Introduction
Patients and methods
Results
No. | Age at MRI (years) | Gender | Syndrome | Complex/simple | Segmentation/formation | Deformities found on X-ray | Deformities found on MRI | Neural pathology | Neurosurgical procedures | Age at first neuro procedure (years) | Orthopedic procedures | Age at first ortho procedure (years) |
---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 9.3 | F | None | Complex | Formation | T4: hemivertebrae | T9: limbus; L4: limbus; L5: spina bifida occulta | Low-lying cord | None | None | ||
2 | 3.1 | M | None | Complex | Formation | C6–T8: complex hypoplastic deformity | None | Chiari 1; C6–T6: meningocele | None | PSIF | 3.2 | |
3 | 7.6 | M | None | Simple | Formation | L3: hemivertebrae | None | T4–L1: syrinx | None | None | ||
4 | 7.2 | F | None | Complex | Combined | T3, T6: hemivertebrae; L5: sacralization of lumbar | None | Chiari 1; C5–T2, T9–T12: syrinx; low-lying cord | None | PSIF, hemivertebrectomy | 7.1 | |
5 | 3.7 | M | None | Complex | Combined | C7, T1: butterfly; T2, T7: hemivertebrae; S1: lumbarization of the sacrum | C6, T5–T7: spina bifida occulta; T6–T8: butterfly | Fatty filum terminale without tethering | None | None | ||
6 | 1.3 | M | None | Complex | Formation | T4,T5: hemivertebrae; T6: butterfly; L3: hemivertebrae | None | L1: arachnoid cyst | None | PSIF, hemivertebrectomy | 2.4 | |
7 | 0.9 | F | None | Simple | Formation | T7, L5: hemivertebrae | None | Fatty filum terminale | None | None | ||
8 | 1.1 | M | None | Simple | Formation | T4, L2: hemivertebrae | None | Fatty filum terminale without tethering | None | Hemivertebrectomy | 4.5 | |
9 | 10.1 | F | None | Simple | Formation | T10: hemivertebrae | T3, T5: butterfly; S1: lumbarization of the Sacrum | T4–T9: syrinx | None | PSIF, ASIF, hemivertebrectomy | 10.3 | |
10 | 2.7 | M | None | Complex | Combined | T6–T10: complex hypoplastic deformity; T11: butterfly; S1: lumbarization of the sacrum | None | Fatty filum terminale | None | None | ||
11 | 4.1 | M | None | Complex | Combined | L1: hemivertebrae; L3–L5: bar; S1–S5: hypoplasia | L3–L4: bony septum | L3–S3: diastematomyelia; low lying cord, tethered cord, lipoma | Detethering, split cord repair | 4.8 | Hemivertebrectomy, segmental instrumentation, posterior convex fusion, removal of hardware | 4.4 |
12 | 9.4 | F | None | Simple | Combined | L5: hemivertebrae; S1: lumbarization of the sacrum | None | T6–T12: Syrinx; low-lying cord, fatty filum terminal | Detethering | 10.7 | None | |
13 | 1.9 | M | None | Complex | Formation | T3, T5, T7: hemivertebrae | C1–C2: complex fusion | Cerebellar cyst | None | None | ||
14 | 13.6 | M | None | Simple | Formation | T6, T8: hemivertebrae | T5, T7, T9, T10: butterfly; S1: spina bifida occulta | T6–T8: diastematomyelia; low-lying cord, fatty filum terminale | Detethering, laminectomy | 13.9 | PSIF, osteotomy | 14.6 |
15 | 14.2 | F | None | Complex | Combined | C6–T4: complex combination of formation and segmentation defects; L3–L4: block | None | C5–C6: syrinx; L1: diastematomyelia; low-lying cord | None | Anterior fusion (prior to study period), osteotomy, costectomy, laminectomy | 2.5 (prior to study), 14.4 (during study) | |
16 | 2.7 | F | None | Complex | Combined | C3: butterfly; T9–T10: congenital bar; T11–T12: block | None | Chiari 1 | None | Hemivertebrectomy, PSIF | 3.1 | |
17 | 4.5 | F | Beckwith-Wiedemann syndrome | Simple | Formation | L3: hemivertebrae | C6–C7: block; L2–L3: block; L3–S3: spina bifida occulta | T12: high terminating cord | None | None | ||
18 | 7.2 | M | Cerebrocostomandibular syndrome | Complex | Combined | L4–L5: bar; S1: hemivertebrae | C2–C3: block | S1: lipomyelomengocele | None | VEPTR x3 | 8.6 | |
19 | 12.3 | F | Fetal alcohol syndrome, Klippel Feil | Complex | Combined | C5, C7: hemivertebrae; T1–T2: block; T3–T4: bar | C2–C3: block; T4: butterfly | Low-lying cord, tethered cord, fatty filum terminale | None | PSIF | 14.4 | |
20 | 1.3 | M | Goldenhar syndrome | Complex | Combined | C4–C7: butterfly; C6–C7: bar; T2: hemivertebra | None | Low-lying cord; T2–L3: lipoma | None | None | ||
21 | 9.2 | F | Jarco-Levin | Complex | Combined | T7–T12: hemivertebrae; L1: butterfly; L3–L4: block vertebrae | 8th cervical vertebrae; L4–L5: hemivertebrae; L1 butterfly | Low-lying cord, fatty filum terminale, tethered cord | Detethering | 1.2 (prior to study) | None | |
