Veronika Stiles University of Michigan HYGDCE 484 November 25,2008

 Confusing terminology  A single classification is necessary  International Society for the Study of Vascular Anomalities classification system (1996): Soft tissue vascular lesions Vascular tumors Vascular Malformations (e.g., hemangioma) (e.g., Sturge-Weber Syndrome) 1. Enjolras O, Mulliken JB. Vascular tumors and vascular malformations (new issues). Adv Dermatol. 1997;13:

 Benign proliferation of endothelial cells  Most common neoplasm of the infancy  Does not exhibit unlimited growth potential  Types: capillary (small vessel); cavernous (large vessel); and mixed 2. Ibsen OA, Phelan JA. Oral Pathology for the dental hygienist. 3rd ed. New York: W.B. Saunders Company; © DermAtlas

 Unclear  Possible role of angiogenesis is identified:  Excess of stimulating cytokines, or…  Decrease in angiogenesis inhibitors  No genetic component is reported 3. Katz DA. Hemangioma [Internet]. Emedicine from WebMD; c [updated 2008 Nov 17; cited 2008 Nov 21]. Available from:

 30% are visible at birth  Majority arises between weeks 1-4  Go through slow regression “involution”  Involution can last 3-10 years  By age 5, 50% of infants no longer have it 4. Vascular Birthmarks Foundation.org [Internet]. Latham (NY): Vascular Birthmarks Foundation; c [cited 2008 Nov 21]. Available from: 5. Correa PH, Nunes LC, Johann AC, Ferreira de Aguiar MC, Gomez RS, Mesquita RA. Prevalence of oral hemangioma, vascular malformation and varix in a Brazilian population. Braz Oral Res Jan- Mar; 21 (1): 40-5.

 Females are 5 times more susceptible  Twins  Caucasians  Low birth weight infants (>2.2 lbs): 26% chance of developing hemangiomas 4. Vascular Birthmarks Foundation.org [Internet]. Latham (NY): Vascular Birthmarks Foundation; c [cited 2008 Nov 21]. Available from: Available from: Microsoft Office Word 2007

 83% occur in head and neck area  Tongue (ventral) is most common intraoral site  Followed by lips and buccal mucosa  Variably sized deep red or blue lesions  Frequently blanch under pressure 2.Ibsen OA, Phelan JA. Oral Pathology for the dental hygienist. 3 rd ed. New York: W.B. Saunders Company; Vascular Birthmarks Foundation.org [Internet]. Latham (NY): Vascular Birthmarks Foundation; c [cited 2008 Nov 21]. Available from: © DermAtlas Ventral of the tongue Upper lip

 Usually asymptomatic to the patient  Compression of tissues around it  Formation of fissures  Macroglossia if the tongue is affected  Formation of ulcers and hemorrhages  Functional and aesthetic problems 6. Donnelly LF, Adams DM, Bisset GS 3 rd. Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic. AJR Am J Roentgenol. 2000; 174(3): Jackson IT, Carreno R, Potparic Z, Hussain K. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plas Reconstr Surg. 1993; 91 (7) : © DermAtlas

 Keep in mind possibility of severe bleeding  Severe hemorrhage can complicate treatment  Interference with eating, breathing, speaking  Rapid tumor enlargement  Early detection and biopsy are necessary  Treatment: sclerotherapy, corticosteroids, interferon α, laser, embolization, surgery 2. Ibsen OA, Phelan JA. Oral pathology for the dental hygienist. 3 rd ed. New York: W.B. Saunders Company; Vascular Birthmarks Foundation.org [Internet]. Latham (NY): Vascular Birthmarks Foundation; c [cited 2008 Nov 21]. Available from: 5. Correa PH, Nunes LC, Johann AC, Ferreira de Aguiar MC, Gomez RS, Mesquita RA. Prevalence of oral hemangioma, vascular malformation and varix in a Brazilian population. Braz Oral Res Jan- Mar; 21 (1): 40-5.

