Poland-Moebius syndrome: a case with oral anomalies.
The combination of Moebius and Poland anomalies is rarely described in the literature. While some authors believe this association is an independent syndrome, others think that Poland, Moebius and Poland-Moebius syndromes are variations of the same condition. We report a case of Poland-Moebius syndrome in a 6-year-old girl who presented with bilateral convergent strabismus, the inability to abduct her eyes beyond the midline and brachydactyly of her right hand. Oral manifestations included incompetent lips and an abnormal tongue. Other facial features included hypoplasia of her mandible and her left ear at a slightly lower level than her right ear. Panoramic and lateral skull radiographs confirmed the absence of certain teeth and the hypoplastic mandible. The diagnosis of Poland-Moebius syndrome was made on the basis of cranial nerve involvement and oro-facial manifestations.
Domingos
A C
AC
Department of Dental and Maxillofacial Radiology, Universidade Estadual de Campinas, Piracicaba, Brazil. andreadomingos@yahoo.com.br
Lopes
S L C P
SL
Almeida
S M
SM
Boscolo
F N
FN
Whaites
E J
EJ
D000848:Anodontia / Q000209:etiology; D002648:Child; D005260:Female; D005385:Fingers / Q000002:abnormalities; D006801:Humans; D008046:Lip / Q000002:abnormalities; D008334:Mandible / Q000002:abnormalities; D020331:Mobius Syndrome / Q000150:complications*; D011045:Poland Syndrome / Q000150:complications*; D013285:Strabismus / Q000151:congenital; D014059:Tongue / Q000002:abnormalities