Vascular Anomalies of the Mid‐ and Lower Face










Atlas of Oral and Maxillofacial Radiology, First Edition. Bernard Koong.
© 2017 John Wiley & Sons Ltd. Published 2017 by John Wiley & Sons Ltd.
191
Vascular anomalies comprise two broad categories: vascular
tumours (proliferative neoplasms) and vascular malformations.
Ultrasound and magnetic resonance angiography (MRA) can
assist in characterising the vascular flow dynamics.
VASCULAR TUMOURS (PROLIFERATIVE
NEOPLASMS)
12.1 Haemangioma
(Figures12.1 and12.2)
Most common lesion in this grouping of true vascular
neoplasms.
Can be subcategorised into infantile and congenital types.
Infantile.
Present in the postnatal period or first 6 months of life
with approximately two‐thirds arising in the head and
neck region; commonly involving the parotid glands,
orbits, nasal cavity, subglottis, anterior and posterior
regions of the neck.
Demonstrate a triphasic growth pattern with prolifera-
tive, plateau and involution phases, reaching a maximum
size between 3 and 5 months.
Lesions usually begin to involute by 1 year. Approximately
half of these lesions will show near complete resolution
by 5 years.
Congenital; present at birth and further classified as:
Non‐involuting congenital haemangioma: grow propor-
tionally with the growth of the patient.
Rapidly involuting congenital haemangioma: maximal in
size at birth but involute in the first 12–18 months.
First‐line treatment is medical, utilising propranolol, with
surgery reserved for complicated (ulcerating, bleeding) or
refractory lesions.
Radiological features
The purpose of imaging is to characterise, locally stage for the
deep extent and assess for multiplicity.
Haemangiomas present clinically as a raised ‘strawberry‐like
plaque, which may be classified as cutaneous, subcutaneous,
visceral or mixed. Assessment of deep lesions and definition
of locoregional extent in children should be undertaken with
ultrasound and magnetic resonance imaging (MRI).
During the proliferating phase, haemangiomas are well
defined but of mixed echogenicity on ultrasound with low‐
resistance high flows on Doppler studies.
On MRI haemangiomas are of heterogeneous and usually
slightly hyperintense signal relative to muscle on T2‐
weighted imaging, with flow voids and intense contrast
enhancement.
MRA may show associated anomalies such as in PHACES
(posterior fossa malformations–haemangiomas–arterial
anomalies–cardiac defects–eye abnormalities–sternal cleft
and supraumbilical raphe) syndrome.
Differential diagnosis
Key radiological differences
Venocavernous
malformation
Insinuating, trans‐spatial, variable enhance-
ment, phleboliths.
Arteriovenous
malformation
Flow voids, minor soft tissue component.
Hypervascular
primary neoplasms
Avidly contrast enhancing, flow voids.
Hypervascular
metastases
Avidly contrast enhancing, flow voids,
known primary tumour.
CHAPTER 12
Vascular Anomalies oftheMid‐ andLower Face
Michael Bynevelt, Andrew Thompson and Bernard Koong

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Atlas of Oral and Maxillofacial Radiology, First Edition. Bernard Koong. © 2017 John Wiley & Sons Ltd. Published 2017 by John Wiley & Sons Ltd.191Vascular anomalies comprise two broad categories: vascular tumours (proliferative neoplasms) and vascular malformations. Ultrasound and magnetic resonance angiography (MRA) can assist in characterising the vascular flow dynamics.VASCULAR TUMOURS (PROLIFERATIVE NEOPLASMS)12.1 Haemangioma (Figures12.1 and12.2)• Most common lesion in this grouping of true vascular neoplasms.• Can be subcategorised into infantile and congenital types. ◦Infantile. ■ Present in the postnatal period or first 6 months of life with approximately two‐thirds arising in the head and neck region; commonly involving the parotid glands, orbits, nasal cavity, subglottis, anterior and posterior regions of the neck. ■ Demonstrate a triphasic growth pattern with prolifera-tive, plateau and involution phases, reaching a maximum size between 3 and 5 months. ■ Lesions usually begin to involute by 1 year. Approximately half of these lesions will show near complete resolution by 5 years. ◦Congenital; present at birth and further classified as: ■ Non‐involuting congenital haemangioma: grow propor-tionally with the growth of the patient. ■ Rapidly involuting congenital haemangioma: maximal in size at birth but involute in the first 12–18 months.• First‐line treatment is medical, utilising propranolol, with surgery reserved for complicated (ulcerating, bleeding) or refractory lesions.Radiological features• The purpose of imaging is to characterise, locally stage for the deep extent and assess for multiplicity.• Haemangiomas present clinically as a raised ‘strawberry‐like’ plaque, which may be classified as cutaneous, subcutaneous, visceral or mixed. Assessment of deep lesions and definition of locoregional extent in children should be undertaken with ultrasound and magnetic resonance imaging (MRI).