Pediatric dentistry plays an important part in the orthodontic treatment of patients with rare orofacial diseases. Interactions between these two disciplines are numerous and particularly noteworthy in the following pathologies:

1. cleft lip and palate;

2. ectodermal dysplasia;

3. amelogenesis imperfecta;

4. dentinogenesis imperfecta.

After reviewing the main characteristics of these pathologies, we will highlight the fundamental role of pediatric dentistry in the early diagnosis, the prevention and the dental care throughout the orthodontic treatment. A close cooperation between the orthodontist and the pediatric dentist is a key factor to a successful treatment.

This review of ectodermal dyplasias (ED) presents the particular syndromes that might present to the otolaryngologist for management and discusses the ear, nose and throat manifestations of the condition.

Hypohidrotic ectodermal dysplasia (HED) is a rare condition characterized by abnormalities to ectodermal derived tissues although other organs or systems are frequently involved. Patients with HED can have a number of symptoms that may lead them to present to the otolaryngologist. We present a case of a 37-year-old female with HED who initially presented with nasal obstruction but then very rapidly developed stridor due to a tracheal squamous cell carcinoma. We suggest a possible association between HED and carcinoma of the upper respiratory tract that has not previously been reported.

Hypohidrotic ectodermal dysplasia is an hereditary condition of the ectodermal tissues which may escape recognition because of lack of clinical awareness due to its rarity. Otorhinolaryngological features of this syndrome include chronic respiratory tract infections, persistent foul-smelling nasal discharge and crust formation, and hearing problems.

The condition is usually an X-linked recessive disease affecting mainly ectodermal tissue, although nonectodermal tissue may also be affected. The notation ‘ectodermal’ is used because ectodermal tissues are always involved. The syndrome is characterized by complete or partial absence of sweat glands, sparse hair growth, absent or deformed peg teeth, sparse sebaceous glands, occasional absence of salivary and lacrimal glands, scanty mucous glands and deficient cilia.

We describe two children with this disorder with specific reference to the effect on the upper respiratory tract.