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Syndactyly

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Congenital Anomalies of the Upper Extremity

Abstract

Syndactyly is a condition well known to the pediatric surgeon due to it being the second most common congenital upper limb defect. Ranging from simple interdigital webbing, syndactyly is frequently seen in association with soft tissue and bone variances in the hand as well as in syndromes associated with abnormalities of the other organ systems. Coming from the Greek syn (“συν” meaning together) and dactyly (“δακτυλος” meaning digits) it describes an embryological failure of finger separation. Although common in some species, including birds and kangaroos, it has a major aesthetic and functional significance for humans born with the condition.

This chapter focuses on the current understanding of the genetic and molecular causes of syndactyly. It will also discuss the varying clinical presentations as well as highlight its management.

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References

  1. Canale ST, Beaty JH. Campbell’s operative orthopaedics, vol. 4. 11th ed. Philadelphia: Mosby Elsevier; 2008. p. 4403–4.

    Google Scholar 

  2. Green DP, Hotchkiss RN, Pederson WC, Wolfe SW. Green’s operative hand surgery, vol. 2. 5th ed. Philadelphia, PA: Mosby Elsevier; 2005. p. 1381–2.

    Google Scholar 

  3. Burke FD, McGrouther DA, Smith PJ. Principles of hand surgery, chapter 15. Edinburgh: Churchill Livingstone; 1989. p. 256.

    Google Scholar 

  4. Eaton CJ, Lister GD. Syndactyly. Hand Clin. 1990;6(4):555.

    Article  CAS  PubMed  Google Scholar 

  5. Kozin SH. Syndactyly. J Am Soc Surg Hand. 2001;1:1–13.

    Article  Google Scholar 

  6. Benson MKD, Fixen JA, Macnicol MF, Parsch K. Children’s orthopaedics and fractures. 2nd ed. Edinburgh: Churchill Linigston; 2002. p. 306–307.

    Google Scholar 

  7. Netscher DT, Baumholtz MA. Treatment of congenital upper extremity problems. Plast Reconstr Surg. 2007;119(5):101e–29.

    Article  PubMed  CAS  Google Scholar 

  8. Hogan BL. Morphogenesis. Cell. 1999;96:225–33.

    Article  CAS  PubMed  Google Scholar 

  9. Oligny LL. Human molecular embryogenesis: an overview. Pedatr Dev Pathol. 2001;4:324–43.

    Article  CAS  Google Scholar 

  10. Mariani FV, Martin GR. Deciphering skeletal patterning: clues from the limb. Nature. 2003;423:319–25.

    Article  CAS  PubMed  Google Scholar 

  11. Saunders JW. The proximo-distal sequence of origin of the parts of the chick wing and the role of the ectoderm. J Exp Zool. 1948;108(3):363–403.

    Article  PubMed  Google Scholar 

  12. Saunders JW, Gasseling MT. Ectodermal-mesodermal interactions in the origin of limb symmetry. In: Fleischmajer RE, Billingham R, editors. Epithelial-mesenchymal interactions. Baltimore: Williams & Wilkins; 1968. p. 78–97.

    Google Scholar 

  13. Todt WL, Fallon JF. Posterior apical ectodermal ridge removal in the chick wing bud triggers a series of events resulting in defective anterior pattern formation. Development. 1987;101(3):501–15.

    Article  CAS  PubMed  Google Scholar 

  14. Ahmed H, Akbari H, Emami A, Akbari MR. Genetic overview of syndactyly and polydactyly. Plast Reconstr Surg Glob Open. 2017;5(11):e1549.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Manouvrier-Hanu S, Holder-Espinasse M, Lyonnet S. Genetics of limb anomalies in humans. Trends Genet. 1999;15(10):409–17.

    Article  CAS  PubMed  Google Scholar 

  16. Ingham PW, McMahon AP. Hedgehog signalling in animal development. Genes Dev. 2001;15:3059–87.

    Article  CAS  PubMed  Google Scholar 

  17. Riddle RD, Johnson RL, Laufer E, Tabin C. Sonic hedgehog mediates the polarizing activity of the ZPA. Cell. 1993;75(7):1401–16.

    Article  CAS  PubMed  Google Scholar 

  18. Vortkamp A, Lee K, Lanske B, Segre GV, Kronenberg HM, Tabin CJ. Regulation of rate of cartilage differentiation by Indian hedgehog and PTH-related protein. Science. 1996;273(5275):613–22.

    Article  CAS  PubMed  Google Scholar 

  19. Naski MC, Omitz DM. FGF signalling in skeletal development. Front Biosci. 1998;3:D781–94.

    Article  CAS  PubMed  Google Scholar 

  20. Ohbayashi N, Shibayama M, Kurotaki Y, Imanishi M, Fujimori T, Itoh N, et al. FGF18 is required for normal cell proliferation and differentiation during osteogenesis and chondrogenesis. Genes Dev. 2002;16:870–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Chung UI, Schipani E, McMahon AP, Kronenberg HM. Indian hedgehog couples chondrogenesis to osteogenesis in endochondral bone development. J Clin Invest. 2001;107:295–304.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Klopocki E, Lohan S, Brancati F, Koll R, Brehm A, Seemann P, et al. Copy-number variations involving the IHH locus are associated with syndactyly and craniosynostosis. Am J Hum Genet. 2011;88(1):70–5. Epub 2010 Dec 17.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Gofflot F, Hars C, Illien F, Chevy F, Wolf C, Picard JJ, et al. Molecular mechanisms underlying limb anomalies associated with cholesterol deficiency during gestation: implications of hedgehog signalling. Hum Mol Genet. 2003;12(10):1187–98.

    Article  CAS  PubMed  Google Scholar 

  24. Litingtung Y, Dahn RD, Yina L, Fallon JF, Chiang C. Shh and Gli3 are dispensable for limb skeleton formation but regulate digit number and identity. Nature. 2002;418:979–83.

    Article  CAS  PubMed  Google Scholar 

  25. Welscher P, Zuniga A, Kuijper S, Drenth T, Goedemans HJ, Meijlink F, et al. Progression of vertebrate limb development through Shh-mediated counteraction of Gli3. Science. 2002;298(5594):827–30.

    Article  CAS  Google Scholar 

  26. Chiang C, Litingtung Y, Harris MP, Simandl BK, Li Y, Beachy PA, et al. Manifestation of the limb prepattern: limb development in the absence of sonic hedgehog function. Dev Biol. 2001;236:421–35.

