General and orofacial aspects of pychnodysostosis: report of two dental surgical intercurrences

Authors

DOI:

https://doi.org/10.33448/rsd-v10i14.21692

Keywords:

Pycnodysostosis; Bone diseases; Surgery oral; Postoperative complications.

Abstract

The aim of this article is to discuss the clinical, diagnostic and treatment aspects of dental surgical complications in patients with pychnodysostosis, through two clinical cases. Female patient, with physical characteristics of pychnodysostosis, 27 years old, complaining of pain, which appeared after undergoing a tooth extraction in the region of element 38. After clinical and radiographic examinations, a mandible fracture was found in the procedure site. The fracture was treated using a submandibular approach and fixation with a reconstruction plate, but without showing radiographic signs of reliable bone consolidation. The second case is a 23-year-old female patient with pychnodysostosis, who reported recurrent pain in the left mandibular body and frequent odors from the oral cavity, after undergoing a tooth extraction that had not healed. The treatment of the symptoms presented was performed using a delicate curettage at the site, where the presence of a cleavage plane was observed. The specimen was sent for histopathological analysis, which later showed a picture compatible with osteomyelitis with foci of bone necrosis. To date, the current literature does not present a description of a detailed clinical protocol for patients with this condition. However, some precautions must be taken to prevent bone fractures, such as avoiding mechanical trauma and invasive surgical approaches, in addition to maintaining regular dental follow-up.

References

Alves, D., Berini Aytés, L., & Gay Escoda, C. (2008). Pycnodysostosis. A report of 3 clinical cases. Med Oral Patol Oral Cir Bucal, 13, E633–5.

Alves, N., & Cantín, M (2014). Clinical and radiographic maxillofacial features of pycnodysostosis. Int J Clin Exp Med, 7, 492–6.

Bitu, C., Kaupilla, J., Teppo, S., & Vilen, S. (2012). Expression of cysteine protease cathepsin K by oral tongue SCCs in vivo and in vitro. Oral Surg Oral Med Oral Pathol Oral Radiol, 114 (4), 109–110.

Cui, Y. (2012). Uma revisão sistemática de distúrbios esqueléticos genéticos em revistas biomédicas chinesas entre 1978 e 2012. Orphanet J Rare Dis, 22 (8), 7–55.

Darcan, S., Akisu, M., Taneli, B., & Kendir, G. (1996). A case of pycnodysostosis with growth hormone deficiency. Clin Genet 50, 422-5.

Edelson, J., Obad, S., Geiger, R., On, A., Artul, H. (1992). Pycnodysostosi. Aspectos ortopédicos com descrição de 14 novos casos. Clin Orthop Relat Res, 280, 263–276.

Elmore, S. M. (1967). Pycnodysostosis: A review. J Bone Joint Surg Am, 49, 153–62.

Farias, J. G. (2003). Picnodisostose Associada a Osteomielite da Maxila [Picnodisostose Associada à Osteomielite da Maxila]. Rev Bras CiêncSaúde, 7 (2), 5.

Gelb, B. D., Shi, G. P., Chapman, H. A., & Desnick, R. J. (2002). Pycnodysostosis, a lysosomal disease caused by cathepsin K deficiency. Curll Mol Med, 2 (5), 36–38.

Girbal, I., Nunes, T., Medeira, A., & Bandeira, T. (2013). Pycnodysostosis with novel gene mutation and severe obstructive sleep apnoea: management of a complex case. BMJ case reports, 2013, bcr2013200590. https://doi.org/10.1136/bcr-2013-200590

Hepp, N., Frederiksen, A. L., Dunø, M., Jørgensen, N. R., et al (2019). Multiple Fractures and Impaired Bone Fracture Healing in a Patient with Pycnodysostosis and Hypophosphatasia. Calcif Tissue Int, 105 (6), 681-686.

Hernández-Alfaro, F., Arenaz, J. B., Serrat, M. S., & Bueno, J. M. (2011). Orthognathic surgery in pycnodysostosis: a case report. Int J Oral Maxillofac Surg, 4 (1), 110–113.

Kamat, S, Sankar, K, Eswari, N. J, Gahlawat, V, Jude, B. N., & Negi, A. (2015). Management of chronic suppurative osteomyelitis in a patient with pycnodysostosis by intra-lesional antibiotic therapy. J Nat Sci Biol Med, 6, 464–7.

Kirita, T, Sugiura, T, Horiuchi, K, Morimoto, Y, Yazima, H, & Sugimura, M (2001). Mandibular reconstruction using a vascularised fibula osteocutaneous flap in a patient with pyknodysostosis. Br J Plast Surg, 54,712–4.

Khoja, A, Fida, M, & Shaikh, A (2015). Pycnodysostosis with Special Emphasis on Dentofacial Characteristics. Case Rep Dent. 2015, 817989.

Marouteaux, P., & Lamy M. (1995). The malady of toulouse-lautrec. Jama, 191 (3), 715–717.

Mujawar, Q., Naganoor, R., Patil, H., Thobbi, A. N., Ukkali, S., & Malagi, N. (2009). Pycnodysostosis with unusual findings: a case report. Cases J, 2, 65-44.

Muto, T, Michiya, H, Taira, H, Murase, H, & Kanazawa, M (1991). Pycnodysostosis. Report of a case and review of the Japanese literature, with emphasis on oral and maxillofacial findings. Oral Surg Oral Med Oral Pathol, 72, 449–55.

Quezado, R (2003). Picnodisostose: relato de dois casos [Pycnodysostosis: report of two cases]. Arq Bras Endocrinol Metab, 47 (1), 95–101.

Sedano, H., Gorlin, R., & Anderson, V. (1968). Pycnodysostosis: clinical and genetic considerations. Sou J Dis Child, 116 (7), 70–77.

Soliman, A. T., Ramadan, M. A., Sherif, A., Aziz, Bedair, E. S., & Rizk, M. M. (2001). Pycnodysostosis: clinical, radiologic, and endocrine evaluation and linear growth after growth hormone therapy. Metab Clin Exp, 50, 905–911.

Wen, X., Yi, L. Z., Liu, F., Wei, J. H., & Xue, Y. (2016). The role of cathepsin K in oral and maxillofacial disorders. Oral Dis, 22, 109–15.

Xue, Y., Cai, T., Shi, S., et al (2011). Clinical and animal research findings in pycnodysostosis and gene mutations of cathepsin K from 1996 to 2011. Orphanet J Rare Diseases, 11 (2), 6–20.

Published

25/10/2021

How to Cite

SUASSUNA, T. M. .; ABREU, N. M. R. de; AGUIAR, Ávilla P.; NOLETO, J. W.; SAMPAIO, F. C. General and orofacial aspects of pychnodysostosis: report of two dental surgical intercurrences. Research, Society and Development, [S. l.], v. 10, n. 14, p. e69101421692, 2021. DOI: 10.33448/rsd-v10i14.21692. Disponível em: https://www.rsdjournal.org/index.php/rsd/article/view/21692. Acesso em: 25 apr. 2024.

Issue

Section

Health Sciences