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Angiomas or vascular abnormalities [EN]

Angiomas or vascular abnormalities mainly comprise haemangiomas of the infancy and vascular malformations : capillary malformations (capillary nevus or port wine stain), lymphovascular, veinous and arterial malformations.



HEMANGIOMAS:

hemangiomeHemangiomas are found in 4 to 10% of infants, with a marked female predominance, increased prevalence in low birth weight neonates. Prenatal associations include multiple gestation, placenta praevia, and pre-eclampsia. Hemangiomas typically appear during the first weeks of life. Their natural history includes an early stage of rapid proliferation followed by a plateau and a slow involution over 5 to 6 years. The rapid growth phase is variable in duration and may last for a few weeks to one year.  The involution process begins at approximately 12 months of age and continues over the next 5 to 7 years. Hemangioma become progressively paler and less firm at palpation. At the age of 5-6, almost 70% of hemangiomas completely disappear, although minimal skin sequels remain in about 30% of cases. Cutaneous hemangiomas can be superficial, deep or mixed. Sometimes they are found in visceral locations (liver mostly). Superficial lesions are well delimited and often feature the classic so-called strawberry hemangioma. Those within the deep dermis or subcutaneous tissues often present as pale blue or purple firm and elastic masses. Finally, mixed forms associate a superficial component surrounding a deeper sub-cutaneous haemangioma. It should be noted that many hemangiomas do not need any treatment, however, 5-15% of  them require consulting a specialist because of complications (superficial ulceration, expanding volume with a risk of compression of vital organs). In accordance with the type of hemangioma and child’s age, various treatment can be proposed included betablockers, corticosteroids, laser or surgery.



PORT WINE STAINS (superficial vascular capillary malformations, capillary nevus):

angiome planCapillary nevus is present at birth and persists over life. The lesion has no tendency to expand but will increase proportionally to the growth curve. Capillary nevus is mostly an aesthetic problem; however its localisation in a certain skin area may be associated with complications. Sturge-Weber-Krabbe syndrome associates capillary nevus of the face and meningeal angioma with or without glaucoma. The risk area for capillary nevus corresponds to the ophtalmic branch of the trigeminal nerve (forehead and upper eyelid). This type of capillary nevus should be distinguished from another form of vascular birthmark that affects symmetrically the forehead and one or the two eyelids. In addition, capillary nevi of the lower limb may be the cause of abnormal growth in length and diameter of the involved limb that necessitates an orthopaedic coverage. Currently the most effective treatment for the capillary nevus is the pulsed dye laser. Several treatment sessions are necessary to obtain satisfactory results (4 to 6 usually). In most cases, the pulsed dye laser only allows to make the lesion paler.



LYMPHOVASCULAR MALFORMATIONS OR LYMPHANGIOMAS

Lymphangiomas may present as soft subcutaneous cysts and are named cystic hygroma. They are rather localized in the head and neck region. The main complication of these lesions is the occurrence of painful inflammatory episodes, which may lead to a secondary cure. Lymphangioma circumscriptum is another type of lymphangioma and the commonest type. It presents as small hemorrhagic or translucent vesicles that may arise on the surface of the skin. These vesicles may be isolated or grouped resembling frog spawn. Lymphangioma may be present more atypically with only hypertrophy of the subcutaneous tissues without cutaneous symptoms. The treatment of lymphangiomas is difficult. Macro (large) cystic lesions of lymphangioma may be surgically removed or treated with sclerotherapy. Microcystic lesions are usually not accessible to therapy.



VENOUS MALFORMATIONS

Venous malformations present as single or multiple compressible lesions, blue or purple resembling varicose veins that tend to increase in size during efforts.  
They may become painful because of  thrombosis. These malformations are usually under the influence of hormones. Indeed, they often aggravate at puberty or during pregnancy. Treatment is difficult and is not the rule unless the lesions are limited and accessible for surgery. In most cases, definitive therapy is not possible and partial therapy such as sclerosis of larger lesions is proposed and/or surgical removal to decrease size of the lesions.



ARTERIOVENOUS MALFORMATIONS

During childhood, arteriovenous malformations are usually in a dormancy phase. At that time, they may mimic a capillary nevus. Clinical signs that should alert include increased local heat and extension/increased volume of the lesion which is unusual in the case of a capillary nevus. In the context of puberty, the lesion may increase. Evolution during lifetime, and especially during pregnancy, is unpredictable and the malformation may develop and expand into the neighboring tissues (skin, muscles, or even bones). Treatment is very challenging and typically care requires a multidisciplinary approach (dermatologists, radiologists and surgeons).


Links:

http://www.anomalie-vasculaire.com/


Publications:

Moehrle M, Léauté-Labrèze C, Schmidt V, Röcken M, Poets CF, Goelz R. Topical Timolol for Small Hemangiomas of Infancy. Pediatr Dermatol. 2012 Apr 4. doi:10.1111/j.1525-1470.2012.01723.x. [Epub ahead of print] PubMed PMID: 22471694.

Cante V, Pham-Ledard A, Imbert E, Ezzedine K, Léauté-Labrèze C. First report of topical timolol treatment in primarily ulcerated perineal haemangioma. Arch Dis Child Fetal Neonatal Ed. 2012 Mar;97(2):F155-6. Epub 2011 Dec 21. PubMed PMID: 22190187.

Thoumazet F, Léauté-Labrèze C, Colin J, Mortemousque B. Efficacy of systemic propranolol for severe infantile haemangioma of the orbit and eyelid: a case tudy of eight patients. Br J Ophthalmol. 2012 Mar;96(3):370-4. Epub 2011 Jun 14. PubMed PMID: 21673014.

