Anesthetic Management of Arthrogryposis Multiplex Congenita in Children: A Retrospective Review of 27 Cases

Abstract

Background: Arthrogryposis Multiplex Congenita, is a rare non-progressive congenital syndrome spectrum that causes multiple joint contractures and fibrosis with an intact sensory system and normal intellect. Pediatric patients with AMC frequently present for multiple orthopedic surgeries requiring general anesthesia. Children with arthrogryposis have micrognathia, a high arched palate, a short and rigid webbed neck making tracheal intubation difficult and at times impossible. The extensive contractures, tense skin, minimal muscle mass and subcutaneous tissue pose additional challenges for intraoperative positioning and intravenous access. Aims: To emphasize the anesthetic challenges encountered in this group of patients Methods: A retrospective case note review was performed on 27 children with AMC syndrome over a 5 year period. They belonged to ASA class 1 and 2 with age group between Day15 of life to 14 years of age and weighing from 2.5 kg to 64 kg. Results: In the majority of cases, the course of general anaesthesia was normal. Clinically, the incidence of Larsen’s syndrome in our series was 18%, one child had Freeman Sheldon syndrome and the rest 77% fit into the AMC group. Difficult airway was encountered in 22% of patients with 18% of them having difficult intravenous access. 25% of children presented for multiple orthopedic corrective procedures. Conclusions: Children with AMC have unique needs which need to be recognized preoperatively and dealt adequately. Difficult airway needs to be anticipated which warrants the presence of not only a sophisticated airway cart but also a highly experienced anesthesiologist. These children come for multiple repeat procedures and issues like difficult intravenous access and positioning need to be addressed as well. Keywords: Children, Arthrogryposis multiplex congenital, difficult airway, anesthesia, hyperthermia, intravenous access