EPIDERMOLYSIS BULLOSA DENTAL CARE

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Plastique Hôpital Universitaire Necker Enfants Malades, Paris, France 2) Centre de Référence des

1 Mme Necker, 1775 EPIDERMOLYSIS BULLOSA DENTAL CARE Dr Bothild Kverneland 1,2), Dr Ioana Pavlov 1,2), Pr Arnaud Picard 1,2,3), Pr Marie-PauleVazquez 1,2,3) Service de Chirurgie Maxillo-Faciale et Plastique Hôpital Universitaire Necker Enfants Malades, Paris, France 2) Centre de Référence des Malformations Rares de la Face et de la Cavité Buccale 3) Faculté de Médecine Paris-Descartes, Université Paris 5 1)

2 WHY? WHEN? HOW?

3 WHY?

4 WHY? TO PREVENT AND AVOID CATASTROPHIES! - PAIN - INFECTIONS - LACK OF ESTHETICS - TREATMENT DIFFICULTIES - COST

WHY? ANAEROBES AEROBES SOCIO-ECONOMIC DIFFICULTIES, IMMIGRATION YOUNG AGE DISEASE MICROSUBSTRAT ORGANISMES HOST + TIME IMMUNO_ DEFICIENCY REDUCED SALIVA FLOW, XEROSTOMIA SALIVARY CLEARANCE PH

5 WHY? ANAEROBES AEROBES SOCIO-ECONOMIC DIFFICULTIES, IMMIGRATION YOUNG AGE DISEASE MICROSUBSTRAT ORGANISMES HOST + TIME IMMUNO_ DEFICIENCY REDUCED SALIVA FLOW, XEROSTOMIA SALIVARY CLEARANCE PH TOOTH MORPHOLOGY LACK OF SPACE, DIFFICULT ORAL HYGIENE HARD-TISSUE QUALITY BAD FOOD HABITS CARBOHYDRATES FREQUENT FOOD INTAKE MIXED FOOD LIQUID MEALS EXISTENT CARIES? PSYCHOLOGICAL AND MECHANICAL STRESS

TOOTH MORPHOLOGY LACK OF SPACE, DIFFICULT ORAL HYGIENE HARD-TISSUE QUALITY BAD FOOD HABITS

6 WHY? 1976: Crawford et al: - extraction of all teeth? 1999: Wright et al: - dental treatment is possible! 2008: Skogedal et al: - good oral health is possible! Crawford EG, Burkes EJ Briggaman RA: Hereditary epidermolysis bullosa: oral manifestations and dental therapy, O Surg. O Med. O Pathol, 42, (1976) Wright JT: Oral Manifestations of Epidermolysis Bulosa in EG Clinical Epidemiologiec, and Laboratrory Advances and the Findings of the National EB Registry , The John Hopkins Universidty Press Baltimore (1999 Skogedal N, Saltnes S, Storhaug K: Recessive Dystrophic epidermolyis bullosa (RDEB) Caries preventionand preventive extractions of molars. Clinical presentation of 3 cases, 2008, Ref Type Conference Proceeding

O Pathol, 42, 490-500 (1976) Wright JT: Oral Manifestations of Epidermolysis Bulosa in EG Clinical Epidemiologiec, and Laboratrory Advances and the Findings of the National EB Registry

7 WHEN? - 6 months: FIRST DECIDUAL TEETH - 3 years: TEMPORARY DENTURE COMPLETE - 6 years: FIRST PERMANENT MOLARS, INCISORS (mixed denture) - 9 years: PREMOLARS, CANINES - 12 years: SECOND PERMANENT MOLAR (young adult denture) MEET THE DENTAL TEAM AS SOON AS POSSIBLE, ADAPTED INTERVALS

9 years: PREMOLARS, CANINES - 12 years: SECOND PERMANENT MOLAR (young

8 ORAL MANIFESTATIONS Epidermolysis Bullosa Simplex Junctional Epidermolysis Bullosa Dystrophique RecessiveEpidermolysis Bullosa

9 ORAL MANIFESTATIONS EBS Gingival blisters, erosions, ulcers Gingival retractions Hypoplastic enamel? épiderme EBS derme hypoderme TREATMENT POSSIBILITIES:

10 ORAL MANIFESTATIONS EBJ Enamel defect Retained/impacted teeth Bullous lesions, ulcers, erosions Gingival retractions épiderme derme EBJ hypoderme TREATMENT POSSIBILITIES:

