Abstract
Aim
To describe dental and dentofacial characteristics observed in patients diagnosed with osteopetrosis and to advise a dental care approach in these patients.
Methods
Four patients were clinically diagnosed with osteopetrosis, characterised by increased bone density, bone marrow failure, blindness and deafness due to compression of cranial nerves. All patients were dentally screened at different ages (2.5–31 years) and three of them were treated with a haematopoietic stem cell transplantation (HSCT) at the age of 6 months, 1 and 3.1 years.
Results
All patients showed similar dental characteristics but varying severity and extent. Dental pits, abnormalities in form, agenesis and enamel deformations are seen. The eruption of the permanent dentition occurs at a slow rate, primary teeth can persist, have no successor, and aberrant form of the primary/permanent teeth can delay eruption. Uneven surfaces and atypical dental crowns combined with visual impairment make brushing of the teeth and plaque removal more difficult to manage.
Conclusion
Dental problems such as delay in tooth eruption, crown anomalies and agenesis are seen in the patients diagnosed with osteopetrosis, although the severity and extensiveness of the symptoms differ and possibly depend on the age of the patient at HSCT. Treatment management: Frequent dental follow-up examinations are necessary for guiding the eruption and professional dental cleanings. Aid in the eruption can be helpful. In the case of surgical interventions, an antibiotic prophylaxis is advised. A fluoride treatment can be added to prevent caries. The role of HSCT in dental findings needs further research.
Similar content being viewed by others
References
Allen G, Logan R, Gue S. Oral manifestations of cancer treatment in children: a review of the literature. Clin J Oncol Nurs. 2010;14(4):481–90 (available from PM: 20682504).
Bansal V, Kumar S, Arunkumar KV, Mowar A, Khare G. Dental management in autosomal recessive (intermediate) osteopetrosis: a case report. Pediatr Dent. 2010;32(7):542–5 (available from PM: 21462770).
Del FA, Cappariello A, Teti A. Genetics, pathogenesis and complications of osteopetrosis. Bone. 2008;42(1):19–29 (available from PM: 17936098).
Driessen GJ, Gerritsen EJ, Fischer A, et al. Long-term outcome of haematopoietic stem cell transplantation in autosomal recessive osteopetrosis: an EBMT report. Bone Marrow Transpl. 2003;32(7):657–63 (available from PM: 13130312).
Gawade PL, Hudson MM, Kaste SC, et al. A systematic review of dental late effects in survivors of childhood cancer. Pediatr Blood Cancer. 2014;61(3):407–16 (available from PM: 24424790).
Helfrich MH. Osteoclast diseases and dental abnormalities. Arch Oral Biol. 2005;50(2):115–22 (available from PM: 15721137).
Jalevik B, Fasth A, Dahllof G. Dental development after successful treatment of infantile osteopetrosis with bone marrow transplantation. Bone Marrow Transpl. 2002;29(6):537–40 (available from:PM: 11960278).
Lam DK, Sandor GK, Holmes HI, Carmichael RP, Clokie CM. Marble bone disease: a review of osteopetrosis and its oral health implications for dentists. J Can Dent Assoc. 2007;73(9):839–43 (available from PM: 18028760).
Makarem A, Lotfi N, Danesh-sani SA, Nazifi S. Osteopetrosis: oral and maxillofacial manifestations. Int J Head Neck Surg. 2012;3:115–7.
Meikle MC. The tissue, cellular, and molecular regulation of orthodontic tooth movement: 100 years after Carl Sandstedt. Eur J Orthod. 2006;28(3):221–40 (available from PM: 16687469).
Schulz A, Moshous D, Steward CG, Villa A. Osteopetrosis–Consensus guidelines of the ESID and EBMT Working Party Inborn Errors. 2-12-2011. Ref Type: Online Source.
Sekerci AE, Sisman Y, Ertas ET, Sahman H, Aydinbelge M. Infantile malignant osteopetrosis: report of 2 cases with osteomyelitis of the jaws. J Dent Child (Chic.). 2012;79(2):93–9 (available from PM: 22828766).
Sobacchi C, Schulz A, Coxon FP, Villa A, Helfrich MH. Osteopetrosis: genetics, treatment and new insights into osteoclast function. Nat Rev Endocrinol. 2013;9(9):522–36 (available from PM: 23877423).
Stark Z, Savarirayan R. Osteopetrosis. Orphanet J Rare Dis. 2009;4:5 (available from: PM: 19232111).
Steward CG. Hematopoietic stem cell transplantation for osteopetrosis. Pediatr Clin North Am. 2010;57(1):171–80 (available from PM: 20307717).
Tyrovola JB, Spyropoulos MN, Makou M, Perrea D. Root resorption and the OPG/RANKL/RANK system: a mini review. J Oral Sci. 2008;50(4):367–76 (available from PM: 19106463).
Villa A, Guerrini MM, Cassani B, Pangrazio A, Sobacchi C. Infantile malignant, autosomal recessive osteopetrosis: the rich and the poor. Calcif Tissue Int. 2009;84(1):1–12 (available from PM: 19082854).
Wang H, Pan M, Ni J, et al. ClC-7 deficiency impairs tooth development and eruption. Sci Rep. 2016;6:19971 (available from PM: 26829236).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Ethical approval
“All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.”
Rights and permissions
About this article
Cite this article
Detailleur, V., Vansteenkiste, G., Renard, M. et al. Dental care approach in patients with osteopetrosis. Eur Arch Paediatr Dent 17, 435–443 (2016). https://doi.org/10.1007/s40368-016-0251-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s40368-016-0251-y