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Syndactyly type 4(SDTY4)

MedGen UID:
350013
Concept ID:
C1861355
Disease or Syndrome
Synonyms: HAAS TYPE SYNDACTYLY; Polysyndactyly type Haas; Syndactyly, type IV
SNOMED CT: Syndactyly type 4 (719158007); Haas type syndactyly (719158007)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): LMBR1 (7q36.3)
 
Monarch Initiative: MONDO:0008515
OMIM®: 186200
Orphanet: ORPHA93405

Definition

Syndactyly type IV (SDTY4) is characterized by complete syndactylism of all the fingers accompanied by polydactyly and cup-shaped hands due to flexion of the fingers (summary by Sato et al., 2007). [from OMIM]

Clinical features

From HPO
Polydactyly
MedGen UID:
57774
Concept ID:
C0152427
Congenital Abnormality
A congenital anomaly characterized by the presence of supernumerary fingers or toes.
Postaxial polydactyly
MedGen UID:
67394
Concept ID:
C0220697
Congenital Abnormality
Polydactyly refers to the occurrence of supernumerary digits and is the most frequent of congenital hand and foot deformities. Based on the location of the extra digits, polydactyly can be classified into preaxial, involving the thumb or great toe; postaxial, affecting the fifth digit; and central, involving the 3 central digits. Postaxial polydactyly (PAP) is further subclassified into 2 types: in type A, a well-formed extra digit articulates with the fifth or a sixth metacarpal, whereas in type B, a rudimentary, poorly developed extra digit is present (summary by Umm-e-Kalsoom et al., 2012). Genetic Heterogeneity of Postaxial Polydactyly Other forms of postaxial polydactyly type A include PAPA2 (602085) on chromosome 13q21; PAPA3 (607324) on chromosome 19p13; PAPA4 (608562) on chromosome 7q22; PAPA5 (263450) on chromosome 13q13; PAPA6 (615226), caused by mutation in the ZNF141 gene (194648) on chromosome 4p16; PAPA7 (617642), caused by mutation in the IQCE gene (617631) on chromosome 7p22; PAPA8 (618123), caused by mutation in the GLI1 gene (165220) on chromosome 12q13; PAPA9 (618219), caused by mutation in the CIBAR1 gene (617273) on chromosome 8q22; and PAPA10 (618498), caused by mutation in the KIAA0825 gene (617266) on chromosome 5q15.
Triphalangeal thumb
MedGen UID:
66029
Concept ID:
C0241397
Congenital Abnormality
A thumb with three phalanges in a single, proximo-distal axis. Thus, this term applies if the thumb has an accessory phalanx, leading to a digit like appearance of the thumb.
Supernumerary metacarpal bones
MedGen UID:
639615
Concept ID:
C0545617
Congenital Abnormality
The presence of more than the normal number of metacarpal bones.
1-5 finger complete cutaneous syndactyly
MedGen UID:
350015
Concept ID:
C1861357
Finding
6 metacarpals
MedGen UID:
348337
Concept ID:
C1861360
Finding
1-5 finger cutaneous syndactyly
MedGen UID:
869302
Concept ID:
C4023728
Congenital Abnormality
A soft tissue continuity in the anteroposterior axis between the thumb to the little finger that extends distally to at least the level of the proximal interphalangeal joints (complete syndactyly of all fingers of the hand).
2-3 toe syndactyly
MedGen UID:
1645640
Concept ID:
C4551570
Congenital Abnormality
Syndactyly with fusion of toes two and three.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSyndactyly type 4
Follow this link to review classifications for Syndactyly type 4 in Orphanet.

