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Recent trends in management of vascular malformation

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Recent trends in management of vascular malformation

  1. 1. Recent Trends in Management of Vascular Malformations Moderator-Prof A K Khanna Speaker- Awaneesh Katiyar
  2. 2. • A thought -BIRTH MARK GIVEN BY GOD • VASCULAR MALFORMATION – INBORN ERROR OF VASCULAR MORPHOGENESIS
  3. 3. Classification of Vascular Anomalies • In 1863 Virchow – attempt to classify Vascular lesions 1. Angioma a) Angioma Simplex b) Angioma Cavernosum c) Angioma Racemosum 2. Lymphangioma
  4. 4. • In 1982 , Mulliken and Glowacki • 49 specimen , 2 categories- Hemangioma/malformation(23) • Hemangioma – proliferative(14) and involutary(12) • Clinical history ,Endothelial hyperplasia incorporation with tritiated thymidine and basement membrane.
  5. 5. Classification of vascular lesions in infants and children(Mulliken,1982) Vascular lesions Hemangiomas Malformation Proliferative phase Involuting Phase Capillary Venous Arterial Lymphatic Fistulae
  6. 6. Hamburg Classification,1988 Type Truncular Extratruncular Predominantly Arterial Defects Aplasia or Obstructive Dilation Infiltrating Limited Predominantly venous Defects Aplasia or Obstructive Dilation Infiltrating Limited Predominantly Lymphatic Defects Aplasia or Obstructive Dilation Infiltrating Limited Predominantly Arteriovenous shunting Defects Deep Superficial Infiltrating Limited Combined / mixed vascular defects Arterial and venous Hemolymphatic Infiltrating hemolymphatic Limited hemolymphaticBased on seventh Meeting of the International Workshop on Vascular Malformations. Hamburg, Germany, 1988.
  7. 7. Classification of Vascular Anomalies By Jackson, 1993 From Jackson IT, Carreno R, Potparic Z, et al. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg 1993;91:1217. Vascular Anomalies Hemangiomas Vascular Malformation Lymphatic malformation Low flow High flow
  8. 8. Classification of Vascular Anomalies ISSVA ,Italy, 1996 Vascular Tumors Vascular Malformation Simple Combined Hemangioma Capillary(C) Arteriovenous Fistula(AVF) Others Lymphatic (L) AVM Venous(V) CVM LVM CAVM CLAVM Based on Scientific Committee of the Eleventh Meeting of the International Society for the Study of Vascular Anomalies. Rome, Italy, 1996.
  9. 9. Classification of Vascular Anomalies ISSVA , Melbourne,2014 Vascular tumors Vascular Malformation Benign Simple Locally Aggressive or Borderline Combined Malignant of major named vessels associated with other anomalies Provisionally unclassified vascular anamolies
  10. 10. Vascular Malformation Simple Combined of major named vessels Aka “channel”or “truncular”Vascular Anomalies associated with other anomalies Provisionally unclassified Capillary Malformation CVM,CLM Affect lymphatic veins Arteries Klippel-trenauanay Verucous Hemangioma Parke -Weber Angiokeratoma Lymphatic Malformation LVM,CLVM Servelle- Martorell Kaposiform lymphangiomatosis Sturge-Weber others Venous Malformation CAVM Anomalies of origin course number length diameter communication persistence (of embryonal vessel) maffucci Macrocephaly-CM Arteriovenous malformation CLAVM Microcephaly-CM CLOVES Arteriovenous fistula Others Proteus Bannayan-Riley-Ruvalcaba Limb CM+congenital non pregressive limb hypertrophy
  11. 11. Hemangioma Vs Vascular Malformaton Hemangiomas Vascular Malformations May or May not be present at birth Always present at birth but may not be evident Grow rapidly and slowly involute Progress in proportion with body Involute spontaneously Usually don’t involute Females are more affected Equally affected True benign neoplasm Defect of morphogenesis Associated with endothelial hyperplasia with rapidly dividing endothelial cells Ectasia of abnormal vessels Mast cells increase in proliferative phase No evidence hemangioma VMs Gerald M. Legiehn, Ca,Manraj K.S. Heran, Classification, Diagnosis, and Interventional Radiologic Management of Vascular Malformations Orthop Clin N Am 37 (2006) 435–474 hemangioma VMs
