Venous malformation
Lymphatic malformation
Arteriovenous malformation
Growth is PROPORTIONATE to body size

8. AV Malformations - Clinical Picture
Commonly manifest later in childhood or adolescence
Pulsatile lesions that can cause clinical bruit, pain, bleeding, and ulceration

9. AV Malformations - Imaging
CT and MRI
Large dilated vascular structures with arteries and veins being usually indistinguishable
Contrast enhanced CT demonstrates abnormal nidus of vessels along the right face with dilated vascular channels, likely draining veins

10. AV Malformations - Imaging
ANGIOGRAPHY
GOLD STANDARD
Early venous shunting, arterial feeders, venous drainage
Lateral Projection
- ECA Injection
Early Arterial
Mid Arterial

11. AV Malformations - Staging
Stage
Description 1
Quiescent: stable 2
Increasing in size 3
Symptomatic: pain, bleeding, disfigurement, disruption of function 4
Decompensating: high-output cardiac failure
Schobinger classification helps guide management for AV malformations. Treatment of symptomatic patient's with stage II or above AV malformations.

12. AV Malformations - Rx Gold Standard Resistant Malformations
Preoperative angiography with selective embolization of the nidus and draining veins followed by definitive resection within hours.
Resistant Malformations
Angiography with combination of selective embolization of the nidus and percutaneous sclerotherapy. With limited angiographic access to the nidus, emerging percutaneous cryotherapy is an option.

13. AV Malformations - Rx
T1 MRI with frank enhancement of mandibular AVM. This was treated with percutaneous sclerotherapy of the hypervascular mass with near complete devascularization.

14. Venous Malformations – Clinical Picture
Soft, compressible, bluish nonpulsatile mass
Expansion on ipsilateral jugular vein compression, performance of the Valsalva maneuver, or dependent positioning

15. Venous Malformations - Imaging
MRI (Best Imaging)
Crucial to assess multispatial extent
Isointense -T1-weighted
Hyperintense - T2-weighted
Intense enhancement

16. Venous Malformations - Imaging
MRI
Distinguishing features include DISCRETE AREAS OF HOMOGENEOUS HIGH SIGNAL INTENSITY (venous lakes) and PHLEBOLITHS
T2-W
T1-W
Post Gad T1-W

17. Venous Malformations - Imaging
MR images demonstrate phlebolith (arrows) seen as signal voids on both T1-W and T2-W images on the right side of the mandible

18. Venous Malformations - ImagingCT
Muscle attenuation on non-contrast images with variable patterns of enhancement.
LOW-FLOW lesions can demonstrate PHLEBOLITHS.
Contrast enhanced CT image demonstrates enhancing venous malformation in the right forehead

19. Venous Malformations - ImagingCT
Muscle attenuation on non-contrast images with variable patterns of enhancement.
LOW-FLOW lesions can demonstrate PHLEBOLITHS.
Contrast enhanced CT image demonstrates venous malformation with phleboliths in the right parapharyngeal space

20. Venous Malformations - Treatment
Many venous malformations do not require specific management
Intervention depends on
Hematologic evaluation
Size of the lesion
Functional impact on the patient

21. Hematologic Evaluation
Venous Malformations - Treatment
Hematologic Evaluation
Fibrinogen and D-dimer levels can detect the presence of localized intravascular coagulopathy, which may progress to disseminated intravascular coagulopathy with aggressive therapy.
If elevated, should start prophylactic Lovenox.

22. Venous Malformations - Treatment
Sclerotherapy
Small & well circumscribed
The sclerosants administered are typically 95% ethanol or sodium tetradecyl sulfate (Sotradecol)
Sclerotherapy
Large & infiltrating
+ Surgery Debulking
For large, infiltrating malformations that are difficult to delineate, combined sclerotherapy with ablative and reconstructive surgery is indicated
Sirolimus is an emerging treatment when extensive venous malformations are refractory to other treatments.

23. Venous Malformations - Treatment
Sclerotherapy procedure of right facial venous malformation using ultrasound guidance, 20 gauge angiocatheter access, and injection of bleomycin sclerosant.

24. Lymphatic Malformations - Clinical Picture
Arises from sequestered portions of the embryonic lymphatic anlage
CLINICAL EXAM
Manifest as soft, colorless masses
60% apparent at birth, while 80-90% manifest by 2 years
Head and neck are the most common site of distribution
Growth proportionate to body size

25. Lymphatic Malformations
Type I lymphatic malformations (macrocystic)
Generally arise below the mylohyoid and occur in the anterior and posterior triangles of the neck.
Historically termed cystic hygromas
>2 cm in size
Type II lymphatic malformations (microcystic)
Generally located above the mylohyoid commonly involving the tongue, floor of the mouth, cheeks, lips, and sometimes the parotid gland
Historically termed lymphangiomas
<2 cm in size

26. Lymphatic Malformations - Imaging
Low Density- CT
High T2 Signal - MRI
No significant enhancement
(Venous components of complex lesions may enhance)
Multiloculated fluid intensity lesion in a neonate with minimal enhancement

27. Lymphatic Malformations - Imaging
D.D.
Branchial cleft cyst
Thymic cyst
Abscess
Multiloculated fluid intensity lesion in a child with rim enhancement. The high pre-contrast T1 signal image suggests protein content.

28. Lymphatic Malformations - Imaging
Venolymphatic malformation with mixed signal intensity (fluid signal and enhancing components).
Venous malformation in white *Both have phleboliths

29. Lymphatic Malformations - Rx
Sclerotherapy is advocated for large macrocystic lesions with alcohol, doxycycline, or bleomycin
Surgical excision with careful preservation of involved neurovascular structures and laser photocoagulation serve as important therapeutic adjuncts

30. Lymphatic Malformations -Rx
Sclerotherapy
-Ultrasound of lymphatic malformation with macrocystic and microcystic components. -Large macrocystic components were accessed with needle aspirating fluid (yellow LM and red LVM) -Following aspiration, doxycycline mixed with contrast were injected into the lymphatic malformation.

