Tags

Type your tag names separated by a space and hit enter

Sprengel's deformity in Klippel-Feil syndrome.
Spine (Phila Pa 1976). 2007 Aug 15; 32(18):E512-6.S

Abstract

STUDY DESIGN

A retrospective study.

OBJECTIVES

To address the role of congenitally fused cervical segments, the degree of cervical scoliosis, and other risk factors on the presence of Sprengel's deformity (SD) in young patients with Klippel-Feil syndrome (KFS).

SUMMARY OF BACKGROUND DATA

Numerous abnormalities are associated with KFS, one of the most common being SD. It has been postulated that more severe forms of KFS may be more associated with extraspinal manifestations, such as SD.

METHODS

Thirty KFS patients from a single institution were reviewed. Cervical neutral lateral/dynamic/anteroposterior and thoracic anteroposterior plain radiographs were assessed. Radiographically, occipitalization (O-C1), number of congenitally fused segments (C1-T1), classification type (Types I-III), degree of cervical scoliosis, and the presence of SD was assessed. Clinical chart review entailed patient demographics and evidence of the clinical assessment of SD. The threshold for statistical significance was P < 0.05.

RESULTS

There were 11 males (36.7%) and 19 females (63.3%) with a mean age of 13.5 years (range, 2.7-26.3 years). Occipitalization was present in 10 (33.3%) individuals and C2-C3 was the most common level fused (70.0%). The mean number of congenitally fused segments was 3.3 (range, 1-6 levels). The mean degree of cervical scoliosis was 17.3 degrees (range, 0 degrees-67 degrees). There were 6 (20%) Type I, 15 Type II (50.0%), and 9 Type III (30%) patients. SD was noted in 5 (16.7%) of the patients. Four patients had unilateral, whereas 1 patient had bilateral SD. There was 4.0 and 3.1 mean number of congenitally fused segments in patients with or without SD, respectively. SD did not occur in Type I patients (single fused block). The presence of SD was found to be nonsignificant regarding sex type (P = 0.327), presence of occipitalization (P = 0.300), number of congenitally fused segments (P = 0.246), specific congenitally fused segments (P > 0.05), classification type (P > 0.05), and scoliosis (P = 0.702).

CONCLUSION

SD occurred in 16.7% of KFS patients. Sex type, number of congenitally fused segments, specific fused patterns, occipitalization, classification type, and the degree of cervical scoliosis did not seem to be significantly associated with the presence of SD in KFS patients in our series. Thorough examination for the presence and degree of SD in KFS is necessary, irrespective of the extent of cervical abnormalities. Alternatively, the treating physician should not dismiss a thorough cervical spine examination in patients with SD, evaluating factors that may predispose the KFS patient to an increased risk of neurologic injury.

Authors+Show Affiliations

Graduate Division, Harvard University, Cambridge, MA, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

17700431

Citation

Samartzis, Dino, et al. "Sprengel's Deformity in Klippel-Feil Syndrome." Spine, vol. 32, no. 18, 2007, pp. E512-6.
Samartzis D, Herman J, Lubicky JP, et al. Sprengel's deformity in Klippel-Feil syndrome. Spine. 2007;32(18):E512-6.
Samartzis, D., Herman, J., Lubicky, J. P., & Shen, F. H. (2007). Sprengel's deformity in Klippel-Feil syndrome. Spine, 32(18), E512-6.
Samartzis D, et al. Sprengel's Deformity in Klippel-Feil Syndrome. Spine. 2007 Aug 15;32(18):E512-6. PubMed PMID: 17700431.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Sprengel's deformity in Klippel-Feil syndrome. AU - Samartzis,Dino, AU - Herman,Jean, AU - Lubicky,John P, AU - Shen,Francis H, PY - 2007/8/19/pubmed PY - 2007/9/25/medline PY - 2007/8/19/entrez SP - E512 EP - 6 JF - Spine JO - Spine VL - 32 IS - 18 N2 - STUDY DESIGN: A retrospective study. OBJECTIVES: To address the role of congenitally fused cervical segments, the degree of cervical scoliosis, and other risk factors on the presence of Sprengel's deformity (SD) in young patients with Klippel-Feil syndrome (KFS). SUMMARY OF BACKGROUND DATA: Numerous abnormalities are associated with KFS, one of the most common being SD. It has been postulated that more severe forms of KFS may be more associated with extraspinal manifestations, such as SD. METHODS: Thirty KFS patients from a single institution were reviewed. Cervical neutral lateral/dynamic/anteroposterior and thoracic anteroposterior plain radiographs were assessed. Radiographically, occipitalization (O-C1), number of congenitally fused segments (C1-T1), classification type (Types I-III), degree of cervical scoliosis, and the presence of SD was assessed. Clinical chart review entailed patient demographics and evidence of the clinical assessment of SD. The threshold for statistical significance was P < 0.05. RESULTS: There were 11 males (36.7%) and 19 females (63.3%) with a mean age of 13.5 years (range, 2.7-26.3 years). Occipitalization was present in 10 (33.3%) individuals and C2-C3 was the most common level fused (70.0%). The mean number of congenitally fused segments was 3.3 (range, 1-6 levels). The mean degree of cervical scoliosis was 17.3 degrees (range, 0 degrees-67 degrees). There were 6 (20%) Type I, 15 Type II (50.0%), and 9 Type III (30%) patients. SD was noted in 5 (16.7%) of the patients. Four patients had unilateral, whereas 1 patient had bilateral SD. There was 4.0 and 3.1 mean number of congenitally fused segments in patients with or without SD, respectively. SD did not occur in Type I patients (single fused block). The presence of SD was found to be nonsignificant regarding sex type (P = 0.327), presence of occipitalization (P = 0.300), number of congenitally fused segments (P = 0.246), specific congenitally fused segments (P > 0.05), classification type (P > 0.05), and scoliosis (P = 0.702). CONCLUSION: SD occurred in 16.7% of KFS patients. Sex type, number of congenitally fused segments, specific fused patterns, occipitalization, classification type, and the degree of cervical scoliosis did not seem to be significantly associated with the presence of SD in KFS patients in our series. Thorough examination for the presence and degree of SD in KFS is necessary, irrespective of the extent of cervical abnormalities. Alternatively, the treating physician should not dismiss a thorough cervical spine examination in patients with SD, evaluating factors that may predispose the KFS patient to an increased risk of neurologic injury. SN - 1528-1159 UR - https://www.unboundmedicine.com/medline/citation/17700431/Sprengel's_deformity_in_Klippel_Feil_syndrome_ L2 - http://dx.doi.org/10.1097/BRS.0b013e318133fcf8 DB - PRIME DP - Unbound Medicine ER -