Professional guidelines

PubMed

Wei X, Huang G, Gui B, Xie B, Chen S, Fan X, Chen Y
Mol Genet Genomic Med 2022 Apr;10(4):e1901. Epub 2022 Mar 2 doi: 10.1002/mgg3.1901. PMID: 35235708Free PMC Article
Biamino E, Canale A, Lacilla M, Marinosci A, Dagna F, Genitori L, Peretta P, Silengo M, Albera R, Ferrero GB
Int J Pediatr Otorhinolaryngol 2016 Jun;85:95-8. Epub 2016 Apr 11 doi: 10.1016/j.ijporl.2016.03.038. PMID: 27240504
Roscioli T, Elakis G, Cox TC, Moon DJ, Venselaar H, Turner AM, Le T, Hackett E, Haan E, Colley A, Mowat D, Worgan L, Kirk EP, Sachdev R, Thompson E, Gabbett M, McGaughran J, Gibson K, Gattas M, Freckmann ML, Dixon J, Hoefsloot L, Field M, Hackett A, Kamien B, Edwards M, Adès LC, Collins FA, Wilson MJ, Savarirayan R, Tan TY, Amor DJ, McGillivray G, White SM, Glass IA, David DJ, Anderson PJ, Gianoutsos M, Buckley MF
Am J Med Genet C Semin Med Genet 2013 Nov;163C(4):259-70. Epub 2013 Oct 11 doi: 10.1002/ajmg.c.31378. PMID: 24127277

Recent clinical studies

Etiology

Yuan F, Zhong L, Chung KC
Plast Reconstr Surg 2018 Oct;142(4):963-971. doi: 10.1097/PRS.0000000000004798. PMID: 29994847Free PMC Article
Hwang JH, Kim HW, Lee DH, Chung JH, Park H
J Hand Surg Eur Vol 2015 Oct;40(8):855-61. Epub 2015 Mar 31 doi: 10.1177/1753193415580066. PMID: 25827142
Huang F, Sweet R, Tewfik TL
Int J Pediatr Otorhinolaryngol 2004 Apr;68(4):495-501. doi: 10.1016/j.ijporl.2003.11.010. PMID: 15013619
Rider MA, Grindel SI, Tonkin MA, Wood VE
J Hand Surg Br 2000 Aug;25(4):376-81. doi: 10.1054/jhsb.2000.0447. PMID: 11058008
Abraham E, Waxman B, Shirali S, Durkin M
J Pediatr Orthop 1999 May-Jun;19(3):404-10. PMID: 10344329

Diagnosis

Schierz IAM, Piro E, Giuffrè M, Pinello G, Angelini A, Antona V, Cimador M, Corsello G
J Matern Fetal Neonatal Med 2022 Dec;35(23):4513-4520. Epub 2020 Dec 1 doi: 10.1080/14767058.2020.1854213. PMID: 36062518
Valente HB, Silva VEDS, Barros TRM, Vanderlei FM, Laurino MJL, Botta AFB, Vanzella LM, Bongiovani AC, Vanderlei LCM
Rev Assoc Med Bras (1992) 2022 Apr;68(4):450-455. doi: 10.1590/1806-9282.20210919. PMID: 35649066
García-Zamora E, Naz-Villalba E, Pampín-Franco A, García-Iñigo FJ, López-Estebaranz JL
Clin Exp Dermatol 2020 Jun;45(4):515-517. Epub 2020 Jan 4 doi: 10.1111/ced.14154. PMID: 31900952
Wang B, Li N, Geng J, Wang Z, Fu Q, Wang J, Xu Y
Congenit Anom (Kyoto) 2017 Jan;57(1):4-7. doi: 10.1111/cga.12173. PMID: 27254532
Sharma A, Patel N, Arora S, Ramachandran R
Acta Anaesthesiol Belg 2014;65(4):179-82. PMID: 25622384

