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Symptomatic cervical disc herniation in a pediatric Klippel-Feil patient: the risk of neural injury associated with extensive congenitally fused vertebrae and a hypermobile segment.
Spine (Phila Pa 1976) 2006; 31(11):E335-8S

Abstract

STUDY DESIGN

A case report.

OBJECTIVE

To report the occurrence of a herniated cervical disc following a high-impact activity in a pediatric Klippel-Feil patient who presented with spinal cord compression, myelopathy, and myelomalacia requiring posterior instrumented fusion followed by an anterior discectomy and fusion at the hypermobile nonfused segment.

SUMMARY OF BACKGROUND DATA

The primary hallmark of Klippel-Feil syndrome (KFS) is the presence of at least one congenitally fused cervical segment. Studies have reported the potential risk of cervical injury from hypermobility associated with the nonfused cervical segment in KFS. The manifestation of a cervical disc herniation in the pediatric KFS patient is rare. To the authors' knowledge, the development of a symptomatic cervical herniated disc attributed to mechanical fatigue following a high-impact activity has not been addressed in the literature with respect to the pediatric KFS patient having extensive cervical fusion and a hypermobile segment.

METHODS

A 16.8-year-old KFS boy with occipitalization of C1 and fusion of C2-C3 and C4-T1 presented with myelopathy, severe cord compression, and myelomalacia stemming from a left-sided herniated cervical disc at C3-C4 with onset following an 8-foot high rooftop jump. On radiographic evaluation, the patient's C3-C4 segment was hypermobile.

RESULTS

The patient was operatively managed via a same-day combined posterior-anterior procedure. The posterior aspect of the procedure entailed a posterior lateral mass plate-screw fixation at C3-C4 with autologous iliac crest bone fusion. Anteriorly, a discectomy was performed at C3-C4 with application of an interbody tricortical autograft. After surgery, the patient wore a halo vest for 3 months, followed by a soft collar for an additional 3 months. On final follow-up at 39 months, the patient was asymptomatic with no instrumentation-related complications, fusion of the posterior graft-bed and anterior interbody graft was noted, and cervical alignment was maintained.

CONCLUSIONS

A hypermobile segment in the pediatric KFS patient is a risk factor that may lead to cord compression. A symptomatic herniated cervical disc may develop from an excessive mechanical load stress in a pediatric KFS patient with multiple fused segments. In such a patient, a same-day combined posterior-anterior procedure provides cord decompression and stabilizes the spine with a favorable outcome.

Authors+Show Affiliations

Division of Health Sciences, University of Oxford, Oxford, England.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

16688024

Citation

Samartzis, Dino, et al. "Symptomatic Cervical Disc Herniation in a Pediatric Klippel-Feil Patient: the Risk of Neural Injury Associated With Extensive Congenitally Fused Vertebrae and a Hypermobile Segment." Spine, vol. 31, no. 11, 2006, pp. E335-8.
Samartzis D, Lubicky JP, Herman J, et al. Symptomatic cervical disc herniation in a pediatric Klippel-Feil patient: the risk of neural injury associated with extensive congenitally fused vertebrae and a hypermobile segment. Spine. 2006;31(11):E335-8.
Samartzis, D., Lubicky, J. P., Herman, J., Kalluri, P., & Shen, F. H. (2006). Symptomatic cervical disc herniation in a pediatric Klippel-Feil patient: the risk of neural injury associated with extensive congenitally fused vertebrae and a hypermobile segment. Spine, 31(11), pp. E335-8.
Samartzis D, et al. Symptomatic Cervical Disc Herniation in a Pediatric Klippel-Feil Patient: the Risk of Neural Injury Associated With Extensive Congenitally Fused Vertebrae and a Hypermobile Segment. Spine. 2006 May 15;31(11):E335-8. PubMed PMID: 16688024.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Symptomatic cervical disc herniation in a pediatric Klippel-Feil patient: the risk of neural injury associated with extensive congenitally fused vertebrae and a hypermobile segment. AU - Samartzis,Dino, AU - Lubicky,John P, AU - Herman,Jean, AU - Kalluri,Prakasam, AU - Shen,Francis H, PY - 2006/5/12/pubmed PY - 2006/9/23/medline PY - 2006/5/12/entrez SP - E335 EP - 8 JF - Spine JO - Spine VL - 31 IS - 11 N2 - STUDY DESIGN: A case report. OBJECTIVE: To report the occurrence of a herniated cervical disc following a high-impact activity in a pediatric Klippel-Feil patient who presented with spinal cord compression, myelopathy, and myelomalacia requiring posterior instrumented fusion followed by an anterior discectomy and fusion at the hypermobile nonfused segment. SUMMARY OF BACKGROUND DATA: The primary hallmark of Klippel-Feil syndrome (KFS) is the presence of at least one congenitally fused cervical segment. Studies have reported the potential risk of cervical injury from hypermobility associated with the nonfused cervical segment in KFS. The manifestation of a cervical disc herniation in the pediatric KFS patient is rare. To the authors' knowledge, the development of a symptomatic cervical herniated disc attributed to mechanical fatigue following a high-impact activity has not been addressed in the literature with respect to the pediatric KFS patient having extensive cervical fusion and a hypermobile segment. METHODS: A 16.8-year-old KFS boy with occipitalization of C1 and fusion of C2-C3 and C4-T1 presented with myelopathy, severe cord compression, and myelomalacia stemming from a left-sided herniated cervical disc at C3-C4 with onset following an 8-foot high rooftop jump. On radiographic evaluation, the patient's C3-C4 segment was hypermobile. RESULTS: The patient was operatively managed via a same-day combined posterior-anterior procedure. The posterior aspect of the procedure entailed a posterior lateral mass plate-screw fixation at C3-C4 with autologous iliac crest bone fusion. Anteriorly, a discectomy was performed at C3-C4 with application of an interbody tricortical autograft. After surgery, the patient wore a halo vest for 3 months, followed by a soft collar for an additional 3 months. On final follow-up at 39 months, the patient was asymptomatic with no instrumentation-related complications, fusion of the posterior graft-bed and anterior interbody graft was noted, and cervical alignment was maintained. CONCLUSIONS: A hypermobile segment in the pediatric KFS patient is a risk factor that may lead to cord compression. A symptomatic herniated cervical disc may develop from an excessive mechanical load stress in a pediatric KFS patient with multiple fused segments. In such a patient, a same-day combined posterior-anterior procedure provides cord decompression and stabilizes the spine with a favorable outcome. SN - 1528-1159 UR - https://www.unboundmedicine.com/medline/citation/16688024/Symptomatic_cervical_disc_herniation_in_a_pediatric_Klippel_Feil_patient:_the_risk_of_neural_injury_associated_with_extensive_congenitally_fused_vertebrae_and_a_hypermobile_segment_ L2 - http://dx.doi.org/10.1097/01.brs.0000217628.32344.73 DB - PRIME DP - Unbound Medicine ER -