22 | 1.2 | M | Kabuki syndrome | Simple | Segmentation | T4–T5: bar | None | Fatty filum terminale | None | None | ||
23 | 9.9 | M | Multiple pterygium | Complex | Combined | T6–T8: hemivertebrae | T5–T9: bar | Low-lying cord | None | PSIF x 2; hardware removal | 10.5 | |
24 | 12.6 | M | Neurofibromatosis Type 1 | Complex | Formation | T6: hemivertebrae; T7–T9: pedicle hypoplasia | C3–C6: dorsal scalloping | C3: glioma; C3–C6: dural ectasia; C3–C7: pseudomeningocele; T7–T9: pseudomeningocele, dural ectasia | None | PSIF | 12.6 | |
25 | 13.9 | M | VACTERL | Complex | Combined | T4: hemvertebrae; T5–T9: block; T9: anterior wedge | T2–T3 bar; T11–T12: block; S1: spina bifida occulta | Tethered cord, fatty filum terminale | Detethering, S1 laminectomy | 13.9 | None | |
26 | 7.2 | M | VACTERL | Complex | Combined | C6–T4: complex combination of hemivertebrae, butterfly, and segmentation defects | None | Low-lying cord; fatty filum terminale | None | None | ||
27 | 4.6 | M | VACTERL | Complex | Combined | T1, T10: hemivertebrae; S1: lumbarization of the sacrum. | C1: platybasia; C3: hemivertebrae | Low-lying cord, fatty filum terminale, tethered cord | None | None | ||
28 | 1.2 | F | VACTERL | Simple | Formation | T7, L1: hemivertebrae | None | Low-lying cord | None | PSIF, hemivertebrectomy | 1.6 | |
29 | 0.4 | M | VACTERL | Complex | Combined | L4: hemivertebra; L5–S1: block | None | Low-lying, tethered cord, fatty filum terminale | Detethering | 1.1 | None | |
30 | 13.0 | F | VACTERL | Complex | Combined | T3–T4: bar; T5: hemivertebra | C1: craniocervical junction abnormality; C1–C2: hypoplasia, T8: butterfly; S1–S2: lumbarization | Low-lying cord | None | PSIF x2; Smith−Peterson osteotomy | 13.0 | |
31 | 0.7 | F | VACTERL | Complex | Combined | T6–T7, T8–T9: bar; T8: butterfly | S1: lumbarization; absence of coccyx | Low-lying cord | None | None | ||
32 | 5.8 | F | VACTERL | Simple | Formation | L5: anterior wedge | None | Tethered cord, fatty filum terminale | Detethering, fatty filum resection | 6.5 | None | |
33 | 7.9 | F | VACTERL | Complex | Formation | T9–T10: hemivertebrae; L4: butterfly | None | T6: syrinx | None | PSIF | 10.9 | |
34 | 1.4 | F | VACTERL | Complex | Formation | T3, T5, T6: butterfly | T1–T2, T5–T7: block | T1–T2: syrinx | None | None | ||
35 | 11.9 | F | VACTERL, arythrogryposis | Complex | Combined | T8-T9: bar; T12: hemivertebrae; L1: butterfly; L2: hemivertebrae; L3: butterfly; S1: hemivertebrae | L4: stenosis; L5-S1: large neural foramen; S2: abnormal articulation with ilium | Low-lying cord; tethered cord | None | PSIF, diskectomy, bar release | 4.8 (prior to study) | |
36 | 4.2 | M | VACTERL, Caudal regression syndrome | Complex | Formation | S1–S5: hypoplasia; Co1–Co5: absence of coccyx | C1: hypoplasia; L5: bony septum | Tethered cord; T8–S1: syrinx, diastematomyelia | Detethering, laminectomy | 4.4 | None | |
37 | 3.8 | F | VACTERL, Caudal regression syndrome | Complex | Formation | S3–S5: hypoplasia; Co1–Co5: absence of coccyx | None | Blunted spinal cord | None | None | ||
38 | 0.7 | F | VACTERL, Caudal regression Syndrome | Complex | Formation | L5: Anterior wedge; L4-S1: widening of interpedicular space | Co1-Co5: absence of coccyx | Blunted spinal cord | None | None | ||
39 | 0.8 | M | VACTERL, Caudal regression syndrome | Complex | Formation | T8: hemivertebra; L4: butterfly; S1–S5: hypoplasia | C1–C2: stenosis; Co1–Co5: absence of coccyx | Low-lying cord, tethered cord | Detethering, laminectomy | 3.0 | None | |
40 | 3 day | F | VACTERL, Caudal regression | Complex | Formation | T10–L5: hemivertebrae | None | Blunted spinal cord | None | None | ||
41 | 6.3 | F | VACTERL, fanconi anemia | Complex | Combined | T3–T6: bar; T4: hemivertebrae; S1: lumbarization of the sacrum | C3–C4, C6–C7: bar; C7–T1, L3–L5: block | Chiari 1, tethered cord (previous) | Detethering | Prior to study (0.04) | PSIF, osteotomy | 6.59 (during study) |
Level and number
Vertebral level |
N
| No spinal cord anomaly | Spinal cord anomaly | Incidence (%) |
---|---|---|---|---|
Cervical | 10 | 3 | 7 | 70 |
Thoracic | 58 | 27 | 31 | 54 |
Lumbar | 31 | 12 | 19 | 61 |
Sacral/coccygeala
| 15 | 2 | 13 | 87 |
Complexity and etiology
Bony anomaly |
N
| No spinal cord anomaly | Spinal cord anomaly | Incidence (%) |
p value |
---|---|---|---|---|---|
Simplea
| 30 | 19 | 11 | 37 | 0.011 |
Complexb
| 45 | 15 | 30 | 67 | |
Segmentationc
| 2 | 1 | 1 | 50 | 0.065 |
Formationd
| 47 | 26 | 21 | 45 | |
Mixed | 26 | 7 | 19 | 73 |