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190 Reid C. Mahoney, et al.
Internal jugular (IJ) and external jugular (EJ) aneurysms tend to be benign, fusiform, and
most often present as an asymptomatic neck mass.
(16)
Right-sided IJ aneurysms are more
common than left-sided IJ aneurysms (Figure18.3). EJ aneurysms are equally distributed
between left and right sided. Some patients have discomfort from a neck aneurysm, and this
is more common in EJ than IJ aneurysms; however, voice changes, cough, and dysphagia
are only associated with IJ aneurysms, although the rates of these symptoms are all <5%.
(17)
Rupture has not been reported. Although thrombosis has been reported in several case studies, the rise of pulmonary embolus appears low.
(4)
Neck masses encompass many different pathologies, including neoplasm and cysts, but
painless masses that enlarge with Valsalva maneuver should incorporate internal jugular
venous aneurysms into the differential diagnosis. IJ venous aneurysms are often diagnosed in
the pediatric population, but a wide range of ages at diagnosis have been reported. Pediatric
patients tend to have aneurysmal degeneration of the right side, whereas older patients tend
to have left-sided aneurysms attributed to calcified arch vessels causing external compression
or traumatic forces.
(18)
Following history and physical, ultrasound is often the only imaging
necessary for diagnosis. If there are equivocal findings on ultrasound or an operation is being
planned, CT or MRI can be performed. Serial imaging and conservative management are
an appropriate treatment strategy for asymptomatic patients once diagnosis is confirmed.
Intervention should be considered for growth, pain, localized compression symptoms, and
cosmetic reasons. Surgical intervention options include aneurysmectomy and venorrhaphy,
bypass, or ligation. Ligation with aneurysm excision is an appropriate option for EJ aneurysms. Patency of the contralateral internal jugular vein should be confirmed if ligation is
performed, as there are reports of intracranial hypertension after right side IJ ligation.
(17)
Thoracic venous aneurysms include subclavian, innominate, and superior vena cava (SVC)
aneurysms. Several case reports and case series have been published encompassing aneurysms
in these locations.
reasons, frequently by chest X-ray when a widened mediastinum is found.
(1)
Most are diagnosed incidentally when imaging is performed for other
(1)
Providers must
be aware of the possibility that a thoracic or mediastinal mass may indeed be a venous aneurysm, as biopsy or surgical intervention can be catastrophic.
(19)
Most thoracic venous aneurysms are managed nonoperatively with low rates of significant complications. Subclavian
vein aneurysm patients have been treated nonoperatively, with endovascular surgery, or with
open surgery, all of whom recovered without complications in the follow-up period.
(1)
Central thoracic venous aneurysms, including the innominate artery and the SVC, tend to
have low rates of complications including rupture and pulmonary embolism. It should be
noted that fusiform SVC aneurysms portend a more benign course while saccular aneurysms,
usually post-infectious or inflammatory, tend to present with more complications.
(20)
Surgical
intervention on any central thoracic venous aneurysm is difficult and should be preceded with
appropriate preoperative risk stratification and patient discussion. There are case reports
describing intraoperative death including massive pulmonary embolism from manipulation
of the aneurysm.
(1)
Contained ruptures of the SVC have been managed nonoperatively with
patient survival, adding to the concept that risks associated with surgical intervention most
often outweigh benefit.
(3)
Abdominal Venous Aneurysms
Portal Venous System
Superior mesenteric venous aneurysms, as part of the portal venous system, are often described
in conjunction with portal venous aneurysms (Figure18.4). Cirrhosis or malignancy have
been associated with PV/SMV aneurysm.
(21)
Diagnosis can be by ultrasound as an unexpected
Evidence-Based Management of Venous Aneurysms 191
Figure 18.4 Aneurysm of the portal superior mesenteric vein confluence.
finding in a patient being assessed for other pathology, but CT and MRI remain reasonable
options for diagnosis in the face of equivocal ultrasound findings. Patients tend to present
without symptoms, but vague abdominal pain and GI bleeding can be seen,
(1)
and there are
reports of patients with biliary or duodenal compressive symptoms from aneurysmal degeneration of the portal venous system.