    Article  CAS  PubMed  Google Scholar 

  27. Kraus P, Fraidenraich D, Loomis CA. Some distal limb structures develop in mice lacking sonic hedgehog signalling. Mech Dev. 2001;100:45–58.

    Article  CAS  PubMed  Google Scholar 

  28. Drossopoulou G, Lewis KE, Sanz-Ezquerro JJ, Nikbakht N, McMahon AP, Hofmann C, et al. A model for anteroposterior patterning of the vertebrate limb based on sequential long and short range Shh signalling and bmp signalling. Development. 2000;127:1337–48.

    Article  CAS  PubMed  Google Scholar 

  29. Kawakami Y, Capdevilla J, Buscher D, Itoh T, Rodríguez Esteban C, Izpisúa Belmonte JC. WNT signals control FGF-dependent limb initiation and AER induction in the chick embryo. Cell. 2001;104:891–900.

    Article  CAS  PubMed  Google Scholar 

  30. Rankin J, Strachan T, Lako M, Lindsay S. Partial cloning and assignment of WNT6 to human chromosome band 2q35 by in situ hybridization. Cytogenet Cell Genet. 1999;84:50–2.

    Article  CAS  PubMed  Google Scholar 

  31. Parr BA, Shea MJ, Vassileva G, McMahon AP. Mouse WNT genes exhibit discrete domains of expression in the early embryonic CNS and limb buds. Development. 1993;119(1):247–61.

    Article  CAS  PubMed  Google Scholar 

  32. Khan S, Basit S, Zimri F, Ali N, Ali G, Ansar M, et al. A novel homozygous missense mutation in WNT10B in familial split-hand/foot malformation. Clin Genet. 2012;82(1):48–55.

    Article  CAS  PubMed  Google Scholar 

  33. Mori C, Nakamura N, Kimura S, Irie H, Takigawa T, Shiota K. Programmed cell death in the interdigital tissue of the fetal mouse limb is apoptosis with DNA fragmentation. Anat Rec. 1995;242:103–10.

    Article  CAS  PubMed  Google Scholar 

  34. Nishii K, Tsuzuki T, Kumai M, Takeda N, Koga H, Aizawa S, et al. Abnormalities of developmental cell death in Dad1-deficient mice. Genes. 1999;4(4):243–52.

    CAS  Google Scholar 

  35. Salas-Vidal E, Valencia C, Covarrubias L. Differential tissue growth and patterns of cell death in mouse limb autopod morphogenesis. Dev Dyn. 2001;220:295–306.

    Article  CAS  PubMed  Google Scholar 

  36. Merino R, Rodriguez-Leon J, Macias D, Gañan Y, Economides AN, Hurle JM. The BMP antagonist Gremlin regulates outgrowth, chondrogenesis and programmed cell death in the developing limb. Development. 1999;126:5515–22.

    Article  CAS  PubMed  Google Scholar 

  37. Crocoll A, Herzer U, Ghyselinck NB, Chambon P, Cato AC. Interdigital apoptosis and downregulation of BAG-1 expression in mouse autopods. Mech Dev. 2002;111:149–52.

    Article  CAS  PubMed  Google Scholar 

  38. Heymer J, Rüther U. Syndactyly of Ft/+mice correlates with an imbalance in bmp4 and fgf8 expression. Mech Dev. 1999;88(2):173–81.

    Article  CAS  PubMed  Google Scholar 

  39. Schwabe GC, Mundlos S. Genetics of congenital hand anomalies. Handchir Mikrochir Plast Chir. 2004;36:85–97.

    Article  CAS  PubMed  Google Scholar 

  40. Francis PH, Richardson MK, Brickell PM, Tickle C. Bone morphogenetic proteins and a signalling pathway that controls patterning in the developing chick limb. Development. 1994;120:209–18.

    Article  CAS  PubMed  Google Scholar 

  41. Lyons KM, Hogan BL, Robertson EJ. Colocalization of BMP 7 and BMP 2 RNAs suggests that these factors cooperatively mediate tissue interactions during murine development. Mech Dev. 1995;50:71–83.

    Article  CAS  PubMed  Google Scholar 

  42. Laufer E, Dahn R, Orozco OE, Yeo CY, Pisenti J, Henrique D, et al. Expression of radical fringe in limb-bud ectoderm regulates apical ectodermal ridge formation. Nature. 1997;386:366–73.

    Article  CAS  PubMed  Google Scholar 

  43. Ganan Y, Macias D, Duterque-Coquillaud M, Ros MA, Hurle JM. Role of TGF beta s and BMPs as signals controlling the position of the digits and the areas of interdigital cell death in the developing chick limb autopod. Development. 1996;122:2349–57.

    Article  CAS  PubMed  Google Scholar 

  44. Zuzarte-Luis V, Hurle JM. Programmed cell death in the developing limb. Int J Dev Biol. 2002;46:871–6.

    CAS  PubMed  Google Scholar 

  45. Guha U, Gomes WA, Kobayashi T, Pestell RG, Kessler JA. In vivo evidence that BMP signalling is necessary for apoptosis in the mouse limb. Dev Biol. 2002;249(1):108–20.

    Article  CAS  PubMed  Google Scholar 

  46. Yokouchi Y, Sakiyama J, Kameda T, Iba H, Suzuki A, Ueno N, et al. BMP 2/4 mediate programmed cell death in chicken limb buds. Development. 1996;122:3725–34.

    Article  CAS  PubMed  Google Scholar 

  47. Zou H, Niswander L. Requirement for BMP signalling in interdigital apoptosis and scale formation. Science. 1996;272:738–41.

    Article  CAS  PubMed  Google Scholar 

  48. Arteaga-Solis E, Gayraud B, Lee SY, Shum L, Sakai L, Ramirez F. Regulation of limb patterning by extracellular microfibrils. J Cell Biol. 2001;154(2):275–81.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Dahn RD, Fallon JF. Interdigital regulation of digit identity and homeotic transformation by modulated BMP signalling. Science. 2000;289:438–41.

    Article  CAS  PubMed  Google Scholar 

  50. McCulloch DR, Nelson CM, Dixon LJ, Silver DL, Wylie JD, Lindner V, et al. ADAMTS metalloproteases generate active versican fragments that regulate interdigital web regression. Dev Cell. 2009;17(5):687–98.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  51. Talamillo A, Delgado I, Nakamura T, de-Vega S, Yoshitomi Y, Unda F, et al. Role of Epiprofin, a zinc-finger transcription factor, in limb development. Dev Biol. 2010;337(2):363–74.

