Background: Achondroplasia is a relatively frequent genetic disorder
that may lead to limb weakness, motormental retardation, hydrocephaly,
and respiratory disorders. In this pathology, foramen magnum stenosis
and accompanying disorders like respiratory depression is well
documented. Case Presentation: A 2.5 year-old child with the diagnosis
of achondroplasia admitted to our clinic with severe respiratory
depression, limb weakness, and motor mental retardation as well as
sympathetic dysfunction. In radiologic evaluations, severe foramen
magnum stenosis was detected. The patient was operated and posterior
fossa decompression was accomplished to prevent compression of
respiratory centers and neurons. Conclusion: This case is unique with
the narrowest foramen magnum reported up to date and the sympathetic
dysfunction which is not reported as a complication in achondroplasic
patients. The authors review the relevant literature, focusing on the
indications for cervicomedullary decompression in infants with
achondroplasia