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Ulnar deviation of finger

MedGen UID:
68543
Concept ID:
C0231679
Finding
Synonyms: Ulnar deviation of fingers; Ulnar deviation of the fingers; Ulnar drift
SNOMED CT: Ulnar deviation of fingers (367510009); Ulnar drift (367510009)
 
HPO: HP:0009465

Definition

Bending or curvature of a finger toward the ulnar side (i.e., away from the thumb). The deviation is at the metacarpal-phalangeal joint, and this finding is distinct from clinodactyly. [from HPO]

Conditions with this feature

Congenital contractural arachnodactyly
MedGen UID:
67391
Concept ID:
C0220668
Congenital Abnormality
Congenital contractural arachnodactyly (CCA) appears to comprise a broad phenotypic spectrum. Classic CCA is characterized by arachnodactyly; flexion contractures of multiple joints including elbows, knees, hips, ankles, and/or fingers; kyphoscoliosis (usually progressive); a marfanoid habitus (a long and slender build, dolichostenomelia, pectus deformity, muscular hypoplasia, highly arched palate); and abnormal "crumpled" ears. At the mildest end, parents who are diagnosed retrospectively upon evaluation of their more severely affected child may show a lean body build, mild arachnodactyly, mild contractures without impairment, and minor ear abnormalities. At the most severe end is "severe CCA with cardiovascular and/or gastrointestinal anomalies," a rare phenotype in infants with pronounced features of CCA (severe crumpling of the ears, arachnodactyly, contractures, congenital scoliosis, and/or hypotonia) and severe cardiovascular and/or gastrointestinal anomalies. Phenotypic expression can vary within and between families.
Diastrophic dysplasia
MedGen UID:
113103
Concept ID:
C0220726
Disease or Syndrome
Diastrophic dysplasia (DTD) is characterized by limb shortening, normal-sized head, hitchhiker thumbs, spinal deformities (scoliosis, exaggerated lumbar lordosis, cervical kyphosis), and contractures of the large joints with deformities and early-onset osteoarthritis. Other typical findings are ulnar deviation of the fingers, gap between the first and second toes, and clubfoot. On occasion, the disease can be lethal at birth, but most affected individuals survive the neonatal period and develop physical limitations with normal intelligence.
Autosomal recessive multiple pterygium syndrome
MedGen UID:
82696
Concept ID:
C0265261
Congenital Abnormality
Multiple pterygium syndromes comprise a group of multiple congenital anomaly disorders characterized by webbing (pterygia) of the neck, elbows, and/or knees and joint contractures (arthrogryposis) (Morgan et al., 2006). The multiple pterygium syndromes are phenotypically and genetically heterogeneous but are traditionally divided into prenatally lethal (253290) and nonlethal (Escobar) types.
C syndrome
MedGen UID:
167105
Concept ID:
C0796095
Disease or Syndrome
The C syndrome, also known as Opitz trigonocephaly syndrome, is a malformation syndrome characterized by trigonocephaly, severe mental retardation, hypotonia, variable cardiac defects, redundant skin, and dysmorphic facial features, including upslanted palpebral fissures, epicanthal folds, depressed nasal bridge, and low-set, posteriorly rotated ears (summary by Kaname et al., 2007). C syndrome shows phenotypic overlap with Bohring-Opitz syndrome, or C-like syndrome (605039), a disorder with more severe features than C syndrome, caused by heterozygous mutation in the ASXL1 gene (612990) on chromosome 20q11.
Epiphyseal dysplasia, Baumann type
MedGen UID:
322764
Concept ID:
C1835830
Disease or Syndrome
Whistling face syndrome, recessive form
MedGen UID:
376364
Concept ID:
C1848470
Disease or Syndrome
Whistling face syndrome is characterized by an atypical facial appearance with anomalies of the hands and feet. Most cases show autosomal dominant inheritance: see distal arthrogryposis 2A (DA2A; 193700). There are rare reports of presumably autosomal recessive inheritance (summary by Altunhan et al., 2010).
Digitotalar dysmorphism; ulnar drift, hereditary
MedGen UID:
342156
Concept ID:
C1852085
Disease or Syndrome
Fuhrmann syndrome
MedGen UID:
346429
Concept ID:
C1856728
Disease or Syndrome
This syndrome has main characteristics of bowing of the femora, aplasia or hypoplasia of the fibulae and poly, oligo and syndactyly. It has been reported in 11 patients. Most of the patients also had a hypoplastic pelvis and hypoplasia of the fingers and fingernails. Some had congenital dislocation of the hip, absence or fusion of tarsal bones, absence of various metatarsals and hypoplasia and aplasia of the toes. The syndrome is caused by a partial loss of WNT7A function (gene mapped to 3p25).
Microcephalic osteodysplastic primordial dwarfism, type 3
MedGen UID:
349167
Concept ID:
C1859439
Disease or Syndrome
COG1 congenital disorder of glycosylation
MedGen UID:
443957
Concept ID:
C2931011
Disease or Syndrome
An extremely rare form of carbohydrate deficient glycoprotein syndrome with, in the few cases reported to date, variable signs including microcephaly, growth retardation, psychomotor retardation and facial dysmorphism.
Cardiac, facial, and digital anomalies with developmental delay
MedGen UID:
1648330
Concept ID:
C4748484
Disease or Syndrome
CAFDADD is a multisystemic developmental disorder with variable cardiac and digital anomalies and facial dysmorphism. Some patients may have seizures and ocular/aural abnormalities (Tokita et al., 2018).
Stüve-Wiedemann syndrome 1
MedGen UID:
1803541
Concept ID:
C5676888
Disease or Syndrome
Stuve-Wiedemann syndrome is an autosomal recessive disorder characterized by bowing of the long bones and other skeletal anomalies, episodic hyperthermia, respiratory distress, and feeding difficulties usually resulting in early death (Dagoneau et al., 2004). See also 'classic' Schwartz-Jampel syndrome type 1 (SJS1; 255800), a phenotypically similar but genetically distinct disorder caused by mutation in the HSPG2 gene (142461) on chromosome 1p36. Genetic Heterogeneity of Stuve-Wiedemann Syndrome Stuve-Wiedemann syndrome-2 (STWS2; 619751) is caused by mutation in the IL6ST gene (600694) on chromosome 5q11.

