M. LAADHARI, A. AISSA, M. KHERIFECH, I. MEZHOUD, K. BEN HELAL*, M. ALLANI, R. ALOUINI
Medical Imaging Ibn El Jazzar Hospital Kairouan
* Department of Pediatrics Ibn El Jazzar Hospital Kairouan
Tunisia
PAN ARAB 2012- PEDIATRICS : PD 9
Venous vascular malformation type of soft tissue discovery at puberty
INTRODUCTION Vascular malformations are a spectrum of unknown injury interesting mainly the pediatric population
Venous malformations are the most common vascular malformations
In case of complex or atypical clinical presentation, the doppler ultrasound and MRI are the two noninvasive imaging techniques which are essential :
To achieve the positive diagnosis towards differential diagnosis
To make an assessment of local and regional expansion referred to pre-therapeutic and prognostic and monitor spontaneous or on treatment of injuries
OBJECTIVES
Illustrate a case of vascular malformation hemodynamically inactive venous type.
Demonstrate the role of different imaging means (standard X-ray, doppler ultrasound, cross-sectional imaging) in the diagnostic confirmation.
OBSERVATION
A 12-year-old patient without a history disease, which was presented to the ED with a painful swelling of the forearm lasting for two days with a history of trauma two weeks ago.
Clinical examination: swelling of the medial surface soft, movable relative to the two planes, painful without cutaneous signs in regard.
An X-ray standard, a doppler ultrasound, a CT scan supplemented by an MRI were performed.
Soft tissue mass with round opacity tone
calcium without adjacent bone
changes.
OBSERVATIONX-ray standard face of the
forearm
Multiple structures tubulated, tortuous hypoechoic, heterogeneous, infiltrating the subcutaneous fat, compressible with multiple hyperechoic spots followed by posterior acoustic shadowing (phlebolites).
No flow at color Doppler and pulse.
OBSERVATIONDoppler Ultra sound
Lesion on the soft tissu, containing many heterogeneous hyperdense calcifications of varying size, with enhancement
after injection discreet locations.
OBSERVATIONC T scann
Training oval in the subcutaneous and muscular tissue composed of contiguous structures serpiginous franc hyperT2, isoT1 with intralesional structures in focal hypoT2 EG and T2
are compatible with phleboliths.
OBSERVATIONM R I(1,5T)
Sequence -coronale -STIR-Sequence -coronale -FSE T1-
OBSERVATIONM R I
Sequence –axiale-FSE T1,FSET2 and T2*
T1
T2
T2*
OBSERVATIONM R I
Precoce and moderate enhancement, heterogeneous and "clumps" after injection.
Axial-FSE T1 Fatsat afterGadolinium
Coronal-FSE T1 Fatsat after Gadolinium
MIP
Coronal-FSE T1 Fatsat after Gadolinium
OBSERVATIONM R I
DISCUSSION
Prerequisite: know the classification of superficial vascular abnormalities.
Many sources of terminological confusion misdiagnosis and inappropriate treatment.
Classification (1996) adopted by the International Society for the
Study of Vascular Anomalies (ISSVA)
VASCULAR TUMORS : abnormal endothelial cell proliferation
Vascular Malformations : embryological vessel abnormalities without abnormal cell proliferation
DISCUSSION
Infantile hemangioma: the most common tumor in infants
Congenital hemangiomas (RICH and NICH), kaposiform hemangioendothelioma, tufted angioma
Exeptionnel: hemangiopericytoma, fibrosarcoma, rhabdomyosarcoma infant …
DISCUSSIONClassification – Vascular
tumor
Classified according to hemodynamic data
(classification more relevant)
Slow flow malformations (hemodynamically
inactive): capillary, venous ,lymphatic, combination of these malformations
Fast flow malformations (hemodynamically active):
Those with a blood component
Fistula or arteriovenous malformation
DISCUSSIONClassification – Vascular
malformations
DISCUSSION
VASCULARMALFORMATIONS
Congenital lesions Present at birth Always sometimes late clinical manifestation (until adolescence)
Lack of spontaneous regression, persistence throughout life with growth proportional to that of the child
Possible phases of thrust if trauma, infection, hormonal changes (puberty, pregnancy)
DISCUSSIONVascular Malformation –
General
Treatment usually necessary + + + Treatment is conditioned by hemodynamic characteristics of the vascular malformations
Importance of the distinction between congenital malformations and slow flow to fast flow
DISCUSSIONVascular Malformation –
General
Place of imaging very limited
Superficial anomaly hardly visible on imaging (sometimes skin thickening and subcutaneous)
