Interdisciplinary Treatment of a Young Patient with Ectodermal Dysplasia with the Aid of Mini-Implants for Orthodontic Anchorage

Special Article - Malocclusion

Austin Dent Sci. 2018; 3(1): 1015.

Interdisciplinary Treatment of a Young Patient with Ectodermal Dysplasia with the Aid of Mini-Implants for Orthodontic Anchorage

Kolokitha OE1*, Chatzistavrou E2 and Kotsiomiti E3

¹Department of Orthodontics, School of Dentistry, Aristotle University of Thessaloniki, Greece

²Private Practice, Thessaloniki, Greece

³Department of Removable Prosthodontics, School of Dentistry, Aristotle University of Thessaloniki, Greece

*Corresponding author: Olga-Elpis Kolokitha, Department of Orthodontics, School of Dentistry, Aristotle University of Thessaloniki, GR-54124, Thessaloniki, Greece

Received: January 01, 2018; Accepted: February 13, 2018; Published: February 20, 2018

Abstract

Ectodermal Dysplasia (ED) is a rare congenital disease affecting several ectodermal structures such as skin, hair, nail, glands, and teeth. Oligodontia constitutes a problem for the young patient since it affects its functional, esthetic, psychological and social well-being. This clinical report presents the implementation of mini-implants for skeletal anchorage in a 15-year old boy with the characteristic features of ED, and with only 8 permanent teeth present. Missing multiple permanent teeth complicates a usual orthodontic treatment. However, with the aid of mini-implants, Orthodontics assisted in the alignment, levelling and improvement of the bite of the existing teeth, to produce a preprosthetic distribution of the available teeth as possible. A multi-disciplinary approach on behalf of the dental specialists contributed to the improvement of the patient’s needs such as mastication, speech, esthetics, as well as selfesteem. The interdisciplinary coordinated treatment proved to be very effective for the benefit of the patient.

Keywords: Ectodermal dysplasia; Oligodontia; Orthodontic anchorage; Mini-implants; Interdisciplinary management

Introduction

Ectodermal Dysplasia (ED) was first described as a syndrome in the medical literature by Thurnam [1] in 1848, when he reported two maternal first cousins with an unusual condition affecting the skin, hair and teeth. The term was coined by Weech [2] in 1929. ED is a hereditary disorder, characterized by the abnormal development of tissues of ectodermal origin, such as skin, nail, hair and teeth. The triad of nail dystrophy (onychodysplasia), alopecia or hypotrihosis (scanty, fine light hair on the scalp and eyebrows), and palmoplantar hyperkeratosis is usually accompanied by a lack of sweat glands (hypohidrosis) and a partial or complete absence of primary and/or permanent dentition [3].

Two major types of ectodermal dysplasia are described, based on the number and functioning of the sweat glands:

a) X-linked anhidrotic or hypohidrotic (HED), where sweat glands are either absent or significantly reduced in number (Christ- Siemens-Touraine syndrome) and

b) Hidrotic where sweat glands are normal and the condition is inherited as autosomal dominant (Clouston’s syndrome).

The dentition and hair are affected similarly in both types, but the hereditary patterns and nail and sweat gland manifestations tend to differ [4].

Although more than 170 conditions under the umbrella term of “ectodermal dysplasia” have been described, these disorders are considered to be relatively rare [5]. Its estimated incidence is 1:100,000 births, with a mortality rate of 28% in males by 3 years of age due to intermittent hyperthermia. ED is more common and severe in males than in females.

The different types of ectodermal dysplasia are caused by the mutation or deletion of certain genes located on different chromosomes. More specifically, mutations in the Ectodysplasin A (EDA), Ectodysplasin A Receptor (EDAR) and Ectodysplasin A Receptor Associated Death-Domain (EDARADD) genes cause HED. The EDA, EDAR and EDARADD genes provide instructions for making proteins (ectodysplasin A) that work together during embryonic development. Defective ectodysplasin A formation prevents normal interactions between the ectoderm and the mesoderm and hence impairs the normal development of hair, sweat glands and teeth [6,7].

