CHANDS

Ectodermal dysplasia associated with ankyloblepharon. CHANDS is an acronym for curly hair, ankyloblepharon, and nail dysplasia, syndrome.

Baughman Syndrome.

Fewer than 10 cases have been described. Autosomal recessive mode of inheritance with pseudodominance has been suggested. Consanguinity seems to be a risk factor.

The main clinical signs are listed in the acronym: curly hair, ankyloblepharon (fusion of the eyelids), and nail dysplasia (hypoplastic, hyperconvex, grooved). Other signs may include abnormal gait, ataxia, speech defect, hypermetropia, and strabismus. One case also had fibrous adhesions between maxilla and mandibula at the level of the premolars, but also from the buccal mucosa to the alveolar ridges, which basically resulted in the inability to open the mouth.

In the presence of interalveolar fusion and inability to open the mouth (in the case reported by Ohishi et al., interalveolar distance was 4 mm at birth), airway management is very difficult. In this report, surgery was postponed until 1 year of age because feeding (surprisingly) was not an issue. At the time of surgery, mouth opening was 6 mm preoperatively and 12 mm after cutting of the fibrous bands. Endotracheal intubation was performed, but the technique used had not been described. Either awake fiberoptic nasal intubation or, if this option is not available, tracheotomy under local anesthesia is the technique of choice. As illustrated by this case, separation of the mandibula and maxilla does not immediately improve the situation from an anesthetic point of view because ankylosis of the temporomandibular joints is present. Other anesthetic implications are limited to eye protection (in Ohishi's report, the eyelids were separated immediately after birth by an ophthalmologist, and apparently no anesthetic was given at that time).

Ectodermal Dysplasia: A group of inherited disorders characterized by disturbances in one or more ectodermal structures and their accessory appendages. The absent or deficient function of at least two derivatives of the ectoderm constitutes a form of ED.

More than 150 different forms of ED have been described.