(22)
Given the preponderance for these aneurysms to be
associated with other pathology, it is difficult to determine the true significance of aneurysmal
degeneration in these vessels, but case reports describe operative intervention for complications including thrombosis or rupture.
with aneurysmal degeneration of the portal venous system without known liver disease.
(21)
Areview by Laurenzi et al included 128 patients
(23)
Thirty-two of these patients underwent surgical intervention, the majority of which underwent aneurysmectomy with venorrhaphy. Eighty percent, however, were treated nonoperatively, and this appears to be a viable management strategy for asymptomatic or high-risk
surgical patients.
Inferior Vena Cava
Inferior vena cava (IVC) aneurysms are also very uncommon. They may be primary, congenital, associated with trauma, arteriovenous fistula, inflammation, or neoplasm. Primary IVC
aneurysms may represent a congenital abnormality of persistence, degradation, or fusion of
the embryonic cardinal veins from which the IVC is formed (Figure18.5).
(24)
Patient presentation ranges from asymptomatic and discovered on incidental imaging finding to acute thrombosis to pulmonary embolism.
(9)
Ultrasound can be used for diagnosis, but the retrohepatic
and suprahepatic IVC is best imaged utilizing CT or MRI.
Gradman and Steinberg described a classification system for IVC aneurysms that has
proven useful in describing IVC aneurysms and, as such, can aid in guiding clinical management (Figure18.6).
(25)
Wang et al, based on a literature review, summarized IVC aneurysms
192 Reid C. Mahoney, et al.
Figure 18.5 Intraoperative photo of infrarenal inferior vena cava aneurysm.
Figure 18.6 Gradman and Steinberg classification of inferior vena cava aneurysms.
according to the Gradman and Steinberg classification system.
(9)
Type IIVC aneurysms represent 35% of IVC aneurysms, are often fusiform or asymptomatic, and do not appear to
be associated thrombosis or VTE complications. Given the complexity of surgical management, type IIVC aneurysms are likely best served with conservative management; successful
nonoperative management is reported in over 90% of patients.
(9)
Type II IVC aneurysms,
18% of IVC aneurysms, appear to be mostly congenital, and more than 90% are saccular.
Most patients present with abdominal or back pain as well as limb swelling. IVC thrombosis pulmonary embolism occurs in approximately one-third of these patients. Type III IVC
aneurysms appear to be the most common type, accounting for 45% of IVC aneurysms.
They tend to present with symptoms including back pain, abdominal pain, leg swelling,
IVC thrombosis (42%), DVT (45%), and PE (12%). More than 90% are saccular, and more
than 90% are congenital. Type IV IVC aneurysms are rare, accounting for <10% of IVC
Evidence-Based Management of Venous Aneurysms 193
aneurysms. All reported cases are saccular and congenital. There was one death from rupture
in this subgroup.
(9)
Based on these findings, surgically fit patients that have a type II–IV IVC
aneurysm should be intervened on. However, small asymptomatic IVC aneurysms can likely
be followed with serial imaging. Montero-Baker et al consider flow dynamics of type II–IV
IVC aneurysms to be different than type Iand therefore, if surgically accessible, recommend
surgical intervention to avoid complications of thrombosis and pulmonary embolism.
(26)
CONCLUSIONS
Venous aneurysms may occur in nearly all veins. Their management depends on several
important factors including anatomic location, size, and clinical presentation. Decisions
regarding management can have significant impact on the patient and should be based on
available evidence as discussed in this chapter. Popliteal venous aneurysms are associated
with significant clinical problems including pulmonary embolus, are surgically accessible,
and therefore should be dealt with promptly. Upper extremity, cervical, and superficial lower
extremity venous aneurysms tend to have a more indolent course. Thoracic and abdominal
venous aneurysms require an individualized approach with treatment algorithms proposed
for IVC aneurysms based on anatomic location.
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3. Gloviczki P (Eds.). Handbook of Venous and Lymphatic Disorders: Guidelines of the American
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Chapter 19
Diagnosis and Management of
Vascular Malformations
Steven M. Farley
INTRODUCTION
The starting point to the management of vascular malformations is nomenclature. Vascular
anomalies are divided into vascular tumors and vascular malformations. Hemangioma is
a term commonly misused to describe any vascular anomaly. In reality, a hemangioma is a
specific disease – a benign vascular tumor most commonly diagnosed in infants. As a benign
tumor, a hemangioma goes through stages of growth and involution. Avascular malformation is not a tumor but an error of embryogenesis.
