Literature DB >> 16172901

Infantile scoliosis in Beals syndrome: the use of a non-fusion technique for surgical correction.

Anthony Gwynne Martin1, Pedro R Foguet, David S Marks, A G Thompson, A H Child.   

Abstract

Beals syndrome (congenital contractural arachnodactyl) is a genetic disorder of the connective tissue phenotypically related to Marfan syndrome. It is characterised by dolichostenomelia, arachnodactyly, multiple joint contractures, crumpled ears, hypoplastic muscles and scoliosis. The latter, the most important clinical feature of this rare condition, presents in the infantile and juvenile age group and has a tendency to rapid progression. Bracing often fails to control the scoliosis and surgery is the recommended treatment. We present our experience of two cases managed with the paediatric Isola instrumentation and a non-fusion technique.

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Year:  2005        PMID: 16172901      PMCID: PMC3489316          DOI: 10.1007/s00586-005-0980-9

Source DB:  PubMed          Journal:  Eur Spine J        ISSN: 0940-6719            Impact factor:   3.134


  19 in total

1.  The operative treatment of progressive early onset scoliosis. A preliminary report.

Authors:  J F Patterson; J K Webb; R G Burwell
Journal:  Spine (Phila Pa 1976)       Date:  1990-08       Impact factor: 3.468

2.  Ectopia lentis and aortic root dilatation in congenital contractural arachnodactyly.

Authors:  E Bawle; M H Quigg
Journal:  Am J Med Genet       Date:  1992-01-01

Review 3.  Posterior instrumentation in scoliosis.

Authors:  J K Webb; R G Burwell; A A Cole; I Lieberman
Journal:  Eur Spine J       Date:  1995       Impact factor: 3.134

4.  Spontaneous fusion in scoliosis after instrumentation without arthrodesis.

Authors:  J R Fisk; H A Peterson; R Laughlin; R Lutz
Journal:  J Pediatr Orthop       Date:  1995 Mar-Apr       Impact factor: 2.324

5.  Submuscular Isola rod with or without limited apical fusion in the management of severe spinal deformities in young children: preliminary report.

Authors:  L C Blakemore; P V Scoles; C Poe-Kochert; G H Thompson
Journal:  Spine (Phila Pa 1976)       Date:  2001-09-15       Impact factor: 3.468

6.  Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5. The International Marfan Syndrome Collaborative Study.

Authors:  P Tsipouras; R Del Mastro; M Sarfarazi; B Lee; E Vitale; A H Child; M Godfrey; R B Devereux; D Hewett; B Steinmann
Journal:  N Engl J Med       Date:  1992-04-02       Impact factor: 91.245

7.  Contractural arachnodactyly with mitral regurgitation and iridodonesis.

Authors:  I C Huggon; J P Burke; J F Talbot
Journal:  Arch Dis Child       Date:  1990-03       Impact factor: 3.791

8.  Clinical and genetic associations in Marfan syndrome and related disorders.

Authors:  F Ramirez; B Lee; E Vitale
Journal:  Mt Sinai J Med       Date:  1992-09

Review 9.  Harrington instrumentation without arthrodesis and consecutive distraction program for young children with severe spinal deformities. Experience and technical details.

Authors:  C A Tello
Journal:  Orthop Clin North Am       Date:  1994-04       Impact factor: 2.472

10.  Developmental expression of fibrillin genes suggests heterogeneity of extracellular microfibrils.

Authors:  H Zhang; W Hu; F Ramirez
Journal:  J Cell Biol       Date:  1995-05       Impact factor: 10.539

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  1 in total

1.  Genetic variants in GPR126 are associated with adolescent idiopathic scoliosis.

Authors:  Ikuyo Kou; Yohei Takahashi; Todd A Johnson; Atsushi Takahashi; Long Guo; Jin Dai; Xusheng Qiu; Swarkar Sharma; Aki Takimoto; Yoji Ogura; Hua Jiang; Huang Yan; Katsuki Kono; Noriaki Kawakami; Koki Uno; Manabu Ito; Shohei Minami; Haruhisa Yanagida; Hiroshi Taneichi; Naoya Hosono; Taichi Tsuji; Teppei Suzuki; Hideki Sudo; Toshiaki Kotani; Ikuho Yonezawa; Douglas Londono; Derek Gordon; John A Herring; Kota Watanabe; Kazuhiro Chiba; Naoyuki Kamatani; Qing Jiang; Yuji Hiraki; Michiaki Kubo; Yoshiaki Toyama; Tatsuhiko Tsunoda; Carol A Wise; Yong Qiu; Chisa Shukunami; Morio Matsumoto; Shiro Ikegawa
Journal:  Nat Genet       Date:  2013-05-12       Impact factor: 38.330

  1 in total

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