Léauté-Labrèze C, Prey S, Ezzedine K. Infantile haemangioma: part I. Pathophysiology, epidemiology, clinical features, life cycle and associated structural abnormalities. J Eur Acad Dermatol Venereol. 2011 Nov;25(11):1245-53. doi: 10.1111/j.1468-3083.2011.04102.x. Epub 2011 May 14. PubMed PMID: 21569112.

Léauté-Labrèze C, Prey S, Ezzedine K. Infantile haemangioma: part II. Risks, complications and treatment. J Eur Acad Dermatol Venereol. 2011 Nov;25(11):1254-60. doi: 10.1111/j.1468-3083.2011.04105.x. Epub 2011 May 14. PubMed PMID: 21569113.

Saint-Jean M, Léauté-Labrèze C, Mazereeuw-Hautier J, Bodak N, Hamel-Teillac D, Kupfer-Bessaguet I, Lacour JP, Naouri M, Vabres P, Hadj-Rabia S, Nguyen JM, Stalder JF, Barbarot S; Groupe de Recherche Clinique en Dermatologie Pédiatrique. Propranolol for treatment of ulcerated infantile hemangiomas. J Am Acad Dermatol. 2011 May; 64(5): 827-32. Epub 2011 Feb 25. PubMed PMID: 21353332.

Mazereeuw-Hautier J, Hoeger PH, Benlahrech S, Ammour A, Broue P, Vial J, Ohanessian G, Léauté-Labrèze C, Labenne M, Vabres P, Rössler J, Bodemer C. Efficacy of propranolol in hepatic infantile hemangiomas with diffuse neonatal hemangiomatosis. J Pediatr. 2010 Aug;157(2):340-2. Epub 2010 May 20. PubMed PMID: 20488455.

Léauté-Labrèze C, Sans-Martin V. [Infantile hemangioma]. Presse Med. 2010 Apr;39(4):499-510. Epub 2010 Mar 5. Review. French. PubMed PMID: 20207100.

Sans V, de la Roque ED, Berge J, Grenier N, Boralevi F, Mazereeuw-Hautier J, Lipsker D, Dupuis E, Ezzedine K, Vergnes P, Taïeb A, Léauté-Labrèze C.

Propranolol for severe infantile hemangiomas: follow-up report. Pediatrics. 2009 Sep;124(3):e423-31. Epub 2009 Aug 10. PubMed PMID: 19706583.

Léauté-Labrèze C, Taïeb A. [Efficacy of beta-blockers in infantile capillary haemangiomas: the physiopathological significance and therapeutic consequences]. Ann Dermatol Venereol. 2008 Dec;135(12):860-2. Epub 2008 Nov 20. Review. French. PubMed PMID: 19084699.

Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taïeb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008 Jun 12;358(24):2649-51. PubMed PMID: 18550886.

Lacoste A, Léauté-Labrèze C. [Neonatal haemangiomatosis]. Ann Dermatol Venereol. 2007 Aug-Sep;134(8-9): 694-8; quiz 693, 699. French. PubMed PMID: 7925701.

Stockman A, Boralevi F, Taïeb A, Léauté-Labrèze C. SACRAL syndrome: spinal dysraphism, anogenital, cutaneous, renal and urologic anomalies, associated with an angioma of lumbosacral localization. Dermatology. 2007;214(1): 40-5. PubMed PMID: 17191046.

Léauté-Labrèze C, Stockmann A. [Genital region angiomas]. Ann Dermatol Venereol. 2004 Oct;131(10):901-5. Review. French. PubMed PMID: 15697084.

Agesta N, Boralevi F, Sarlangue J, Vergnes P, Grenier N, Léauté-Labrèze C. Life-threatening haemorrhage as a complication of a congenital haemangioma. Acta Paediatr. 2003 Oct;92(10):1216-8. PubMed PMID: 14632343.

Léauté-Labréze C, Boralevi F, Pedespan JM, Meymat Y, Taïeb A. Pulsed dye laser for Sturge-Weber syndrome. Arch Dis Child. 2002 Nov;87(5):434-5. PubMed PMID: 12390926; PubMed Central PMCID: PMC1763097.

Thédenat B, Léauté-Labrèze C, Boralevi F, Roul S, Labbé L, Marlière V, Taïeb A. [Blood pressure monitoring in infants with hemangiomas treated with corticosteroids]. Ann Dermatol Venereol. 2002 Feb;129(2):183-5. French. PubMed PMID: 11937956.

Bakhach S, Grenier N, Berge J, Léauté-Labrèze C, Chateil JF, Douws C, Vergnes P, Taïeb A. [Color Doppler sonography of superficial capillary hemangiomas]. J Radiol. 2001 Nov;82(11):1613-9. French. PubMed PMID: 11894546.

Enjolras O, Mulliken JB, Wassef M, Frieden IJ, Rieu PN, Burrows PE, Salhi A, Léauté-Labreze C, Kozakewich HP. Residual lesions after Kasabach-Merritt phenomenon in 41 patients. J Am Acad Dermatol. 2000 Feb;42(2 Pt 1):225-35. PubMed PMID: 10642677.

Léauté-Labrèze C, Taïeb A. Caution with regard to the efficacy of interferon alfa-2b in the treatment of giant hemangiomas. Arch Dermatol. 1998 Oct;134(10):1297-8. PubMed PMID: 9801696.

 

 
 
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