    Article  CAS  PubMed  Google Scholar 

  52. Ota S, Zhou ZQ, Keene DR, Knoepfler P, Hurlin PJ. Activities of N-Myc in the developing limb link control of skeletal size with digit separation. Development. 2007;134(8):1583–92.

    Article  CAS  PubMed  Google Scholar 

  53. Vortkamp A, Gessler M, Grzeschik KH. GLI3 zinc-finger gene interrupted by translocations in Greig syndrome families. Nature. 1991;352:539–40.

    Article  CAS  PubMed  Google Scholar 

  54. Johnston JJ, Sapp JC, Turner JT, Amor D, Aftimos S, Aleck KA, et al. Molecular analysis expands the spectrum of phenotypes associated with GLI3 mutations. Hum Mutat. 2010;31(10):1142–54.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Ricks CB, Masand R, Fang P, Roney EK, Cheung SW, Scott DA. Delineation of a 1.65 Mb critical region for hemihyperplasia and digital anomalies on Xq25. Am J Med Genet A. 2010;152A(2):453–8.

    Article  CAS  PubMed  Google Scholar 

  56. Schmidt K, Hughes C, Chudek JA, Goodyear SR, Aspden RM, Talbot R, et al. Cholesterol metabolism: the main pathway acting downstream of cytochrome P450 oxidoreductase in skeletal development of the limb. Mol Cell Biol. 2009;29(10):2716–29.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  57. Lv D, Luo Y, Yang W, Cao L, Wen Y, Zhao X, et al. A novel single-base deletion in ROR2 causes atypical brachydactyly type B1 with cutaneous syndactyly in a large Chinese family. J Hum Genet. 2009;54(7):422–5.

    Article  CAS  PubMed  Google Scholar 

  58. Böse K, Nischt R, Page A, Bader BL, Paulsson M, Smyth N. Loss of nidogen-1 and -2 results in syndactyly and changes in limb development. J Biol Chem. 2006;281(51):39620–9.

    Article  PubMed  CAS  Google Scholar 

  59. Liu Y, Liu C, Yamada Y, Fan CM. Growth arrest specific gene 1 acts as a region-specific mediator of the Fgf10/Fgf8 regulatory loop in the limb. Development. 2002;129(22):5289–300.

    Article  CAS  PubMed  Google Scholar 

  60. Dauwerse JG, de Vries BB, Wouters CH, Bakker E, Rappold G, Mortier GR, et al. A t(4;6)(q12;p23) translocation disrupts a membrane-associated O-acetyl transferase gene (MBOAT1) in a patient with a novel brachydactyly-syndactyly syndrome. Eur J Hum Genet. 2007;15(7):743–51.

    Article  CAS  PubMed  Google Scholar 

  61. Jiang R, Lan Y, Chapman HD, Shawber C, Norton CR, Serreze DV, et al. Defects in limb, craniofacial, and thymic development in Jagged2 mutant mice. Genes Dev. 1998;12(7):1046–57.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. Sidow A, Bulotsky MS, Kerrebrock AW, Bronson RT, Daly MJ, Reeve MP, et al. Serrate2 is disrupted in the mouse limb-development mutant syndactylism. Nature. 1997;389(6652):722–5.

    Article  CAS  PubMed  Google Scholar 

  63. Hwang SJ, Beaty TH, McIntosh I, Hefferon T, Panny SR. Association between homeobox-containing gene MSX1 and the occurrence of limb deficiency. Am J Med Genet. 1998;75(4):419–23.

    Article  CAS  PubMed  Google Scholar 

  64. Goodman FR. Limb malformations and the human HOX genes. Am J Med Genet. 2002;112(3):256–65.

    Article  PubMed  Google Scholar 

  65. McKusick VA. www.usfca.edu/Library/databases/OMIM/ and Mendelian inheritance in man. 12th ed. Baltimore: Johns Hopkins University Press; 1998.

  66. Temtamy SA, McKusick VA. The genetics of hand malformations. New York, NY: Alan R. Liss New York; 1978. p. 301–22.

    Google Scholar 

  67. Goldstein DJ, Kambouris M, Ward RE. Familial crossed polysyndactyly. Am J Med Genet. 1994;50:215–23.

    Article  CAS  PubMed  Google Scholar 

  68. Malik S, Percin FE, Ahmad W, Percin S, Akarsu NA, Koch MC, et al. Autosomal recessive mesoaxial synostotic syndactyly with phalangeal reduction maps to chromosome 17p13.3. Am J Med Genet A. 2005;134(4):404–8.

    Article  PubMed  Google Scholar 

  69. Jordan D, Hindocha S, Dhital M, Saleh M, Khan W. The epidemiology, genetics and future management of syndactyly. Open Orthop J. 2012;6:14–27.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  70. Percin EF, Percin S. Two unusual types of syndactyly in the same family; Cenani-Lenz type and “new” type versus severe type I syndactyly? Genet Couns. 2003;14(3):313–9.

    CAS  PubMed  Google Scholar 

  71. Bosse K, Betz RC, Lee YA, Wienker TF, Reis A, Kleen H, et al. Localization of a gene for syndactyly type 1 to chromosome 2q34-q36. Am J Hum Genet. 2000;67(2):492–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  72. Ghadami M, Majidzadeh-A K, Haerian BS, Damavandi E, Yamada K, Pasallar P, et al. Confirmation of genetic homogeneity of syndactyly type 1 in an Iranian family. Am J Med Genet. 2001;104(2):147–51.

    Article  CAS  PubMed  Google Scholar 

  73. Robin NH, Segel B, Carpenter G, Muenke M. Craniosynostosis, Philadelphia type: a new autosomal dominant syndrome with sagittal craniosynostosis and syndactyly of the fingers and toes. Am J Med Genet. 1996;62:184–91.

    Article  CAS  PubMed  Google Scholar 

  74. Jain M, Wallis D, Robin NH, De Vrieze FW, Hardy JA, Ghadami M, et al. Locus homogeneity between syndactyly type 1A and craniosynostosis Philadelphia type? Am J Med Genet A. 2008;146A:2308–11.

