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Syndactyly type 5(SDTY5)

MedGen UID:
350010
Concept ID:
C1861348
Congenital Abnormality
Synonyms: Syndactyly with associated metacarpal and metatarsal fusion; SYNDACTYLY WITH METACARPAL AND METATARSAL FUSION; Syndactyly, type V
SNOMED CT: Syndactyly type 5 (719159004)
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): HOXD13 (2q31.1)
 
Monarch Initiative: MONDO:0008516
OMIM®: 186300
Orphanet: ORPHA93406

Definition

A very rare congenital limb malformation with characteristics of postaxial syndactyly of hands and feet, associated with metacarpal and metatarsal fusion of fourth and fifth digits. So far, less than ten reports have been described in the literature. Soft tissue syndactyly (involving the third and fourth fingers and the second and third toes) may be present. The locus associated with SD5 maps to 2q31-q32. Mutations in the HOXD13 gene may be causative. The condition is inherited as an autosomal dominant trait. [from SNOMEDCT_US]

Clinical features

From HPO
Brachydactyly
MedGen UID:
67454
Concept ID:
C0221357
Congenital Abnormality
Digits that appear disproportionately short compared to the hand/foot. The word brachydactyly is used here to describe a series distinct patterns of shortened digits (brachydactyly types A-E). This is the sense used here.
3-4 toe syndactyly
MedGen UID:
371723
Concept ID:
C1834062
Finding
Syndactyly with fusion of toes three and four.
4-5 toe syndactyly
MedGen UID:
324891
Concept ID:
C1837836
Finding
Syndactyly with fusion of toes four and five.
Short distal phalanx of finger
MedGen UID:
326590
Concept ID:
C1839829
Finding
Short distance from the end of the finger to the most distal interphalangeal crease or the distal interphalangeal joint flexion point. That is, hypoplasia of one or more of the distal phalanx of finger.
4-5 metacarpal synostosis
MedGen UID:
395341
Concept ID:
C1859768
Anatomical Abnormality
Absent distal interphalangeal creases
MedGen UID:
350011
Concept ID:
C1861349
Finding
Absence of the distal interphalangeal flexion creases of the fingers.
Enlarged proximal interphalangeal joints
MedGen UID:
348334
Concept ID:
C1861350
Finding
Cutaneous finger syndactyly
MedGen UID:
866898
Concept ID:
C4021254
Congenital Abnormality
A soft tissue continuity in the A/P axis between two fingers that extends distally to at least the level of the proximal interphalangeal joints, or a soft tissue continuity in the A/P axis between two fingers that lies significantly distal to the flexion crease that overlies the metacarpophalangeal joint of the adjacent fingers.
Joint contracture of the hand
MedGen UID:
56382
Concept ID:
C0158113
Finding
Contractures of one ore more joints of the hands meaning chronic loss of joint motion due to structural changes in non-bony tissue.
Camptodactyly of finger
MedGen UID:
98041
Concept ID:
C0409348
Finding
The distal interphalangeal joint and/or the proximal interphalangeal joint of the fingers cannot be extended to 180 degrees by either active or passive extension.
Carpal synostosis
MedGen UID:
98468
Concept ID:
C0431863
Congenital Abnormality
Synostosis (bony fusion) involving one or more bones of the carpus (scaphoid, lunate, triquetrum, trapezium, trapezoid, capitate, hamate, pisiform).

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSyndactyly type 5
Follow this link to review classifications for Syndactyly type 5 in Orphanet.

Professional guidelines

PubMed

Theman TA, Upton J, Taghinia AH, Firriolo JM, Nuzzi LC, Labow BI
J Hand Surg Am 2018 Nov;43(11):1042.e1-1042.e8. Epub 2018 Jun 8 doi: 10.1016/j.jhsa.2018.03.050. PMID: 29891270
Fearon JA, Podner C
Plast Reconstr Surg 2013 Jan;131(1):132-142. doi: 10.1097/PRS.0b013e3182729f42. PMID: 23271523
Lumenta DB, Kitzinger HB, Beck H, Frey M
J Hand Surg Am 2010 Aug;35(8):1323-9. Epub 2010 Jul 16 doi: 10.1016/j.jhsa.2010.04.033. PMID: 20638200

