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Klippel-Feil syndrome presenting with bilateral thoracic outlet syndrome.
Spine (Phila Pa 1976) 2004; 29(9):E189-92S

Abstract

STUDY DESIGN

This report aims, in the light of the recent literature, to describe the clinical features of bilateral thoracic outlet syndrome in a case of Klippel-Feil syndrome, the results of electrophysiologic evaluation, and the outcome after surgical intervention.

OBJECTIVES

Cervical ribs in the context of Klippel-Feil syndrome as the cause of bilateral thoracic outlet syndrome is discussed.

SUMMARY OF BACKGROUND DATA

In Klippel-Feil syndrome, congenital fusion of cervical vertebrae occurs and may also be associated with various anomalies, including musculoskeletal anomalies. The typical neurologic defects in this syndrome are caused by compression of the cervical cord and/or the corresponding roots.

METHODS

A 25-year-old woman had experienced, for 3 years, sensory symptoms, mainly numbness and pain, in both arms and episodic color changes of the hands that were aggravated by certain activities.

RESULTS

Radiologic examination revealed Klippel-Feil syndrome type I, accompanied by bilateral cervical ribs. Electrophysiologic evaluation demonstrated impairment of nerve conduction, as indicated by F wave changes, after the arms were raised. The patient underwent successful decompression of the neurovascular structures at the thoracic aperture.

CONCLUSIONS

It is often difficult to diagnose thoracic outlet syndrome by conventional neurophysiology. Dynamic changes in F waves appear to be a useful finding. In the absence of symptoms of myeloradiculopathy, thoracic outlet syndrome could be the sole manifestation of Klippel-Feil syndrome.

Authors+Show Affiliations

Department of Neurosurgery, University of Patras, Rio, Greece.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

15105685

Citation

Konstantinou, Dimitris T., et al. "Klippel-Feil Syndrome Presenting With Bilateral Thoracic Outlet Syndrome." Spine, vol. 29, no. 9, 2004, pp. E189-92.
Konstantinou DT, Chroni E, Constantoyiannis C, et al. Klippel-Feil syndrome presenting with bilateral thoracic outlet syndrome. Spine. 2004;29(9):E189-92.
Konstantinou, D. T., Chroni, E., Constantoyiannis, C., & Dougenis, D. (2004). Klippel-Feil syndrome presenting with bilateral thoracic outlet syndrome. Spine, 29(9), pp. E189-92.
Konstantinou DT, et al. Klippel-Feil Syndrome Presenting With Bilateral Thoracic Outlet Syndrome. Spine. 2004 May 1;29(9):E189-92. PubMed PMID: 15105685.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Klippel-Feil syndrome presenting with bilateral thoracic outlet syndrome. AU - Konstantinou,Dimitris T, AU - Chroni,Elisabeth, AU - Constantoyiannis,Constatinos, AU - Dougenis,Dimitris, PY - 2004/4/24/pubmed PY - 2004/6/3/medline PY - 2004/4/24/entrez SP - E189 EP - 92 JF - Spine JO - Spine VL - 29 IS - 9 N2 - STUDY DESIGN: This report aims, in the light of the recent literature, to describe the clinical features of bilateral thoracic outlet syndrome in a case of Klippel-Feil syndrome, the results of electrophysiologic evaluation, and the outcome after surgical intervention. OBJECTIVES: Cervical ribs in the context of Klippel-Feil syndrome as the cause of bilateral thoracic outlet syndrome is discussed. SUMMARY OF BACKGROUND DATA: In Klippel-Feil syndrome, congenital fusion of cervical vertebrae occurs and may also be associated with various anomalies, including musculoskeletal anomalies. The typical neurologic defects in this syndrome are caused by compression of the cervical cord and/or the corresponding roots. METHODS: A 25-year-old woman had experienced, for 3 years, sensory symptoms, mainly numbness and pain, in both arms and episodic color changes of the hands that were aggravated by certain activities. RESULTS: Radiologic examination revealed Klippel-Feil syndrome type I, accompanied by bilateral cervical ribs. Electrophysiologic evaluation demonstrated impairment of nerve conduction, as indicated by F wave changes, after the arms were raised. The patient underwent successful decompression of the neurovascular structures at the thoracic aperture. CONCLUSIONS: It is often difficult to diagnose thoracic outlet syndrome by conventional neurophysiology. Dynamic changes in F waves appear to be a useful finding. In the absence of symptoms of myeloradiculopathy, thoracic outlet syndrome could be the sole manifestation of Klippel-Feil syndrome. SN - 1528-1159 UR - https://www.unboundmedicine.com/medline/citation/15105685/Klippel_Feil_syndrome_presenting_with_bilateral_thoracic_outlet_syndrome_ L2 - http://dx.doi.org/10.1097/00007632-200405010-00023 DB - PRIME DP - Unbound Medicine ER -