Venous malformation!

AIM : ROLE OF IMAGING IN AIDING  PLANNING RX

Vascular malformations are classified as high-flow or low-flow or a combination of the

two.

The distinction between high flow and lowflow is important:

High-flow anomalies are treated by transcatheter embolization, whereas lowflow

lesions are treated by the percutaneous injection of sclerosing agents (sclerotherapy).

Low-flow malformations include capillary, lymphatic, and venous types.

High-flow malformations can be arterial or arteriovenous.

MARKS OF VASCULAR ORIGIN.

• A classification ------
• Hemangiomas and congenital vascular tumors
• Infantile
• Congenital
• Noninvoluting
• Intramuscular
• Kaposiform hemangioendothelioma
• Vascular malformations
• High-flow
• Arteriovenous malformation
• Arteriovenous fistula
• Low-flow
• Capillary malformation (“port-wine stain”)
• Venous malformation
• Lymphatic malformation
• Combined
• High-flow
• Parkes-Weber syndrome: capillary, arterial, venous
• malformation in a limb, with limb overgrowth
• Low-flow
• Klippel-Trenaunay syndrome: capillary-lymphaticvenous
• malformation in a lower extremity with
• limb overgrowth
• Maffucci syndrome: lesions resembling venous
• malformations, with enchondromatosis

Summary                                                                          

MATERIAL AND METHODS:

• 15y boy, having bluish discoloration of lateral aspects of thigh and leg, with increasing pain and swelling about the lesion, present since birth with more of discomfort since past 2-3yrs.
• No history of trauma/surgical intervention revealed.
• Local examination revealed compressible swelling with no abnormal bruit/pulsation with normal local temperature. Size of swelling increases on standing/walking or running.
• Underwent: Doppler US,CT angio and MRI.

IMAGE GALLERIA                                                                                                                                                                                                                                                      

RESULTS :ULTRASOUND AND DOPPLER

RESULTS :CT SCAN PERIPHERAL ANGIO  

RESULTS :MRI

DISCUSSION: DIFFERENTIATING THE DIFFERENTIALS

What is : KTS?

KLIPPEL-TRENAUNAY SYNDROME

KTS is a slow-flow combined vascular anomaly (capillary-lymphatic-venous malformation) that is typically associated with marked overgrowth of the leg and geographic capillary stains. The condition may rarely be associated with hypotrophy. Anomalous lateral veins, which are typically on the lateral aspect of the thigh, become prominent because of incompetent valves and deep venous anomalies. Thrombophlebitis and pulmonary embolism are the main complications in these patients.

Imaging, typically MRI and/or MRA, is mainly used to evaluate the extent and patency of the deep veins, as shown in the image below.

 MRI findings are variable because of the wide range in the severity of this disorder.

The lymphatic component of the disease may be macrocystic or microcystic. Increased fat is often identified in the areas of soft-tissue overgrowth.

In severe cases, the deep venous system is anomalous (hypoplastic or interrupted), and drainage occurs mainly via markedly dilated, anomalous superficial veins.

The pathognomonic marginal vein of Servelle is frequently identified in the lateral calf and thigh. MRV is the imaging modality of choice in the evaluation of the patency of deep veins before attempts at surgery. Contrast-enhanced angiography may reveal discrete microarteriovenous fistulas.

ASSOCIATES

• Proteus syndrome
• The major clinical features of Proteus syndrome, a rare vascular anomaly, include verrucous nevus, lipomas and/or lipomatosis, macrocephaly, asymmetric limbs with partial gigantism of the hands and feet, and cerebriform plantar thickening. The characteristic MRI finding is abundant fat deposition in the malformation. MRA and MRV can elucidate the composition of focal masses and the underlying vascular anatomy.
• Maffucci syndrome
• In Maffucci syndrome, low-flow vascular malformations are associated with bony exostoses and enchondromas. The osseous lesions usually appear in children, and vascular lesions manifest in older patients. Bony and vascular lesions may be unilateral or bilateral. Vascular lesions are low-flow vascular malformations similar to VMs. Patients typically have spindle-cell hemangioendotheliomas, a reactive vascular proliferation within a pre-existing vascular malformation. Malignant transformation, most commonly chondrosarcoma, occurs in 20-30% of patients.
• Parkes -Weber syndrome
• This rare fast-flow combined vascular malformation usually involves a lower limb, and it is usually associated with a geographic stain over the enlarged limb. Symptoms include cutaneous warmth and a bruit or thrill on clinical examination, all of which are more suggestive of a complex vascular malformation than a simple CM. MRIs and MRAs show enlarged extremity muscles and bones with an abnormal signal intensity and contrast enhancement pattern; they also show generalized arterial and venous dilatation in the involved extremity

CONCLUSION :

• Multimodality approach, clinical picture as well as understanding the natural course of the disease aids in  patient management

CASE OF THE MONTH

CLINICAL PROFILE:

A 39 year-old man presented with recent onset of headache and convulsions.

