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Radiological Spectrum of Congenital Spine Abnormalities 2008

General Spine

Razia Rehmani, MD,
Steven Lev, MD, Non ASSR Member
Jim Ferreti, MD, Non ASSR Member
Kadakia Satish, MD, Non ASSR Member
Margarita Revzin, MD, Non ASSR Member
Devorra Balsad: Nm, MD, Non ASSR Member

Scientific Poster

Exhibit Panels: 3

Purpose

To demonstrate the broad range of the developmental spinal disorders. Our classification is based on an understanding of embryology as well as a consideration of pertinent clinical criteria. We underscore the key radiological findings with simplified diagrams to help distinguish these often similar appearing entities. We also review the goals of therapy.

Methods & Materials

We retrospectively reviewed the imaging findings (plain films, ultrasound, MDCT and MRI) of patients with developmental spinal pathologies at our institution. We categorized these disorders, when possible, according to abnormalities at specific stages of embryological differentiation. Important clinical signs such as whether the malformation was open or closed, were also noted.

Results

Notochordal anomalies include neurenteric cyst and diastematomyelia, both with possible associated vertebral anomalies. Our diastatomyelia cases include cord tethering and both fibrous and bony spurs. Arachnoid cyst and epidermoid may mimic neurenteric cyst, and may be associated with bony remodeling. Another cystic lesion we display for comparison is an unusual case of a degenerating schwanomma. Neurulation disorders are of two types - premature dysjunction, where the neural tube separates from the overlying ectoderm early, and nondysjunction. The former contain fat, such as lipomyelomeningocoele (closed defect with a subcutaneous mass), intradural lipoma and dermoid. The latter may have ectodermal tracts and include myelocoele and myelomenigocoele (both open defects). Caudal cell mass anomalies include terminal myelocystocoele, anterior sacral meningocoele and sacrococcygeal teratoma. Bony disorders include those of vertebral formation (hemivertebrae) and segmentation failure (block vertebra and Klippel-Feil Syndrome). The radiologist should search for associated neural and visceral malformations. Scoliosis requires exclusion of underlying causes such as neoplasm or infection. Disorders of dural expansion include syndromes such as Marfans and Neurofibromatosis. We present several examples illustrating the spectrum of NF1, including a remarkable case of a large plexiform multi-level tumor. NF2 may have multiple inherited tumors (MISME). One case exhibits a bizarre lobulated intraspinal schwanomma. Normal variants include filum fibrolipoma, with no associated cord tethering, ventriculus terminalis and conjoined nerve roots. Both prenatal and postnatal ultrasound is the initial imaging modality used for detection and evaluation. Conventional radiography can help target spinal canal enlargement, interpedicular widening and vertebral anomalies. MDCT, with its capability to provide rapid sagittal and coronal 2D reformatted images and 3D reconstructions, is an essential visual aide and can assist in surgical planning. MR is critical in depicting associated nonosseous abnormalities, such as tethered cord and sinus tracts. Early therapy, such as cord untethering, closure and repair of open spinal dysraphisms and correction of scoliosis is imperative in preventing progressive neurological impairment.

Conclusion

Congenital disorders of the spine are vast and complex. A logical three-tiered approach based on imaging findings, embryology and clinical observations is crucial in sifting through these diverse entities.

References

1) Osborn A, Blaser S, Salzman K, et al. Neurenteric cyst. Diagnostic Imaging: Brain. Amirsys 2004;I-7:40-41
2) Barkovich AJ, Naidich TP. Congenital anomalies of the spine. In: Norman D, eds. Contemporary neuroimaging. Vol 1, Pediatric neuroimaging. 2nd ed. New York, NY: Raven, 1995; 477-540.
3) Byrd SE, Darling CF, McLone DG. Developmental disorders of the pediatric spine. Radiol Clin North Am 1991; 29:711-752
4)Naidich TP, Zimmerman RA, McLone DG, Raybaud CA, Altman NR, Braffman BH. Congenital anomalies of the spine and spinal cord. In: Atlas SW, eds. Magnetic resonance imaging of the brain and spine. 2nd ed. New York, NY: Lippincott-Raven, 1996; 1265-1338.
5)Zieger M, Dorr U, Schulz RD. Pediatric spinal sonography. II. Malformations and mass lesions. Pediatr Radiol 1988; 18:105-111.
6)Kriss VM, Kriss TC, Desai NS, Warf BC. Occult spinal dysraphism in the infant. Clin Pediatr 1995; 34:650-654.
7)Pediatric Neuroradiology, Brain. Head, Neck and Spine.
Tortori-Donati, Paolo, Rossi, Andrea, AJNR Am. J. Neuroradiol., Aug 2007; 28: 1420 - 1421.

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