As such, most malformations do not grow and certainly do not involute. Many parents are
told their child’s malformation should regress or disappear but see no improvement as the
child grows. Another challenge with terminology has been the use of different nomenclature
systems over time and a lack of consensus on terminology. The International Society for the
Study of Vascular Anomalies (ISSVA) has created a single nomenclature system.2 Referencing
this document is initially challenging due to the high level of detail regarding syndromes and
genetic information.
Once identified as a vascular malformation, in clinical practice, a malformation can be best
described as low flow or high flow. Low flow malformations are more common, can be purely
venous or have a lymphatic component as well. High flow lesions reference a high volume of
blood flow through the malformation and denote an arterial component to the lesion. High
flow lesions are generally more difficult to manage. They will grow over time with low sheer,
high flow characteristics. High flow lesions are more likely to bleed, be painful and cause
wounds or tissue loss. Moreover, control of the high flow lesion is more difficult and complex.
1
LOW FLOW MALFORMATIONS
Presentation
A low flow lesion can present in a variety of ways. Some lesions are superficial and are visible
on physical exam with a blueish lesion in the skin. Other lesions cause local tissue swelling,
and a visible bump alerts the patient or family. With regard to symptoms, patients can present
with venous type complaints – pain, throbbing, local swelling, heaviness, phlebitis – in any
location of the body. At times, low flow lesions are asymptomatic and are an incidental finding from cross-sectional imaging. Age at presentation varies, but often lesions are identified
by the teenage years.
Imaging
A plain radiograph can be a route to diagnosis. Low flow lesions often demonstrate phleboliths, small calcific spheres (Figure19.1). Phleboliths are thought to form due to intermittent episodes of thrombosis in the irregular flow channels in a low flow lesion. Ultrasound
DOI: 10.1201/9781003316626-22 195
196 Steven M. Farley
Figure 19.1 Phleboliths in a low flow vascular malformation. Fluoroscopy of the right leg, arrow points to
phleboliths.
duplex can identify low flow lesions. On B-mode, compressible vascular channels and lakes
can be seen. On color mode and Doppler, chaotic and multidirectional low velocity flow is
seen within the vascular channels. Phleboliths can demonstrate post-acoustic shadowing and
post-phlebitic vessel wall thickening is often present. Computed tomography (CT) imaging is
not generally helpful except to exclude a high flow lesion. Magnetic resonance imaging (MRI)
is the primary modality to fully characterize a low flow lesion. MRI provides excellent soft
tissue resolution with the ability to differentiate bone, muscle, tendon, fat, skin and malformation. Contrast-assisted, dynamic MRA can also help determine if a vascular malformation
is a high flow lesion through evaluation of the arterial system.
Treatment
Conservative management with elevation, compression garments and non-steroidal antiinflammatories remain the first-line therapy. Low flow lesions do not grow, and symptoms
are often mild to moderate. Phlebitis can develop intermittently and present with periods of
more intense pain, but these symptoms will alleviate in a few weeks.
Patients with daily pain, difficulty sleeping or working will often request treatment.
Malformations are often insinuated into the deeper structures such as muscle, tendon, nerves
and near bone. Surgical excision is often not curative, and symptoms recur. The morbidity of
surgery often does not justify the treatment; therefore, embolization is the generally preferred
invasive treatment option.
Choice of liquid embolic agents varies among providers. Embolic agents can be categorized
by the efficacy compared to the side effect profile. Absolute alcohol is considered the most
Diagnosis and Management of Vascular Malformations 197
powerful agent with a mechanism of direct denaturation of proteins. The potency of alcohol
is balanced by its higher side effect profile characterized by damage to nearby structures such
as nerves, tendons and skin. If injected superficially, alcohol can blister and ulcerate the skin.
Afurther concern regarding alcohol use relates to total dose or volume. Higher doses can
result in alcohol intoxication and sudden pulmonary venous hypertension.
Detergents are another class of liquid embolic agents. These chemicals, such as sodium
tetradecyl sulfate and polidocanol, are commonly used for the treatment of varicose veins.