Dental anomalies are associated with 80% of cases and may include anodontia, hypodontia, misshapen teeth, taurodontia, supernumerary teeth, neonatal teeth, natal teeth, retained primary teeth, enamel hypoplasia and lack of an alveolar ridge [8]. According to Shah and Shah (2014) [9], hypodontia resulting from ED appears to follow a definite pattern. Hypohidrotic Ectodermal Dysplasia frequently exhibits the most severe dental anomalies. The average number of missing permanent teeth is reported as 23.7 [10]. The maxillary central incisors, maxillary first molars, and maxillary canines are the teeth most often present while mandibular anterior teeth are the least likely to be present [11,12]. The maxillary central incisors and the maxillary and mandibular canines are usually peg-shaped conical in shape. The alveolar process fails to develop at edentulous sites and may be poorly developed even at dentate sites [13-16]. The deficient alveolar growth and decreased vertical dimension of occlusion results in a small lower vertical face height [17]. These features give the child a distinctly senile facial appearance similar to an edentulous patient [16].

Typically, the face of a patient with ectodermal dysplasia is characterized by square aspect of the forehead, frontal bossing, sunken cheeks, hyper pigmentation of the periorbital skin, prominent supraorbital ridges, saddle nose, large low-set ears and protuberant lips [18]. Other symptoms include usually dry, scaly, and easily irritated skin; sweat glands that may be absent or few in number or non-functioning; eyebrows, eyelashes and other body hair that may be sparse or absent; and hair that may be fragile, dry, and generally disorderly because of the lack of sebaceous glands [19]. Salivary glands, including the intraoral accessory glands are sometimes hypoplastic resulting in xerostomia and dry, cracked lips with pseudorhagades formation [20].

A young patient with ectodermal dysplasia is facing several functional, esthetic, psychosocial and psychological problems [9,16]. Tooth agenesis has been shown to have a significant impact upon the oral health related quality of life. The oral conditions imposed by this syndrome necessitate multidisciplinary dental treatment for balanced development of both the functioning and esthetic aspects of the dentition of an ED patient [21].

Dental management of ectodermal dysplasia traditionally involves the provision of a series of complete or removable partial dentures during the growing years [22-29] and definitive habilitation following completion of jaw growth [30-31]. The role of orthodontics is equally important, starting from the initial visit of the patient to the pediatric dentist. The absence of many primary and permanent teeth, combined with the underdeveloped alveolar processes, leaves the existing, usually conical in shape teeth, often with unfavourable positions (such as rotations and retroclinations) [32]. Alignment and proper positioning of the existing teeth is a prerequisite for a successful rehabilitation of the dentition. However, with the limited number of permanent teeth present, anchorage remains an issue. Mini-implants offer many opportunities in this field. It is the intent of this article to present a young patient with ectodermal dysplasia who benefited from the use of mini-implants for proper positioning of the existing teeth with the simultaneous use of removable prosthesis.

Case Presentation

A 15-year old boy with ectodermal dysplasia presented in the Graduate Orthodontic Clinic of the University with chief complaint the multiple missing teeth. He had sparse, fine silky hair, sparse eyebrows and eyelashes, dry skin, hyper pigmentation around the eyes and mouth, large low-set ears, depressed nasal bridge and protuberant lips (Figure 1a,b). The lateral cephalogram taken at the time of the initial examination showed a concave hard tissue and soft tissue profile, a posterior position of the maxilla and a forward chin position in relation to the cranial base, an increased labial inclination of the upper incisors in relation to their osseous base and the cranium (Figure 2). The intraoral examination revealed the presence of only 8 permanent teeth (#16,13,11,21,23,33,43,47) and a retained primary tooth (#62). The panoramic x-ray revealed numerous agenesis in the permanent dentition (20 permanent teeth were congenitally absent) and obvious resorption of the root of #62 (Figure 3). His maxillary incisors, already built with composite resin to camouflage their conical shape, were unaesthetically proclined with an enlarged midline diastema (5 mm) and the maxillary canines were conical and labially inclined. Intraorally, the mucosa was dry and the mandible exhibited narrow knife-edge shaped alveolar ridges (Figure 4a,b,c,d,e).