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Anaesthetic Implications of children with Larsen syndrome

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Original Article

Author Details : Anandkumar , Serina Ruth Salins*

Volume : 6, Issue : 3, Year : 2019

Article Page : 430-436

https://doi.org/10.18231/j.ijca.2019.083

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Abstract

Objectives: Larsen Syndrome (LRS) is a rare osteochondrodysplasia, presenting with large-joint dislocations, foot deformities and craniofacial abnormalities. These often require early and multiple orthopaedic interventions to maintain age appropriate function which necessitate repeated anaesthesia. Other than the skeletal deformities, this syndrome is also associated with other multisystem (cardiac, spinal, respiratory) involvement, a thorough knowledge of which is essential to optimise perioperative outcomes. We analysed the clinical data in our cohort of children with LRS undergoing orthopaedic intervention and detail the anaesthetic management in these children. The commonest problems involved the airway and cervical spine and these required proactive management. The primary objective is to note the anaesthetic challenges for children affected with Larsen Syndrome.

Materials and Methods: This retrospective study done at a tertiary care centre, includes, all children or families with Larsen syndrome who had been identified in the paediatric orthopaedic clinic from January 2005 to December 2015. After acquiring Institution ethics committee approval, data was collected from the hospital registry for all cases. Anaesthesia protocol was observed in the perioperative period. Difficult airway and cervical neck instability were noted and management discussed. Statistical analysis was not required.
Results: There were ten children from ten families diagnosed with LRS. Systemic and skeletal anomalies were noted.
Conclusion: Larsen syndrome is a well-known entity, even amongst the Indian race. Carefully management in the entire perioperative care, is required for safer outcomes, reduced length of stay in the hospital and improved patient and provider satisfaction.

Keywords: Larsen syndrome, Paediatric anaesthesia, Difficult airway, Cervical spine stabilisation.

How to cite : Anandkumar, Salins S R, Anaesthetic Implications of children with Larsen syndrome. Indian J Clin Anaesth 2019;6(3):430-436

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