    Article  PubMed  PubMed Central  Google Scholar 

  75. Rossant J. ENU mutants from the Center of Modeling Human Disease. MGI Direct Data Submission. 2004;Accession ID MGI:3032560.

    Google Scholar 

  76. Malik S, Schott J, Ali SW, Oeffner F, Amin-ud-Din M, Ahmad W, et al. Evidence for clinical and genetic heterogeneity of syndactyly type I: the phenotype of second and third toe syndactyly maps to chromosome 3p21.31. Eur J Hum Genet. 2005;13:1268–74.

    Article  CAS  PubMed  Google Scholar 

  77. Sarfarazi M, Akarsu AN, Sayli BS. Localization of the syndactyly type II (synpolydactyly) locus to 2q31 region and identification of tight linkage to HOXD8 intragenic marker. Hum Mol Genet. 1995;4:1453–8.

    Article  CAS  PubMed  Google Scholar 

  78. Dai L, Heng ZC, Zhu J, Cai R, Mao M, Wang H, et al. Mutation analysis of HOXD13 gene in a Chinese pedigree with synpolydactyly. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2005;22(3):277–80.

    CAS  PubMed  Google Scholar 

  79. Wajid M, Ishii Y, Kurban M, Dua-Awereh MB, Shimomura Y, Christiano AM. Polyalanine repeat expansion mutations in the HOXD13 gene in Pakistani families with synpolydactyly. Clin Genet. 2009;76(3):300–2.

    Article  CAS  PubMed  Google Scholar 

  80. Muragaki Y, Mundlos S, Upton J, Olsen BR. Altered growth and branching patterns in synpolydactyly caused by mutations in HOXD13. Science. 1996;272(5261):548–51.

    Article  CAS  PubMed  Google Scholar 

  81. Akarsu AN, Stoilov I, Yilmaz E, Sayli BS, Sarfarazi M. Genomic structure of HOXD13 gene: a nine polyalanine duplication causes synpolydactyly in two unrelated families. Hum Mol Genet. 1996;5:945–52.

    Article  CAS  PubMed  Google Scholar 

  82. Goodman FR, Majewski F, Collins AL, Scambler PJ. A 117-kb microdeletion removing HOXD9-HOXD13 and EVX2 causes synpolydactyly. Am J Hum Genet. 2002;70:547–55.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  83. Goodman FR, Mundlos S, Muragaki Y, Donnai D, Giovannucci-Uzielli ML, Lapi E, et al. Synpolydactyly phenotypes correlate with size of expansions in HOXD13 polyalanine tract. Proc Natl Acad Sci U S A. 1997;94:7458–63.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  84. Malik S, Girisha KM, Wajid M, Roy AK, Phadke SR, Haque S, et al. Synpolydactyly and HOXD13 polyalanine repeat: addition of 2 alanine residues is without clinical consequences. BMC Med Genet. 2007;8:78.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  85. Zhao X, Sun M, Zhao J, Leyva JA, Zhu H, Yang W, et al. Mutations in HOXD13 underlie syndactyly type V and a novel brachydactyly-syndactyly syndrome. Am J Hum Genet. 2007;80(2):361–71.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  86. Ghoumid J, Andrieux J, Sablonniere B, Odent S, Philippe N, Zanlonghi X, et al. Duplication of chromosome 2q31.1-q31.2 in a family presenting syndactyly and nystagmus. Eur J Hum Genet. 2011;19(11):1198–201.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  87. Debeer P, Schoenmakers EF, Twal WO, Argraves WS, De Smet L, Fryns JP, et al. The fibulin-1 gene (FBLN1) is disrupted in a t (12;22) associated with a complex type of synpolydactyly. Med Genet. 2002;39(2):98–104.

    Article  CAS  Google Scholar 

  88. Debeer P, Schoenmakers EF, De Smet L, Van de Ven WJ, Fryns JP. Co-segregation of an apparently balanced reciprocal t(12;22)(p11.2;q13.3) with a complex type of 3/3′/4 synpolydactyly associated with metacarpal, metatarsal and tarsal synostoses in three family members. Clin Dysmorphol. 1998;7(3):225–8.

    Article  CAS  PubMed  Google Scholar 

  89. Debeer P, Schoenmakers EF, Thoelen R, Holvoet M, Kuittinen T, Fabry G, et al. Physical map of a 1.5 mb region on 12p11.2 harbouring a synpolydactyly associated chromosomal breakpoint. Eur J Hum Genet. 2000;8(8):561–70.

    Article  CAS  PubMed  Google Scholar 

  90. Malik S, Abbasi AA, Ansar M, Ahmad W, Koch MC, Grzeschik KH. Genetic heterogeneity of synpolydactyly: a novel locus SPD3 maps to chromosome 14q11.2-q12. Clin Genet. 2006;69(6):518–24.

    Article  CAS  PubMed  Google Scholar 

  91. Malik S, Grzeschik KH. Synpolydactyly: clinical and molecular advances. Clin Genet. 2008;73(2):113–20.

    Article  CAS  PubMed  Google Scholar 

  92. Kuru I, Samli H, Yucel A, Bozan ME, Turkmen S, Solak M. Hypoplastic synpolydactyly as a new clinical subgroup of synpolydactyly. Hand Surg Br. 2004;29(6):614–20.

    Article  Google Scholar 

  93. Ikegawa M, Han H, Okamoto A, Matsui R, Tanaka M, Omi N, et al. Syndactyly and preaxial synpolydactyly in the single Sfrp2 deleted mutant mice. Dev Dyn. 2008;237(9):2506–17.

    Article  CAS  PubMed  Google Scholar 

  94. Johnston O, Kirby VV. Syndactyly of the ring and little finger. Am J Hum Genet. 1955;7:80–2.

    CAS  PubMed  PubMed Central  Google Scholar 

  95. De Smet L, Mulier T, Fabry G. Syndactyly of the ring and small finger. Genet Couns. 1994;5:45–9.

    PubMed  Google Scholar 

  96. Schrander-Stumpel CTRM, de Groot-Wijnands JBG, de Die-Smulders C, Fryns JP. Type III syndactyly and oculodentodigital dysplasia: a clinical spectrum. Genet Couns. 1993;4:271–6.

    CAS  PubMed  Google Scholar 

  97. Gladwin A, Donnai D, Metcalfe K, Schrander-Stumpel C, Brueton L, Verloes A, et al. Localization of a gene for oculodentodigital syndrome to human chromosome 6q22-q24. Hum Mol Genet. 1997;6(1):123–7.

