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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.
©2001-2008 DermAtlas
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
© 2001-2008 DermAtlas © 2001-2008 DermAtlas
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. [email protected] ). Ask the SWS expert [Internet]. Message to: Veronika Stiles (University of Michigan Degree Completion E-Learning Dental Hygiene Program, Ann Arbor, MI. [email protected]).
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