CT scan performed elsewhere, showed a space-occupying lesion in right parietal area, which was predominantly hypodense with a few hyperdense areas within it. (? Calcification / haemorrhage).

Patient was referred to us for MRI of the brain.

MRI of the Brain was performed, both before and after intravenous contrast (Gadolinium) injection. 

FINDINGS:

A large, fairly well defined area of altered signal intensity was noted involving the right parietal region white matter. The signal intensity pattern was suggestive of an intraparenchymal haematoma with perifocal oedema ( Fig. 1).

Fig 1: a) T1W sagittal image and b) Gradient Echo axial image showing the haematoma in right parietal region.

Multiple tiny nodular areas of profound low signal intensity on all sequences were noted at the central and anterior portion of the lesion suggestive of ‘Flow voids’ which on IV contrast were confirmed to be multiple linear radiating vascular channels giving ‘spider like’ appearance.  A dilated venous channel of average diameter 6mm was noted extending from lateral aspect of this area and running supero-medially to drain into the superior sagittal sinus( Fig.2).

FIG.2 Coronal post contrast scan showing a prominent ‘collector vein’( yellow arrow) draining into the superior sagittal sinus.

A diagnosis of Developmental Venous Anomaly/ Venous Angioma was made, based on these imaging findings.

Digital subtraction angiography of the cerebral vessels was performed via right common femoral arterial route using a headhunter catheter at our institute under all aseptic precautions.

Multiple tiny radiating vascular channels of average diameter 1 to 2mm were noted in the right posterior parietal region in the venous phase of right internal carotid artery injection giving ‘Medusa head’ appearance on lateral view.

These small veins converged on a large vein measuring approximately 0.6cm in diameter draining into the superior sagittal sinus after a curvilinear course. The diagnosis of a Developmental Venous Anomaly (DVA) / Venous angioma was confirmed.

Fig.3 DSA (late venous phase) after right Internai carotid artery injection , showing the medullary veins forming medusa head ( blue arrow) and draining into the collector vein( black arrow).

Patient is advised a follow up MRI after 6 weeks, to look for any associated vascular malformation like a cavernous angioma.

DISCUSSION:

Venous angioma of the brain consists of multiple, radially oriented dilated medullary veins that drain into a transparenchymal venous stem. It is probably the most common anomaly of the intracranial vasculature. Venous angiomas are considered to be prone to cause intracranial haemorrhage.

Imaging

Venous angiomas of the brain have many appearances, ranging from a small, single draining vein, involving at most one small portion of brain to a large hemispheric venous anomaly draining an entire hemisphere. Venous angiomas can occur almost anywhere, but favored sites include the frontal lobes and the posterior fossa. They are typically unilateral, although bilateral ones have been reported, particularly in the posterior fossa.

Angiography:  The most common angiographic descriptions of venous angioma include a ‘caput medusae’ appearance of deep, medullary veins during the early to middle venous phase, accompanied by a single large draining vein, most often extending transhemispherically to a superficial cortical vein or dural sinus.

CT:  With contrast-enhanced CT, the draining vein of a venous angioma is readily detected as a linear or curvilinear focus of enhancement, typically coursing from the deep white matter to a cortical vein or a deep vein or to a dural sinus.

MRI:  On MR images, venous angiomas characteristically have a transhemispheric flow void on both T1- and T2-weighted images. After the administration of MR contrast material because of slow flow, significant enhancement of both the draining vein and often the caput medusae of medullary veins may be seen. In complicated venous angioma, associated hemorrhage, cavernous angioma or, exceptionally, nonhemorrhagic venous infarction may be seen.

Treatment

Treatment such as surgical removal of the venous angioma usually isn't recommended because the lesions typically cause no problems and surgical intervention may lead to complications such as stroke. Occasionally, venous malformations can be associated with other types of vascular malformations that may require treatment.

CASE OF THE MONTH

CLINICAL PROFILE:

A 39 year-old man presented with recent onset of headache and convulsions.

CT scan performed elsewhere, showed a space-occupying lesion in right parietal area, which was predominantly hypodense with a few hyperdense areas within it. (? Calcification / haemorrhage).

Patient was referred to us for MRI of the brain.