    Article  CAS  PubMed  Google Scholar 

  98. Paznekas WA, Boyadjiev SA, Shapiro RE, Daniels O, Wollnik B, Keegan CE, et al. Connexin 43 (GJA1) mutations cause the pleiotropic phenotype of oculodentodigital dysplasia. Am J Hum Genet. 2003;72:408–18.

    Article  CAS  PubMed  Google Scholar 

  99. Richardson R, Donnai D, Meire F, Dixon MJ. Expression of Gja1 correlates with the phenotype observed in oculodentodigital syndrome/type III syndactyly. J Med Genet. 2004;41(1):60–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  100. Dobrowolski R, Hertig G, Lechner H, Wörsdörfer P, Wulf V, Dicke N, et al. Loss of connexin43-mediated gap junctional coupling in the mesenchyme of limb buds leads to altered expression of morphogens in mice. Hum Mol Genet. 2009;18(15):2899–911.

    Article  CAS  PubMed  Google Scholar 

  101. Fenwick A, Richardson RJ, Butterworth J, Barron MJ, Dixon MJJ. Novel mutations in GJA1 cause oculodentodigital syndrome. Dent Res. 2008;87(11):1021–6.

    Article  CAS  Google Scholar 

  102. Amador C, Mathews AM, Del Carmen MM, Laughridge ME, Everman DB, Holden KR. Expanding the neurologic phenotype of oculodentodigital dysplasia in a 4-generation Hispanic family. J Child Neurol. 2008;23(8):901–5.

    Article  PubMed  Google Scholar 

  103. Debeer P, Van Esch H, Huysmans C, Pijkels E, De Smet L, Van de Ven W, et al. Novel GJA1 mutations in patients with oculo-dento-digital dysplasia (ODDD). Eur J Med Genet. 2005;48(4):377–87.

    Article  PubMed  Google Scholar 

  104. Jamsheer A, Wisniewska M, Szpak A, Bugaj G, Krawczynski MR, Budny B, et al. A novel GJA1 missense mutation in a polish child with oculodentodigital dysplasia. Appl Genet. 2009;50(3):297–9.

    Article  CAS  Google Scholar 

  105. Dobrowolski R, Sasse P, Schrickel JW, Watkins M, Kim JS, Rackauskas M, et al. The conditional connexin43G138R mouse mutant represents a new model of hereditary oculodentodigital dysplasia in humans. Hum Mol Genet. 2008;17(4):539–54.

    Article  CAS  PubMed  Google Scholar 

  106. Dobrowolski R, Sommershof A, Willecke K. Some oculodentodigital dysplasia-associated Cx43 mutations cause increased hemichannel activity in addition to deficient gap junction channels. J Membr Biol. 2007;219(1–3):9–17.

    Article  CAS  PubMed  Google Scholar 

  107. van Es RJ, Wittebol-Post D, Beemer FA. Oculodentodigital dysplasia with mandibular retrognathism and absence of syndactyly: a case report with a novel mutation in the connexin 43 gene. Int J Oral Maxillofac Surg. 2007;36(9):858–60.

    Article  PubMed  Google Scholar 

  108. Vreeburg M, de Zwart-Storm EA, Schouten MI, Nellen RG, Marcus-Soekarman D, Devies M, et al. Skin changes in oculo-dento-digital dysplasia are correlated with C-terminal truncations of connexin 43. Am J Med Genet A. 2007;143(4):360–3.

    Article  CAS  PubMed  Google Scholar 

  109. Wiest T, Herrmann O, Stögbauer F, Grasshoff U, Enders H, Koch MJ, et al. Clinical and genetic variability of oculodentodigital dysplasia. Clin Genet. 2006;70(1):71–2.

    Article  CAS  PubMed  Google Scholar 

  110. Haas SL. Bilateral complete syndactylism of all fingers. Am J Surg. 1940;50:363–6.

    Article  Google Scholar 

  111. Sato D, Liang D, Wu L, Pan Q, Xia K, Dai H, et al. Syndactyly type IV locus maps to 7q36. J Hum Genet. 2007;52:561–4.

    Article  CAS  PubMed  Google Scholar 

  112. Rambaud-Cousson A, Dudin AA, Zuaiter AS, Thalji A. Syndactyly type IV/hexadactyly of feet associated with unilateral absence of the tibia. Am J Med Genet. 1991;40:144–5.

    Article  CAS  PubMed  Google Scholar 

  113. Gillessen-Kaesbach G, Majewski F. Bilateral complete polysyndactyly (type IV Haas). Am J Med Genet. 1991;38:29–31.

    Article  CAS  PubMed  Google Scholar 

  114. Wieczorek D, Pawlik B, Li Y, Akarsu NA, Caliebe A, May KJ, et al. A specific mutation in the distant sonic hedgehog (SHH) cis-regulator (ZRS) causes Werner mesomelic syndrome (WMS) while complete ZRS duplications underlie Haas type polysyndactyly and preaxial polydactyly (PPD) with or without triphalangeal thumb. Hum Mutat. 2010;31(1):81–9.

    Article  CAS  PubMed  Google Scholar 

  115. Sun M, Ma F, Zeng X, Liu Q, Zhao XL, Wu FX, et al. Triphalangeal thumb-polysyndactyly syndrome and syndactyly type IV are caused by genomic duplications involving the long range, limb-specific SHH enhancer. J Med Genet. 2008;45:589–95.

    Article  CAS  PubMed  Google Scholar 

  116. Wang ZQ, Tian SH, Shi YZ, Zhou PT, Wang ZY, Shu RZ, et al. A single C to T transition in intron 5 of LMBR1 gene is associated with triphalangeal thumb-polysyndactyly syndrome in a Chinese family. Biochem Biophys Res Commun. 2007;355(2):312–7.

    Article  CAS  PubMed  Google Scholar 

  117. Kemp T, Ravn J. Ueber erbliche Hand-und Fussdeformitaeten in einem 140-koepfigen Geschlecht, nebst einigen Bemerkungen ueber Poly-und Syndaktylie beim Menschen. Acta Psychiatr Neurol Scand. 1932;7:275–96.

