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dystrophic epidermolysis bullosa (RDEB)—Clinical practice guidelines

24. What is the best frequency of maintenance recall appointments after implant supported

rehabili-tation in patients with RDEB?

Summary of evidence

Most authors recommend monitoring the patient 1 and 3 months after implant placement and implant rehabil-itation. Thereafter, the patient should be engaged in a monitoring schedule. The appointments should be every 2 or 3 months the first year,20,21,25–29,31and thereafter on a biannual schedule.30 Panoramic radiographs should be taken after 6 months, 12 months, and every 12 months thereafter (Image 4.26).20,21,25,26,31 Most authors do not remove the rehabilitation to perform the cleaning ses-sion. Only one author removed the abutments for hygiene purposes.24

Level of evidence

∙ Nonanalytic studies (3)

Recommendation

∙ The patients should be clinically evaluated at 1 week, 1 month, and 3 months after the implant surgery.

I M A G E 4 . 2 6 One-year follow-up panoramic radiograph

∙ After the implant rehabilitation, the patient should be monitored after 1 week and 1 month.

∙ Dental hygiene sessions should be scheduled every 3 to 6 months thereafter.

∙ Radiographs should be obtained every 12 months.

∙ Prosthesis should only be removed if there is a problem, not for standard cleaning.

Images

We would like to acknowledge the support of patients, clin-icians, and researches from different clinical centers glob-ally for collaborating by providing images for the present guideline. Written informed consent has been obtained for all images where patients can be recognized. Images pro-vided by Dr. Marcelo Guzman-Letelier, Dr. Antonio Oli-vares, and Dr. Susanne Krämer.

R E F E R E N C E S

1. Stavropoulos F, Abramowicz S. Management of the oral surgery patient diagnosed with epidermolysis bullosa: report of 3 cases and review of the literature. J Oral Maxillofac Surg.

2008;66(3):554-559.

2. Wright JT. Oral manifestations of epidermolysis bullosa. In: J-D Fine, ed.Epidermolysis Bullosa Clinical, Epidemiologic, and Lab-oratory Advances and the Findings of the National Epidermolysis Bullosa Registry. Baltimore: The Johns Hopkins University Press;

1999:236-256.

3. De Benedittis M, Petruzzi M, Favia G, Serpico R. Oro-dental manifestations in Hallopeau-Siemens-type recessive dystrophic epidermolysis bullosa.Clin Exp Dermatol. 2004;29(2):128-132.

4. Silva LCP, Cruz RA, Abou-Id LR, Brini LNB, Moreira LS. Clini-cal evaluation of patients with epidermolysis bullosa: review of the literature and case reports.Spec Care Dentist. 2004;24(1):22-27.

5. Peñarrocha-Diago M, Serrano C, Sanchis JM, Silvestre FJ, Bagán JV. Placement of endosseous implants in patients with oral epi-dermolysis bullosa.Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol. 2000;90(5):587-590.

2009;67(7):1499-1502.

9. Lindemeyer R, Wadenya R, Maxwell L. Dental and anaesthetic management of children with dystrophic epidermolysis bullosa.

Int J Paediatr Dent. 2009;19(2):127-134.

10. Serrano-Martínez MC, Bagán J V, Silvestre FJ, Viguer MT. Oral lesions in recessive dystrophic epidermolysis bullosa.Oral Dis.

2003;9(5):264-268.

11. Krämer SM, Serrano MC, Zillmann G, et al. Oral health care for patients with epidermolysis bullosa - best clinical practice guide-lines.Int J Paediatr Dent. 2012;22(Suppl. 1):1-35.

12. Kummer TR, Nagano HCM, Tavares SS, Santos BZD, Miranda C. Oral manifestations and challenges in dental treatment of epidermolysis bullosa dystrophica. J Dent Child (Chic).

2013;80(2):97-100.

13. Puliyel D, Chiu C, Habibian M. Restorative and periodontal challenges in adults with dystrophic epidermolysis bullosa.J Calif Dent Assoc. 2014;42(5):313-318.

14. Feijoo JF, Bugallo J, Limeres J, et al. Inherited epidermolysis bul-losa: an update and suggested dental care considerations.J Am Dent Assoc. 2011;142(9):1017-1025.

15. Reichart PA, Schmidt-Westhausen AM, Khongkhunthian P, Strietzel FP. Dental implants in patients with oral mucosal diseases - a systematic review.J Oral Rehabil. 2016;43(5):388-399.

16. Diz P, Scully C, Sanz M. Dental implants in the medically com-promised patient.J Dent. 2013;41(3):195-206.

17. Candel-Marti M-E, Ata-Ali J, Oltra D, Peñarrocha-Diago M, Bagán J-V. Dental implants in patients with oral mucosal alterations: An update.Med Oral Patol Oral Cir Bucal.

2011;16(6):e787-e793.

18. Chrcanovic BR, Gomez RS. Dental implants in patients with epi-dermolysis bullosa: a systematic review.Oral Maxillofac Surg.

2019;23(4):389-394.

19. Scottish Intercollegiate Guidelines Network., Harbour RT, Forsyth L. SIGN 50: A Guideline Developer’s Handbook.

Ediburgh, Scotland: Scottish Intercollegiate Guidelines Net-work; 2008:102 p.

20. Peñarrocha M, Rambla J, Balaguer J, et al. Complete fixed pros-theses over implants in patients with oral epidermolysis bullosa.