Professional guidelines

PubMed

George S, Rizkallah M, Leviet D, Leclercq C, El Abiad R
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Rahimi F, Bee C, Debicki D, Roberts AC, Bapat P, Jog M
Can J Neurol Sci 2013 Sep;40(5):663-9. doi: 10.1017/s031716710001489x. PMID: 23968939
Sebastin SJ, Chung KC
J Pediatr Rehabil Med 2011;4(2):119-30. doi: 10.3233/PRM-2011-0169. PMID: 21955970

Recent clinical studies

Etiology

Suzuki A, Kawabata H, Hayashi J, Tamura D
J Hand Surg Asian Pac Vol 2019 Mar;24(1):17-23. doi: 10.1142/S2424835519500048. PMID: 30760140
Bertelli JA, Tavares KE
Hand Surg Rehabil 2018 Dec;37(6):368-371. Epub 2018 Oct 23 doi: 10.1016/j.hansur.2018.09.006. PMID: 30361046
Oster LH, Blair WF, Steyers CM, Flatt AE
J Hand Surg Am 1989 Nov;14(6):963-71. doi: 10.1016/s0363-5023(89)80045-0. PMID: 2584657
Linscheid RL, Dobyns JH
J Hand Surg Am 1985 Nov;10(6 Pt 1):821-9. doi: 10.1016/s0363-5023(85)80157-x. PMID: 4078264
Ogden JA, Watson HK, Bohne W
J Bone Joint Surg Am 1976 Jun;58(4):467-75. PMID: 818089