 AKA cystic hygroma, lymphangioma, hemangiolymphangioma  Benign tumor composed of lymphatic vessels  Etiology is unclear  Errors of the vascular morphogenesis  Most are congenital 2. Ibsen OA, Phelan JA. Oral pathology for the dental hygienist. 3rd ed. New York: W.B. Saunders Company; Vascular Birthmarks Foundation.org [Internet]. Latham (NY): Vascular Birthmarks Foundation; c [cited 2008 Nov 21]. Available from: 9. Tatulescu D, Secheli I, Secheli M. An unusual case of vascular malformation requiring additional orthodontic, swallowing and voice therapy after intralesional Bieomycin injection. German Medical Science. 2006; 27 (6). © DermAtlas

 Infection-related: 70-80% experience infection  Airway related: may lead to airway obstruction 10.Edwards SP. Oral lymphoangiomas [Internet]. Emedicine from WebMD; c [updated 2006 Apr 12; cited 2008 Nov 21]. Available from: Available from:

 Superficial lesions: pebby and clear/bluish Image 1:Oral representation: Lower lip  Deeper lesions: soft and ill-defined  Macrosystic lesions: fluctuant and transilluminating 10.Edwards SP. Oral lymphoangiomas [Internet]. Emedicine from WebMD; c [updated 2006 Apr 12; cited 2008 Nov 21]. Available from: Available from:

 Oral lesions  compromised airway  Lesions tend to recur  Dental caries is prevalent due to poor OH  Oral-systemic link: spread of odontogenic infection to a LM can be life-threatening  Premature tooth loss compromises diet 10.Edwards SP. Oral lymphoangiomas [Internet]. Emedicine from WebMD; c [updated 2006 Apr 12; cited 2008 Nov 21]. Available from:

 Maxillofacial skeleton dysmorphogenesis  Due to pressure effects and high blood flow  Speech pathologies  Abnormal mobility and morphology of tongue  Palatal hypomobility  Neonate feeding difficulties  Complications from surgical treatment 10.Edwards SP. Oral lymphoangiomas [Internet]. Emedicine from WebMD; c [updated 2006 Apr 12; cited 2008 Nov 21]. Available from:

 Due to multiple dental complications of LM,  aggressive dental care is indicated  meticulous oral hygiene is emphasized  more frequent dental recall appointments  pediatric DDS should be involved early for LM children 10.Edwards SP. Oral lymphoangiomas [Internet]. Emedicine from WebMD; c [updated 2006 Apr 12; cited 2008 Nov 21]. Available from:

 Congenital neurological disorder  Seizures  Large unilateral port-wine stain on forehead  Covers upper eyelid also  Caused by overabundance of capillaries around nerve V  Loss of nerve cells in the area of the stain 11.National Institute of Neurological Disorders and Stroke. NINDS Sturge-Weber Syndrome Information Page [Internet]. Bethesda (MD): Office of Communications and Public Liaison, National Institute of Neurological Disorders and Stroke, National Institutes of Health. [updated 2007 Feb 14; cited 2008 Nov 23]. Available from: © DermAtlas

 Teeth might come in early or late  Gingival hypertrophy is common  Periodontal management is often recommended  Prior to any invasive surgery, MRI/MRA or CAT of the face are indicated  Meticulous oral hygiene is emphasized 12. Comi A (Director of Hunter Nelson Sturge-Weber Center, Kennedy Krieger Institute, Baltimore, MD. ). Ask the SWS expert [Internet]. Message to: Veronika Stiles (University of Michigan Degree Completion E-Learning Dental Hygiene Program, Ann Arbor, MI Nov 19, 09:18 [cited 2008 Nov 23]. © DermAtlas

 My aunt Liliya’s face has been disfigured by a large unilateral port-wine stain: R cheek, eye  When she moved to Germany, laser surgery has corrected the port-wine stain appearance  Former Russian president, Mikhail Gorbachev, has been affected by a port-wine stain Available from: