ORAL VASCULAR MALFORMATIONS:

HEMANGIOMA, LYMPHANGIOMA, AND

STURGE-WEBER SYNDROME

Veronika Stiles

University of Michigan

HYGDCE 484

November 25,2008

CLASSIFICATION SYSTEM 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;13:375-423.   

HEMANGIOMA: DEFINITION AND HISTOLOGY

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; 2000.

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ETIOLOGY OF HEMANGIOMAS

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; c1996-2008 [updated 2008 Nov 17; cited 2008 Nov 21]. Available from: http://www.emedicine.com/orthoped/topic499.htm

HEMANGIOMA DEVELOPMENT

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; c2000-2008 [cited 2008 Nov 21]. Available from: http://www.birthmark.org/hemangiomas.php5. 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. 2007 Jan- Mar; 21 (1): 40-5.

HEMANGIOMA: SUSCEPTIBLE POPULATIONS

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; c2000-2008 [cited 2008 Nov 21]. Available from: http://www.birthmark.org/hemangiomas.php

Available from: Microsoft Office Word 2007

HEMANGIOMA: CLINICAL APPEARANCE

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. 3rd ed. New York: W.B. Saunders Company; 2000.4. Vascular Birthmarks Foundation.org [Internet]. Latham (NY): Vascular Birthmarks Foundation; c2000-2008 [cited 2008 Nov 21]. Available from: http://www.birthmark.org/hemangiomas.php

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Ventral of the tongueUpper lip

HEMANGIOMA: DENTAL COMPLICATIONS

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 3rd. Vascular malformations and hemangiomas: a practical approach in a multidisciplinary clinic. AJR Am J Roentgenol. 2000; 174(3): 597-608.7. 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) :1216-30.

© 2001-2008 DermAtlas © 2001-2008 DermAtlas

HEMANGIOMA: DENTAL CONSIDERATIONS

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. 3rd ed. New York: W.B. Saunders Company; 2000.4. Vascular Birthmarks Foundation.org [Internet]. Latham (NY): Vascular Birthmarks Foundation; c2000-2008 [cited 2008 Nov 21]. Available from: http://www.birthmark.org/hemangiomas.php5. 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. 2007 Jan- Mar; 21 (1): 40-5.

LYMPHATIC MALFORMATION (LM): DEFINITION AND ETIOLOGY

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; 2000.8.Vascular Birthmarks Foundation.org [Internet]. Latham (NY): Vascular Birthmarks Foundation; c2000-2008 [cited 2008 Nov 21]. Available from: http://www.birthmark.org/lm.php9. 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).

© 2001-2008 DermAtlas

LYMPHATIC MALFORMATION: MORBIDITY

Infection-related: 70-80% experience infection

Airway related: may lead to airway obstruction

10.Edwards SP. Oral lymphoangiomas [Internet]. Emedicine from WebMD; c1996-2008 [updated 2006 Apr 12; cited 2008 Nov 21]. Available from: http://www.emedicine.com/derm/topic650.htm

Available from: http://www.birthmarks.us/LM.htm

LYMPHATIC MALFORMATION: PHYSICAL APPEARANCE

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; c1996-2008 [updated 2006 Apr 12; cited 2008 Nov 21]. Available from: http://www.emedicine.com/derm/topic650.htm

Available from: http://www.birthmarks.us/LM.htm

LYMPHATIC MALFORMATIONS: DENTAL COMPLICATIONS 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; c1996-2008 [updated 2006 Apr 12; cited 2008 Nov 21]. Available from: http://www.emedicine.com/derm/topic650.htm

OTHER DENTAL COMPLICATIONS

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; c1996-2008 [updated 2006 Apr 12; cited 2008 Nov 21]. Available from: http://www.emedicine.com/derm/topic650.htm

ADVICE FROM A DENTAL PROFESSIONAL

Due to multiple dental complications of LM,aggressive dental care is indicatedmeticulous oral hygiene is emphasizedmore frequent dental recall appointmentspediatric DDS should be involved early for

LM children

10.Edwards SP. Oral lymphoangiomas [Internet]. Emedicine from WebMD; c1996-2008 [updated 2006 Apr 12; cited 2008 Nov 21]. Available from: http://www.emedicine.com/derm/topic650.htm

STURGE-WEBER SYNDROME: DEFINITION 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: http://www.ninds.nih.gov/disorders/sturge_weber/sturge_weber.htm

© 2001-2008 DermAtlas

SWS: DENTAL COMPLICATIONS

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. Comi@kennedykrieger.com ). Ask the SWS expert [Internet]. Message to: Veronika Stiles (University of Michigan Degree Completion E-Learning Dental Hygiene Program, Ann Arbor, MI. gveronik@umich.edu).

2008 Nov 19, 09:18 [cited 2008 Nov 23].

© 2001-2008 DermAtlas

ORAL VASCULAR MALFORMATIONS: A PERSONAL TOUCH

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: http://en.wikipedia.org/wiki/PORT_wine_stain