Severe mid-cervical kyphosis with cord compression in Larsen's syndrome and diastrophic dysplasia: unrelated syndromes with similar radiologic findings and neurosurgical implications.
Mid-cervical kyphosis in diastrophic dysplasia (DD) with cord compression and weakness has been recognized for the last three decades. A strikingly similar situation exists in Larsen's syndrome (LS), an otherwise unrelated problem of weakness with lay joints and dislocations with typical facies. Forty patients with DD were studied of whom seven had cervical spine kyphosis: one patient had spontaneous correction over the ensuing two decades. Seven patients with LS were studied of whom four had cervical spine changes with cord compression. There was one sudden death among the patients with LS and no cases of spontaneous correction. Muscle weakness in DD and LS should lead to evaluation of cervical spine kyphosis with cord compression. Plain films, CT and recently MRI are useful.
Forese
L L
LL
Department of Orthopaedic Surgery, Columbia-Presbyterian Medical Center, New York, USA.
Berdon
W E
WE
Harcke
H T
HT
Wagner
M L
ML
Lachman
R
R
Chorney
G S
GS
Roye
D P
DP
D002574:Cervical Vertebrae / Q000000981:diagnostic imaging / Q000473:pathology; D002675:Child, Preschool; D005147:Facial Bones / Q000002:abnormalities; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007592:Joint Diseases / Q000150:complications / Q000151:congenital*; D004204:Joint Dislocations / Q000150:complications / Q000151:congenital*; D007738:Kyphosis / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D010009:Osteochondrodysplasias / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D011859:Radiography; D013117:Spinal Cord Compression / Q000209:etiology*; D013577:Syndrome