    Article  Google Scholar 

  118. Robinow M, Johnson GF, Broock GJ. Syndactyly type V. Am J Med Genet. 1982;11:475–82.

    Article  CAS  PubMed  Google Scholar 

  119. Kjaer KW, Hansen L, Eiberg H, Utkus A, Skovgaard LT, Leicht P, et al. A 72-year-old Danish puzzle resolved—comparative analysis of phenotypes in families with different-sized HOXD13 polyalanine expansions. Am J Med Genet. 2005;138A:328–39.

    Article  Google Scholar 

  120. Cenani A, Lenz W. Totale Syndaktylie und totale radioulnare Synostose bie zwei Bruedern. Ein Beitrag zur Genetik der Syndaktylien Ztschr Kinderheilk. 1967;101:181–90.

    Article  CAS  Google Scholar 

  121. Liebenam L. Ueber gleichzeitiges Vorkommen von Gliedmassendefekten und osteosklerotischer Systemerkrunkung. Ztschr Mensch Vererbungs-und Konstitutionslehre. 1938;21:697–703.

    Google Scholar 

  122. Borsky AJ. Congenital anomalies of the hand and their surgical treatment. Charles C Thomas: Springfield, IL; 1958.

    Google Scholar 

  123. Yelton CL. Certain congenital limb deficiencies occurring in twins and half siblings. Inter-Clinic Inform Bull. 1962;1:1–7.

    Google Scholar 

  124. Drögemüller C, Leeb T, Harlizius B, Tammen I, Distl O, Höltershinken M, et al. Congenital syndactyly in cattle: four novel mutations in the low density lipoprotein receptor-related protein 4 gene (LRP4). BMC Genet. 2007;8:5.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  125. Simon-Chazottes D, Tutois S, Kuehn M, Evans M, Bourgade F, Cook S, et al. Mutations in the gene encoding the low-density lipoprotein receptor LRP4 cause abnormal limb development in the mouse. Genomics. 2006;87(5):673–7.

    Article  CAS  PubMed  Google Scholar 

  126. Li Y, Pawlik B, Elcioglu N, Aglan M, Kayserili H, Yigit G, et al. LRP4 mutations alter Wnt/beta-catenin signaling and cause limb and kidney malformations in Cenani-Lenz syndrome. Am J Hum Genet. 2010;86(5):696–706.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  127. Bacchelli C, Goodman FR, Scambler PJ, Winter RM. Cenani-Lenz syndrome with renal hypoplasia is not linked to FORMIN or GREMLIN. Clin Genet. 2001;59:203–5.

    Article  CAS  PubMed  Google Scholar 

  128. Dimitrov BI, Voet T, De Smet L, Vermeesch JR, Devriendt K, Fryns JP, et al. Genomic rearrangements of the GREM1–FMN1 locus cause oligosyndactyly, radio-ulnar synostosis, hearing loss, renal defects syndrome and Cenani–Lenz-like non-syndromic oligosyndactyly. J Med Genet. 2010;47(8):569–74.

    Article  CAS  PubMed  Google Scholar 

  129. Harpf C, Pavelka M, Hussl H. A variant of Cenani-Lenz syndactyly (CLS): review of the literature and attempt of classification. Br J Plast Surg. 2005;58(2):251–7.

    Article  CAS  PubMed  Google Scholar 

  130. Orel H. Kleine Beitrage zur Vererbungswissenschaft. Synostosis Metacarpi Quarti et Quinti Z Anat. 1928;14:244–52.

    Google Scholar 

  131. Lonardo F, Della Monica M, Riccardi G, Riccio I, Riccio V, Scarano G. A family with X-linked recessive fusion of metacarpals IV and V. Am J Med Genet. 2004;124A:407–10.

    Article  PubMed  Google Scholar 

  132. Holmes LB, Wolf E, Miettinen OS. Metacarpal 4-5 fusion with X-linked recessive inheritance. Am J Hum Genet. 1972;24:562–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  133. Lerch H. Erbliche Synostosen der Ossa metacarpalia IV und V. Z Orthop. 1948;78:13–6.

    Google Scholar 

  134. Faiyaz-Ul-Haque M, Zaidi SHE, King LM, Haque S, Patel M, Ahmad M, et al. Fine mapping of the X-linked split-hand/split-foot malformation (SHFM2) locus to a 5.1-Mb region on Xq26.3 and analysis of candidate genes. Clin Genet. 2005;67:93–7.

    Google Scholar 

  135. Percin EF, Percin S, Egilmez H, Sezgin I, Ozbas F, Akarsu AN. Mesoaxial complete syndactyly and synostosis with hypoplastic thumbs: an unusual combination or homozygous expression of syndactyly type I. J Med Genet. 1998;35(10):868–74.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  136. Malik S, Arshad M, Amin-ud-Din M, Oeffner F, Dempfle A, Haque S, et al. A novel type of autosomal recessive syndactyly: clinical and molecular studies in a family of Pakistani origin. Am J Med Genet. 2004;126A:61–7.

    Article  PubMed  Google Scholar 

  137. Losch G, Duncker H. Acrosyndactylism. Transactions of the International Society of Plastic and Reconstructive Surgeons, 5th congress. Butterworth Pty: Melbourne; 1971.

    Google Scholar 

  138. Patterson T. Congenital ring constrictions. Br J Plast Surg. 1961;14:1–31.

    Article  CAS  PubMed  Google Scholar 

  139. Torpin R, Faulkner A. Intrauterine amputation with the missing member found in the fetal membranes. JAMA. 1966;198:185–7.

    Article  CAS  PubMed  Google Scholar 

  140. Upton J. Congenital anomalies of the hand and forearm. In: McCarthy JG, May Jr JW, Littler JW, editors. The hand, vol. 8. Plastic surgery. Philadelphia: WB Saunders; 1990. p. 5213–5398.

    Google Scholar 

  141. Walsh RJ. Acrosyndactyly. A study of twenty-seven patients. Clin Orthop. 1970;71:99–111.

    CAS  PubMed  Google Scholar 

  142. Maisels D. Acrosyndactyly. Br J Plast Surg. 1962;15:166–72.

    Article  CAS  PubMed  Google Scholar 

  143. Poland A. Deficiency of the pectoral muscles. Guys Hosp Rep. 1841, VI:191–3.

    Google Scholar 

  144. Clarkson P. Poland’s syndactyly. Guys Hosp Rep. 1962;111:335–46.

    CAS  PubMed  Google Scholar 

  145. Bouvet J, Leveque D, Bernetieres F, Gros JJ. Vascular origin of Poland syndrome: a comparative rheographic study of the vascularisation of the arms in eight patients. Eur J Pediatr. 1978;128:17–26.

