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Haemangiomas And Vascular Malformations

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  • 1.  
  • 2. Vascular Anomalies; Haemangiomas and Vascular Malformations Dr. Mohamed Makboul, MD Plastic Surgery Department Assiut University
  • 3.
    • The term hemangioma refers to the common tumor of infancy that has a proliferative endothelium, and exhibits rapid postnatal growth and slow regression during childhood; this tumor never appears in an adolescent or adult.
    • Whereas vascular malformations are comprised of abnormally formed channels that are lined by stable endothelium, present at birth, never regress and often expand.
  • 4. Vascular anomalies can be classified into two unique groups:-
    • Hemangiomas
    • Vascular malformations which subcategories into :
      • Slow-flow
        • Capillary
        • Lymphatic
        • Venous
      • Fast-flow
        • Arterial arterial (aneurysm, stenosis, ectasia)
        • Arteriovenous fistulae (AVF)
        • Arteriovenous malformation (AVM)
  • 5. Haemangioma
  • 6. Haemangioma
    • Hemangioma is the most common tumor of infancy, appears in neonatalhood, usually within the first 2 weeks and has a defined natural history.
  • 7. Clinical Features
    • The natural history of Hemangioma is characterized by three phases:
        • Proliferating Phase
        • Involuting Phase
        • Involuted Phase
  • 8. Proliferating Phase
    • Hemangioma grows rapidly during the first 6 to 8 months of infancy, the skin becomes raised, bosselated, and a vivid crimson color.
    • Palpation at this stage reveals a tense and noncompressible mass.
  • 9. Day of Birth 1 month 3 month 2 month
  • 10. Involuting Phase
    • Hemangioma reaches its peak before the first year; and for a time thereafter, growth is proportionate to that of the child.
    • The first signs of the involuting phase appear as the crimson color fades to a dull purplish color, the skin gradually pales, and the tumor feels less tense.
    • The involuting phase continues until the child is 5 to 10 years of age.
  • 11. Involuted Phase
    • Regression is complete in 50% of children by age 5 years and in 70% by age 7 years, with continued improvement until age 10 to 12 years.
    • The hemangioma may persist in the form of residual tumor, loose skin, telangiectasias, or scarring.
  • 12. Hemangiomas can be superficial, deep, or visceral in location
    • Superficial lesions: often exhibit the classic crimson color of the so-called strawberry hemangioma.
    • Deep lesions: Are those within the deep dermis or subcutaneous tissues often present as pale blue or purple masses that may be confused with venous malformations.
    • Visceral lesions: Are not apparent on physical examination, and in 50% of cases, there are no accompanying cutaneous hemangiomas. In this setting, their presence may only be suggested by physiologic findings such as hepatomegaly, congestive heart failure, or stridor.
  • 13. DIAGNOSTIC IMAGING
    • Hemangiomas are readily distinguished from other tumors by:
      • Ultrasonography (US)
      • Computerized tomography (CT)
      • Magnetic resonance imaging (MRI)
      • Arteriography
  • 14. DIFFERENTIAL DIAGNOSIS
    • Hemangiomas must be differentiated from:
      • Macular stains,
      • Vascular malformations, and
      • Other vascular tumors of infancy.
  • 15. Macular Stains
    • The most common type of vascular birthmark is the macular stain.
    • These are flat lesions, ranging in color from pink to red, seen in as many as 40 percent of newborns.
    • Typically seen in the neck, glabella, eyelid, and forehead, these probably are physiologic phenomena that will resolve with time.
  • 16. Vascular Malformations
    • The natural history of vascular malformations is distinct from hemangiomas.
    • Boys and girls are affected equally.
    • All malformations are present at birth.
    • The physical appearance of vascular malformations is dependent on the type of vessels involved.
    • Venous malformations may appear as bluish masses and be confused with subcutaneous hemangiomas, but they are distinguished by the ability to be emptied of blood with compression.
  • 17.
    • Malformations grow commensurately with the child and do not undergo the rapid proliferative growth phase exhibited by hemangiomas.
    • The greatest distinction between hemangiomas and malformations is that the former spontaneously involute and the latter do not.
    • Hemangiomas, unlike vascular malformations, rarely cause bony distortion or hypertrophy.
    Vascular Malformations (cont.)
  • 18. Other Vascular Tumors of Infancy
    • Pyogenic granuloma
    • Is an acquired vascular lesion that closely resembles hemangioma upon clinical and microscopic examination.
    • They tend to occur on the skin and mucosa of older children and young adults, with a mean age of 6.7 years.
    • Pyogenic granulomas arise suddenly and usually without a history of trauma.
    • Frequently located on the cheeks, eyelids, extremities.
    • The natural history is one of superficial ulceration and repetitive episodes of bleeding.
  • 19. COMPLICATIONS
    • Ulceration
    • Infection
    • Visual Impairment
    • Airway Obstruction
    • Auditory Canal Obstruction
    • Congestive Heart Failure : usually occurs in one of two settings: diffuse neonatal hemangiomatosis or large visceral hemangiomas
  • 20. MANAGEMENT
    • In most instances, parental education and reassurance will suffice.
