Hemangiomas are the most common tumor in infancy. They are abnormal endothelial proliferations and are considered to be true tumors. They are found more commonly in females, with a female/male ratio of 3:1. They are usually seen in the head and neck region (60%, not 20%) but may occur on the trunk, limbs, or intracranially. Hemangiomas can be divided into infantile and congenital subtypes. Most are infantile and these develop shortly after birth appearing as a small red spot. Congenital hemangiomas however are fully grown at birth and are classified into two forms, rapidly involuting (rapidly involuting congenital hemangioma [RICH]) or noninvoluting (noninvoluting congenital hemangioma [NICH]). Most patients with hemangiomas have a single lesion, although some do have more than one. Presentation of multiple hemangiomas is most often as part of a syndrome such as Maffucci’s or PHACE syndrome.1–3
The mainstay of treatment for hemangiomas is conservative management given that the vast majority are self-limiting and resolve over time. This infant is at risk of further bleeding episodes and should be seen again in clinic, but does not require further intervention as present. Hemangiomas may be treated with surgery, laser therapy, steroids, or other injectable agents if they are causing visual or airway obstruction, or bleeding and ulceration that is difficult to control with conservative measures.1,2 There has been recent interest in the use of beta-blocker medication in the form of systemic propranolol or topical timolol for treatment of ulcerated hemangiomas and results are promising. Starkey and Shahidullah3 published a comprehensive review of evidence on this topic in 2011. Propranolol is thought to restrict growth in hemangiomas by causing vasoconstriction, inhibition of vascular endothelial growth factor (VEGF), and induction of apoptosis. Typical doses are 1–3 mg/kg/day for up to 12 months. Potential side effects include bradycardia, bronchoconstriction, and hypotension. Bleomycin is a chemotherapeutic agent that has also shown promising results in the management of ulcerated hemangiomas and other vascular malformations.4 Steroids may be injected locally or given systemically to arrest growth of hemangiomas but do not cause regression. Laser therapy can be used for hemangiomas in the acute setting to help with ulceration, or in the later stages of regression to remove residual color from the area.5
3. Drolet BA, Esterly NB, Frieden IJ. Hemangiomas in children. N Engl J Med 1999;341(3):173–181
A lobular capillary hemangioma is more commonly known as a pyogenic granuloma. The latter is a misnomer as it is neither pyogenic nor a granuloma. A lobular capillary hemangioma is a rapidly growing skin lesion seen in infants and young children which develops over a short time interval such as just a few weeks. Growth tends to plateau within 1 month and the size remains static around 6–7 mm in diameter. Although it is often thought to occur following trauma, it is more likely to occur spontaneously. The main problem with such lesions is that they are friable and tend to bleed regularly. They are often therefore best treated with surgical excision and direct closure. In contrast, congenital hemangiomas are present at birth and do not further grow beyond this. They are subclassified into those which rapidly involute (RICH), those which do not involute (NICH), and those which partially involute (PICH). NICH and PICH lesions will generally be treated surgically, while RICH do not usually require this. Infantile hemangiomas do develop spontaneously; however, they do so much earlier in life than the pyogenic granuloma in this case (Fig. 22.1).1
5. Blei F, Guarini A. Current workup and therapy of infantile hemangiomas. Clin Dermatol 2014;32(4):459–470 CLINICAL COURSE OF HEMANGIOMAS 3. You are discussing long-term outcomes with the family of a 6-week-old infant with a 1-cm infantile hemangioma to the cheek. Which one of the following should you tell them? The vast majority of infantile hemangiomas will involute spontaneously and parents and other family members should be reassured about this. However, a number of patients will have residual fullness at the site of the hemangioma that may require surgical excision. Others may have residual discoloration that may benefit from laser therapy. Infantile hemangiomas appear shortly after birth and have an initial phase of rapid growth. This often lasts for 6–18 months before involution begins. Therefore, this child is likely to have further growth of the hemangioma before involution. Involution has traditionally been said to have occurred in 50% of patients by the age of 5, 70% by the age of 7, and 90% by the age of 9. However, this overestimates the duration of hemangioma involution as most often involution is complete by age 4. Congenital hemangiomas differ in their ability to spontaneously involute. They are of two types: RICH (rapidly involuting) and NICH (noninvoluting). Both are present at birth but only RICH involute spontaneously. They do so within the first year of life. NICH lesions do not respond well to pharmacotherapy and may need surgery if problematic.1,2
