Abstract
Cervical spine radiographs of 33 patients with Klippel-Feil syndrome were studied for patterns of bony fusion and presence of wasp-waist sign. Five patients were found to have the classic features of massive cervical fusion. Two patients with two adjacent levels of bony fusion showed a wasp-waist sign. Fusion at one level of the cervical spine accounted for 26 remaining cases. The wasp-waist sign was observed in 14 of the cases in which there was complete vertebral interbody fusion, making this finding a valuable radiologic sign. Partial anterior or posterior vertebral interbody fusion, or isolated fusion of the neural arch, however, may or may not be associated with the wasp-waist sign. Klippel-Feil syndrome, easy to recognize when presenting with classic features or when associated with the wasp-waist sign, may be confused with a variety of other entities.
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References
Bland HT (1987) Disorders of the cervical spine. Saunders, Philadelphia, pp 298–312
Brown MW, Templeton AW, Hodges FJ (1964) The incidence of acquired and congenital fusions in the cervical spine. AJR 92:1255–1259
Canale ST, Beaty JH (1991) Operative pediatric orthopedics. Mosby Yearbook, St Louis, pp 439–444
Connor JM, Evans DAP (1982) Fibrodysplasia ossificans progressiva: the clinical features and natural living history of 34 patients. J Bone Joint Surg [Br] 64:76–83
Connor JM, Smith R (1982) The cervical spine in fibrodysplasia ossificans progressiva. Br J Radiol 55:492–496
Dolan DK (1977) Development abnormalities of the cervical spine below the axis. Radiol Clin North Am 15:167–174
Feil A (1919) L'absence et la diminution des vertèbres cervicales (étude clinique et pathogénique): le syndrome de la réduction numérique cervicale. Thesis, Sorbonne University, Paris
Forsythe M, Rothman RH (1978) New concepts in the diagnosis and treatment of infection of the cervical spine. Orthop Clin North Am 9:1039–1051
Frymoyer JW (1991) The adult spine. Raven Press, New York, pp 1025–1037
Hanai K, Inouye Y, Kawai K, et al (1982) Anterior decompression for myelopathy resulting from ossification of the posterior longitudinal ligament. J Bone Joint Surg [Br] 64:561–564
Hensinger RN (1991) The congenital anomalies of the cervical spine. Clin Orthop Rel Res 264:16–38
Hensinger RN, Lang JR, McEwen GD (1974) The Klippel-Feil syndrome: a constellation of related anomalies. J Bone Joint Surg [Am] 56:1246–1253
Hirabayashi K, Satomi K, Sapaki T (1988) Ossification of the posterior longitudinal ligament in the cervical spine. Lippincott, Philadelphia, pp 678–692
Holdsworth FW (1963) Fractures, dislocations, and fracture-dislocations of the spine. J Bone Joint Surg [Br] 45:810–823
Horlyck E, Rabeck M (1974) Cervical spine injuries. Acta Orthop Scand 45:845–853
Hsu LCS, Leong JC (1984) Tuberculosis of the lower cervical spine (C2–C7). J Bone Joint Surg [Br] 66:1–5
Klippel M, Feil A (1912) Anomalie de la colonne vertébrale par l'absence des vertèbres cervicales; cage thoracique remontant jusqu'a la base du crâne. Bull Hem Soc Anat Paris 87:185–188
Labhart A (1986) Clinical endocrinology, theory and practice. Springer, Berlin Heidelberg New York, pp 721–728
Lawry LB (1977) The Klippel-Feil anomalad as part of the fetal alcohol syndrome. Tetralogy 16:53–66
Lifshitz F (1990) Pediatric endocrinology, 2nd edn. Dekker, New York, pp 387–388
Martel W, Holt JF, Cassidy JT (1962) Roentgenologic manifestations of juvenile rheumatoid arthritis. AJR 88:400–422
Martin NS (1970) Tuberculosis of the spine. J Bone Joint Surg [Br] 52:613–628
Pizzutillo PD (1989) The Klippel-Feil syndrome. In: Cervical Spine Research Society Staff (eds) The cervical spine, 2nd edn. Lippincott, Philadelphia, pp 258–271
Putman EC, Ravin C (1988) Textbook of diagnostic imaging, Saunders, Philadelphia, pp 1440–1442
Ratanasiri T, Thiramont N (1991) The ossification of the posterior longitudinal ligament in Thailand. J Jpn Orthop Assoc S468
Resnick D, Niwayama G (1988) Diagnosis of bone and joint disorders, 2nd edn. Saunders, Philadelphia, pp 1069–1099
Resnick D, Niwayama G (1988) Diagnosis of bone and joint disorders. 2nd edn. Saunders, Philadelphia, pp 3574–3578
Rogers WA (1957) Fractures and dislocations of the cervical spine: an end result study. J Bone Joint Surg [Am] 39:341–376
Saliere D, Cler D, Bisson M, Massiasp (1984) Atteinte du rachis cervical aucours des arthrites chroniques juveniles. A partir de vingt-neuf observations evoluant depuis plus de cinq ans. Sem Hop Paris 2:97–100
Shoul IM, Ritvo M (1952) Clinical and roentgenological manifestations of the Klippe-Feil syndrome. AJR 88:369–383
Southwell RB, Reynolds AF, Badger VM, Sherman FC (1980) Klippel-Feil syndrome with cervical cord compression resulting from cervical subluxation in association with an omo-vertebral bone. Spine 5:480–482
Tachdjian MO (1990) Pediatric orthopedics, 2nd edn. Saunders, Philadelphia, pp 128–136
Tredwell PJ, Smith DF, Macleod PJ, et al (1982) Cervical spine anomalies in fetal alcohol syndrome. Spine 7:331–334
Tuli SM (1975) Tuberculosis of the spine. Am Publishing, New Delhi, pp 29–47
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Nguyen, V.D., Tyrrel, R. Klippel-Feil syndrome: patterns of bony fusion and wasp-waist sign. Skeletal Radiol. 22, 519–523 (1993). https://doi.org/10.1007/BF00209100
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DOI: https://doi.org/10.1007/BF00209100