Anesthetic considerations in Sheldon-Hall syndrome

Paediatr Anaesth. 2014 May;24(5):538-40. doi: 10.1111/pan.12303.

Abstract

Arthrogryposis is characterized by multiple, nonprogressive joint contractures which may be caused by maternal disorders such as oligohydramnios as well as fetal akinesia resulting from primary disorders of muscle, connective tissue, or neurologic tissue. Its prevalence is about 1 : 3000. Distal arthrogryposis (DA) is a heterogenous group of genetic disorders with a characteristic flexion of the joints of the hands and feet divided into different types with additional features. Sheldon-Hall Syndrome (SHS), also known as distal arthrogryposis type 2A (DA2A), has some nonorthopedic features of specific importance to anesthetic care.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple
  • Androstanols / therapeutic use*
  • Anesthetics, Intravenous*
  • Arthrogryposis
  • Child, Preschool
  • Clubfoot / surgery
  • Fentanyl
  • Humans
  • Intubation, Intratracheal / methods*
  • Male
  • Methohexital
  • Neuromuscular Nondepolarizing Agents / therapeutic use*
  • Rocuronium
  • Tooth Abnormalities / surgery

Substances

  • Androstanols
  • Anesthetics, Intravenous
  • Neuromuscular Nondepolarizing Agents
  • Methohexital
  • Fentanyl
  • Rocuronium

Supplementary concepts

  • Distal arthrogryposis type 2B