• During the proliferating phase, haemangiomas are well defined but of mixed echogenicity on ultrasound with low‐resistance high flows on Doppler studies.• On MRI haemangiomas are of heterogeneous and usually slightly hyperintense signal relative to muscle on T2‐weighted imaging, with flow voids and intense contrast enhancement.• MRA may show associated anomalies such as in PHACES (posterior fossa malformations–haemangiomas–arterial anomalies–cardiac defects–eye abnormalities–sternal cleft and supraumbilical raphe) syndrome.Differential diagnosisKey radiological differencesVenocavernous malformationInsinuating, trans‐spatial, variable enhance-ment, phleboliths.Arteriovenous malformationFlow voids, minor soft tissue component.Hypervascular primary neoplasmsAvidly contrast enhancing, flow voids.Hypervascular metastasesAvidly contrast enhancing, flow voids, known primary tumour.CHAPTER 12Vascular Anomalies oftheMid‐ andLower FaceMichael Bynevelt, Andrew Thompson and Bernard Koong Extensive trans-spatial lesion involving the right side of the face, masticator space and right orbit. Clinical appearances and ultrasound appearances were consistent with an infantile haemangioma (a) (b)Figure12.1 Infantile haemangioma: fat‐saturated T2‐weighted (a) and T1‐weighted (b) coronal MRI.(a) (b)(c) (d) Well-circumscribed, intensely enhancing mass with the typical appearances of a haemangioma(e) The lesion was moderately ‘vascular’ on initial imaging but demonstrated involution over 1 year with medical therapyFigure12.2 Infantile haemangioma of the right parotid and buccal spaces: fat‐saturated T2‐weighted axial MRI (a); T1‐weighted axial MRI (b); fat‐saturated gadolinium‐enhanced T1‐weighted axial MRI (c); Doppler ultrasound of the lesion at diagnosis (d); and 12 months post treatment (e). Vascular Anomalies oftheMid‐ andLower Face 19312.2 Other lesions included inthis grouping• Tufted angioma.• Kaposiform and spindle cell haemangioendotheliomas.• Pyogenic granulomas.• Haemangiopericytoma.VASCULAR MALFORMATIONS• A spectrum of non‐proliferative lesions resulting from errors in vascular formation. Uncomplicated lesions increase in size proportionally with patient growth.• Can be grouped according to the morphology of their con-stituent vascular structures (capillary, venous, cavernous, lymphatic, arterial or combined).• Lesions with metameric associations can be grouped as a craniofacial arteriovenous or craniofacial venous metameric syndrome.• Intralesional blood flow rates, demonstrated on colour Doppler ultrasound, facilitate categorisation.Complications• Often cause any of these lesions to rapidly increase in size, become painful and may compromise adjacent structures. These include: ◦intralesional thrombosis ◦inflammation and infection ◦haemorrhage – importantly, possible intraosseous and transosseous communications are a consideration prior to local surgical procedures, including local anaesthetic injections and dentoalveolar/orofacial procedures. ◦Kasabach–Merritt syndrome: a consumptive coagulopathy and thrombocytopenia.12.3 Low‐flow lesionsVenolymphatic malformations or lymphangiomas(Figure12.3)• Congenital lesions consisting of malformed lymphatic channels, the majority of which are found in the head and neck region, most commonly in the posterior triangle. The lesions can also be found in the orbit, floor of the mouth and in the oral tongue.• Usually present before 2 years old as a compressible swelling with a yellow‐red discoloration of the overlying skin when presenting as a mucosal lesion.• Slow growing unless complicated by infection, inflammation or haemorrhage, when they can suddenly increase in size.• Aside from conservative management, which may be employed for small microcystic lesions, treatment options include percutaneous sclerotherapy and surgery.Radiological features• The purpose of imaging is to characterise, locally stage for the deep extent and assess for multiplicity.• Structurally are cystic, with variable signal on MRI reflecting cyst content, which may be haemorrhagic or proteinaceous.• Septated macrocystic lesions (>1 cm) are more commonly present at birth, in contrast to the more prevalent microcystic (<1 cm) lesions that present later in age.• Variable enhancement is observed following intravenous contrast administration; pure lymphangiomas are non‐ enhancing.• Appearances on ultrasound and MRI vary in accordance with the size of the constituent cysts. The microcystic type is dif-fusely hyperechoic on ultrasound without demonstrable flow, hypointense on T1- and hyperintense on T2‐weighted MRI. The macrocystic subtype are septated anechoic structures, which may reveal fluid–fluid levels, but no flow with Doppler interrogation.