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A Case of Occipitocervical Fixation for Orthotopic Os Odontoideum in a 2-year-old Child with Progressive Floppy
Yosuke Hashimoto1, Hisaaki Uchikado1,2, Takehiro Makizono1
1Department of Neurosurgery, Kurume University School of Medicine, Asahi-machi, Kurume, Japan.
Insights
Craniocervical instability in infants, a rare cause of floppy infant syndrome, presents significant treatment challenges. Surgical intervention halted progressive paralysis in a 2-year-old diagnosed with orthotopic os odontoideum.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Developmental Pediatrics
Background:
- Craniocervical instability is a rare cause of infantile hypotonia (floppy infant syndrome).
- Treatment is complicated by underdeveloped bone structures in infants.
- Progressive neurological decline can occur if left untreated.
Abstract:
Floppy infants caused by craniocervical instability in childhood are extremely rare. Furthermore, due to underdeveloped bone structures, treatment for it is extremely difficult. The patient was a 2-year-old boy who was referred at the age of 1 year for psychomotor developmental delay. The cause was unknown, so he was placed under observation. At the age of 2 years, he remained a floppy infant and had not yet acquired motor skills, so he was referred again. Neurologically, he was in a state of progressive motor paralysis of the limbs. Magnetic resonance imaging revealed damage to the medulla oblongata, and he was diagnosed with orthotopic os odontoideum. A C1 laminectomy was performed, with posterior fixation from a C2 translaminar screw to the occipital bone. The progression of the condition has halted, and the patient is currently undergoing rehabilitation. Here, we report the pathological diagnosis, surgical treatment, and orthotopic os odontoideum timing, a condition presenting with progressive craniocervical instability in infants.
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