Pediatric Spinal Anomalies

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1 Department of Radiology University of California San Diego Pediatric Spinal Anomalies John R. Hesselink, M.D.

2 Spine Embryogenesis 1. Primitive streak 2. Proliferation of cells at primitive pit (Hensen's node) 3. Cells enter pit & migrate cephalad to form notochordal process 4. Notochord induces overlying neural plate Barkovich, Pediatric Neuroimaging Raven Press, New York, 1995

3 Formation of the Spinal Cord Neurulation Canalization & retrogressive differentiation

4 Formation of the Vertebral Column Membrane development Chondrification Ossification

5 Normal Pediatric Spine Stage I: Birth to 1 Month Cartilage Ossified vertebra Disk Vertebra Disk T1-weighted image T2-weighted image

6 Normal Pediatric Spine Stage II: 1 Month to 6 Months Disk Vertebra Vertebra Disk T1-weighted image T2-weighted image

7 Spine Anomalies Embryogenesis Split Notochord from endodermectoderm adhesion Neural plate Neural crest Cutaneous ectoderm Neural ectoderm Notochord Formation of neural folds & groove Premature Dysjunction Neurulation Non-Dysjunction Dysjunction

8 Anomalies of Notochord Formation Adhesion between endoderm & ectoderm splits notochord Diastematomyelia Split notochord syndrome Dorsal enteric anomalies (fistula, sinus, cyst)

9 Dorsal Enteric Cyst

10 Spine Development Premature Dysjunction Lipomyelo- Lipomyelocele Intradural meningocele lipoma

11 Anomalies of Premature Dysjunction Intradural Lipoma Lipomyelocele

12 Spine Development Non-Dysjunction Myelocele Myelomeningocele Dorsal dermal sinus Hemimyelocele Chiari II Myelocele Myelomeningocele

13 624 History: Newborn male with a mass on the lower back

14 Dx: Myelomeningocele {Page 2}

15 1 Myelomeningocele 2 3

16 Postoperative myelomeningocele with clinical deterioration Postop hematoma Postop infection Residual or recurrent tumor Cord ischemia/infarction Myelomalacia Arachnoid cyst Diastematomyelia Syringohydromyelia Cord retethering

17 Dorsal Dermal Sinus A disorder of nondysjunction Dorsal opening in a hyperpigmented patch or a hairy nevus Terminates in spinal canal (extradural or intradural) Associated with epidermoid or dermoid in 50% May have spina bifida History of meningitis

18 1 Dorsal dermal sinus with dermoid/epidermoid tumor 2 3

19 History: 9 y/o boy with a lumbar nevus & brisk leg reflexes 307

20 Lumbar nevus {Page 2}

21 {Page 3} Dx: Dorsal dermal sinus with an intradural epidermoid

22 Hemimyelocele 1 2

23 {Page 2}

24 History: One day old boy born with an occipital mass 39

25 {Page 2} Dx: Chiari III - occipital meningoencephalocele - small posterior fossa - low tonsils, culpocephaly - scalloped clivus, beaked tectum - stenogyria

26 Lower Spine Development Canalization Caudal cell mass forms Microcysts coalesce to form neural tube Retrogressive Differentiation Neural tube decreases in size Neural tube forms caudal conus medullaris & filum terminale

27 Spine Development Caudal Cell Mass Anomalies Fibrolipomas of filum terminale Tight filum terminale syndrome Caudal regression Anterior sacral meningoceles Sacrococcygeal teratomas

28 Fibrolipoma of the Filum Terminale

29 Congenital scoliosis secondary to tethered cord

30 Spinal Dysraphism Critical Neural Features Position of conus Location of neural placode Fibromuscular tethering bands Ventral & dorsal nerve roots Hydrosyringomyelia

31 Tethered Spinal Cord Associated Anomalies Myelomeningocele Lipomyeloschisis Diastematomyelia Dorsal dermal sinus Caudal regression

32 Caudal Regression Syndrome

33 Anterior Sacral Meningocele

34 Sacrococcygeal Teratoma

35 Spinal Anomalies of Unknown Origin Myelocystocele Simple meningoceles Lateral meningoceles Syringohydromyelia

36 Myelocystocele

37 History: 67 y/o male with NF1 408

38 Dx: Meningocele {Page 2}

39 Postoperative Myelocystocele

40 Syringohydromyelia Etiology Chiari I 42% 28% 15% Post-Traumatic 15% Idiopathic Neoplastic

41 Chiari I with Syringohydromyelia

42 History: 22 y/o male with brain stem dysfunction Dx: Chiari I malformation with syringohydromyelia 339

43 History: 45 y.o. male with a progressive myelopathy C1 C5 C6 101

44 Myelopathy {Page 2} T5 T6 T9

45 Spinal Cord Cysts Evidence for Benign Syrinx Fluid isointense to CSF Smooth, well-defined internal margins Thinned adjacent parenchyma Cord atrophy No contrast enhancement Presence of Chiari malformation