• Computed tomography (CT) is often the imaging modal-ity of choice if the patient presents urgently with a com-plication, potentially requiring urgent therapeutic intervention.Differential diagnosisKey radiological differencesBranchial cleft cyst Location: in the region of the angle of mandible.Ranula No contrast enhancement.Dermoid cyst High T1 signal.Thyroglossal duct remnantRelated to hyoid and midline position.Capillary malformations• These lesions are clinically readily apparent and, although typically isolated, have important syndromic associations such as Sturge–Weber syndrome (with a ‘trigeminally’ distributed lesion or ‘port‐wine stain’ on the face, with leptomeningeal, choroidal and oral mucosal lesions) and Klippel–Trénaunay syndrome.• Treatment includes pulsed laser therapy. 194 Atlas of Oral and Maxillofacial RadiologyVenocavernous malformations (Figures 12.4–12.8)• Composed of dilated dysplastic venous spaces, which may be focal and encapsulated or diffuse with trans‐spatial insinuation.• Often extend around a neurovascular bundle, along and through the fascial planes, and into deep anatomic subsites in the neck, such as the masticator and parapharyngeal spaces. Lesions can be in part or entirely intraosseous.• The patient may have multiple cutaneous and mucosal venous malformations as part of an inherited disorder such as the blue rubber naevus syndrome.• Lesions are bluish, may be compressible and/or expand on Valsalva manoeuvre.• Percutaneous sclerotherapy is the primary treatment option. Focal lesions are typically sequestered but drain into normal adjacent veins. Diffuse lesions may have extensive regional venous drainage, which, if not recognised, may cause sys-temic toxicity with sclerotherapy.Radiological features• The purpose of imaging is to characterise, locally stage for the deep extent and assess for multiplicity. Cone beam computed tomography (CBCT) is insufficient.• MRI characteristics of these lesions include high signal on T2‐weighted imaging and isointense on T1‐weighted imaging relative to skeletal muscle.• Fat‐saturated T2‐weighted imaging provides the best infor-mation related to the local extent of lesions, their intrinsic flow rates and soft tissue/calcified components.• Large lesions are often cystic, exhibiting prominent vascular channels, and are hyperintense with septations. Small lesions reveal less prominent vascular channels and tend to be more ‘solid’ with intermediate signal intensities.• On T1‐weighted imaging lesions are lobulated and associated localised fat hypertrophy has been described.• With intravenous contrast medium, homogeneous or hetero-geneous enhancement patterns are seen.Macrocysticanechoic spaces(a) (b) (c) (d)Prominent capsular and septal enhancementLarge trans-spatial multiseptated high T2 and isodense T1 signal mass centred on the rightfloor of the mouth Prominent septal flowFigure12.3 Inflamed lymphangioma: Doppler ultrasound of the lesion (a); fat‐saturated T2‐weighted coronal MRI (b); T1‐weighted coronal MRI (c); and fat‐saturated gadolinium‐enhanced T1‐weighted coronal MRI (d). Vascular Anomalies oftheMid‐ andLower Face 195(a) (b)Ill-defined isointense lesion centred on the left side of the tongue, displacing and involving the intrinsic musculature and left sublingual space High-signal lesion with foci of low signal representing prominent vascular flow voidsFigure12.4 Venocavernous malformation: T1‐weighted coronal MRI (a) and fat‐saturated T2‐weighted coronal MRI (b).(a) (b) (c) (d) *****High T2 and T1 signal lesion within the right masseter muscle with surrounding strandingand symptomatically painful with ipsilateral facial nerve symptomsThe lesion demonstrated moderate enhancement and regressed over the following few weeksFigure12.5 Thrombosed venocavernous malformation, multifocal venous malformations (asterisks): fat‐saturated T2‐weighted axial MRI (a); T1‐weighted coronal MRI (b); fat‐saturated gadolinium‐enhanced T1‐weighted axial MRI (c); and fat‐saturated T2‐weighted coronal MRI (d). Vascular tissueSpiculated appearance(a)(b)Figure12.6 Right mandibular low‐flow vascular malformation: non‐contrast axial bone (a) and soft tissue (b) MDCT images. (Courtesy of Professor Paul Monsour, School of Dentistry, The University of Queensland, Australia.)Phleboliths Vascular tissue associatedwith the phleboliths(a) (b)Figure12.7 Bilateral low‐flow vascular malformations adjacent to the maxilla: non‐contrast axial bone (a) and soft tissue (b) MDCT images.Delayed phasecontrast enhancementEnlarged muscles with no arterial phase enhancement(a) (b) (c)Figure12.8 Right hemifacial low‐flow vascular malformation involving the masticatory muscles: postcontrast axial (a) and coronal (b,c) MDCT images. Vascular Anomalies oftheMid‐ andLower Face 197• MRA and magnetic resonance venography (MRV) may reveal localised and remote associated vascular changes and multi-focal lesions. An associated enlarged draining vein or anoma-lous venous drainage may be seen.