1
Venous vascular malformation type of soft tissue
discovery at puberty

•  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

2
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

3
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.

4
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.

5

• OBSERVATION
• X-ray standard face of the forearm

• Soft tissue mass with round opacity tone calcium
  without adjacent bone changes.

6

• OBSERVATION
• Doppler Ultra sound

• 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.
  

7

• OBSERVATION
• C T scann

• Lesion on the soft tissu, containing many
  heterogeneous hyperdense calcifications of
  varying size, with enhancement after injection
  discreet locations.

8

• OBSERVATION
• M R I(1,5T)

Sequence -coronale -STIR-
Sequence -coronale -FSE T1-

• 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.

9

• OBSERVATION
• M R I

T1
Sequence axiale-FSE T1,FSET2 and T2
T2
10

• OBSERVATION
• M R I

Axial-FSE T1 Fatsat after Gadolinium
MIP
Precoce and moderate enhancement, heterogeneous
and "clumps" after injection.
Coronal-FSE T1 Fatsat after Gadolinium
11

• OBSERVATION
• M R I

Coronal-FSE T1 Fatsat after Gadolinium
12
DISCUSSION

• Prerequisite know the classification of
  superficial vascular abnormalities.
• Many sources of terminological confusion
  misdiagnosis and inappropriate treatment.

13
Classification (1996) adopted by the
International Society for the Study of Vascular
Anomalies (ISSVA)
DISCUSSION

• VASCULAR TUMORS abnormal endothelial cell
  proliferation
• Vascular Malformations  embryological vessel
  abnormalities without abnormal cell proliferation

14
DISCUSSION Classification Vascular tumor

• Infantile hemangioma the most common tumor in
  infants
• Congenital hemangiomas (RICH and NICH),
  kaposiform hemangioendothelioma, tufted angioma
• Exeptionnel hemangiopericytoma, fibrosarcoma,
  rhabdomyosarcoma infant

15
DISCUSSION Classification Vascular
malformations

•  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

16
DISCUSSIONVASCULARMALFORMATIONS
17
DISCUSSION Vascular Malformation General

• 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)

18
DISCUSSION Vascular 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

19

• DISCUSSION
• A slow flux malformation of capillary type

• 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)

20

• DISCUSSION
• A slow flux malformation of Venous 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)

21

• DISCUSSION
• A 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

22

• DISCUSSION
• A 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)

23

• DISCUSSION
• A 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

24

• DISCUSSION
• A 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) gt 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

25

• DISCUSSION
• A 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

26

• DISCUSSION
• A 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 ltor 6 s component malformation with
  arterial or capillary
• Late ifgt 6 s pure venous malformation

27

• DISCUSSION
• A 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.

28

• DISCUSSION
• A 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

29
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.

30
CONCLUSION

• 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).