title: Isolated atlantal stenosis in a patient with idiopathic growth hormone deficiency, and Klippel-Feil and Duane's syndromes.
abstract: OBJECTIVE
  Isolated atlantal stenosis is very rare with less than ten cases reported, mostly in adult Asians; however, this pathology should be recognized by the neurosurgeon. An unusual case of this rare clinical entity is reported in conjunction with multiple other anomalies.
  
  
  METHODS
  We report a Caucasian girl with symptomatic stenosis of the first cervical vertebrae who presented with episodes of loss of tone with subsequent falling, facial cyanosis, urinary incontinence, hand weakness, and difficulties with swallowing. This patient also had the diagnoses of Duane's and Klippel-Feil syndromes, and idiopathic growth hormone deficiency. In addition, this patient was found to have retroflexion of the odontoid process, which further compromised the spinal canal. Flexion-extension radiographs failed to identify cervical spine instability.
  
  
  METHODS
  Following suboccipital craniectomy and the removal of the posterior arch of the atlas, the patient's symptoms were resolved and her urinary incontinence improved.
  
  
  CONCLUSIONS
  We theorize that our case represents a variant of Wildervanck syndrome. Further case reports are necessary to verify that isolated atlantal stenosis is a component of this constellation of findings. Furthermore, our case demonstrates that degenerative changes of the spine are not necessary for the production of symptoms in cases of isolated stenosis of the atlas.
authors.affiliation: Pediatric Neurosurgery, Children's Hospital, Birmingham, AL 35233, USA. richard.tubbs@ccc.uab.edu
authors.forename: R Shane
authors.initials: RS
authors.lastname: Tubbs
authors.forename: W Jerry
authors.initials: WJ
authors.lastname: Oakes
authors.forename: Jeffrey P
authors.initials: JP
authors.lastname: Blount
mesh_terms: D002648:Child; D004370:Duane Retraction Syndrome / Q000150:complications* / Q000473:pathology / Q000532:radiotherapy; D004393:Dwarfism, Pituitary / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006801:Humans; D007714:Klippel-Feil Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D011859:Radiography; D013130:Spinal Stenosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology