Ectodermal dysplasia combined with congenital heart
XTE-Syndrome; Moynahan Syndrome type III.
This is an extremely rare,
autosomal dominant inherited form of ectodermal dysplasia.
Characterized by xeroderma, talipes, and tooth
enamel defects. Further signs include growth and mild mental retardation,
congenital mitral stenosis, cleft palate, absent eyelashes of the lower lid,
short-lasting skin vesicles, reduced number of sweat glands associated with
hypohidrosis and increased photosensitivity. Hair, finger, and toe nails can
From an anesthetic point of view, a
preoperative echocardiography to assess cardiac function and the degree of
mitral stenosis is recommended. Depending on the procedure, patients may
need bacterial endocarditis prophylaxis. Xeroderma may also involve the
lacrimal system and care must be taken to avoid corneal ulcerations during
anesthesia. Vascular access may be challenging in the presence of the skin
changes. Environmental temperature must be well controlled to avoid hyperthermia.
In the face of decreased sweating,
atropine should not be used and hyperthermia must be avoided.
Moynahan EJ: XTE syndrome (xeroderma, talipes and enamel defect): A new
heredo-familial syndrome. Two cases. Homozygous inheritance of a dominant
gene. Proc R Soc Med