11 ORAL MANIFESTATIONS dreb Gingival blisters, erosions, ulcers Gingival retractions Microstomia Reduced oral opening Vestibular obliteration Ankyloglossie Dental crowding Halitosis épiderme derme EBDr hypoderme TREATMENT POSSIBILITIES:

opening Vestibular obliteration Ankyloglossie Dental

12 HOW?

13 HOW? ANAEROBES AEROBES SOCIO-ECONOMIC DIFFICULTIES, IMMIGRATION YOUNG AGE DISEASE MICROSUBSTRAT ORGANISMES HOST BAD FOOD HABITS CARBOHYDRATES FREQUENT FOOD INTAKE MIXED FOOD LIQUID MEALS + SOLUTIONS IMMUNO_ DEFICIENCY REDUCED SALIVA FLOW, XEROSTOMIA SALIVARY CLEARANCE PH TOOTH MORPHOLOGY LACK OF SPACE, DIFFICULT ORAL HYGIENE HARD-TISSUE QUALITY EXISTENT CARIES? PSYCHOLOGICAL AND MECHANICAL STRESS

SOLUTIONS IMMUNO_ DEFICIENCY REDUCED SALIVA FLOW, XEROSTOMIA SALIVARY CLEARANCE PH TOOTH MORPHOLOGY

14 HOW? RESPECT THE 4 TRADITIONNAL PILLARS! - Alimentation - Hygiène - Fluor - Regular visits for preventive measures

15 CONCLUSION -

16 MERCI

17 BIBLIOGRAPHY BIBLIOGRAPHIE - American Academy of Pediatric Dentistry, Guidelines on Infant Oral Health Care, American Academy of Pediatric Dentistry Reference Manual , Chicago, 2009, pp Wells M, Oral health status of children with craniofacial anomalies. Pediatr Dent May-Jun;35(3):E Picard A et Al. Calendrier et protocole interventionnel des fentes labiopalatines au sein du Service de chirurgie maxillo-faciale et plastique le l Hôpital D Enfants Trousseau (AP-HP, Paris), Rev Orthop Dento Faciale 2011;45: Crawford EG, Burkes EJ Briggaman RA: Hereditary epidermolysis bullosa: oral manifestations and dental therapy, O Surg. O Med. O Pathol, 42, (1976) - Wright JT: Oral Manifestations of Epidermolysis Bulosa in EG Clinical Epidemiologiec, and Laboratrory Advances and the Findings of the National EB Registry , The John Hopkins Universidty Press Baltimore ( Skogedal N, Saltnes S, Storhaug K: Recessive Dystrophic epidermolyis bullosa (RDEB) Caries preventio nand preventive extractions of molars. Clinical presentation of 3 cases, 2008, Ref Type Conference Proceeding - Kverneland B: Pourquoi a-t-on mal aux dents?, CLUD Kverneland B et al: Les épidermolyses bulleuses de l enfant, Revue francophone d odontologie pédiatrique 2011 Vol06N4,pp Huckert M et al: Epidermolyses bulleuses dystrophique, particularités de la prise en charge sous anesthesie generale, Revue francophone d odontologie pédiatrique 2011 Vol06N4,pp170-5

Calendrier et protocole interventionnel des fentes labiopalatines au sein du Service de chirurgie maxillo-faciale et plastique le l Hôpital D Enfants Trousseau (AP-HP, Paris), Rev Orthop Dento

19 LES EPIDERMOLYSES BULLEUSES HEREDITAIRES LES ATTEINTES BUCCALES Manifestations Directes de la génodermatose (EB) 1: Bulles orales 2: Microstomie labiale 3: Ouverture buccale réduite 4: Synéchies vestibulaires 5: Ankyloglossie 6: Récessions gingivales 7: Volume global oral réduit 8: Hyposialie, xérostomie 9: Agueusie 10: Amélogénèse défectueuse - Solutions? - Attente thérapie spécifique. Manifestations Infectieuses et Multifactorielles (indirectes) de la maladie 1: Hygiène difficile 2: Stomatite 3: Glossite 4: Parodontopathies 5: Halitose 6: DDM encombrement, malpositions 7: Caries 8: Pertes dentaires 9: Alimentation per os difficile 10: Gastrostomiae - Solutions?

défectueuse - Solutions? - Attente thérapie spécifique.

20 LES EPIDERMOLYSES BULLEUSES HEREDITAIRES L HYGIENE BUCCO-DENTAIRE 90 MM, 2 ans, score: =12/100 score spécifiq u e 80 score nonuspécifiqe SOMME DES SCORES 70 Age d'examen (an) 60 NN, 9 ans, score: = 70/ OO, 12 ans score: = 81/

60 NN, 9 ans, score: 28 + 42 = 70/100 50 40 30 OO, 12 ans score: 31 + 50 = 81/100 20 10 0

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