Search for underlying vascular malformation or associated anomalies (vascular
syndromes) + + +
DISCUSSIONA slow flux malformation of
capillary type
Most common vascular malformation Old "cavernous hemangioma" (confusing terminology)
Dysplastic veins: venous ectasia or true venous lakes (= cavities with vascular endothelial lining)
Often evident at birth Often asymptomatic, sometimes painful if:
Thrust thrombosis secondary to intralesional or hormonal changes
Depth extension of the muscle
Joint damage
Headquarters: head and neck region (40%), extremities (40%), trunk (20%)
DISCUSSIONA slow flux malformation of
Venous type
Two categories :
Heredatery veinous malformations
Venous malformations common (our case)
The most common Location : Cervicofacial + + and members Often later onset Usual complications: thrombosis in situ always find a localized intravascular coagulation
Treatment only in cases of functional impairment or significant aesthetic
DISCUSSIONA slow flux malformation of
Venous type
Members
« Clinical presentation » Possible with cutaneous, subcutaneous, muscle and joint
Pain due to thrombosis localized to gravity or nerve compression
In case of joint damage: recurrent effusions and hemorrhagic reaction with possible cartilage destruction (type hemophilic arthropathy)
DISCUSSIONA slow flux malformation of
Venous type
« X-ray standard »
Mass of soft tissue Non-specific but inconstant pathognomonic phleboliths (round opacities tone calcium)
Possible bone remodeling adjacent lesions extended
DISCUSSIONA slow flux malformation of
Venous type
« Color and pulsed doppler ultrasound »
Two types of venous malformations
Cavitary +++ Gaps
Phlebolite
Slow venous flow monophasic
No flow (16%): thrombosis or technical limitations (very slow flow below the detection flux) => to Valsalva maneuver
Component two-phase : flow capillary-associated (slow arterial flow)
Dysplasique Multiple varicose dilatations
Multiple structures tubulées tortuous, anechoic, infiltrating the subcutaneous fat, muscle-tendon structures ...
Slow venous flow
DISCUSSIONA slow flux malformation of
Venous type
« CT scann »
Little use
More sensitive than plain radiography for detecting phleboliths
Detection of any fatty component and detection of bone underlying
DISCUSSIONA slow flux malformation of
Venous type
« IRM »PRECONISED PROTOCOLE
Importance of T2 FS or STIR sequences + + +
SE T1 staging (anatomical balance), EG T2 (phleboliths, hemosiderin)
T1 FS gado (evaluation of perfusion)
3D dynamic MR angiography with injection
EG 3D T1 gadolinium bolus (2 ml / s) and subtraction
Dynamic MRI: evaluation of time between the onset of arterial enhancement and early enhancement of the lesion : Early if <or = 6 s: component malformation with arterial
or capillary
Late if> 6 s : pure venous malformation
DISCUSSIONA slow flux malformation of
Venous type
« IRM »HABITUEL ASPECTS
Serpiginous structures, tubulated or multilocular masses in connection with venous lakes separated by septa
Isosignal or hypo-signal on T1, frank hyper-signal on T2.
More heterogeneous signal on T1 if bleeding or thrombosis.
Hypo-signal areas on T2 (phleboliths, thrombi, septa). Hypointense on all sequences (phleboliths). EG asignal on T2 (slow flow). Progressive enhancement "patchy" or "clumps" of circulating areas.
DISCUSSIONA slow flux malformation of
Venous type
« Per-cutanous phlebography »
Not useful for diagnosis Stage 1 of treatment by sclerotherapy (in puncture of the malformation with needle 20-22 G)
Optimal evaluation of the anatomy of the MV and its venous drainage
DISCUSSIONA slow flux malformation of
Venous type
CONCLUSION
The superficial vascular abnormalities are a diagnostic and therapeutic challenge that must be based on a multidisciplinary approach.
Their diagnosis is based primarily on clinical examination.
Vascular malformations are usually present at birth and do not regress spontaneously.
Venous malformations are the most common vascular malformations and arteriovenous malformations are vascular malformations, the most dangerous, with unpredictable and difficult to treat.
It is important to distinguish between slow flow vascular malformations (capillary, venous, lymphatic) and vascular malformations fast flow (arteriovenous) that fall under different therapeutic management
Interest of 3D MR angiography with dynamic gadolinium-enhanced and high temporal resolution (5 s).
CONCLUSION