    Article  CAS  PubMed  Google Scholar 

  146. Fraser FC, Ronen GM, O’Leary E. Pectoralis major defect and Poland sequence in second cousins: extension of the Poland sequence spectrum. Am J Med Genet. 1989;33:468–70.

    Article  CAS  PubMed  Google Scholar 

  147. Bouwes-Bavinck J, Weaver D. Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Mobius anomalies. Am J Med Genet. 1986;23:903–18.

    Article  Google Scholar 

  148. Wilson M, Louis DS, Stevenson TR. Poland’s syndrome: variable expression and associated anomalies. J Hand Surg Am. 1988;13:880–2.

    Article  CAS  PubMed  Google Scholar 

  149. Karnak I, Tanyel FC, Tunçbilek E, Unsal M, Büyükpamukçu N. Bilateral Poland anomaly. Am J Med Genet. 1998;75(5):505–7.

    Article  CAS  PubMed  Google Scholar 

  150. Wilkie AOM, Slaney SF, Oldridge M, Poole MD, Ashworth GJ, Hockley AD, et al. Apert syndrome results from localized mutations of FGFR2 and is allelic with Crouzon syndrome. Nat Genet. 1995;9:165–72.

    Article  CAS  PubMed  Google Scholar 

  151. Howard TD, Paznekas WA, Green ED, Chiang LC, Ma N, Ortiz de Luna RI, et al. Mutations in TWIST, a basic helix-loop-helix transcription factor, in Saethre-Chotzen syndrome. Nat Genet. 1997;15:36–41.

    Article  PubMed  Google Scholar 

  152. Paznekas WA, Cunningham ML, Howard TD, Korf BR, Lipson MH, Grix AW, et al. Genetic heterogeneity of Saethre-Chotzen syndrome, due to TWIST and FGFR mutations. Am J Hum Genet. 1998;62:1370–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  153. Muenke M, Schell U, Hehr A, Robin NH, Losken HW, Schinzel A, et al. A common mutation in the fibroblast growth factor receptor 1 gene in Pfeiffer syndrome. Nat Genet. 1994;8:269–74.

    Article  CAS  PubMed  Google Scholar 

  154. Rossi M, Jones RL, Norbury G, Bloch-Zupan A, Winter R. The appearance of the feet in Pfeiffer syndrome caused by FGFR1 P252R mutation. Clin Dysmorphol. 2003;12:269–74.

    Article  PubMed  Google Scholar 

  155. Jenkins D, Seelow D, Jehee FS, Perlyn CA, Alonso LG, Bueno DF, et al. RAB23 mutations in Carpenter syndrome imply an unexpected role for hedgehog signaling in cranial-suture development and obesity. Am J Hum Genet. 2007;80:1162–70. Note: Erratum: Am. J. Hum. Genet. 81: 1114 only, 2007.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  156. Cohen DM, Green JG, Miller J, Gorlin RJ, Reed JA. Acrocephalopolysyndactyly type II—Carpenter syndrome: clinical spectrum and an attempt at unification with Goodman and Summitt syndromes. Am J Med Genet. 1987;28:311–24.

    Article  CAS  PubMed  Google Scholar 

  157. McGregor L, Makela V, Darling SM, Vrontou S, Chalepakis G, Roberts C, et al. Fraser syndrome and mouse blebbed phenotype caused by mutations in FRAS1/Fras1 encoding a putative extracellular matrix protein. Nat Genet. 2003;34:203–8.

    Article  CAS  PubMed  Google Scholar 

  158. Shafeghati Y, Kniepert A, Vakili G, Zenker M. Fraser syndrome due to homozygosity for a splice site mutation of FREM2. Am J Med Genet. 2008;146A:529–31.

    Article  CAS  PubMed  Google Scholar 

  159. Carney TJ, Feitosa NM, Sonntag C, Slanchev K, Kluger J, Kiyozumi D, et al. Genetic analysis of fin development in zebrafish identifies furin and hemicentin1 as potential novel Fraser syndrome disease genes. PLoS Genet. 2010;6(4):e1000907.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  160. Garagnani L, Smith GD. Syndromes associated with syndactyly. In: The pediatric upper extremity. New York: Springer; 2014. p. 1–31.

    Google Scholar 

  161. Malik S, Ahmad W, Grzeschik KH, Koch MC. A simple method for characterising syndactyly in clinical practice. Genet Couns. 2005;16:229–38.

    CAS  PubMed  Google Scholar 

  162. Winter RM, Tickle C. Syndactylies and polydactilies: embryological overview and suggested classification. Eur J Hum Genet. 1993;1:96–104.

    Article  CAS  PubMed  Google Scholar 

  163. Man LX, Chang B. Maternal cigarette smoking during pregnancy increases the risk of having a child with a congenital digital anomaly. Plast Reconstr Surg. 2006;117(1):301–8.

    Article  CAS  PubMed  Google Scholar 

  164. Luo JY, Fu CH, Yao KB, Hu RS, Qy D, Liu ZY. A case-control study on genetic and environmental factors regarding polydactyly and syndactyly. Zhonghua Liu Xing Bing Xue Za Zhi. 2009;30(9):903–6.

    PubMed  Google Scholar 

  165. Lorea P, Coessens BC. Evolution of surgical techniques for skin release. Eur J Plast Surg. 2001;24:275–81.

    Article  Google Scholar 

  166. Oda T, Pushman AG, Chung KC. Treatment of common congenital hand conditions. Plast Reconstr Surg. 2010;126(3):121e–33.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  167. Kettelkamp DB, Flatt AE. An evaluation of syndactylia repair. Surg Gynecol Obstet. 1961;113:471–8.

    CAS  PubMed  Google Scholar 

  168. Dao K, Shin AY, Billings A. Surgical treatment of congenital syndactyly of the hand. J Am Acad Orthop Surg. 2004;12:39–48.

    Article  PubMed  Google Scholar 

  169. Hutchinson DT, Frenzen SW. Digital syndactyly release. Tech Hand Up Extrem Surg. 2010;14(1):33–7.

    Article  PubMed  Google Scholar 

  170. Deunck J, Nicolai JP, Hamburg SM. Long-term results of syndactyly correction: full-thickness versus split-thickness skin grafts. J Hand Surg Br. 2003;28(2):125–30.

