Hemangioma

00:00 In this lecture, we're going to discuss Hemangiomas and Nevi in Children. "So a mother comes in and consults a dermatologist because she is concerned about a lesion on the left side of her child's face, which you can see here. The lesion is red, protuberant and sharply demarcated.

00:21 Recently, it has been rapidly increasing in size, becoming more dome-shaped and elevated." What do you think the diagnosis is? Yeah, that's a hemangioma. So let's talk about hemangiomas.

00:35 It's a benign vascular proliferation, it's most common neoplasm of childhood and about 7% of of all benign tumors of infancy and childhood are hemangiomas. There is an increase number in these hemangiomas of normal vessels filled with blood which makes excision of them very challenging, they can bleed a lot. They're generally present at birth and then they have a marked growth in the first few months of life. There is usually an overlying erythematous skin.

01:11 So, sometimes they may have a bluish discoloration and this may be sign of something called a cavernous hemangioma. A cavernous hemangioma has a large cavern in it where there's a lot of blood or _____. These are generally soft to the touch. There's another type of hemangioma we should be aware of which are port-wine stains. These are not elevated off the skin. They are purplish, red lesion that's flat and macular. They do not enlarge over time. They're usually on the face and they're usually unilateral and they can be associated with some syndromes. An example of some syndromes with port-wine stains are Klippel-Trenaunay-Weber syndrome.

01:57 These patients often have hemihypertrophy or an enlargement of 1 side of the body, say a leg that's bigger than the other, and there will be similar lesions on those extremities or Sturge-Weber syndrome. Sturge-Weber syndrome is an involvement of one of these in generally the trigeminal area of the face. They can have an underlying abnormality resulting in seizure disorders, a delay in their cognition and other problems. Here's an example of a patient with Sturge-Weber. You can see it respects the midline and doesn't cross over. It's involving a trigeminal area, in this case V1 branch of the trigeminal nerve and this, if you see this, is highly likely to be a case of Sturge-Weber. Another type of hemangioma, which is much less concerning is salmon patches. These typically exist on the nape of the neck or right over the glabella on the forehead. The ones on the back of the neck we affectionately call a stork bite as if the baby is being delivered by stork or on the face we call them angel kisses. These are a distended capillary bed and they are in normal variant in people. They exist, in fact, in 40% of newborns and they can persist for life. There are some people, for example, who you can't see them until they get angry and then they show up a little bit. Another lesion we should consider is the pyogenic granuloma. This is a bright red pedunculated lesion that's often engorged with blood, a very bloody lesion, so it bleeds easily. These lesions tend to show up in children or sometimes in pregnant women and they really can happen at any time in life. They are very rapidly growing. It's basically a vascular overgrowth of granulation tissue. About 1 in 4 of these patients have a lesion that develops after a trauma or an injury and thus the encouragement of granulation tissue growth and often they are ulcerated. These can usually be easily removed.

04:11 Another lesion is the spider angioma. This is a lesion with a localized area of distended capillaries and looks a little bit perhaps like a spider with little arms coming out. It is a hereditary hemorrhagic telangiectasia and we can see it in some syndromes such as for example CREST syndrome, which usually happens later in adolescence or in young adulthood. Another lesion is a cherry angioma. These are very small, benign, red-domed lesions and they're usually on the trunk and there is really no consequence. Histologically, these lesions are little vascular channels in a bed of fibrous connective tissue. If they're traumatic they can grow a little bit but they're generally tolerated fine. Now, let's talk a little bit about those infant hemangiomas, the one that we showed up earlier that was growing rapidly on that child over the previous month. These often have spontaneous involution and here you can see a child who had a very large hemangioma and you can see how it's gradually getting smaller over time. So many times we just watch these patients, we don't actually do anything for them. However, there are medications that can encourage them to resolve and the medication we use the most is propranolol. We don't really understand why propranolol helps with these lesions but it does. If the lesion is particularly invasive or if it's obstructing somewhere, for example if it's obstructing an airway, we can use laser ablation as well and rarely we can use systemic steroids which might help.

05:57 One complication you should be aware of is Kasabach-Merritt syndrome. This is an unusual cause of pediatric disseminated intravascular coagulation. This happens in children with large hemangiomas and sometimes is a result of a hemangioma that is inside the body. This can be very challenging to deal with. Generally, these patients have a microangiopathic anemia and DIC with platelet consumption as a result of blood getting sheared apart as it goes through that hemangioma.