A case report of arthrogryposis multiplex congenita in monochorionic biamniotic twins
DOI:
https://doi.org/10.18597/rcog.357Keywords:
arthrogryposis, diseases in twins, birth defectAbstract
Arthrogryposis multiplex congenita (AMC) is a group of rarely occurring musculoskeletal disorders, characterised by multiple joint contractures affecting the muscles of the upper and lower limbs and the back. This condition’s aetiology remains unknown and many causal agents have been implicated, particularly viral infections and intra-uterine restrictive conditions.
Objective: contributing a case of AMC in a monochorionic biamniotic twin pregnancy to the literature and reviewing this congenital malformation’s aetiology and physiopathogeny.
Case presentation: a second-born male twin, weighing 1345 gr, shortening of the four limbs in all segments was observed, accompanied by joint contractures leading to marked hand and foot deviation towards the mid line and the impossibility of active movement.
Discussion: AMC being displayed in one of the identical twins mentioned in the present report suggests that this condition did not take place because of chromosomal alteration or a hereditary disease. Rather, it was presented because of one or several external factors (probably mechanical) affecting the foetus which may have jeopardised the normal development of the joints and led to AMC appearing. This is the fourth report of similar characteristics in the literature reviewed.
Author Biographies
Felipe Ruiz-Botero
Harry Pachajoa
Wilmar Saldarriaga
Carolina Isaza
References
Alves PV, Zhao L, Patel PK, Bolognese AM. Arthrogryposis: diagnosis and therapeutic planning for patients seeking orthodontic treatment or orthognathic surgery. J Craniofac Surg 2007;18:838-43.
Mennen U. Arthrogryposis multiplex congenita: functional classification and the AMC disc-o-gram. J Hand Surg 2004;29:363-7.
López R, Gutiérrez M, Quero O, Méndez M. Artrogriposis múltiple congénita. Reporte de un caso. MedULA 2003;9:61-4.
Mennen U, Williams E. Arthrogryposis multiplex congenita in a monozygotic twin an intrauterine lesion? J Hand Surg 1996;21:647-8.
Hillman JW, Jonson TH. Arthrogryposis multiplex congenita in twins. J Bone Joint Surg Am1952;34:211-4.
Bürglen L, Amiel J, Viollet L, Lefebvre S, Burlet P, Clermont O, et al. Survival motor neuron gene deletion in the arthrogryposis multiplex congenitaspinal muscular atrophy association. J Clin Invest 1996;98:1130-2.
Mennen U, van Heest A, Ezaki MB, Tonkin M, Gericke G. Arthrogryposis multiplex congenita. J Hand Surg 2005;30:468-74.
Drachman DB, Banker BQ. Arthrogryposis multiplex congenita. Case due to disease of the anterior horn cells. Arch Neurol 1961;5:77-93.
Drachman DB, Coulombre AJ. Experimental clubfoot and arthrogryposis multiplex congenita. Lancet 1962;15:523-6.
Saito Y, Hayashi M, Miyazono Y, Shimogama T, Ohno K. Arthrogryposis multiplex congenita with callosal agenesis and dentato-olivary dysplasia. Brain Dev 2006;28:261-4.
How to Cite
Downloads
Downloads
Published
Issue
Section
| Article metrics | |
|---|---|
| Abstract views | |
| Galley vies | |
| PDF Views | |
| HTML views | |
| Other views | |