• When involving the bones of the jaw, CT may demonstrate a well‐marginated or ill‐defined lesion. A variety of bone changes can be seen in the lesion, which have been described as ‘lace like’, ‘sunburst’, ‘soap bubble’, ‘honeycomb’ or ‘tennis racket’. A periosteal reaction may also be seen.Prominent feeding and draining vessels may result in curvi-linear or serpiginous lucencies within the mandible and may enlarge the mandibular canal.• When involving teeth, there is usually root resorption and tooth displacement.• Phleboliths may also be demonstrated.• Multidetector computed tomography (MDCT) angiography may also assist with lesion characterisation and staging.• Doppler ultrasound assists in the portrayal of vascular chan-nels and their associated flow rates. Lesions with smaller vas-cular channels are generally of increased echogenicity and are less compressible. Phleboliths appear as echogenic foci that cause posterior acoustic shadowing.• The general appearance of the lesion is often quite ‘aggressive’ when inflamed or with superimposed thrombosis. Malignancy and inflammatory lesions are a necessary consideration in this setting. • MDCT is often the imaging modality of choice if the patient presents as an emergency with a complication, potentially requiring urgent therapeutic intervention.Differential diagnosisKey radiological differencesHaemangioma High T2 signal, avidly enhancing, clinical.Lymphatic malformationNo contrast enhancement, fluid–fluid levels.Arteriovenous malformation (slowflow)Flow voids, minor soft tissue component.12.4 High‐flow lesionsArteriovenous malformations (Figure12.9)• Composed of single or multiple arteriovenous fistulae bypass-ing the normal highly resistive capillary bed, are often trau-matic but also present at birth, arising in the face or cavernous sinus region. The associated vessels in this category possess a true vessel wall structure.• Despite the rapid flows, a lesion may not be apparent clini-cally prior to detailed clinical and radiological investigation.• Clinically they are pink/blue lesions, often exhibiting a palpable thrill or audible bruit, and may grow in stages enlarging from quiescence to that which causes systemic cardiovascular decompensation.Radiological features• The purpose of imaging is to characterise, locally stage for the deep extent and assess for multiplicity. CBCT is insufficient.• Jaw based lesions usually present as a lucent (unilocular) or variably multilocular well‐defined corticated lesion and most commonly these are seen in the posterior mandible. Occasionally, the borders are ill defined in appearance, espe-cially on intraoral and panoramic radiographs.• Mandibular canal and associated foramina are often enlarged and are curvilinear or serpiginous in outline.• Small lucent channel‐like lucencies at the jaw cortices are often present.• Occasionally, a spiculated periosteal response is seen and intralesional phleboliths may be identified.• When involving teeth, there is usually root resorption and tooth displacement.• MDCT angiography provides a structural anatomical assess-ment. Similarly, MRI, MRA and MRV demonstrate a tangled mesh of dilated arteries and veins, containing flow voids. Susceptibility‐weighted sequences can demonstrate associ-ated haemorrhagic foci and contrast studies will highlight some components of slow flow and tissue injury.• Conventional or catheter angiography (internal and external carotid artery) can confirm the suspected diagnosis, providing more refined information as regards arterial ‘feeders’, draining veins, flow characteristics, nidal configuration as well as col-lateral vessels which may contain dangerous anastomoses.• MDCT is often the imaging modality of choice if the patient presents as an emergency with a complication, potentially requiring urgent therapeutic intervention.• Doppler ultrasound demonstrates high systolic and diastolic flow, and arteriovenous shunting with arterial waveforms inveins.• Lesions often appear quite ‘aggressive’ when inflamed or with superimposed thrombosis.Differential diagnosisKey radiological differencesHaemangioma High T2 signal, avidly enhancing, clinical.Lymphatic malformationNo contrast enhancement, fluid–fluid levels.Venocavernous malformationInsinuating, trans‐spatial, variable enhance-ment, phleboliths.Vascular tumour Avidly contrast enhancing, flow voids.Hypervascular metastasesAvidly contrast enhancing, flow voids, known primary tumour. 198 Atlas of Oral and Maxillofacial RadiologyEnlarged mandibular foramen and canalLucent channels at the mandibular corticesWidened follicular space with displaced developing crownRoot resorptionTortuous feeder and draining vessels(a)(b) (c) (d)Figure12.9 Left mandibular arteriovenous malformation: panoramic radiograph (a), axial non‐contrast MDCT (b,c) and corrected sagittal MDCT angiogram (d) images.

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