Recent clinical studies

Etiology

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
Hermida A, Jedraszak G, Kubala M, Mathiron A, Berna P, Bennis Y, Hermida JS
Gene 2021 Apr 20;777:145465. Epub 2021 Jan 29 doi: 10.1016/j.gene.2021.145465. PMID: 33524520
Baek GH, Kim J
J Hand Surg Asian Pac Vol 2016 Oct;21(3):283-91. doi: 10.1142/S2424835516400087. PMID: 27595943
Stoler JM, Rosen H, Desai U, Mulliken JB, Meara JG, Rogers GF
J Craniofac Surg 2009 Sep;20(5):1375-7. doi: 10.1097/SCS.0b013e3181ae42e4. PMID: 19816260
Colombani PM
Semin Pediatr Surg 2003 May;12(2):94-9. doi: 10.1016/s1055-8586(02)00018-5. PMID: 12728394

Diagnosis

Levy RJ, Timothy KW, Underwood JFG, Hall J, Bernstein JA, Pașca SP
Pediatr Neurol 2023 Jan;138:101-106. Epub 2022 Nov 2 doi: 10.1016/j.pediatrneurol.2022.10.013. PMID: 36436328
McVeigh TP, Soye JA, Gordon E, Lynch SA
Am J Med Genet A 2018 May;176(5):1180-1183. Epub 2018 Feb 10 doi: 10.1002/ajmg.a.38632. PMID: 29427337
Stoler JM, Rosen H, Desai U, Mulliken JB, Meara JG, Rogers GF
J Craniofac Surg 2009 Sep;20(5):1375-7. doi: 10.1097/SCS.0b013e3181ae42e4. PMID: 19816260
Cerruti Mainardi P
Orphanet J Rare Dis 2006 Sep 5;1:33. doi: 10.1186/1750-1172-1-33. PMID: 16953888Free PMC Article
Colombani PM
Semin Pediatr Surg 2003 May;12(2):94-9. doi: 10.1016/s1055-8586(02)00018-5. PMID: 12728394

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
Gao R, Yan H, Duan J, Gao Y, Cao C, Li L, Guo L
Sci Rep 2022 Mar 22;12(1):4858. doi: 10.1038/s41598-022-08705-z. PMID: 35318355Free PMC Article
Hermida A, Jedraszak G, Kubala M, Bourgain M, Bodeau S, Hermida JS
Rev Esp Cardiol (Engl Ed) 2022 May;75(5):447-448. Epub 2021 Nov 26 doi: 10.1016/j.rec.2021.11.003. PMID: 34844894
Lolis MS, Bowe WP, Shalita AR
Med Clin North Am 2009 Nov;93(6):1161-81. doi: 10.1016/j.mcna.2009.08.008. PMID: 19932324
Cornejo-Roldan LR, Roessler E, Muenke M
Hum Genet 1999 May;104(5):425-31. doi: 10.1007/s004390050979. PMID: 10394936

Prognosis

Hermida A, Jedraszak G, Kubala M, Mathiron A, Berna P, Bennis Y, Hermida JS
Gene 2021 Apr 20;777:145465. Epub 2021 Jan 29 doi: 10.1016/j.gene.2021.145465. PMID: 33524520
Nur BG, Pehlivanoğlu S, Mıhçı E, Calışkan M, Demir D, Alper OM, Kayserili H, Lüleci G
Pediatr Neurol 2014 May;50(5):482-90. Epub 2014 Jan 11 doi: 10.1016/j.pediatrneurol.2014.01.023. PMID: 24656465
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
Cerruti Mainardi P
Orphanet J Rare Dis 2006 Sep 5;1:33. doi: 10.1186/1750-1172-1-33. PMID: 16953888Free PMC Article

Clinical prediction guides

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
Baek GH, Kim J
J Hand Surg Asian Pac Vol 2016 Oct;21(3):283-91. doi: 10.1142/S2424835516400087. PMID: 27595943
Wang B, Xu B, Cheng Z, Zhou X, Wang J, Yang G, Cheng L, Yang J, Ma X
Clin Chim Acta 2012 Jul 11;413(13-14):1049-52. Epub 2012 Feb 22 doi: 10.1016/j.cca.2012.02.015. PMID: 22374128
Sarfarazi M, Akarsu AN, Sayli BS
Hum Mol Genet 1995 Aug;4(8):1453-8. doi: 10.1093/hmg/4.8.1453. PMID: 7581388
Rasmussen SA, Frias JL
Clin Genet 1988 Jan;33(1):5-10. doi: 10.1111/j.1399-0004.1988.tb04258.x. PMID: 3342547

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