Author: Singaravelu S.; Ahmed D.; Frias C.; Tierney J.
Abstract: Purpose/Objective: Patients with Escobar syndrome pose specific challenges to the anaesthetist. We present our anaesthetic expereince for LSCS in a woman with this condition. Materials and Methods: Short statured primi with Escobar syndrome was admitted for LSCS at full term. She had severe kyphoscoliosis, restricted hand movments, atrophic shoulder, webbed neck and limited mouth opening. Lumbar spine was easily palpable. Spinal anaesthetic was administered with 2.4 mls 0.5% Bupivacaine and 300 mcg Diamorphine. 20 minutes later, block level was T4 on right and T6 on left side. GA was planned. Tracheal intubation was succesful with awake FOI and Remifentanil TCI. GA maintained with TIVA Propofol and Remifentanil. Both mother and baby had satisfactory outcome. Results: Main anaesthetic consideration in Escobar syndrome is difficulty with central neuraxial block due to kyphoscoliosis. Following succesful CNB, local anaesthetic spread may fail due to abnormal curvature or septal bands. They also have difficult airway due to limited mouth opening and joint webbing and may need awake FOI. Their short stature warrants smaller ET tubes for intubation. Due to Malignant Hyperthermia risks, consideration should be given to using TIVA and Rocuronium for anaesthetic maintenance. Conclusions: This is the first case report of succesful anesthetic management of patient with Escobar syndrome for LSCS following unsuccesful spinal anaesthesia. Careful planning, back-up plans and clear communication between staff is crucial to succesful anaesthetic conduct for LSCS in patients with complex syndromes like Escobar syndrome.
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