A rare autosomal dominant ectodermal dysplasia
characterized by defects of the nail plates of the fingers (onychorrhexis)
and toes (koilonychia). Familial hypodontia with normal hair and sweat gland
Witkop Syndrome; Dysplasia of Nails with Hypotonia.
Described by C.J. Witkop in 1965.
Condition is frequent among Dutch Mennonites in Canada.
Autosomal dominant. Mapping found linkage in
the region of the MSX1 locus.
In a model of MSX1-deficient mice, histologic
analysis confirmed that the MSX1 expression in mesenchyme of developing nail
beds showed thinner nail and tooth development disrupted.
Nail dysplasia, hypoplastic dentition, normal hair,
normal sweat and salivary gland function.
A variable number of teeth may be hypoplastic,
the permanent incisors and second molars are most commonly affected.
Complete adontia is rare. The nails are thin, centrally hollow, and brittle.
Bilateral polycystic ovaries have been reported with the syndrome.
Inspect dentition for teeth at risk
of being dislodged during laryngoscopy and tracheal intubation. Special investigations as
indicated by the proposed procedure.
The presence of hypodontia may be
associated with a higher risk of dental damage during direct laryngoscopy.
Otherwise, there are no specific considerations other than those relating to
the proposed surgery.
There are no known specific
implications with this condition.
Murdoch-Kinch C, Miles D, Poon C: Hypodontia and nail dysplasia syndrome.
Oral Surg Oral Med Oral Pathol