J Korean Acad Pediatr Dent.  2018 May;45(2):257-263. 10.5933/JKAPD.2018.45.2.257.

Dental Management in a Patient with Infantile Osteopetrosis : A Case Report with a 7-Year follow-up

• Cheon M
• Yang S
• Kim J
• Choi N
• Kim S

• KMID: 2430485
• DOI: http://doi.org/10.5933/JKAPD.2018.45.2.257

Abstract

Osteopetrosis is characterized by impaired osteoclast function and increased bone density. Infantile osteopetrosis is a severe form of the disease and has characteristics such as diffusely sclerotic skeleton, pancytopenia, cranial nerve entrapment, infection susceptibility, and abnormal craniofacial appearance. Patients with infantile osteopetrosis often experience developmental delay, and may have a short life span. A 14-month-old girl with osteopetrosis presented to the department of pediatric dentistry. Incipient caries on deciduous incisors were observed. The patient revisited 4 years of age. Besides medical problems, oral complications such as growth retardation, narrow upper arch, crowding, dental caries, and abnormal tooth development were observed. After consultation with her pediatrician, dental treatments were performed on the deciduous molars under sedation after a prophylactic antibiotic injection. At a periodic follow-up, multiple deciduous teeth were treated and extracted, and oral-rehabilitation with a removable partial denture was initiated. Patient with osteopetrosis are highly susceptible to infection because of their compromised immune system and problems associated with wound healing that lead to osteomyelitis or sepsis development. Active participation in dental care for sugar intake management and proper oral hygiene are obligatory.

Keyword

Infantile osteopetrosis; Oral complication; Dental management; Oral rehabilitation

MeSH Terms

Bone Density
Cranial Nerves
Crowding
Dental Care
Dental Caries
Denture, Partial, Removable
Female
Follow-Up Studies*
Humans
Immune System
Incisor
Infant
Molar
Oral Hygiene
Osteoclasts
Osteomyelitis
Osteopetrosis*
Pancytopenia
Pediatric Dentistry
Sepsis
Skeleton
Tooth
Tooth, Deciduous
Wound Healing