Extremely rare syndrome with dextrocardia and facial,
ophthalmologic, and neurologic abnormalities.
Dextrocardia with Unusual Facies and Microphthalmia
Two presumably unrelated Arab
children, both born to consanguineous parents, have been described,
suggesting that autosomal recessive transmission is most likely.
The two children had dextrocardia associated with
sloping forehead, microphthalmia, prominent nose, micrognathia, and plantar
folding. One child had a cleft palate, mental retardation, and
choreoathetosis; the other child had vertebral fusion defects and
Prior to anesthesia, associated
malformations must be assessed. Difficult tracheal intubation should be
expected secondary to the described malformations. Adequate anxiolysis often
is helpful in the management of mentally retarded patients.
Goldenhar Syndrome: Common birth defect of vascular origin
involving first and second branchial arch derivatives, resulting mainly in
facial and vertebral anomalies.
Aughton DJ: New syndrome? Clinical anophthalmia, dextrocardia, and
skeletal anomalies in an infant born to consanguineous parents. Am J Med Genet
Nachlieli T, Gershoni-Baruch R: Dextrocardia, microphthalmia, cleft palate,
choreoathetosis, and mental retardation in an infant born to consanguineous
parents. Am J Med Genet