  12. 12. Hemangioma Vs Vascular Malformaton Hemangiomas Vascular Malformations Rare causes bony /cartilagenous distortion or hypertrophy Low flow malformation usually causes skeletal / bone hypertrophy Due to mass effect may cause depression over bone High flow causes destructive bony changes Angioigraphy – well circumscribed mass with intense prolong tissue staining Diffuse lesion without intervening parenchymal staining Feeding arteries may form equitorial network in periphery Depends on predominantly channel type
  13. 13. Vascular tumors- Benign Hemangiomas infantile congenital Presentation Site Involution Sex Skin color GLUT-1
  14. 14. Pyogenic Granuloma (Lobular capillary Hemangioma) • Solitary, red papule, grows rapidly, forming a stalk. • Lesions are less than 1 cm in diameter. • presentation is inversely correlated with age
  15. 15. Uncommon Vascular tumors • Kaposiform hemangioendothelioma • Associated with kasabach- merritt syndrome
  16. 16. Angiosarcoma • Rare • Aggressive malignant vascular neoplasm • Female preponderance • Average age of onset 3.7yrs • poor prognosis Angiosarcoma in a 2-year-old female patient with an abdominal mass. Axial contrast-enhanced CT image shows a heterogeneously enhancing right hepatic mass.Note focal pooling of contrast (arrow).
  17. 17. Vascular Malformation Slow/low flow Fast/high flow CM VM LM AVM AVFAM
  18. 18. Capillary Malformation • Naevus flammeus neonatorum (“angel’s kiss” or “stork bite” or “port-wine stains” • In new born • At face and nuchal region • Red macular lesions • Persist for throughout life. • CMs may occurs in association with syndromes , best known SWS.
  19. 19. Associated syndromes with CMs • Sturge-Weber Syndrome • Cutis Marmorata Telangiectatica Congenita • Macrocephaly–Capillary Malformation
  20. 20. Venous Malformation • “cavernous Hemangioma” A misnomer. • Always present at birth –may not be obvious. • various presentions. • Include from small swelling to large body deforming swelling lethal outcomes.
  21. 21. • “Genuine Diffuse Phlebectasia of Bockenheimer” – Extensive Venous Malformation of limb – Associated with limb discrepency • Kasabach- merritt phenomenon • Hemolytic anemia • Thrombocytopenia • hypofibrogenemia
  22. 22. Syndromes associated with VMs • Glomuvenous Malformation. • Cutaneomucosal Venous Malformation. • Blue Rubber Bleb Nevus Syndrome.
  23. 23. Lymphatic Malformation Microcystic LM or Lymphangiomas (<2cm) Macrocystic LM or Cystic Hygroma (>2cm) Visceral LM or Lymphangiomatosis Common cystic LM
  24. 24. Congenital/Primary Lymphedema • “Milroy Disease”. • Anomalous Lymphatic Channels • Maily manifest at birth • Compression, suction lipectomy • lymphaticovenous anastomosis
  25. 25. Arterial Malformation • Aneurysms, • Fistulae, • Ectasias, • Stenoses
  26. 26. Arteriovenous Malformation • Congenital high-flow vascular malformations. • Growth Triggered by – Puberty – Trauma • Pathogenesis – TGF-ß signaling and a genetic two hit hypothesis(Knudson’s).
  27. 27. • Extracranial Vs Intracranial Arteriovenous malformation in a 16-year-old male patient with a left chest wall mass.
  28. 28. Schobinger Staging System for Arteriovenous Malformation Stage I Quiescence Cutaneous blush, skin warmth, arteriovenous shunt on Doppler ultrasound Stage II Expansion Darkening blush, lesion shows pulsation, thrill and bruit Stage III Destruction Steal, distal ischemia, pain, dystrophic skin changes, ulceration, necrosis, soft tissue and bony changes Stage IV Decompensation High-output cardiac failure Based on Eighth Meeting of the International Workshop on Vascular Malformations. Amsterdam, Netherlands,1990.