Ulceration is the most common complication of a hemangioma, but even this is relatively uncommon occurring in <5% of patients. Ulceration is one of the main reasons for clinical presentation and consideration during treatment of a hemangioma. Beta-blockers can be useful in this situation as can laser therapy or steroid injection. Other common complications include bleeding, infection, and emotional/psychological issues. Hemangiomas located close to the periorbital region risk visual obstruction that if untreated can lead to anisometropia, a condition where two eyes have significantly unequal refractive power. Each eye can be near sighted (myopic) or farsighted (hyperoptic) or a combination of both. This can cause problems with binocular vision. The patient will tend to see a smaller image in one eye and a larger one in the other or in development the brain may select to ignore the weaker eye and the neglected eye becomes progressively weaker (amblyopia). Hemangiomas located around the oral or VASCULAR ANOMALIES TUMORS Infantile RICH Venous Capillary Lymphatic Macrocystic Microcystic AVM NICH MALFORMATIONS Kaposiform hemangioendothelioma Tufted angioma Endothelia cell proliferation (malignant or benign) Congenital malformation of vessels Tumors Malformations Hemangiomas CongenitaI Fig. 22.1 Classifications. (AVM, Arteriovenous malformation; NICH, noninvoluting congenital hemangioma; RICH, rapidly involuting congenital hemangioma.) nasal cavities can affect feeding and risk airway obstruction. Hemangiomas in the perineum can cause problems with toileting and hygiene, with subsequent sores and infection. High output heart failure is associated with very large liver/visceral hemangiomas. Skeletal distortion can occur secondary to pressure from the hemangioma if the two are closely apposed.1–5
Kasabach-Merritt phenomenon involves platelet sequestration within vascular tumor cells which leads to life-threatening thrombocytopenia and coagulopathy. Although infrequent, it is potentially fatal in infants with rapidly growing vascular lesions, and early recognition with prompt management is essential. It occurs in half of all patients with kaposiform hemangioendothelioma (KHE) which is locally aggressive but does not metastasize. It is treated with combination therapy including vincristine (a chemotherapy agent) plus steroids with or without interferon, aspirin, or rapamycin (sirolimus—an antirejection/immunosuppressant medication). Osler-Weber-Rendu syndromes is also known as hereditary hemorrhagic telangiectasia and involves multiple ectatic postcapillary venules in the skin, mucous membranes, and viscera. It is associated with epistaxis and arteriovenous malformations. Klippel-Trenaunay syndrome involves limb overgrowth associated with capillary and venous malformations. There are enlarged lateral leg veins termed the “veins of Servelle.” Parkes-Weber syndrome involves multifocal capillary malformations and arteriovenous malformations and may have associated limb overgrowth. Blue rubber bleb nevus syndrome involves multifocal venous malformations which are most often in the skin, soft tissues, and gastrointestinal tract. Chronic anemia is a common problem for these patients.1–7
Vascular malformations can be subdivided into capillary, venous, arteriovenous, and lymphatic subtypes. They occur because of faulty embryonic morphogenesis. They are always present at birth and grow proportionally with the child, but they do not regress. The autonomic nervous system influences vascular development. This explains why some abnormalities such as port-wine stains occur in specific nerve distributions. During embryonic development, the venous and arterial channels only appear after the initial capillary network has been formed. Errors in this developmental stage are thought to be responsible for development of vascular malformations.1