    Article  Google Scholar 

  171. Lumenta DB, Kitzinger HB, Beck H, Frey M. Long-term outcomes of web creep, scar quality and function after simple syndactyly surgical treatment. J Hand Surg Am. 2010;35(8):1323–9.

    Article  PubMed  Google Scholar 

  172. Kamath JB, Vardhan H, Naik DM, Bansal A, Rai M, Kumar A. A novel method of using mini external fixator for maintaining web space after the release of contracture and syndactyly. Tech Hand Up Extrem Surg. 2013;17(1):37–40.

    Article  PubMed  Google Scholar 

  173. Aydin A, Ozden BC. Dorsal metacarpal island flap in syndactyly treatment. Ann Plast Surg. 2004;52(1):43–8.

    Article  PubMed  Google Scholar 

  174. Sharma RK, Tuli P, Makkar SS, Parashar A. End-f-skin grafts in syndactyly release: description of a new flap for web resurfacing and primary closure of finger defects. Hand. 2009;4(1):29–34.

    Article  PubMed  Google Scholar 

  175. Gao W, Yan H, Zhang F, Jiang L, Wang A, Yang J, et al. Dorsal pentagonal local flap: a new technique of web reconstruction for syndactyly without skin graft. Aesthet Plast Surg. 2011;35(4):530–7.

    Article  Google Scholar 

  176. Wang S, Zheng S, Li N, Feng Z, Liu Q. Dorsal hexagon local flap without skin graft for web reconstruction of congenital syndactyly. J Hand Surg Am. 2020;45(1):63.e1–9.

    Article  Google Scholar 

  177. Yao JM, Shong JL, Sun H. Repair of incomplete simple syndactyly by a web flap on a subcutaneous tissue pedicle. Plast Reconstr Surg. 1997;99:2079–81.

    Article  CAS  PubMed  Google Scholar 

  178. Tadiparthi S, Mishra A, Mcarthur P. A modification of the Chinese island flap technique for simple incomplete syndactyly release. J Hand Surg Eur Vol. 2009;34(1):99–103.

    Article  CAS  PubMed  Google Scholar 

  179. Greuse M, Coessens BC. Congenital syndactyly: defatting facilitates closure without skin graft. J Hand Surg Am. 2001;26:589–94.

    Article  CAS  PubMed  Google Scholar 

  180. Jose RM, Timoney N, Vidyadharan R, Lester R. Syndactyly correction: an aesthetoc reconstruction. J Hand Surg Eur Vol. 2010;35(6):446–50.

    Article  CAS  PubMed  Google Scholar 

  181. Buck-Gramcko D. Congenital malformations: syndactyly and related deformities. In: Higst H, Buck-Gramcko D, Millesi H, et al., editors. Hand surgery. New York: Thieme Medical Publishers; 1988.

    Google Scholar 

  182. Wall LB, Velicki K, Roberts S, Goldfarb CA. Outcomes of pediatric syndactyly repair using synthetic dermal substitute [published online ahead of print, 2020 Feb 12]. J Hand Surg Am. 2020;S0363-5023(19):31544–8.

    Google Scholar 

  183. Jung JJ, Woo AS, Borschel GH. The use of Integra bilaminar dermal regeneration template in Apert syndactyly reconstruction: a novel alternative to simplify care and improve outcomes. J Plast Reconstr Aesthet Surg. 2012;65(1):118e121.

    Article  Google Scholar 

  184. Duteille F, Truffandier MV, Perrot P. 'Matriderm' dermal substitute with split-thickness skin graft compared with full-thickness skin graft for the coverage of skin defects after surgical treatment of congenital syndactyly: results in 40 commissures. J Hand Surg Eur Vol. 2016;41(3):350–1.

    Article  CAS  PubMed  Google Scholar 

  185. Landi A, Garagnani L, Leti Acciaro A, Lando M, Ozben H, Gagliano MC. Hyaluronic acid scaffold for skin defects in congenital syndactyly release surgery: a novel technique based on the regener- ative model. J Hand Surg Eur. 2014;39(9):994–1000.

    Article  CAS  Google Scholar 

  186. Niranjan NS, Azad SM, Fleming ANM, Liew SH. Long-term results of primary syndactyly correction by the trilobed flap technique. Br J Plast Surg. 2005;58:14–21.

    Article  CAS  PubMed  Google Scholar 

  187. Vekris MD, Lykissas MG, Soucacos PN, Korompilias AV, Beris AE. Congenital syndactyly: outcome of surgical treatment in 131 webs. Tech Hand Up Extrem Surg. 2010;14:2–7.

    Article  PubMed  Google Scholar 

  188. Goldfarb CA, Steffen JA, Stutz CM. Complex syndactyly: aesthetic and objective outcomes. J Hand Surg Am. 2012;37:2068–73.

    Article  PubMed  Google Scholar 

  189. Miyamoto J, Nagasao T, Miyamoto S. Biomechanical analysis of surgical correction of syndactyly. Plast Reconstr Surg. 2010;125(3):963–8.

    Article  CAS  PubMed  Google Scholar 

  190. Cromblehome TM, Dirkes K, Whitney TM, Alman B, Garmel S, Connelly RJ. Amniotic band syndrome in fetal lambs: I. Fetoscopic release and morphometric outcome. J Pediatr Surg. 1995;30:974.

    Article  Google Scholar 

  191. Husler MR, Wilson RD, Horri SC, Bebbington MW, Adzick NS, Johnson MP. When is fetoscopic release of amniotic bands indicated? Review of outcome of cases treated in utero and selection criteria for fetal surgery. Prenat Diagn. 2009;29:457–63.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Plastic Surgery, Whiston Hospital, Liverpool, UK

    Daniel J. Jordan

  2. Department of Plastic Surgery, Bedford Hospital, Bedfordshire Hospitals Foundation NHS Trust, Bedford, UK

    Kavish Maheshwari

  3. Department of Plastic Surgery, St. Helens and Knowsley NHS Trust, Liverpool, UK

    Rakhee Nayar

  4. Department of Plastic Surgery, Bedfordshire NHS Trust, Bedford, UK

    Sandip Hindocha

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    Donald R. Laub Jr.

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Jordan, D.J., Maheshwari, K., Nayar, R., Hindocha, S. (2021). Syndactyly. In: Laub Jr., D.R. (eds) Congenital Anomalies of the Upper Extremity. Springer, Cham. https://doi.org/10.1007/978-3-030-64159-7_14

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