Pyknodysostosis: Report of a Rare Case and its Dental Management

AIM: This is a case report of a 16-year-old girl visiting MR Ambedkar Dental College and Hospital (Department of Pedodontics and Preventive Dentistry) for dental treatment. BACKGROUND: Osteopetrosis acroosteolytica or Toulouse-Lautrec syndrome or pyknodysostosis is a rare autosomal recessive bone dysplasia, characterized by osteosclerosis, and short stature. Montanari described a patient with an unusual variation of achondroplasia, which in retrospect was the first case of pyknodysostosis to be reported.(1) CASE DESCRIPTION: A 16-year-old girl reported to the Department of Pediatric and Preventive Dentistry with a chief complaint of pain in the lower left back region of the jaw since past 2 weeks. CONCLUSION: Pyknodysostosis is a rare condition that is diagnosed basically on its clinical and radiographic features. CLINICAL SIGNIFICANCE: It is important to recognize these features so that correct diagnosis can be made. This allows the treatment and prevention of future complications and ensures a better quality of life to the patient. HOW TO CITE THIS ARTICLE: Jawa A, Setty JV, Vijayshankar LV, et al. Pyknodysostosis: Report of a Rare Case and its Dental Management. Int J Clin Pediatr Dent 2020;13(2):192–195.