31. Lymphatic Malformations - Rx
Pre and post sclerotherapy images in lymphatic malformation showed significant reduction in lesion size one year after sclerotherapy

32. Hemangioma - Prototype Vascular Tumor
Infantile Hemangioma is GLUT1 positive
Proliferating and involuting phases
Proliferation
Involution
Birth
6-12 months
Up to 9 years
Typically complete resolution –
50% by 5 years; 70% by 7 years; 90% by 9 years

33. Hemangioma-Classification
Congenital Hemangioma is GLUT1 negative
Rapidly involuting congenital hemangioma (RICH) most commonly involute by 14 months
Partial-involuting congenital hemangioma (PICH)
Non-involuting congenital hemangioma (NICH) can grow in proportion the patients age or stay the same size

34. Hemangioma - Imaging
Clinical history is key to recognizing the proliferating phase CT
Heterogeneity and intense enhancement, often in a delayed manner
Contrast enhanced CT in a 6 month old infant shows
enhancing right neck mass that was progressively getting larger since birth

35. Hemangioma - Imaging
Iso to slightly hyperintense on T1-weighted images
Progressively hyperintense on T2-weighted images and show contrast enhancement
T2-W
T1-W
Post Gad T1-W
MR images demonstrate internal heterogeneity, increased T2 signal, internal flow voids and intense enhancement

36. Hemangioma - Imaging
On angiography, they are high-flow lesions that are well-circumscribed and exhibit a lobular pattern of intense, persistent tissue staining.
POST
ANT POST ANT POST
Lateral projection during internal maxillary artery injection demonstrates intense and persistent tissue staining
ANT

37. Hemangioma -Treatment
Emergency
Potentially life-threatening complication
Urgency
Existing or imminent functional impairment, pain, or bleeding
Structure Anomalies
Identify structural anomalies associated with infantile hemangioma
Elective
Reduce the likelihood of long-term or permanent disfigurement
Treatment when one of the criteria below are met:

38. Hemangioma -Treatment
Observation remains the preferred management option
Vincristine
Pulse dye laser
Beta Blockers
Lesions associated with Kasabach-Merritt phenomenon
Treatment of ulcerating lesions
First line treatment
Steroids, Interferon, and Imiquimod
Surgery
Failure/contraindication to pharmacotherapy and favorable anatomical area for resection or resection of residual fibrofatty tissue after involution
Used less commonly because other treatments have better safety profiles

39. Paraganglioma- Clinical Picture
Jugulotympanic paragangliomas present with pulsatile tinnitus and hearing loss. Physical exam shows a bluish/pulsating mass behind the tympanic membrane.
Carotid Body present as a painless, non-tender, enlarging mass lateral to the tip of the hyoid bone. Physical exam shows a pulsatile mass which moves side to side, but not vertically.
Glomus Vagale present as a painless non-tender mass posterior to the mandible with cranial nerve symptoms such as dysphagia (IX), hoarseness (X), shoulder drop (XI), aspiration (XII).

40. Paraganglioma-Imaging
MRI
SALT AND PEPPER:
SALT– areas of subacute hemorrhage
PEPPER – hypointense serpentine flow voids,
intense enhancement, mildly T2 hyperintense
Carotid body tumor is the most common paraganglioma. The MRI images above show typical splaying of the ICA and ECA flow voids, heterogeneous appearance on T2, and enhancement.

41. Paraganglioma-ImagingCT
Lobular avidly enhancing mass
Glomus vagale displaces the ECA and ICA anterior and medial heterogeneous enhancement on CT.
Malignant paraganglioma encompasses the jugular vein, ECA, and ICA. Malignancy most common in glomus vagale.

42. Paraganglioma-Imaging
Jugulotympanic is the most common primary tumor in the middle ear.
Important to notice asymmetric jugular foramen enlargement.
The CT image shows asymmetrical right jugular foramen enlargement with moth eaten appearance.

43. Paraganglioma-Rx Treatment Surgical resection + pre-op EMBOLIZATION
(with or without radiation therapy for large lesions at the skull base and poor surgical candidates)
Angiography
Prolonged INTENSE TUMOR BLUSH. Performed for preoperative assessment and embolization of dominant arterial feeders.

44. Summary Proportionate growth to body size – Vascular malformations
Rapid growth from birth followed by involution – Infantile hemangioma
Phleboliths – Venous malformations
Hemangiomas– Usually no Rx
Venous and Lymphatic malformations – Sclerotherapy +/- surgery
AV malformations and Paraganglioma – Preop embolization + Surgery

45. References
Mulliken JB et al. Hemangiomas and vascular malformations in infants and children: A classification based on endothelial characteristics. Plast Reconstr Surg 69: , 1982
Mulliken JB et al. Classification of pediatric vascular lesions. Plast Reconstr Surg 70: , 1982
Lowe et al. Vascular Malformations: Classification and Terminology the Radiologist Needs to Know. Semin Roentgenol. 2012; 47 (2):
Dasgupta et al. ISSVA classification. Semin in Pediatric surgery. 23:

46. References
Fowell C et al. Arteriovenous malformations of the head and neck: current concepts in management. Br J Oral Maxillofac Surg [Ahead of print].
Nassiri N et al. Evaluation and management of peripheral venous and lymphatic malformations. 4 (2): , 2016.
Woolen S et al. Paragangliomas of the Head and Neck. Neuroimaging Clin N Am [Ahead of print].
Darrow DH et al. Diagnosis and management of infantile hemangioma. 136 (4):