An Achondroplasic Case with Foramen Magnum Stenosis, Hydrocephaly, Cortical Atrophy, Respiratory Failure and Sympathetic Dysfunction
Küçük Resim Yok
Tarih
2012
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Kowsar Corp
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Background: Achondroplasia is a relatively frequent genetic disorder that may lead to limb weakness, motor-mental 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 infant
Açıklama
Anahtar Kelimeler
Achondroplasia, Foramen Magnum, Respiratory Depression, Sympathetic Dysfunction
Kaynak
Iranian Journal of Pediatrics
WoS Q Değeri
N/A
Scopus Q Değeri
Q3
Cilt
22
Sayı
1
