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Very rare congenital inherited disorder that combines cataract
and dental anomalies.
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Mesiodens-Cataract Syndrome; Cataract-Dental Syndrome;
X-Linked Cataract-Hutchinsonian Theet Syndrome.
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Several kindreds have been described since this
condition was defined in 1974. It is fully expressed in Wales only.
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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).
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Congenital cataracts with impaired vision, extra
incisors, anteverted pinnae, short fourth metacarpals, and variable
anomalies. Mental deficiency is present in some patients.
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Typical features of the disease are cataracts and dental
anomalies; worse in related males than in females.
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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.
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No literature about this condition
and anesthesia. Patients may be blind or mentally handicapped.
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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.
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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]