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[Multiple pterygium syndrome in children. 7 cases].

Abstract

Seven children have been observed and treated for a multiple pterygia syndrome. The characteristics of this affection which is rare and mostly transmitted as an autosomal recessive, are: a growth retardation, a facial dysmorphia, pterygia resulting in flexion deformities, and anomalies of hands, feet and external genitale. But the severity of the syndrome is linked with the spine malformation, whose fusions produce severe and precocious deformities. This syndrome must be differentiated from the popliteal pterygium syndrome, whose repercussions are only functional; from the arthrogryposis multiplex congenita, whose syndrome is already complete at birth; and from the false secondary pterygia (caudal spinal agenesis, campomelic syndrome, quadriceps hypoplasia). The multiple pterygia syndrome imposes a genetic investigation and a serious observation of the spine and of the evolutive flexion deformities.

Authors+Show Affiliations

Service d'Orthopédie Traumatologie Infantile, Hôpital des Enfants Malades, Paris.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

fre

PubMed ID

2142314

Citation

Fenoll, B, et al. "[Multiple Pterygium Syndrome in Children. 7 Cases]." Revue De Chirurgie Orthopedique Et Reparatrice De L'appareil Moteur, vol. 76, no. 2, 1990, pp. 102-11.
Fenoll B, Rigault P, Maroteaux P, et al. [Multiple pterygium syndrome in children. 7 cases]. Rev Chir Orthop Reparatrice Appar Mot. 1990;76(2):102-11.
Fenoll, B., Rigault, P., Maroteaux, P., Padovani, J. P., Guyonvarch, G., & Durand, Y. (1990). [Multiple pterygium syndrome in children. 7 cases]. Revue De Chirurgie Orthopedique Et Reparatrice De L'appareil Moteur, 76(2), 102-11.
Fenoll B, et al. [Multiple Pterygium Syndrome in Children. 7 Cases]. Rev Chir Orthop Reparatrice Appar Mot. 1990;76(2):102-11. PubMed PMID: 2142314.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Multiple pterygium syndrome in children. 7 cases]. AU - Fenoll,B, AU - Rigault,P, AU - Maroteaux,P, AU - Padovani,J P, AU - Guyonvarch,G, AU - Durand,Y, PY - 1990/1/1/pubmed PY - 1990/1/1/medline PY - 1990/1/1/entrez SP - 102 EP - 11 JF - Revue de chirurgie orthopedique et reparatrice de l'appareil moteur JO - Rev Chir Orthop Reparatrice Appar Mot VL - 76 IS - 2 N2 - Seven children have been observed and treated for a multiple pterygia syndrome. The characteristics of this affection which is rare and mostly transmitted as an autosomal recessive, are: a growth retardation, a facial dysmorphia, pterygia resulting in flexion deformities, and anomalies of hands, feet and external genitale. But the severity of the syndrome is linked with the spine malformation, whose fusions produce severe and precocious deformities. This syndrome must be differentiated from the popliteal pterygium syndrome, whose repercussions are only functional; from the arthrogryposis multiplex congenita, whose syndrome is already complete at birth; and from the false secondary pterygia (caudal spinal agenesis, campomelic syndrome, quadriceps hypoplasia). The multiple pterygia syndrome imposes a genetic investigation and a serious observation of the spine and of the evolutive flexion deformities. SN - 0035-1040 UR - https://www.unboundmedicine.com/medline/citation/2142314/[Multiple_pterygium_syndrome_in_children__7_cases]_ DB - PRIME DP - Unbound Medicine ER -