  29. 29. Combined Malformations and Overgrowth Syndromes • Klippel-Trenaunay Syndrome • Maffucci Syndrome • Parkes Weber Syndrome • Capillary Malformation-Arteriovenous Malformation • Bannayan-Riley-Ruvalcaba Syndrome (PTEN Hamartoma Syndrome). • CLOVE(S) (Congenital, Lipomatous, Overgrowth, Vascular Malformations, and Epidermal Nevi) Syndrome
  30. 30. Klippel-Trenaunay Syndrome • Also known as Capillary-Lymphatico-Venous Malformation(CLVM)- slow flow type. • Sporadic • Association with gene not yet known. • Limb hypertrophy present at birth which progressively worsens with growth. • Complication-recurrent infection, thrombophlebitis, 1% systemic complication. • Fast flow associated with – Parkes-Weber syndrome.
  31. 31. Investigation • Ultrasonography with CD- noninvasive , inexpensive and readily available- initial & gold standard. Mixed venous waveforms No doppler flow Arterial waveforms
  32. 32. Computed tomography • To define – bony architecture, identify phleboliths and other dystrophic calcification, to describe hepatic lesions • CECT/ Helical CT -More informative in Arterionenous malformations – arterial , nidus and venous structures.
  33. 33. CECT – venous malformation CECT- AVM in mid arterial phase (A) CECT –AVM around Rt humerus (B) CT angio of same lesion
  34. 34. Magnetic Resonance Imaging • Replaced CT- no ionizing radiation. • Better for serial follow up- after sclero/embolotherapy. • Excellent to evaluate low flow malformation and soft tissue. • Protocol-spin echo or fast spin echo T1 weighted imaging – for baseline • T2 weighted short tau inversion recovery images- hemosiderin, dystrophic calcification or phlebolith,
  35. 35. Gerald M. Legiehn, Manraj K.S. Heran, Classification, Diagnosis, and Interventional Radiologic Management of Vascular Malformations Orthop Clin N Am 37 (2006) 435–474
  36. 36. Management of Vascular Malformation Multi-disciplinary Team
  37. 37. Multimodality approach Vascular Anomalies conservative Drug therapy sclerotherapy embolization endovascular surgery Pychological support
  38. 38. • Surgical Excision –limited knowledge of the natural and biology – poor prognosis • New Endovascular intervention- Embolo/ Sclero therapy/balloon/stenting/ablation
  39. 39. Principles of Management • None of the single modality of treatment can treat any VM alone. • Classify before to intervene- truncular/ Extratruncular • Extratruncular- poor outcome in diffuse variety. • Truncular- Fair out if they intervene early
  40. 40. • Wiser – “Not to intervene”-if nature not known. • Refer to experienced hand / center- where better facilities are available • Life threatening lesions treated – As early as possible
  41. 41. General Measures for Management • Explain the Proper Diagnosis- patient , parent, guardians and to referring doctor. • Treat associated complications – Hemorrhage – Ulcer – Anemia – Recurrent thrombophlebitis – thrombocytopenia
  42. 42. • Graduated compression stocking and Garments • Support and Education. • Refer to and consult other specialist • Family members screening and genetic counseling-for associated syndromes
  43. 43. Indications for therapy • Lee(2005) devised “decision to treat”-At least one absolute and two relative indications. • Absolute indications- 1. Hemorrhage 2. Progressive high output failure 3. Complication secondary to Venous hypertension 4. Lesion located in life threatening area. 5. Lesion located in life treatening vital functions
  44. 44. • Relative indications- 1. Progressive disabling pain or discomfort. 2. Functional disability or impairment affecting daily life and quality of life. 3. Severe cosmetic deformity. 4. Vascular-bone Syndrome- causing growth discrepency. 5. Location at high risk for complication. 6. Recurrent infection or sepsis.
  45. 45. Treatment options Management of Vascular Malformation Non–surgical Surgical •Conservative •Drug therapy • Sclerotherapy • Embolotherapy • Endovascular management • Resective • Recontructive • Endovascular recontructive surgries
  46. 46. Conservative Management • Proper skin care . • Local wound care-prevent hemorrhage. • Compression therapy. • Life style modification and appropriate physical therapy- orthopedic footwear • Psychological support.
