Document Detail

Isolated atlantal stenosis in a patient with idiopathic growth hormone deficiency, and Klippel-Feil and Duane's syndromes.
MedLine Citation:
PMID:  15133703     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE AND IMPORTANCE: 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. CLINICAL PRESENTATION: 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. INTERVENTION: 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.
R Shane Tubbs; W Jerry Oakes; Jeffrey P Blount
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2004-05-07
Journal Detail:
Title:  Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery     Volume:  21     ISSN:  0256-7040     ISO Abbreviation:  Childs Nerv Syst     Publication Date:  2005 May 
Date Detail:
Created Date:  2005-04-27     Completed Date:  2005-07-05     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8503227     Medline TA:  Childs Nerv Syst     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  421-4     Citation Subset:  IM    
Pediatric Neurosurgery, Children's Hospital, Birmingham, AL 35233, USA.
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MeSH Terms
Duane Retraction Syndrome / complications*,  pathology,  radiotherapy
Dwarfism, Pituitary / complications*,  pathology,  radiography
Klippel-Feil Syndrome / complications*,  pathology,  radiography
Magnetic Resonance Imaging / methods
Spinal Stenosis / complications*,  pathology,  radiography

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