Vascular anomalies
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This presentation is intended for diagnosing various vascular lesions on the basis of history and clinical examination. It covers a broad range of anomalies plus you can test yourself in the end.
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Vascular anomalies
- 2. Objective • By the end of the presentation the participants will be able to recognize vascular lesions on clinical appearance.
- 3. ISSVA 2014 • The International Society for the Study of Vascular Anomalies was founded in 1992 during the International Workshop on Vascular Anomalies held in 1990 in Amsterdam. • The term anomalies encompasses hemangiomas and vascular malformations. The purpose of the Society is to promote, on a national and international level, clinical and scientific research in all fields, that will lead to advances in knowledge concerning all aspects of vascular anomalies.
- 5. Vascular anomalies Vascular Tumors Vascular malformations Infantile hemangioma Congenital hemangioma NICH RICH • Capillary malformation • Venous malformation • Lymphatic malformation • Arterial malformation • Arteriovenous fistula • Arteriovenous malformation Combined types Slow flow Fast flow
- 6. Differentiating Features TUMOR • True tumors, with proliferation of the vascular endothelium • >3:1 female : male • Small or absent at birth • Rapid growth during infancy • Self-limited • Diagnosis: Clinical + appearence MALFORMATION • No tumor, comprised of dysplastic vessels • 1:1 female : male • Present at birth • Growth proportional to child • Never disappear • Diagnosis: MRI, Doppler ultrasonography, angiography
- 8. BIRTH 1 YR 2 YRS GROWTH NICH RICH IH Growth patterns ofGrowth patterns of hemangiomashemangiomas RICH:rapidly involuting congenital hemangioma; NICH:noninvoluting congenital hemangioma. IH: Infantile Hemangioma AGE
- 9. RICH
- 10. NICH
- 19. Kaposi sarcoma
- 20. Angiosarcoma
- 22. CM with bone or soft tissue overgrowth
- 25. Nevus simplex
- 27. Lymphatic Malformations In the oral cavity appear as multiple translucent non-compressible cysts or vesicles of <2 cm. containing viscous clear fluid, producing a pebbly or warty surface resembling “frog spawn” or “tapioca pudding”. MICROCYSTIC LM ( Outdated term Lymphangioma) 27
- 28. Macro Cystic LM
- 34. Blue rubber bleb nevus syndrome
- 37. Sporadic AVM
- 38. AVM in HHT
- 40. Conclusion • Vascular tumors are to be differentiated from vascular anomalies. • The distinction is possible by history and careful clinical examination most of the time. • In doubtful cases, biopsy is required.
- 41. Thank you • QUESTIONS? • Or test will follow…
Editor's Notes
- NICHs present as bossed, round-to-ovoid shape lesions in shades of pink to purple. The average diameter is 5 cm. There may be overlying coarse telangiectasia.59 NICHs most commonly affect the head/neck (43%) followed by the limbs (38%) and trunk (19%). As the name implies, NICHs do not undergo involution and persist essentially unchanged.59 Although they are tumors histologically, the static behavior of NICHs resembles that of a malformation.
- Gross= Large, plaque-like, infiltrated, red or purple lesion M/E= Cannonball appearance- vascular tufts of tightly packed capillaries, randomly dispersed in the dermis
- Young adults Skin and subcutis Distal extremities – hand M/E : thin walled cavernous vessels lined by bland flattened endothelium admixed with solid areas composed of plump endothelial cells Recurrence is common (> 50%) with discontinuous growth pattern
- Is a solitary a small red papule that grows rapidly, forming a stalk The malefemale ratio is 2 : 1 It is commonly complicated by bleeding (64.2%) and epidermal ulceration (36.3%) The presentation is inversely correlated with age They are distributed on the head or neck (62%), trunk (19%), upper extremity (13%), or lower extremity (5%) Twentyfive percent of patients have a history of trauma or an underlying cutaneous condition (including capillary malformation, dermatologic disorder, viral infection, or insect bite).