  47. 47. Drug thrapy • Anticoagulation- to prevent recurrent thrombophlebitis • Rheological/venotonic agents –Trental / Daflon • Antiepileptic drugs – for intracranial VM
  48. 48. • A serentipity – in 2008 . • Newborn with proliferative hemangioma with cardiovascular ds.- on propranolol therapy • Shows rapid regression of lesion After 2 months of therapy- 2 mg/kg divided tid
  49. 49. • Corticosteroids • Vincristine and interferon alfa-2a&2b • cyclophosphamide
  50. 50. Sclerotherapy • Sclerotherapy is of irritant solution into lumen of a vessel –causes thrombosis and subsequently fibrosis. • Ideal sclerosing solution – Painless to inject – Free of adverse effects – Specific to affected vessel
  51. 51. Sclerosing solutions Detergent Agent Osmotic Agent Chemical Irritants Sod . Tetradecyl sulphate Palidocanol Sod Murrhuate Ethanolamine oleate Ethanol Hypertonic Saline Hypertonic Saline Dextrose Chromated Glycerin Polyiodinated Iodine
  52. 52. Ethanol sclerotherapy • Gold standard – widely used and accepted percutaneous sclerogent • Readly available , inexpensive, easy to use , long shelf life. • Potent irritent- transmural destruction of vessel wall. • Avoided near to – large nerves, skin, mucosa, genitalia, finger/toes.
  53. 53. • In Europe – only approved for slow flow malformations • Not exceed 1ml/kg at any one visit. • Ethylcellulose and Ethibloc
  54. 54. Bleomycin sclerotherapy • Glycopeptide antibiotic- Streptomyces verticillus. • Most commonly used sclerosing agent for the treatment of vascular anomalies in China. • Sclerotherapy has no great pain or nerve injury-the cervical-facial region. • USG guide is most effective method
  55. 55. • 75 patients(2010)- 42 patients (56%) one time of treatment, 21 (28%) two times, nine (12%) three times, and three (4%) patients received four times. • 84% (63 patients) were cured, 13.33% (10 patients) were basically cured, 2.67% (two patients) were improved, and 0% were ineffective.
  56. 56. Sodium tetradecyl sulphate • An anionic surfactant. • Widely used in for sclerosis of esophageal varices and varicose veins. • Now also in – vascular malformation. • Not effective- as ethanol. • Cause urticaria, anaphylaxis, hemolysis, and hematuria
  57. 57. Sclerotherapy for Venous Malformation • Puig and Associates-classifies for sclerotherapy I II III IV
  58. 58. • Type I and to a lesser extent type II lesions respond best • Higher complication rates -attributed to type III and type IV anatomy
  59. 59. MRI monitoring for sclerothrepy outcomes(A) presclerothrapy (B) 6 month after one session of Ethanol sclerotherapy
  60. 60. Sclerotherapy for Lymphatic Malformation • Newer agent particular for LMs- OK432. • Lyophilized powder made of Streptococcus pyogenes (group A, type 3, Su strain) inoculated with benzylpenicillin. • Multi-institutional randamised trial for OK-432 • 95% of complete for macrocystic and 0-80 % for micro cystic or cavernous subtype
  61. 61. • MRI monitoring for sclerothrepy outcomes(A) presclerothrapy (B) 8 month after two session of OK- 432 sclerothrapy
  62. 62. Percutaneous image guided sclerotherpy • Real time Ultrasound Guided foam sclerotherapy • Fluoroscopic & Ultrasound guided Sclerotherapy(FUGS) (2004)
  63. 63. Side effect and complications of sclerotherapy • Allergic reaction. • Matting - is the appearance of groups of new fine telangiectasias surrounding or replacing a previously treated area in blush-like manner. Resolution occurs spontaneously within a three to twelve month period with 70-80% spontaneous resolution within the first 6months. • Cutaneous necrosis • Superficial thrombophlebitis . • Haematoma formation. • Post sclerotherapy hyper pigmentation • Damage to the nerve- an extra vascular injection may cause pain or Paraesthesia and numbness in the site of distribution of the involved nerve. • Pulmonary embolism - Probably occurs from extension of a superficial thrombus into the deep venous system.