    • Because most hemangiomas undergo spontaneous involution, treatment may be reserved for those lesions of functional or psychological concern.
    • Hemangiomas obstructing the visual axis, airway, and auditory canals, or those associated with congestive heart failure, ulceration, or bleeding, should be considered for treatment.
  • 21.
    • A number of options are available for the treatment of hemangiomas.
    • Effective treatments may be divided into:
      • Those that are nonspecific (e.g., excision, cryotherapy);
      • Antiangiogenic (e.g., corticosteroids, interferon); and
      • Antiproliferative (e.g., chemotherapy, radiation).
  • 22. Surgical Excision
    • Surgical excision may be indicated for:
    • Infancy for obstructing effect,
    • Ulcerating, or
    • Large hemangiomas unresponsive to pharmacologic therapy.
  • 23. Capillary Malformation (CM)
  • 24. CLINICAL PRESENTATION
    • Port wine stains are intradermal capillary or venular malformations that are present at birth.
  • 25.
    • CM is a macular, red, vascular stain that presents at birth and persists throughout life.
    • CM can be localized or extensive, on the face, trunk, or limbs. The incidence rate in newborns is 0.3%.
  • 26.
    • Two-thirds of these lesions darken in color over time.
    • Most CMs are harmless cutaneous birthmarks, but some are red flags that signal underlying abnormalities.
    • CM in a limb often is associated with axial and transverse hypertrophy.
  • 27. Treatment
    • CM is treated by flashlamp pulsed-dye laser; the results are better if initiated in infancy and childhood.
    • Soft-tissue and skeletal hypertrophy also can require surgical therapy.
    • Laser therapy cannot correct soft-tissue overgrowth.
  • 28. Laser treatment Pre Post
  • 29. Hypertrophy of the upper lip, treated by partial debulking
  • 30. Venous Malformation (VM)
  • 31. CLINICAL PRESENTATION
    • Venous malformations are localized or extensive, minor or distorting, single or multiple, and located anywhere on the head, limbs, or trunk.
    • Most VMs are sporadic, blue color, soft, compressible on palpation, a slow refill, and increased size with dependency are pathognomonic for venous malformation.
  • 32. DIAGNOSIS
    • Clinical picture.
    • MRI
  • 33. TREATMENT
    • Sclerotherapy is the primary treatment for VMs, although subsequent surgical resection is often needed.
    VM Upper lip, Post Sclerotherapy and excision
  • 34. VM Rt. Cheek, Post Sclerotherapy
  • 35. Extensive venous malformation of the buttock and right thigh. Staged surgical reduction of the right buttock and thigh
  • 36. Lymphatic Malformation (LM)
  • 37. CLINICAL PRESENTATION
    • LMs can be described as microcystic, macrocystic, and combined lymphaticovenous (LVM) forms.
    • Lymphatic anomalies present in a wide spectrum, from lymphedema to large cystic malformations.
    • LMs are generally detected at birth or shortly, thereafter LMs grow with the child.
  • 38.
    • Lymphatic malformations can affect all areas of the body, but most are located in the cervicofacial region and axilla.
    • LMs are the most common cause of congenital tongue enlargement (macroglossia), lip enlargement (macrocheilia), and ear enlargement (macrotia).
    • Skeletal hypertrophy and distortion in 80% of cervicofacial LMs occur by 10 years of age.
    • LM never regresses.
  • 39. TREATMENT
    • Sclerotherapy has assumed a major role in the management of LM.
    • Surgical resection is the only way to potentially cure LM.
  • 40. Large lymphatic malformation of the right lateral forehead and temple region. After excision.
  • 41. Arteriovenous Malformation (AVM)
  • 42.
    • AVM is a high-flow vascular malformation comprised of micro- and macro-aneriovenous fistulas (AVFs).
    • The epicenter of an AVM is called the nidus and consists of arterial feeders, micro- and macroarteriovenous fistulas (AVFs), and ectatic veins.
  • 43. Clinical Staging System
    • Introduced by Schobinger
    • Stage I (Quiescence): Pink-blush stain, warmth, and AV shunting by Doppler
    • Stage II (Expansion): Same as stage I, plus enlargement, pulsations, thrill, bruit, and tortuous/tense veins
    • Stage III (Destruction): Same as above, plus dystrophic changes, ulceration, bleeding persistent pain, and expansion/ destruction
    • Stage IV (Decompensation): Same as stage II, plus cardiac Failure
  • 44. Diagnosis
    • Confirmation of clinical diagnosis is helpful with Color Doppler examination, Magnetic Resonance Imaging, and Angiography.
  • 45. Treatment
    • Usually AVM is treated whenever endangering signs and symptoms arise, such as ischemic pain, ulceration, bleeding, or increased cardiac output (Stages III and IV ).
    • Arterial embolization is done to temporarily occlude the nidus in preparation for resection 24 to 72 hours later.
  • 46. Questions???
  • 47.