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Anesthetic challenges in the management of Larsen syndrome: A rare congenital anomaly

Larsen syndrome is a rare inherited disease associated with dislocations of multiple joints, typical syndromic facies, and multiple spine abnormalities. They often required multiple corrective orthopedic surgeries to regain their functional ability, thus needing repeated anesthesia. Apart from skele...

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Detalles Bibliográficos
Autores principales: Das, Subhasree, Panda, Aparajita, Jena, Sritam S., Jain, Mantu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10077782/
https://www.ncbi.nlm.nih.gov/pubmed/37032698
http://dx.doi.org/10.4103/sja.sja_518_22
Descripción
Sumario:Larsen syndrome is a rare inherited disease associated with dislocations of multiple joints, typical syndromic facies, and multiple spine abnormalities. They often required multiple corrective orthopedic surgeries to regain their functional ability, thus needing repeated anesthesia. Apart from skeletal deformities, they have predicted difficult airway and need extreme care during intubation and positing of the patient. Abnormal posturing due to spinal deformity and poor pulmonary reserve due to kyphoscoliosis creates an extremely challenging situation for the anesthetist to manage the case during the perioperative period. Here we are describing the perioperative anesthetic management of a patient with Larsen syndrome.