  64. 64. Embolotherapy • Embolic Agents- • Liquid-dehydrated ethanol, • Semiliquid - N-butyl cyanoacrylate glue and ethylene vinyl alcohol copolymer [Onyx]) • Solid (e.g.,coils, particles) embolic agents. • Dominant draining veins -coils, glue, and ethanol. • AVFs use -coils and plugs.
  65. 65. Ineffective AVM embolization techniques The use of glues in AVM embolization
  66. 66. Retrograde venous route to AVM nidus Temporary arterial inflow or venous outflow occlusion during embolosclerotherapy of AVMs.
  67. 67. Permanent arterial inflow occlusion. The judicious use of coils (arrows) and cyanoacrylate (arrowhead) can decrease total arterial inflow to an AVM allowing greater concentrations and ‘‘dwell time’’ of sclerosant to be delivered to the nidus. Permanent venous outflow occlusion. Coil embolization of the venous portion of the AVM nidus can reduce total flow within the lesion allowing greater effectiveness of transarterial sclerosant. This technique also may serve as the most definitive AVM closure technique during multitechnique therapy.
  68. 68. Endovascular Management • In management of truncular Malformation • Thermal or Laser Ablation. • Thermal Ablation – mainly used for Venous incompetence • Minimal role in other Vascular Malformation
  69. 69. Laser Treatment • Commonly used for Venous malformation. • Includes – capillary malformations, localised venous anomalies, diffuse venous ectasias. • Also useful in vascular tumour and truncular venous anomalies.
  70. 70. Laser Ablation –truncular VM • Least local and no systemic side effects • For MV/KTS • Endovenous Laser Ablation – wavelenth of 1310-1470 nm. • Isolated laser ablation - often inadequate • Required – combined with embolization of venous outflow or sclerotherapy(pre and post)
  71. 71. Laser ablation-Extratrunclar VM • Soft Tissue Phlebectasias- perforator vein laser coagulation. • Glomuvenous Malformation -Nd:YAG laser . • Capillary-Lymphatic Malformation- Flash Lamp Pumped Dye Laser (FDL)/KTP • Capillary Malformation- double pulse pulsed Nd:YAG /pulsed dye laser , transcutaneous ice cube- cooled Nd:YAG laser CM- pre and post FDL
  72. 72. Endoscopic laser • Treatment of airway, intestinal and genito- urinary tract lesions. • Nd:YAG laser Blue Rubber Bleb Nevus Syndrome HHT – Nd:YAG Laser
  73. 73. Surgical Management for Vascular Anomalies • “Combined” surgical approach. • To correct hemodynamic derangements. • Various surgical procedures .
  74. 74. Reconstructive surgeries • Reconstructive (open) management -truncular lesions with direct involvements of all the named vessels. • Excision of the web/membrane. • Aneurysm
  75. 75. Resective Surgical Therapy • Extratruncular- combined with the endovascular therapy (embolo/ sclerotherapy. • A “limited” resection.
  76. 76. Baby with CLOVE syndrome – under went serial surgical resection of lesion
  77. 77. Endovascular recontructive surgeries • For treating stenosing truncular VM lesions. • Webs, septum, and stenosis -iliac vein, IVC, jugular vein, and azygous vein. • fail to respond • Excisional surgical therapy with/without bypass reconstruction is the treatment of choice.
  78. 78. Conclusion • Inspite of well developed classification – misnomer still are used. • Don’t intervene – till the nature of lesion is not known. • Controlled aggressive approach is required for management of vascular malformation. • Classify the lesion by Hamburg classification before intervention. Strawberry CavernousXX
  79. 79. • Use of minimal invasive technique always have better outcomes – untill complication not occurred. • Most of the Extratruncular lesion almost always required multimodality of treatment. • Truncular lesion – mostly required surgery • May needs to combined with pre or post sclero/ embolotherapy. • Vascular Malformation with complication or at life threatening location required urgent intervention.

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