Dental Concerns of Children with Cystic Fibrosis – An Overview

Abstract

Cystic fibrosis (CF) is an autosomal recessive disorder occurring in one of every 2000 births. It is the most common genetic disorder affecting whites. The genetically altered protein affects exocrine gland function which leads to micro obstruction of the pancreas, which results in cystic degeneration of the pancreas and ultimately a digestive enzyme deficiency producing malabsorption of nutrients. These children have increased chest diameter, clubbing of fingers and toes, chronic productive cough, decreased exercise tolerance. Cystic fibrosis related diabetes is more common with high incidence of tooth discoloration, low incidence of dental caries, high incidence of mouth breathing and open bite malocclusion associated with chronic nasal and sinus obstruction. It should be preferred to treat in a more upright position to allow them to clear secretions more easily. Extractions can be carried out under local anesthesia. Use of sedative agents that interfere with pulmonary function should be avoided. Along with possible etiological factors, clinical features, diagnosis, tests for CF and treatment options for discoloration of the teeth are discussed.

Effects of cystic fibrosis on the oral cavity

“…The effects on the tooth germ in the so-called pre-eruptive phase result in enamel defects [3]. This term describes a series of enamel appearances whose structure is disturbed during its developmental stages [4]. The wide variety of factors that can affect the developmental process of dental hard tissues means that enamel and dentin defects can present a wide range of clinical features [5]: Disorders in the early stages of enamel development are suggestive of enamel hypoplasia [4], which is a quantitative defect involving the partial or total absence of enamel, it presents as pits, hollows or grooves [6].…”

“…This term describes a series of enamel appearances whose structure is disturbed during its developmental stages [4]. The wide variety of factors that can affect the developmental process of dental hard tissues means that enamel and dentin defects can present a wide range of clinical features [5]: Disorders in the early stages of enamel development are suggestive of enamel hypoplasia [4], which is a quantitative defect involving the partial or total absence of enamel, it presents as pits, hollows or grooves [6]. On the other hand, disorders occurring during the calcification or maturation stage can cause hypomineralization [4], which is a qualitative defect characterized by a white or discolored area with a smooth surface and normal enamel thickness [6].…”

“…CFTR dysfunction in cystic fibrosis patients affects hydration and mucociliary transport in all exocrine glands, including the salivary glands [29] [13] [11], it makes their respective secretions more viscous than under healthy conditions [4]. Salivary gland hypofunction alters the flow and composition of saliva, which may affect nutritional intake, social interaction, and quality of life [40].…”

“…Salivary gland hypofunction alters the flow and composition of saliva, which may affect nutritional intake, social interaction, and quality of life [40]. The submandibular gland may be enlarged; secretion from the sublingual gland is thick and tenacious; The parotid gland, which is not a mucus-secreting gland, may have increased flow with high electrolyte content [4].…”

“…Cystic fibrosis patients often breathe through the mouth, due to frequent upper respiratory tract infections, which promotes malocclusions and predisposes to periodontitis and oral mucosal inflammation [4].…”