Ectrodactyly-ectodermal dysplasia-clefting syndrome: the oral hygiene management of a patient with EEC

Abstract

Ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC) is a rare genetic disorder. EEC was first described in 1970 and occurs in 7 of 10,000 people. This paper discusses the effects of EEC and the dental complications typically associated with this syndrome. It is important for dental practitioners to be aware of EEC due to the number of dental conditions associated with it. A case report is presented to discuss the dental management, especially issues related to oral hygiene, of a patient who had EEC.

Ectrodactyly, Ectodermal Dysplasia, Cleft Lip, and Palate (EEC Syndrome) with Tetralogy of Fallot: A Very Rare Combination

“…Orthopedic management in form of limb reconstructive surgery may be considered in patients that are having functional disability such as ectrodactyly, syndactyly, cleft lip, or palate (33). If teeth are missing, artificial dentures may be necessary and oral hygiene makes an important part of management (34). Use of artificial tears and emollients may be necessary for ectodermal dysplasia for prevention of evaporative eye loss (35).…”

Prosthetic Rehabilitation in Children: An Alternative Clinical Technique

“…Conventional removable complete or partial dentures are usually the treatment of choice for these patients [2, 6]. Providing early prosthetic care for very young completely edentulous patients seems advisable.…”

“…Several treatment strategies have been reported for the management of patients suffering from anodontia, oligodontia, and malformed teeth. Conventional removable complete or partial dentures are usually the most frequent prosthetic treatment plan for these young patients [3–6]. …”

A child with EEC syndrome — a 10 years follow-up

“…Buss et al in their studies diagnosed this type of facial cleft in almost 21% of EEC patients [7]; Yin et al observed both cleft lip and palate in approximately 40% of EEC patients [8]; and Brunner et al reported cleft of the lip with or without cleft of the palate in patients with EEC syndrome [9]. Cleft of the palate coexistent with xerostomy, angular cheilitis, dental hypoplasia, and oligodontia require thorough oral hygienic procedures performed on the patient, and this is possible only if the patient has certain degree of motor and mental fitness [6,10]. Hand malformation is not a significant limitation of manual skill.…”