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)
WHY? WHEN? HOW?
WHY?
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 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
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, 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 236256, 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
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
ORAL MANIFESTATIONS Epidermolysis Bullosa Simplex Junctional Epidermolysis Bullosa Dystrophique RecessiveEpidermolysis Bullosa
ORAL MANIFESTATIONS EBS Gingival blisters, erosions, ulcers Gingival retractions Hypoplastic enamel? épiderme EBS derme hypoderme TREATMENT POSSIBILITIES:
ORAL MANIFESTATIONS EBJ Enamel defect Retained/impacted teeth Bullous lesions, ulcers, erosions Gingival retractions épiderme derme EBJ hypoderme TREATMENT POSSIBILITIES:
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:
HOW?
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
HOW? RESPECT THE 4 TRADITIONNAL PILLARS! - Alimentation - Hygiène - Fluor - Regular visits for preventive measures
CONCLUSION -
MERCI
BIBLIOGRAPHY BIBLIOGRAPHIE - American Academy of Pediatric Dentistry, Guidelines on Infant Oral Health Care, American Academy of Pediatric Dentistry Reference Manual 2008-9, Chicago, 2009, pp 90-92 - Wells M, Oral health status of children with craniofacial anomalies. Pediatr Dent. 2013 May-Jun;35(3):E79-86. - 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;45:291-299 - Crawford EG, Burkes EJ Briggaman RA: Hereditary epidermolysis bullosa: oral manifestations and dental therapy, O Surg. O Med. 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 236-256, The John Hopkins Universidty Press Baltimore (1999 - 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 2012 - Kverneland B et al: Les épidermolyses bulleuses de l enfant, Revue francophone d odontologie pédiatrique 2011 Vol06N4,pp161-70 - 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
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?
LES EPIDERMOLYSES BULLEUSES HEREDITAIRES L HYGIENE BUCCO-DENTAIRE 90 MM, 2 ans, score: 6 + 6 =12/100 score spécifiq u e 80 score nonuspécifiqe SOMME DES SCORES 70 Age d'examen (an) 60 NN, 9 ans, score: 28 + 42 = 70/100 50 40 30 OO, 12 ans score: 31 + 50 = 81/100 20 10 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34