[Larsen syndrome: two reports of cases with spinal cord compromise].
BACKGROUND
Larsen syndrome is characterised by untreatable congenital dislocation of multiple body joints, along with marked foot deformities. These patients have a flattened face with a short nose, a broad depressed nasal bridge and a prominent forehead. In this clinical note our aim is to report two cases that coursed with spinal cord compromise.


METHODS
Case 1: an 18-month-old female with congenital dislocation of knees and hips. The patient had a flat face, sunken root of nose, and carp mouth. Magnetic resonance imaging of the spine showed severe cervical kyphosis secondary to malformation and hypoplasia of the cervical vertebral bodies and important compression of the spinal cord. Clinically, there were also signs of upper motor neuron syndrome, which was especially prominent in the lower limbs. Case 2: a 14-year-old male with a characteristic face and dislocation of the head of the radius. The patient presented amyotrophy of the muscles in the right hand and clinical signs of lower motor neuron syndrome due to neuronal damage secondary to spinal malformations.


CONCLUSIONS
Larsen syndrome is an infrequent osteochondrodysplasia. Alteration of the spine is common and may give rise to spinal cord compression with varying clinical repercussions which require surgical treatment in the early years of the patient's life.
Martín Fernández-Mayoralas
D
D
Sección de Neuropediatría, Hospital de la Zarzuela, Hospital Clínico San Carlos, Madrid, España. dmfmayor@yahoo.es
Fernández-Jaén
A
A
Muñoz-Jareño
N
N
Calleja-Pérez
B
B
San Antonio-Arce
V
V
Martínez-Boniche
H
H
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D000293:Adolescent; D005147:Facial Bones / Q000002:abnormalities*; D005260:Female; D005532:Foot Deformities, Congenital / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D004204:Joint Dislocations / Q000151:congenital*; D008297:Male; D013117:Spinal Cord Compression / Q000175:diagnosis*; D013577:Syndrome