Therapy

Nor AM, Jagdeesh K, Mohd FAS, Kamraul AK, Yusmadi A, Noraslawati R, Malina O, Roziana R
Med J Malaysia 2023 Nov;78(6):756-762. PMID: 38031217
Nopparat J, Khuituan P, Peerakietkhajorn S, Teanpaisan R
PLoS One 2023;18(4):e0284303. Epub 2023 Apr 11 doi: 10.1371/journal.pone.0284303. PMID: 37040355Free PMC Article
Horlenko O, Lenchenko A, Kossey G, Tomey A, Debretseni O
Georgian Med News 2018 Dec;(285):47-51. PMID: 30702069
Mircevsk V, Zogovska E, Chaparoski A, Micunovic M, Filipce V, Mirchevski MM, Kostov M, Мicunovic L
Pril (Makedon Akad Nauk Umet Odd Med Nauki) 2017 Mar 1;38(1):35-40. doi: 10.1515/prilozi-2017-0004. PMID: 28593893
Anderson PJ, Hall CM, Evans RD, Hayward RD, Jones BM
J Pediatr Orthop 1999 Jul-Aug;19(4):504-7. doi: 10.1097/00004694-199907000-00015. PMID: 10413001

Prognosis

Dua H, Edbor A, Asudani B
J Indian Med Assoc 2013 Apr;111(4):268-9, 271. PMID: 24475562
Roscioli T, Elakis G, Cox TC, Moon DJ, Venselaar H, Turner AM, Le T, Hackett E, Haan E, Colley A, Mowat D, Worgan L, Kirk EP, Sachdev R, Thompson E, Gabbett M, McGaughran J, Gibson K, Gattas M, Freckmann ML, Dixon J, Hoefsloot L, Field M, Hackett A, Kamien B, Edwards M, Adès LC, Collins FA, Wilson MJ, Savarirayan R, Tan TY, Amor DJ, McGillivray G, White SM, Glass IA, David DJ, Anderson PJ, Gianoutsos M, Buckley MF
Am J Med Genet C Semin Med Genet 2013 Nov;163C(4):259-70. Epub 2013 Oct 11 doi: 10.1002/ajmg.c.31378. PMID: 24127277
Ramos JM, Davis GJ, Hunsaker JC 3rd, Balko MG
Forensic Sci Med Pathol 2009 Dec;5(4):313-7. Epub 2009 Nov 19 doi: 10.1007/s12024-009-9128-2. PMID: 19924577
Biesecker LG
Orphanet J Rare Dis 2008 Apr 24;3:10. doi: 10.1186/1750-1172-3-10. PMID: 18435847Free PMC Article
Rider MA, Grindel SI, Tonkin MA, Wood VE
J Hand Surg Br 2000 Aug;25(4):376-81. doi: 10.1054/jhsb.2000.0447. PMID: 11058008

Clinical prediction guides

Schierz IAM, Piro E, Giuffrè M, Pinello G, Angelini A, Antona V, Cimador M, Corsello G
J Matern Fetal Neonatal Med 2022 Dec;35(23):4513-4520. Epub 2020 Dec 1 doi: 10.1080/14767058.2020.1854213. PMID: 36062518
Valente HB, Silva VEDS, Barros TRM, Vanderlei FM, Laurino MJL, Botta AFB, Vanzella LM, Bongiovani AC, Vanderlei LCM
Rev Assoc Med Bras (1992) 2022 Apr;68(4):450-455. doi: 10.1590/1806-9282.20210919. PMID: 35649066
Raykova D, Klar J, Azhar A, Khan TN, Malik NA, Iqbal M, Tariq M, Baig SM, Dahl N
PLoS One 2014;9(4):e93607. Epub 2014 Apr 8 doi: 10.1371/journal.pone.0093607. PMID: 24714551Free PMC Article
El Ghouzzi V, Lajeunie E, Le Merrer M, Cormier-Daire V, Renier D, Munnich A, Bonaventure J
Eur J Hum Genet 1999 Jan;7(1):27-33. doi: 10.1038/sj.ejhg.5200240. PMID: 10094188
Sarfarazi M, Akarsu AN, Sayli BS
Hum Mol Genet 1995 Aug;4(8):1453-8. doi: 10.1093/hmg/4.8.1453. PMID: 7581388

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