Absent congenital pedicle syndrome is a posterior arch defect characterized by a host of congenital and mechanical abnormalities that result from disconnection of the anterior and posterior columns of the spinal canal. Absent congenital pedicle syndrome is a rare anomaly that is most commonly diagnosed either incidentally, after evaluation of minor trauma, or after complaints of chronic neck pain.

A 32 year old female with a history of systemic lupus erythematous presented to the Neurosurgery Service with progressive weakness in her upper and lower extremities, clonus and hyperreflexia. MRI revealed congenital absence of the pedicles of C2, C3, C4, C5, and C6 with congenitally narrow canal at C4-5. The patient underwent a staged anterior and posterior cervical decompression and fusion. Postoperatively, she was placed in a halo and at one year follow up she was ambulatory with demonstrated improvement in her weakness and fusion of her cervical spine.

Absent congenital pedicle syndrome is rare with the majority of reported cases treated conservatively. Surgical management is a treatment option reserved for patients with myelopathy or instability 1).

Goodwin CR, Desai A, Khattab M, Elder BD, Bydon A, Wolinsky JP. Cervical Fusion for Absent Pedicle Syndrome presenting with Myelopathy. World Neurosurg. 2015 Sep 16. pii: S1878-8750(15)01180-8. doi: 10.1016/j.wneu.2015.09.017. [Epub ahead of print] PubMed PMID: 26386456.
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