Skip to Main Content

++

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.

Walpole IR, Hockey A, Nicoll A: The Nance-Horan syndrome. J Med Genet 27:632, 1990.  [PubMed: 2246772]
Zhu D, Alcorn DM, Antonarakis SE, et al: Assignment of the Nance-Horan syndrome to the distal short arm of the X chromosome. Hum Genet 86:54, 1990.  [PubMed: 1979306]

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.