Nance-Horan Syndrome

Very rare congenital inherited disorder that combines cataract and dental anomalies.

Mesiodens-Cataract Syndrome; Cataract-Dental Syndrome; X-Linked Cataract-Hutchinsonian Theet Syndrome.

Several kindreds have been described since this condition was defined in 1974. It is fully expressed in Wales only.

Transmitted as an X-linked trait with tentative mapping to the short arm of chromosome X (Xp21.1-p22.3, Xp22.3-p21.1, and Xp22.3-p22.2).

Congenital cataracts with impaired vision, extra incisors, anteverted pinnae, short fourth metacarpals, and variable anomalies. Mental deficiency is present in some patients.

Typical features of the disease are cataracts and dental anomalies; worse in related males than in females.

Males have dense nuclear cataracts and microcorneas, whereas heterozygous females have posterior Y-sutural cataracts, small corneas, and only slightly reduced vision. Teeth show nonsyphilitic hutchinsonian incisors, supernumerary teeth, irregular diastema, and cone-shaped incisors. Dolichocephaly and mandibular prognathism occur, with a high nasal bridge, prominent, anteverted pinnae, and short metacarpals. Developmental delay may be seen. Patients have a marfanoid habitus, and males are often blind.

No literature about this condition and anesthesia. Patients may be blind or mentally handicapped.

Dentition may lead to difficulties in direct laryngoscopy and tracheal intubation, as may oral anomalies. Blind and/or mentally handicapped children need much reassurance. The presence of a parent and/or premedication may help when airway difficulties are not expected.

No specific interactions.