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Tibial hemimelia

MedGen UID:
120551
Concept ID:
C0265633
Congenital Abnormality
Synonyms: Absence of Tibia; Bilateral absence of the tibia
SNOMED CT: Congenital absence of tibia (79177001); Tibial hemimelia (79177001); Absence of tibia (275346000); Agenesis of tibia (1003515004)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
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 individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
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.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0010144
OMIM®: 275220
Orphanet: ORPHA93322

Definition

Tibial hemimelia is a rare anomaly characterized by deficiency of the tibia with relatively intact fibula. Jones et al. (1978) classified the anomaly into 4 types according to radiologic criteria. It may present as an isolated anomaly or be associated with a variety of skeletal and extraskeletal malformations. Tibial hemimelia may also constitute a part of a more complicated malformation complex or syndrome, such as the Gollop-Wolfgang complex (228250) and triphalangeal thumb-polysyndactyly syndrome (see 174500 and 188740) (Matsuyama et al., 2003). McKay et al. (1984) reviewed syndromes of congenital defects in which tibial hemimelia is a feature. [from OMIM]

Clinical features

From HPO
Absent tibia
MedGen UID:
478374
Concept ID:
C3276744
Finding
Absence of the tibia.
Abnormality of the skeletal system
MedGen UID:
867418
Concept ID:
C4021790
Anatomical Abnormality
An abnormality of the skeletal system.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTibial hemimelia
Follow this link to review classifications for Tibial hemimelia in Orphanet.

Recent clinical studies

Etiology

Paththinige CS, Sirisena ND, Escande F, Manouvrier S, Petit F, Dissanayake VHW
BMC Med Genet 2019 Jun 14;20(1):108. doi: 10.1186/s12881-019-0839-2. PMID: 31200655Free PMC Article
Radhakrishna VN, Madhuri V, Palocaren T
J Pediatr Orthop B 2019 Mar;28(2):144-152. doi: 10.1097/BPB.0000000000000540. PMID: 30234726
Simmons ED Jr, Ginsburg GM, Hall JE
J Pediatr Orthop 1996 Jan-Feb;16(1):85-9. doi: 10.1097/00004694-199601000-00017. PMID: 8747361
Schoenecker PL, Capelli AM, Millar EA, Sheen MR, Haher T, Aiona MD, Meyer LC
J Bone Joint Surg Am 1989 Feb;71(2):278-87. PMID: 2918013
Richieri-Costa A
Am J Med Genet 1987 Oct;28(2):325-9. doi: 10.1002/ajmg.1320280209. PMID: 3425613

Diagnosis

Chouery E, Tahan E, Karam R, Pharoun J, Mehawej C, Megarbane A
Am J Med Genet A 2023 Apr;191(4):923-929. Epub 2022 Dec 24 doi: 10.1002/ajmg.a.63094. PMID: 36565049
Kumar Sahoo P, Sahu MM, Prasad Das S
Eur J Med Genet 2019 Jul;62(7):103666. Epub 2019 May 10 doi: 10.1016/j.ejmg.2019.05.005. PMID: 31078790
Radhakrishna VN, Madhuri V, Palocaren T
J Pediatr Orthop B 2019 Mar;28(2):144-152. doi: 10.1097/BPB.0000000000000540. PMID: 30234726
Deimling S, Sotiropoulos C, Lau K, Chaudhry S, Sturgeon K, Kelley S, Narayanan U, Howard A, Hui CC, Hopyan S
J Hum Genet 2016 May;61(5):443-6. Epub 2016 Jan 21 doi: 10.1038/jhg.2015.161. PMID: 26791356
Cho TJ, Baek GH, Lee HR, Moon HJ, Yoo WJ, Choi IH
J Pediatr Orthop B 2013 May;22(3):219-21. doi: 10.1097/BPB.0b013e32835106b2. PMID: 22314436

Therapy

Desai MH, Fyfe N
J Pediatr Orthop B 2009 Nov;18(6):339-40. doi: 10.1097/BPB.0b013e32832feeff. PMID: 19701103
Pandit PB, Chitayat D, Jefferies AL, Landes A, Qamar IU, Koren G
Reprod Toxicol 1994 Jan-Feb;8(1):89-92. doi: 10.1016/0890-6238(94)90072-8. PMID: 8186630
Balog B, Skinner SR
J Pediatr Orthop 1984 Aug;4(4):488-90. doi: 10.1097/01241398-198408000-00021. PMID: 6470123

Prognosis

Radhakrishna VN, Madhuri V, Palocaren T
J Pediatr Orthop B 2019 Mar;28(2):144-152. doi: 10.1097/BPB.0000000000000540. PMID: 30234726
Bergère A, Amzallag-Bellenger E, Lefebvre G, Dieux-Coeslier A, Mezel A, Herbaux B, Boutry N
Diagn Interv Imaging 2015 Sep;96(9):901-14. Epub 2015 Apr 24 doi: 10.1016/j.diii.2014.08.008. PMID: 25920687
Simmons ED Jr, Ginsburg GM, Hall JE
J Pediatr Orthop 1996 Jan-Feb;16(1):85-9. doi: 10.1097/00004694-199601000-00017. PMID: 8747361
Dreyfus M, Baldauf JJ, Rigaut E, Clavert JM, Gasser B, Ritter J
Ultrasound Obstet Gynecol 1996 Mar;7(3):205-7. doi: 10.1046/j.1469-0705.1996.07030205.x. PMID: 8705415
Schoenecker PL, Capelli AM, Millar EA, Sheen MR, Haher T, Aiona MD, Meyer LC
J Bone Joint Surg Am 1989 Feb;71(2):278-87. PMID: 2918013

Clinical prediction guides

Morel G, Duhamel C, Boussion S, Frénois F, Lesca G, Chatron N, Labalme A, Sanlaville D, Edery P, Thevenon J, Faivre L, Fassier A, Prodhomme O, Escande F, Manouvrier S, Petit F, Geneviève D, Rossi M
Hum Mutat 2020 Sep;41(9):1499-1506. Epub 2020 Jul 15 doi: 10.1002/humu.24070. PMID: 32598510
Tsai A, Laor T, Estroff JA, Kasser JR
Pediatr Radiol 2018 Sep;48(10):1451-1462. Epub 2018 May 24 doi: 10.1007/s00247-018-4153-5. PMID: 29797037
Shahcheraghi GH, Javid M
J Pediatr Orthop 2016 Sep;36(6):572-81. doi: 10.1097/BPO.0000000000000513. PMID: 26019025
Wada A, Nakamura T, Urano N, Kubota H, Oketani Y, Taketa M, Fujii T
J Pediatr Orthop B 2015 Mar;24(2):147-53. doi: 10.1097/BPB.0000000000000149. PMID: 25622236
Dreyfus M, Baldauf JJ, Rigaut E, Clavert JM, Gasser B, Ritter J
Ultrasound Obstet Gynecol 1996 Mar;7(3):205-7. doi: 10.1046/j.1469-0705.1996.07030205.x. PMID: 8705415

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