Congenital cervical kyphosis is a rare clinical condition. We describe an infant presenting with tetraparesis, rapidly progressing to phrenic paralysis and fatal respiratory insufficiency. Therapeutic options remain challenging. Surgical arthrodesis is technically difficult, and one report of a therapeutic thermoplastic body splint is described. Final outcome, however, is dismal. No long-term survivors have been described.

1.
Giampietro PF, Dunwoodie SL, Kusumi K, Pourquié O, et al: Progress in the understanding of the genetic etiology of vertebral segmentation disorders of humans. Ann NY Acad Sci 2009;1151:38–67.
2.
Hefti F: Congenital anomalies of the spine. Orthopade 2002;31:34–43.
3.
Forese LL, Berdon WE, Harcke HT, Wagner ML, Lachman R, Chorney GS, Roye DP: Severe mid-cervical kyphosis with cord compression in Larsen’s syndrome and diastrophic dysplasia: unrelated syndromes with similar radiologic findings and neurosurgical implications. Pediatr Radiol 1995;25:136–139.
4.
Zhang D, Herring JA, Swaney SS, McClendon TB, Gao X, Browne RH, Rathjen KE, Johnston CE, Harris S, Wise CA: Mutations responsible for Larsen syndrome cluster in the FLNB protein. J Med Genet 2006;43:e24.
5.
Goumy C, Beaufrère AM, Tchirkov A, Gouas L, Gaspard F, Giollant M, Roucaute T, Veronèse L, Lemery D, Vago P: Larsen-like phenotype associated with partial trisomy 3p and monosomy 5p. Prenatal Diagn 2008;28:131–134.
6.
Genevieve D, Le Merrer M, Feingold J, Munnich A, et al: Revisiting metatropic dysplasia: presentation of a series of 19 novel patients and review of the literature. Am J Med Genet A 2008;146A:992–996.
7.
Akbarnia BA, Heydarian K, Ganjavian MS: Concordant congenital spine deformity in monozygotic twins. J Pediatr Orthop 1983;3:502–504.
8.
Tsirikos AI, McMaster MJ: Congenital anomalies of the ribs and chest wall associated with congenital deformities of the spine. J Bone Joint Surg Am 2005;87:2523–2536.
9.
Hedequist DJ, Hall JE, Emans JB: The safety and efficacy of spinal instrumentation in children with congenital spine deformities. Spine 2004;29:2081–2086.
10.
Kim YJ, Otsuka NY, Flynn JM, Hall JE, Emans JB, Hresko MT: Surgical treatment of congenital kyphosis. Spine 2001;26:2251–2257.
11.
Sakaura H, Matsuoka T, Iwasaki M, Yonenobu K, Yoshikawa H: Surgical treatment of cervical kyphosis in Larsen syndrome: report of 3 cases and review of the literature. Spine 2007;32:E39–E44.
12.
Madera M, Crawford A, Mangano FT: Management of severe cervical kyphosis in a patient with Larsen syndrome. Case report. J Neurosurg Pediatr 2008;1:320–324.
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