- Rare vascular neoplasm that is locally aggressive but does not metastasize Fifty percent of lesions are present at birth and are diagnosed during infancy (58%), early childhood (1 to 10 years; 32%), or late child hood (10 to 20 years; 10%) Has an equal sex distribution, is solitary, and affects the head or neck (40%), trunk (30%), or extremity (30%)
- Retiform hemangioendothelioma (RH) is a rare vascular neoplasm, which was first described in 1994 as a distinctive form of low grade angiosarcoma. It presents clinically as a slow growing asymptomatic solitary nodule or plaque either on extremities or trunk in 2nd-4th decade of life. Tumor has a slow indolent course with very high local recurrence and metastasizes very rarely. On histopathology, arborizing blood vessels are arranged in retiform pattern resembling the normal rete testis. Blood vessels are lined by monomorphic hobnail endothelial cells and infiltrated by lymphocytes. To date, only 32 cases have been reported worldwide.[1]
- a locally aggressive, rarely metastasizing vascular lesion characterized by lymphatic-like channels and papillary endothelial proliferation. These tumors appear to be closely related to retiform hemangioendothelioma
- Early lesions – ecchymotic macules or patches Later lesions – bluish purple papules,nodules, plaques or tumors Regardless of type, it is Borderline malignancy with slowly progressive but may involve internal organs
- Small, skin-colored papules and vesicles on inner part of left thigh (box, area shown in Fig 2, F, 1 day after skin biopsy). B, Easily compressible vascular channels measuring 2 to 3 cm in diameter on back aspect of left foot. C, Magnetic resonance image (MRI) showing continuous spread of vascular malformative process (arrows) from skin the adjacent connective tissue and fat. D, MRI showing dilated vascular channels of varying calibers (arrows) on back aspect of left foot. E, Histopathologic aspect of cutaneous lymphatic malformations showing large cavernous lymph spaces and ectatic lymph vessels (stars). Bar = 0.5 mm. F, Hemangiomatous aspect of vascular skin lesions 1 day after skin biopsy as result of bleeding into lymphatic vessels, giving rise to red coloration of lymphatic skin lesions.
- All the anomalies found are due to dysgenesis of lymphatic microvessels. These dysgenesis ranges from mild to severe and even to aplasia of both, the lymphatic capillaries and collectors[14],[15],[16]. Main features: Present in one or both legs at birth. Lymphedema of PCL persists throughout life but does not appear to affect longevity. As the patient matures, the overlying skin displays a slightly rosy hue, and the size of the edematous parts remains proportional to the remainder of the body. It can rarely present with genital edema, resembling sexual ambiguity
- 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.
- A less common, inheritable form is cutaneomucosal venous malformation (VMCM, OMIM # 600 195).6 VMCMs tend to be multifocal and small.7 They are comprised of grossly dilated vascular spaces that are lined by a single continuous layer of endothelial cells, with areas of relative lack of surrounding mural cells, suggesting a defect in their recruitment.8,
- Blue rubber bleb nevus syndrome is a rare disorder characterized by distinctive cutaneous and gastrointestinal venous malformations that usually cause massive or occult gastrointestinal hemorrhage and iron deficiency anemia secondary to the bleeding episodes. It is even a rare cause of gastrointestinal hemorrhage during childhood.
- Glomus tumors are benign lesions that are derived from the glomus cells surrounding arteriovenous anastomoses that serve as temperature regulators. They can be classified into solitary and multiple, acquired or congenital and, histopathologically, into 3 variants: glomus tumor proper, glomangioma and glomangiomyoma. The diagnosis is more likely GVM if the lesion is pink to bluish purple or dark blue and has a cobblestone-like appearance with minor hyperkeratosis, especially if the lesion is located on an extremity. For segmental GVM, the lesion is pink in infancy and rapidly worsens, thickens, and turns to purple or dark blue. However, the diagnosis is more likely to be VM if there is an isolated bluish mucosal or subcutaneous lesion, involving skin and underlying muscles, or an isolated intramuscular or periarticular vascular mass. Phleboliths are suggestive of VM, and the diagnosis is further suggested if the lesion shrinks by external pressure or when in a dependent position. Venous malformations are typically painful in the morning, probably due to stasis and expansion,1,2whereas GVMs are typically painful when compressed.22 More than 50% of our patients with VM noted increased pain with onset of puberty, menstrual cycles, antiovulant drugs, or pregnancy. This type of hormonal modulation was not reported by patients with GVM.
- They represent a group of congenital malformations that create a direct communication between the arterial and venous systems, through a nidus formed by arteriovenous shunts, along with hypertrophy of the afferent arterial and efferent venous system. AVM is present at birth, but become clinically apparent only during the 4-5th decade of life and is often misdiagnosed due to delay in clinical presentation. The most common site for AVM is the brain, followed by the head, neck, limbs, trunk, and viscera. The majority of the head and neck lesions occur on the cheek, followed by the ear, nose, forehead and upper lip.
- rteriovenous malformations (AVMs) are abnormal tangles of arteries and veins. While many AVMs remain asyptomatic for life, they can cause serious problems when they occur inside the brain as a cerebral AVM, or in the brain’s covering (the dura) as a dural AVM, or in the spinal cord as a spinal AVM. AV fistulas are an abnormal connection between arteries and veins. This condition can occur in the brain, the covering of the brain (dura) and the spinal cord. They can cause symptoms by affecting the surrounding brain or spinal cord, and in some cases from bleeding. An AVF is characterized by a single connection between and artery and a vein, whereas an AVM contains multiple arteries and veins. There are two types of arteriovenous fistulas, congenital and acquired. A congenital arteriovenous fistula is a rare birth defect that formed during fetal development. An acquired arteriovenous fistula is one that develops after a person is born. It usually occurs when an artery and vein that are side-by-side are damaged, and the healing process results in the two becoming linked. For example, after catheterizations, arteriovenous fistulas may occur as a complication of the arterial puncture in the leg or arm.