Port-wine stains or capillary malformations affect 0.3% of newborn babies (not 3%) and most commonly occur on the face (80%). One of the main reasons for treating them early in life is to avoid development of a cobblestone appearance which can occur in adulthood if left untreated. They can correspond to the trigeminal nerve (V1–3) distribution, but not the facial nerve and are sometimes accompanied by ocular and central nervous system disorders. Females are affected three times as often as males. Treatment modalities include potassium titanyl phosphate (KTP), pulsed dye, or neodymium-doped yttrium aluminum garnet (Nd:YAG) laser which is best performed before age 4 (not after). Multimodality treatment is preferred and combines laser treatment with antiangiogenic agents such as imiquimod or rapamycin. Either way, multiple treatments will be required.1
Sturge-Weber syndrome is a condition in which there is a large capillary malformation seen in V1 and commonly V2 trigeminal nerve distribution associated with leptomeningeal malformations, neurologic impairment, seizures, developmental delay, and glaucoma. Therefore, in an infant with capillary malformations in the trigeminal nerve distribution, evaluation for Sturge-Weber syndrome should be made at 6 months of age with a magnetic resonance imaging (MRI) and ophthalmology review. PHACES syndrome involves Posterior fossa malformations, facial Hemangiomas, Arterial abnormalities, Coarctation of the aorta, Eye abnormalities, and anomalies of the Sternum, hence the term “PHACES.” It is not associated with capillary malformations. Maffucci’s syndrome is a rare sporadic condition (less than 200 cases described) that involves enchondromas with multiple cutaneous hemangiomas and lymphangiomas. The enchondromas are benign but have the potential for malignant change leading to chondrosarcomas. von Hippel-Lindau disease is another rare condition, this time inherited, that involves hemangiomas of the retina, hemangioblastomas of the cerebellum, and pancreatic, liver, and adrenal gland cysts. Seizures and mental retardation can also occur. Inheritance is autosomal dominant and results from a mutation in the von Hippel- Lindau tumor suppressor gene. It occurs in around 1 in 30,000 births and patients are at risk of developing various different malignant tumors including renal tumors as well as benign proliferations. CLOVES syndrome is another extreme condition which involves vascular anomalies. It stands for: Congenital, Lipomatous, Overgrowth, Vascular malformations, Epidermal nevi, and Skeletal/Spinal/Scoliosis anomalies. Often, there are large macrocystic lymphatic malformations with truncal fatty overgrowth and scoliosis.1–5
Venous malformations are present at birth (in contrast to congenital hemangiomas) and can grow significantly during pregnancy or puberty if they are hormone sensitive. They decrease in size when elevated and swell in a dependent position. Demonstrating this is a key part of the clinical examination of these lesions. They commonly cause aching when full, especially in the extremities such as where varicose veins are present. Sclerotherapy is a popular treatment modality, with a variety of different injectables, and laser therapy is successful with neodymium-doped yttrium aluminum garnet (Nd:YAG) rather than erbium-doped yttrium aluminum garnet (Re:YAG) laser.1
Clear cutaneous vesicles signify a dermal lymphatic component in a lymphatic malformation. This is termed lymphangioma circumscriptum and is generally part of a microcystic subtype of lymphatic malformation. Bony overgrowth and simultaneous venous anomalies are common associations. Lymphatic malformations frequently become infected and require aggressive antibiotic therapy. Surgery for lymphatic malformations is associated with many complications, including delayed wound healing, infection, and prolonged drainage. Furthermore, surgery is associated with a high recurrence rate. Treating these with intralesional bleomycin has shown promising results.1,2
Arteriovenous malformations (AVMs) are high-flow pulsatile lesions that are difficult to treat surgically and require preoperative embolization to minimize risk of bleeding during resection. It is usual to embolize AVMs 24–72 hours before surgery. The main risk for patients undergoing resection of AVMs is bleeding due to the high- flow and consumptive coagulopathy. The latter must be addressed preoperatively with correction of any clotting dysfunction. Preoperative imaging consists of an MRI to determine the extent of the lesion and surrounding soft tissue involvement. Angiography is required to identify the feeding vessels and vascular anatomy. Recurrence rates are high after surgical excision and for this reason generous margins should be taken where possible. Patients may need not only hypotensive anesthesia but also cardiopulmonary bypass in some situations. Management of these malformations is a multidisciplinary task, given the complexity and high risks of morbidity and mortality.1