Guideline on Oral Health Care/Dental Management of heritable Dental Developmental Anomalies

Meghan Sullivan Walsh March 29, 2011

Literature Review - St. Joseph/LMC Pediatric Dentistry

Guideline on Oral Health Care/Dental Management of heritable Dental Developmental Anomalies

Resident: Meghan Sullivan Walsh

Program: Lutheran Medical Center- Providence

Article Title: Guideline on Oral Health Care/Dental Management of Heritable Dental developmental Anomalies

Authors: Council on Clinical Affairs

Journal: Pediatric Dentistry Reference Manual

Volume (number), Year, Page #’s; V32/NO 6 10/11, 226-231

Major Topic: Guidelines addressing the diagnosis, principles of management,and objectives of therapy of children with heritable dental developmental anomalies. Focus on Amelogenesis Imperfecta, Dentinogenesis Imperfecta and Dentin Dysplasia.

Overview of Method of Research: Electronic MEDLINE search of medical literature related to dental developmental anomalies.


A disruption during histodifferentiation, apposition and mineralization can result in enamel hypoplasia, AI, DI and DD. These dental anomalies can have a profound affect in the individual regarding esthetics, self esteem, mastication, sensitivity, finances and dental treatment. These guidelines are focused on the diagnosis and management of three dental anomalies AI, DI and DD.

Amelogenesis Imperfecta:

-Developmental disturbance that effects normal enamel formation.

-Affects nearly all permanent and primary dentition.

-Estimated frequency of 1:7,000

-May be x-linked recessive, autosomal dominant or recessive or sporadic inheritance.

Clinical Manifestation:

-Accelerated or late tooth eruption

-Enlarged follicles

-Impacted permanent teeth

-Ectopic eruption

-Agenesis of second molars

-Anterior open bite

4 types:

Hypocalcified - normal thickness, smooth surface, less hardness

Hypoplastic pitted -normal thickness, pitted surface, normal hardness

Hypoplastic generalized - reduced thickness, smooth surface, normal hardness

Hypomaturation -normal thickness, chipped surface, less hardness, opaque white coloration.


Timely intervention is critical to prevent potential disfiguring conditions. Preventative care includes early diagnosis, regular periodic examinations, meticulous hygiene, oral rinses and application of fluoride or desensitizing agents. Restorative care will depend on the amount of affected enamel and dentin. Intact enamel can be treated by bleaching or microabrasion. Hypocalcified enamel may require composites, veneers or full coverage restorations. In primary dentition it is essential to maintain adequate arch length which may require composites or full coverage SSC’s. Permanent dentition involves complex treatment with multidisciplinary specialists. Behavior guidance as well as the psychological health of the patient will need to be addressed and managed if necessary.

Dentinogenesis Imperfecta:

-Hereditary developmental disturbance

-May be seen alone or in conjunction with osteogenesis imperfecta

-Frequency of 1 in 8,000

-DI type one associated with COL1A1 and COL1A2 genes

-DI type II and Type III are autosomal dominant linked to 4q12-21 chromosome

Clinical Manifestation:

-Variable blue-gray to yellow-brown discoloration

-opalescent enamel

-frequent enamel fractures with rapid wear and attrition

Three types:

Sheilds Type I - occurs with OI. All teeth in permanent and primary are affected. Bulbous crowns, cervical constriction, thin roots, early obliteration of root canal and pulp chambers. Periapical radioleucencies are common.

Sheilds Type II -also known as hereditary opalescent dentin. Primary and permanent teeth are equally affected. Same radiographic affects as Type I.

Sheilds Type III - Rare. Bell-shaped crowns, shell-like appearance with multiple pulp exposures. Normal thickness of enamel, thin dentin and enlarged pulps.


Prevention of attrition and rapid wear of dentin is crucial. Early identification and prevention including regular examinations, meticulous hygiene, fluoride and desensitizing agents. Routine restorative treatment in mild to moderate cases with full coverage restorations in severe primary and permanent dentition. Success of full coverage crowns dependent on shape of dentition hoping to minimize cervical fracture. Stabilization and/or replacement of vertical dimension is common. Multiple periapical abecesses may require endodontic therapy included apical surgery. Multidisciplinary approach is essential.

Dentin Dysplasia:

-Autosomal dominant pattern of inheritance

-Affects 1:100,000

Two types

Dentin Dysplasia Type I - (Radicular Dentin Dysplasia: Rootless Teeth)

Normal size crowns in permanent and primary dentition. Amber translucency. Short and constricted roots. Primary teeth have obliterated pulps that fill in prior to eruption. Multiple periapical radioleucencies.

Dentin Dysplasia Type II - (Coronal Dentin Dysplasia)

Normal root lengths. Primary teeth are amber colored with bulbous crowns, cervical constriction, thin roots and early pulp obliteration. Permanent teeth have normal coloration with thistle-tube shaped pulp chambers and multiple pulp stones.


Prevention of attrition, optimizing esthetics and preventing caries and periodontal disease are of utmost importance. Early identification and prevention including examinations, meticulous hygiene, oral rinses, fluoride and desensitizing agents. Type I may require prosthetic replacements while Type II may be restored with full coverage restorations. Care should be taken for even shallow restorations which could lead to pulpal exposures. Endodontic therapy is guided by root length.

Key Points: Summary:

Early diagnosis, management and prevention are key towards a successful treatment in the above developmental anomalies. Each case is dependent on the individuals classification and clinical manifestations.

Assessment of the Article: Great review of three common developmental anomalies. In all three anomalies mentioned, primary teeth are affected and early diagnosis, management and treatment will be essential for us a pediatric dentist to establish long term success for our patients.