Souza MA, Valente Soares LAV, Alves dos Santos M Dental abnormalities and oral health in patients with Hypophosphatemic rickets. Clinics. 2010; 65:1023-1026
Martin M, Sabandal I, Robotta P Review of the dental implications of X-linked hypophosphataemic rickets (XLHR). Clin Oral Invest. 2015; 19:759-768
Michałus I, Rusiłska A Rare, genetically conditioned forms of rickets: differential diagnosis and advances in diagnostics and treatment. Clin Genet. 2018; 94:103-114
Al-Jundi SH, Al-Naimy YF, Alsweedan S Dental arch dimensions in children with hypophosphataemic Vitamin D resistant rickets. Eur Archiv Paed Dent. 2010; 11:83-87
Zambrano M, Nikitakis NG, Sanchez-Quevedo MC, Sauk JJ, Sedano H, Rivera H Oral and dental manifestations of vitamin D-dependent rickets type I: report of a pediatric case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003; 95:705-709
Hillman G, Geursten W Pathohistology of undecalcified primary teeth in vitamin D-resistant rickets: review and report of two cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1996; 82:218-224
Cremonesi I, Nucci C, D'Alessandro G, Alkhamis N, Marchionni S, Piana G X-linked hypophosphatemic rickets: enamel abnormalities and oral clinical findings. J Scann Micro. 2014; 36:456-461
Douyere D, Joseph C, Gaucher C, Chaussain C, Courson F Familial hypophosphatemic vitamin D-resistant rickets – prevention of spontaneous dental abscesses on primary teeth: a case report. Oral Med Oral Pathol Oral Radiol Endod. 2009; 107:525-530
Soew WK Diagnosis and management of unusual dental abscesses in children. Aust Dent J. 2003; 48:156-168
Studart Soares EC, Gurgel Costa FW, Rodrigues Ribeiro T, NegreirosNunes Alves AP, SáRorizFonteles C Clinical approach in familial hypophosphatemic rickets: report of three generations. Spec Care Dent. 2013; (33)304-307
Batra P, Tejani Z, Mars M X-Linked Hypophosphatemia: dental and histologic findings. J Can Dent Assoc. 2006; 72:69-71
Perdigao J, Geraldeli A Bonding characteristics of self-etching adhesives to intact versus prepared enamel. J Esthet Rest Dent. 2003; 15:32-42
Papagiannoulis-Alexandridis L, Patelarou N, Kouvelas N The dental management of patients with hypophosphatemic resistant rickets. Odontostomatol Proodos. 1985; 39:15-22
Shroff DV, McWhorter AG, Seale NS Evaluation of aggressive pulp therapy in a population of vitamin D-resistant rickets patients: a follow-up of 4 cases. Pediatr Dent. 2002; 24:347-349
Spontaneous dental abscesses in hereditary hypophosphataemic rickets: a preventive restorative approach in the primary dentition Sara L Hughes Emma J Hingston Dental Update 2024 46:11, 707-709.
Authors
Sara LHughes
BDS, MFDS RCS(Glasg)
Dental Foundation Trainee Year 2, Unit of Paediatric Dentistry, University Dental Hospital, Cardiff, UK
Consultant and Honorary Senior Lecturer in Paediatric Dentistry, Unit of Paediatric Dentistry, University Dental Hospital, Heath Park, Cardiff, CF14 4XY, UK
Patients with Hereditary Hypophosphatemic Rickets (HHR) commonly present with spontaneous dental abscesses and are often faced with a life-long burden of multiple endodontic treatments and/or extractions. Early diagnosis and management is essential to reduce risk of infection and maintain function and wellbeing. This is a case report of a 3-year-old boy with HHR, who presented with recurrent spontaneous abscesses in multiple primary teeth that subsequently required extraction. A preventive/restorative approach to protect the unaffected primary teeth is described and demonstrates how early clinical intervention can result in a symptom- and disease-free patient one year following treatment.
CPD/Clinical Relevance: Oral manifestations are common in patients with HHR and early intervention by the clinician can contribute to reducing the risk of future complications.
Article
A 3-year-old boy, AL, was referred to the Unit of Paediatric Dentistry, at the University Dental Hospital Cardiff, by a consultant oral surgeon regarding recurrent buccal swellings in the region of the LRB and LRC. The patient had originally been referred to the Department of Oral and Maxillofacial Surgery, at Prince Charles Hospital, Merthyr, by the general dental practitioner (GDP). Medically, AL had been diagnosed with Hereditary Hypophosphatemic Rickets (HHR), with X-linked dominant transmission, at the age of one year and was under the care of a paediatric endocrinologist, an orthopaedic surgeon and a consultant nephrologist.
Family members affected by the condition included the patient's mother and maternal grandfather, who had two affected sisters. The condition was known to affect two further generations beyond that.
At AL's initial appointment, his parents reported recurrent gingival swellings since the age of 2½ years that had necessitated the use of antibiotics. On extra-oral examination, the patient was of small stature with bowed legs (Figure 1).
Register now to continue reading
Thank you for visiting Dental Update and reading some of our resources. To read more, please register today. You’ll enjoy the following great benefits: