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

Disclosures No relevant financial disclosures

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

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

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

Role of Imaging Anatomic localization Assessing extent Often no specific features

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

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

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

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

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.

AV Malformations - Rx Gold Standard Resistant Malformations Preoperative angiography with selective embolization of the nidus and draining veins followed by definitive resection within 24-48 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.

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.

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

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

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

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

Venous Malformations - Imaging CT 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

Venous Malformations - Imaging CT 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

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

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.

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.

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

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

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

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

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.

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

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

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.

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

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

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

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

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

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

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:

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

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).

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.

Paraganglioma-Imaging CT 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.

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.

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.

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

References Mulliken JB et al. Hemangiomas and vascular malformations in infants and children: A classification based on endothelial characteristics. Plast Reconstr Surg 69:412-422, 1982 Mulliken JB et al. Classification of pediatric vascular lesions. Plast Reconstr Surg 70:120-121, 1982 Lowe et al. Vascular Malformations: Classification and Terminology the Radiologist Needs to Know. Semin Roentgenol. 2012; 47 (2): 106-17 Dasgupta et al. ISSVA classification. Semin in Pediatric surgery. 23: 158-161. 2014.

References Fowell C et al. Arteriovenous malformations of the head and neck: current concepts in management. Br J Oral Maxillofac Surg. 2016. [Ahead of print]. Nassiri N et al. Evaluation and management of peripheral venous and lymphatic malformations. 4 (2): 257-65, 2016. Woolen S et al. Paragangliomas of the Head and Neck. Neuroimaging Clin N Am. 2016. [Ahead of print]. Darrow DH et al. Diagnosis and management of infantile hemangioma. 136 (4): 1060-104. 2015.