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Gollop-Wolfgang complex(GWC)

MedGen UID:
341622
•Concept ID:
C1856789
•
Disease or Syndrome
Synonyms: Femur bifid with monodactylous ectrodactyly; FEMUR, UNILATERAL BIFID, WITH MONODACTYLOUS ECTRODACTYLY
SNOMED CT: Bifid femur co-occurrent with monodactylous ectrodactyly (716006003); Gollop Wolfgang complex (716006003); Bifid femur with monodactylous ectrodactyly (716006003)
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.
Autosomal recessive inheritance (Orphanet)
Autosomal dominant inheritance (Orphanet)
 
Monarch Initiative: MONDO:0009222
OMIM®: 228250
Orphanet: ORPHA1986

Definition

A very rare malformation with main features of ectrodactyly of the hand and ipsilateral bifurcation of the femur. Approximately 200 cases have been reported worldwide. Congenital aplasia/hypoplasia of the tibia, accompanied by pre-axial oligodactyly or monodactyly of the feet, may also be present. In most cases, the bifurcation of the distal femur is unilateral. Patients are often small. Autosomal dominant and autosomal recessive modes of transmission have been suggested. [from SNOMEDCT_US]

Clinical features

From HPO
Aplasia of the ulna
MedGen UID:
394508
•Concept ID:
C2678397
•
Finding
Missing ulna bone associated with congenital failure of development.
See: Feature record | Search on this feature
Split hand
MedGen UID:
397570
•Concept ID:
C2699510
•
Congenital Abnormality
A condition in which middle parts of the hand (fingers and metacarpals) are missing giving a cleft appearance. The severity is very variable ranging from slightly hypoplastic middle fingers over absent middle fingers as far as oligo- or monodactyl hands.
See: Feature record | Search on this feature
Absent tibia
MedGen UID:
478374
•Concept ID:
C3276744
•
Finding
Absence of the tibia.
See: Feature record | Search on this feature
Foot monodactyly
MedGen UID:
867513
•Concept ID:
C4021896
•
Anatomical Abnormality
See: Feature record | Search on this feature
Bifid femur
MedGen UID:
869397
•Concept ID:
C4023824
•
Anatomical Abnormality
A bifid or bifurcated appearance of the femur as seen on x-rays, possible appearing as a more or less severe bowing of the upper leg. Might be associated with hip dysplasia on the affected side.
See: Feature record | Search on this feature
Hand monodactyly
MedGen UID:
870952
•Concept ID:
C4025415
•
Congenital Abnormality
See: Feature record | Search on this feature

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGollop-Wolfgang complex
Follow this link to review classifications for Gollop-Wolfgang complex in Orphanet.

Recent clinical studies

Etiology

Limb salvage treatment for Gollop-Wolfgang complex (femoral bifurcation, complete tibial hemimelia, and hand ectrodactyly).
Wada A, Nakamura T, Fujii T, Urano N, Yanagida H, Takamura K, Taketa M, Oketani Y, Kubota H
J Pediatr Orthop B 2013 Sep;22(5):457-63. doi: 10.1097/BPB.0b013e3283620640. PMID: 23660549
Tibial developmental field defect in valproic acid embryopathy: Report on three cases.
Alessandri JL, Isidor B, David A, Martin-Coignard D, Ghazouani J, Ramful D, Laville JM, Le Caignec C
Am J Med Genet A 2010 Nov;152A(11):2805-9. doi: 10.1002/ajmg.a.33633. PMID: 20949521
A 5-year follow-up study after knee disarticulation in two cases of Gollop-Wolfgang complex.
Bos CF, Taminiau AH
J Pediatr Orthop B 2007 Nov;16(6):409-13. doi: 10.1097/BPB.0b013e3282f057f3. PMID: 17909338
Tibial agenesis, femoral duplication, and caudal midline anomalies.
Evans JA, Chudley AE
Am J Med Genet 1999 Jul 2;85(1):13-9. doi: 10.1002/(sici)1096-8628(19990702)85:1<13::aid-ajmg5>3.0.co;2-n. PMID: 10377008

Diagnosis

Femoral bifurcation and bilateral tibial hemimelia: case report.
Ondari J, Kinyanjui J, Miano P, Sang E, Oburu E, Maru M
Pan Afr Med J 2018;30:99. Epub 2018 Jun 5 doi: 10.11604/pamj.2018.30.99.11969. PMID: 30344883Free PMC Article
Limb salvage treatment for Gollop-Wolfgang complex (femoral bifurcation, complete tibial hemimelia, and hand ectrodactyly).
Wada A, Nakamura T, Fujii T, Urano N, Yanagida H, Takamura K, Taketa M, Oketani Y, Kubota H
J Pediatr Orthop B 2013 Sep;22(5):457-63. doi: 10.1097/BPB.0b013e3283620640. PMID: 23660549
Tibial hemimelia and femoral bifurcation.
Ugras AA, Sungur I, Akyildiz MF, Ercin E
Orthopedics 2010 Feb;33(2):124-6. doi: 10.3928/01477447-20100104-30. PMID: 20192156
Prenatal diagnosis of Gollop-Wolfgang complex (tibial agenesis and femoral bifurcation).
Mendilcioglu I, Mihci E, Pestereli E, Simsek M
Prenat Diagn 2009 Feb;29(2):182-6. doi: 10.1002/pd.2204. PMID: 19180625

Therapy

Tibial developmental field defect in valproic acid embryopathy: Report on three cases.
Alessandri JL, Isidor B, David A, Martin-Coignard D, Ghazouani J, Ramful D, Laville JM, Le Caignec C
Am J Med Genet A 2010 Nov;152A(11):2805-9. doi: 10.1002/ajmg.a.33633. PMID: 20949521

Prognosis

Limb salvage treatment for Gollop-Wolfgang complex (femoral bifurcation, complete tibial hemimelia, and hand ectrodactyly).
Wada A, Nakamura T, Fujii T, Urano N, Yanagida H, Takamura K, Taketa M, Oketani Y, Kubota H
J Pediatr Orthop B 2013 Sep;22(5):457-63. doi: 10.1097/BPB.0b013e3283620640. PMID: 23660549
Prenatal diagnosis of Gollop-Wolfgang complex (tibial agenesis and femoral bifurcation).
Mendilcioglu I, Mihci E, Pestereli E, Simsek M
Prenat Diagn 2009 Feb;29(2):182-6. doi: 10.1002/pd.2204. PMID: 19180625
A 5-year follow-up study after knee disarticulation in two cases of Gollop-Wolfgang complex.
Bos CF, Taminiau AH
J Pediatr Orthop B 2007 Nov;16(6):409-13. doi: 10.1097/BPB.0b013e3282f057f3. PMID: 17909338

Supplemental Content

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    • ClinVar
      Related medical variations
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    • OMIM
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