MRI of the Brain was performed, both before and after intravenous contrast (Gadolinium) injection. 

FINDINGS:

A large, fairly well defined area of altered signal intensity was noted involving the right parietal region white matter. The signal intensity pattern was suggestive of an intraparenchymal haematoma with perifocal oedema ( Fig. 1).

Fig 1: a) T1W sagittal image and b) Gradient Echo axial image showing the haematoma in right parietal region.

Multiple tiny nodular areas of profound low signal intensity on all sequences were noted at the central and anterior portion of the lesion suggestive of ‘Flow voids’ which on IV contrast were confirmed to be multiple linear radiating vascular channels giving ‘spider like’ appearance.  A dilated venous channel of average diameter 6mm was noted extending from lateral aspect of this area and running supero-medially to drain into the superior sagittal sinus( Fig.2).

FIG.2 Coronal post contrast scan showing a prominent ‘collector vein’( yellow arrow) draining into the superior sagittal sinus.

A diagnosis of Developmental Venous Anomaly/ Venous Angioma was made, based on these imaging findings.

Digital subtraction angiography of the cerebral vessels was performed via right common femoral arterial route using a headhunter catheter at our institute under all aseptic precautions.

Multiple tiny radiating vascular channels of average diameter 1 to 2mm were noted in the right posterior parietal region in the venous phase of right internal carotid artery injection giving ‘Medusa head’ appearance on lateral view.

These small veins converged on a large vein measuring approximately 0.6cm in diameter draining into the superior sagittal sinus after a curvilinear course. The diagnosis of a Developmental Venous Anomaly (DVA) / Venous angioma was confirmed.

Fig.3 DSA (late venous phase) after right Internai carotid artery injection , showing the medullary veins forming medusa head ( blue arrow) and draining into the collector vein( black arrow).

Patient is advised a follow up MRI after 6 weeks, to look for any associated vascular malformation like a cavernous angioma.

DISCUSSION:

Venous angioma of the brain consists of multiple, radially oriented dilated medullary veins that drain into a transparenchymal venous stem. It is probably the most common anomaly of the intracranial vasculature. Venous angiomas are considered to be prone to cause intracranial haemorrhage.

Imaging

Venous angiomas of the brain have many appearances, ranging from a small, single draining vein, involving at most one small portion of brain to a large hemispheric venous anomaly draining an entire hemisphere. Venous angiomas can occur almost anywhere, but favored sites include the frontal lobes and the posterior fossa. They are typically unilateral, although bilateral ones have been reported, particularly in the posterior fossa.

Angiography:  The most common angiographic descriptions of venous angioma include a ‘caput medusae’ appearance of deep, medullary veins during the early to middle venous phase, accompanied by a single large draining vein, most often extending transhemispherically to a superficial cortical vein or dural sinus.

CT:  With contrast-enhanced CT, the draining vein of a venous angioma is readily detected as a linear or curvilinear focus of enhancement, typically coursing from the deep white matter to a cortical vein or a deep vein or to a dural sinus.

MRI:  On MR images, venous angiomas characteristically have a transhemispheric flow void on both T1- and T2-weighted images. After the administration of MR contrast material because of slow flow, significant enhancement of both the draining vein and often the caput medusae of medullary veins may be seen. In complicated venous angioma, associated hemorrhage, cavernous angioma or, exceptionally, nonhemorrhagic venous infarction may be seen.

Treatment

Treatment such as surgical removal of the venous angioma usually isn't recommended because the lesions typically cause no problems and surgical intervention may lead to complications such as stroke. Occasionally, venous malformations can be associated with other types of vascular malformations that may require treatment.

CASE 1 : HODGKIN’S LYMPHOMA – GASTRO INTESTINAL TRACT

18 year old male presented with mass in abdomen. Right hemicolectomy was done which revealed a large fleshy ulceroinfiltrative tumour involving caecum and appendix. Multiple fleshy mesenteric lymphnodes were identified at the level of the tumour.

Histopatholgy of the specimen revealed (Fig 1 A & B) polymorphic population composed of lymphocytes, eosinophils, plasma cells, Hodgkin’s cells and binucleate Reed - Stenberg (RS) cells.   

Figure 1 A 

          Figure 1 B

Photomicrograph (40x) showing polymorphic population composed of lymphocytes, eosinophils, plasma cells, Hodgkin’s cells and binucleate Reed - Stenberg (RS) cells.   

Discussion :

Hodgkin’s Lymphoma is very rare in the gastrointestinal tract. In our case, presence of classical Reed - Stenberg (RS) cells was the diagnostic clue.  Immunohistochemistry was advised.