J Oral Maxillofac Surg. 2007;65(7):103-106.

21. Peñarrocha M, Larrazábal C, Balaguer J, et al. Restoration with implants in patients with recessive dystrophic epidermolysis bullosa and patient satisfaction with the implant-supported superstructure.Int J Oral Maxillofac Implants. 2007;22(4):651-655.

22. Lee H, Al Mardini M, Ercoli C, Smith MN. Oral rehabilitation of a completely edentulous epidermolysis bullosa patient with an implant-supported prosthesis: a clinical report.J Prosthet Dent.

2007;97(2):65-69.

23. Oliveira MA, Ortega KL, Martins FM, Maluf PSZ, Magalhes MG.

Recessive dystrophic epidermolysis bullosa”oral rehabilitation using stereolithography and immediate endosseous implants.

Spec Care Dent. 2010;30(1):23-26.

24. Müller F, Bergendal B, Wahlmann U, Wagner W. Implant-supported fixed dental prostheses in an edentulous patient with dystrophic epidermolysis bullosa. Int J Prosthodont.

2010;23(1):42-48.

25. Peñarrocha-Oltra D, Peñarrocha-Diago M, Balaguer-Martínez J, Ata-Ali J, Peñarrocha-Diago M. Full-arch fixed prosthesis sup-ported by four implants in patients with recessive dystrophic epi-dermolysis bullosa.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2011;112(2):e4-10.

26. Oltra D, Aloy-Prósper A, Ata-Ali J, Peñarrocha-Diago M, Peñarrocha-Peñarrocha-Diago M. Implants placed simultaneously with particulated bone graft in patients diagnosed with reces-sive dystrophic epidermolysis bullosa.J Oral Maxillofac Surg.

2012;70(1):e51-e57.

27. Agustín-Panadero R, Gomar-Vercher S, Peñarrocha-Oltra D, Guzmán-Letelier M, Peñarrocha-Diago M. Fixed full-arch implant-supported prostheses in a patient with epidermoly-sis bullosa: a clinical case history report. Int J Prosthodont.

2015;28(1):33-36.

28. Guzmán M, Jara CC, Peñarrocha-Oltra S, Gomar-Vercher S, Peñarrocha-Diago M. Fixed implant-supported full-arch pros-thesis in epidermolysis bullosa with severe symptoms.J Oral Implantol. 2016;42(6):498-505.

29. Agustín-Panadero R, Serra-Pastor B, Peñarrocha-Oltra D, Peñarrocha-Diago M. Maxillary implant prosthodontic treat-ment using digital laboratory protocol for a patient with epidermolysis bullosa: a case history report.Int J Prosthodont.

2017;30(4):390-393.

30. Molina G, Torassa DLR. Implant-supported dental prostheses in a patient with recessive dystrophic epidermolysis bullosa. Four-year follow up.Methodo. 2017;2(4):129-33.

31. Agustín-Panadero R, Serra-Pastor B, Peñarrocha-Oltra D, Fer-reiroa A, Peñarrocha-Diago M. Digital scanning for implant-supported fixed complete arch dental prostheses for patients with epidermolysis bullosa: a case series evaluation.J Prosthet Dent. 2019;122(4):364-370.

32. Hubbard LD, Mayre-Chilton K. Retrospective longitudinal study of osteoporosis in adults with recessive dystrophic epidermolysis bullosa.Clin Case Reports. 2019;7(1):58-63.

33. Peñarrocha M, Serrano MC, Sanchis JM, et al. Periodoncia.

Monográfico de Osteointegración Octubre-Diciembre2000;10(4).

Artículo Original.

Introduction

Children and adults living with Epidermolysis Bullosa (EB) present fragile skin and mucosa, requiring a special approach from the health care team. When planning a dental procedure under sedation or general anesthesia, the entire clinical team must be aware of the appropriate precautions that individuals with EB may require to receive optimal care. The first clinical practice guideline (CPG) on oral health care for patients with EB was pub-lished in 2012.1As new evidence has been published since it has become necessary to update the guideline ensuring that all new data are incorporated, as well as including more experts from different centers around the world to discuss the different treatment options and to work toward establishing the best CPGs. The present guideline has been entrusted by DEBRA International, the worldwide network working on behalf of those living with EB. It follows a standard methodology based on a systematic review of the currently available scientific evidence and a panel discussion.

Aim

To provide the users with information on the current best practice for managing local anesthesia, principles of seda-tion and general anesthesia for children and adults with EB undergoing dental treatment.

Health question covered in the guideline

Do patients with EB undergoing dental treatment under sedation or general anesthesia need specific precautions or treatment modifications compared to healthy individuals to avoid trauma to the skin and mucosa?

Users

Specialists in Pediatric Dentistry, Special Care Dentistry, Oral and Maxillofacial Surgery, Endodontics,

Periodon-tics, Rehabilitation and General Dental Practitioners, Nurses, Anesthetists, Pediatricians, Dermatologists, and other health care professionals managing patients with EB who need dental treatment under sedation or general anes-thesia.

Target group

These guidelines can be applied to all patients diagnosed with EB regardless of the type of EB. They are therefore applicable to all four major types of EB: EB simplex, junc-tional EB, dystrophic EB, and Kindler EB.

Methodology

Systematic literature searching