Anaesthetic Management of a Patient with Apert Syndrome
Journal Title: Indian Journal of Anesthesia and Analgesia - Year 2017, Vol 4, Issue 4
Abstract
Introduction: Apert syndrome is a type of acrocephalosyndactilia that includes several abnormalities. Number of coexisting conditions should be taken under consideration while giving anaesthesia. We report our experience in the anaesthetic management of case of Apert syndrome, referred to us for syndactyly release in both upper limbs. Case Report: A 13months male child with Apert syndrome, weighing 7.29 kg, scheduled for syndactyly release of both upper limbs (fusion of digit 2 to 4), had facial dysmorphism with macroglossia, craniosynostosis, short nose with widely depressed nasal bridge with bulbous tip, proptosis, wide set eyes, mandibular retraction, shield chest deformity (pectus excavatum), limited mouth opening (M.P. grading III), high arched palate, history of sleep apnoea, patent foramen ovale with left to right shunt, brachycephaly and mild ventriculomegaly. Anaesthetic Management: Case was managed under general anaesthesia with intubation in spontaneously breathing anaesthetised patient without neuromuscular blocking agents. Awake extubation was done with no intraoperative or postoperative complications. Discussion: Anaesthetic management of a patient with Apert syndrome may be challenging. The anaesthesiologist must be ready for airway problems, intubation difficulties, obtaining a good mask seal, difficult IV access. Raised intra cranial pressure, sleep apnea, possibility of mental retardation and other associated anomalies should always be considered. Conclusion: Ever vigilant Anesthesiologist who has armoured himself with all the potential problems likely to arise, in the management of children with this rare genetic syndrome (who may need repetitive surgeries), will make all the difference in the successful outcome of these patients.
Authors and Affiliations
Prashant Rai
Keywords
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- EP ID EP472324
- DOI 10.21088/ijaa.2349.8471.4417.27
- Views 86
- Downloads 0
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