Detergent agents come in varying concentrations with higher concentrations more associated
with complications such as skin damage or deep vein thrombosis. Detergent agents can be
delivered as liquid or can be foamed to expand the volume and displace the blood, allowing for more endothelial contact with the detergent. Hand foam is generated using air by
the Tessari method.3 Alow nitrogen formulation of polidocanol is available commercially
but not FDA approved for treatment of vascular malformations.4 Detergent agents are most
commonly delivered by direct needle puncture of the malformation as seen in Figure19.2.
Digital subtraction angiogram of a low flow vascular malformation, Blue arrow points to
the needle used for access. Red arrow points to the low flow vascular malformation. White
arrow point to normal draining veins
Liquid to solid polymerizing agents can also be utilized for embolization. Cyanoacrylate
is commonly used for GI bleeding and is rapidly polymerizing glue. It is generally injected
transcatheter but can be used through a direct needle puncture. It rapidly solidifies and does
not generally allow for repeat treatments through the same catheter. Ethylene vinyl alcohol,
tradename Onyx, is another polymerizing agent approved for treatment of brain AVMs. It
can also be injected by a transcatheter or direct needle injections. Advantages include a slower
polymerization rate of 30 to 60 seconds. It also comes in two different densities to allow for
different flow and penetration rates. Both liquid embolic agents require careful use to prevent
Figure 19.2 Direct needle puncture of a low flow vascular malformation.
198 Steven M. Farley
non-target embolization and “trapping” or gluing the catheter into the patient. Onyx remains
radiodense and can obscure further imaging by angiography or CT scanning. Surgical excision
of Onyx with electrocautery can create sparks due to the rare earth element tantalum used in
Onyx.5 Bipolar electrocautery can reduce the fire risk during surgery. In general, the use of liquid
embolic agents for venous malformations in clinical practice has been associated with only partial occlusion or recanalization of the treated areas. Also, many low malformations present in
superficial locations, and some patients will complain of the firmness of the glue under the skin.
Mechanical agents can also be used for low flow embolization. Large embryonal veins and
larger diameter vascular channels can be filled with a variety of coils or plugs. However, most
low flow malformations are not well managed with mechanical occlusions devices.
Surgery for extensive malformations involving muscle layers or deeper are rarely curative
as complete removal of the malformation is not possible. Symptom improvement can be seen
with incomplete, debulking-type surgery; however, over the course of years, most patients
have recurrent symptoms. Curative surgery is more likely in lesions confined to the skin and
subcutaneous layers.
HIGH FLOW MALFORMATIONS
Presentation
High flow lesions can be symptomatic or asymptomatic. Anatomically, these lesions can present anywhere on the body with the highest rates in the brain. These lesions are more likely to
grow, to be destructive of local anatomy and to present with bleeding (at times life threatening) and tissue loss (Figure19.3) than their low flow counterparts.
IMAGING
Plain radiographs rarely are helpful with high flow lesions except to evaluate long bone
length or bone loss. Duplex ultrasound can assist in the diagnosis of a high flow lesion. On
B-mode, duplex can identify a vascular lesion and map blood vessels. On color flow and
Figure 19.3 High flow malformation on physical exam. Localized swelling of the fifth digit, skin changes with
early ulceration.
Diagnosis and Management of Vascular Malformations 199
Doppler, high flow lesions will demonstrate mosaicism and high flow, low resistance arterial
flow. Unlike low flow lesions, CT imaging can be helpful with high flow lesions. Arterial
ectasia and early contrast filling of the venous system is often seen with high flow lesions.
Similar to CT, MRI and contrast-enhanced, dynamic MRI will demonstrate arterial ectasia,
the nidus filling and early venous drainage of contrast (Figure19.4). Digital subtraction trans
arterial angiography is diagnostic, demonstrating arterial feeding vessels, nidus filling and
early venous filling as seen in Figure19.5.
Digital subtraction angiogram of the right foot. Ectatic tibial arteries feed the AVM nidus
centrally.
Figure 19.4 Magnetic resonance angiography reconstruction of a high flow pelvic AVM.
Figure 19.5 Angiogram of a high flow vascular malformation.