Article

Cervico-oculo-acoustic (Wildervanck) syndrome: clinicoradiological findings.

Department of Dentistry, Jordanian Royal Medical Services, Al-Huson, Irbid, Jordan.
Case Reports 04/2013; 2013. DOI: 10.1136/bcr-2013-009065
Source: PubMed

ABSTRACT We describe a girl presented with facial asymmetry and oral mucosal cleft and with further investigations other anomalies were found including hearing loss, Duane syndrome, Klippel-Feil anomaly, Chiari malformation and accessory bone mass in mandibular ramus leading to the clinical diagnosis of cervico-oculo-acoustic (Wildervanck) syndrome (COAS). The patient underwent surgical occipital decompression by preforming suboccipital craniectomy and C1 posterior laminectomy to relieve the cerebellar tonsillar herniation. Surgical removal of mandibular bony mass was done and the patient is now under orthodontic treatment to correct facial asymmetry and malocclusion.

Download full-text

Full-text

Available from: Farouk B Rihani, Jan 04, 2014
0 Followers
 · 
86 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Cervico-oculo-acoustic (COA) or Wildervanck syndrome is characterized by the triad of Klippel-Feil anomaly, bilateral abducens palsy with retracted bulbs (Duane 'syndrome') and hearing loss. The clinical findings of this syndrome have been well documented. A few case reports with MRI findings have appeared in the literature showing brainstem and cerebellar hypoplasia and vertebral segmentation anomalies. Our case is unique in that diastematomyelia of the lower medulla and cervical cord was accompanied by vermian hypoplasia, tonsillar herniation and resulting triventricular hydrocephalus in a child with Wildervanck syndrome. This case is presented with MR images. Children with Wildervanck syndrome should be investigated for craniospinal abnormalities with MR imaging.
    Clinical Dysmorphology 05/2002; 11(2):125-8. DOI:10.1097/00019605-200204000-00010 · 0.42 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: We present here the case of a 12-year-old boy who had Klippel-Feil syndrome with renal, cardiac and multiple skeletal anomalies, and we show the relevent three-dimensional computed tomography images. Our patient had a triple renal pelvis, mitral valve prolapsus, multiple cervical vertebrae fusions, cervical ribs, hypoplasia of the right thumb, spina bifida of L5, lumbalization at the right side of S1 and a sacral curved defect. In this study, we discuss the atypical clinical features and the diagnostic value of three-dimensional CT for evaluating the skeletal anomalies of the Klippel-Feil syndrome cases.
    Korean Journal of Radiology 12/2005; 6(4):278-81. DOI:10.3348/kjr.2005.6.4.278 · 1.81 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: The Klippel-Feil syndrome is of special interest to those concerned with cleft palate for the following reasons: (1) Cleft palate is a commonly associated finding: (2) malformations of the cervical vertebrae, in the absence of other stigmata of the syndrome, are a common finding in cleft palate; (3) anomalies of the upper cervical column and cranial base can impede velopharyngeal valving; (4) hearing loss is a common finding in the syndrome irrespective of the presence or absence of cleft palate; (5) cervical anomalies may complicate endotracheal intubation or head extension during pharyngeal surgery, and (5) the short neck may be the primary defect that impedes palatal fusion. This report reviews the literature on 339 patients and seven new cases in an effort to catalog the cranial and extracranial malformations associated with the syndrome and to consider the mode of genetic transmission.
    The Cleft palate journal 02/1980; 17(1):65-88.