Diagnosis

Richards T, Ingham L, Russell I, Newington D
Hand (N Y) 2022 Mar;17(2):266-270. Epub 2020 May 26 doi: 10.1177/1558944720921468. PMID: 32452216Free PMC Article
Han B, Shen K, Wang Z, Xu Y
J Pediatr Orthop 2021 Jan;41(1):28-32. doi: 10.1097/BPO.0000000000001679. PMID: 33086366Free PMC Article
Suzuki A, Kawabata H, Hayashi J, Tamura D
J Hand Surg Asian Pac Vol 2019 Mar;24(1):17-23. doi: 10.1142/S2424835519500048. PMID: 30760140
Bertelli JA, Tavares KE
Hand Surg Rehabil 2018 Dec;37(6):368-371. Epub 2018 Oct 23 doi: 10.1016/j.hansur.2018.09.006. PMID: 30361046
Schroeder NS, Goldfarb CA
Clin Sports Med 2015 Jan;34(1):117-26. Epub 2014 Oct 11 doi: 10.1016/j.csm.2014.09.004. PMID: 25455399

Therapy

Han B, Shen K, Wang Z, Xu Y
J Pediatr Orthop 2021 Jan;41(1):28-32. doi: 10.1097/BPO.0000000000001679. PMID: 33086366Free PMC Article
Woo EHC, White P, Lai CWK
J Hand Surg Am 2019 Jul;44(7):610.e1-610.e15. Epub 2018 Oct 6 doi: 10.1016/j.jhsa.2018.08.006. PMID: 30301644
Lankachandra M, Eggers JP, Bogener JW, Hutchison RL
J Hand Surg Asian Pac Vol 2017 Sep;22(3):350-354. doi: 10.1142/S0218810417500411. PMID: 28774254
Rijnja JP, Kouwenberg PPGM, Ray S, Walbeehm ET
Arch Orthop Trauma Surg 2017 Aug;137(8):1155-1160. Epub 2017 Jun 12 doi: 10.1007/s00402-017-2725-8. PMID: 28608275
Massy-Westropp N, Johnston RV, Hill C
Cochrane Database Syst Rev 2008 Jan 23;2008(1):CD003522. doi: 10.1002/14651858.CD003522.pub2. PMID: 18254021Free PMC Article

Prognosis

Saito S, Makino A, Yamanaka H, Tsuge I, Morimoto N
J Hand Surg Eur Vol 2023 Jan;48(1):10-19. Epub 2022 Oct 6 doi: 10.1177/17531934221126864. PMID: 36204982
Suzuki A, Kawabata H, Hayashi J, Tamura D
J Hand Surg Asian Pac Vol 2019 Mar;24(1):17-23. doi: 10.1142/S2424835519500048. PMID: 30760140
Oster LH, Blair WF, Steyers CM, Flatt AE
J Hand Surg Am 1989 Nov;14(6):963-71. doi: 10.1016/s0363-5023(89)80045-0. PMID: 2584657
Linscheid RL, Dobyns JH
J Hand Surg Am 1985 Nov;10(6 Pt 1):821-9. doi: 10.1016/s0363-5023(85)80157-x. PMID: 4078264
Ogden JA, Watson HK, Bohne W
J Bone Joint Surg Am 1976 Jun;58(4):467-75. PMID: 818089

Clinical prediction guides

Ku KH, Yoon JO, Kim HY, Shin YH, Kim JK
J Hand Surg Am 2023 Aug;48(8):829.e1-829.e9. Epub 2022 Mar 27 doi: 10.1016/j.jhsa.2022.01.029. PMID: 35354533
Richards T, Ingham L, Russell I, Newington D
Hand (N Y) 2022 Mar;17(2):266-270. Epub 2020 May 26 doi: 10.1177/1558944720921468. PMID: 32452216Free PMC Article
Han B, Shen K, Wang Z, Xu Y
J Pediatr Orthop 2021 Jan;41(1):28-32. doi: 10.1097/BPO.0000000000001679. PMID: 33086366Free PMC Article
Suzuki A, Kawabata H, Hayashi J, Tamura D
J Hand Surg Asian Pac Vol 2019 Mar;24(1):17-23. doi: 10.1142/S2424835519500048. PMID: 30760140
Ogden JA, Watson HK, Bohne W
J Bone Joint Surg Am 1976 Jun;58(4):467-75. PMID: 818089

Recent systematic reviews

Massy-Westropp N, Johnston RV, Hill C
Cochrane Database Syst Rev 2008 Jan 23;2008(1):CD003522. doi: 10.1002/14651858.CD003522.pub2. PMID: 18254021Free PMC Article

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