Mucopolysaccharidosis: Anaesthetic Considerations and Clinical Manifestations

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Mucopolysaccharidosis: Anaesthetic Considerations and Clinical Manifestations

Journal Title: IOSR Journal of Dental and Medical Sciences (IOSR-JDMS) - Year 2018, Vol 17, Issue 4

Abstract

Mucopolysaccharidosis(MPS) are a group of inherited metabolic disorders. These disorders are uncommon yet an important topic in continuing education in clinical anaesthesiologyTherefore there is a need to study their clinical features and effect of these features on anaesthetic management with special reference to airway management In the last few years many scientific studies have been published emphasizing the risks involved in anaesthetic management of such patients.These are patients of potential difficult airway and entire anaesthetic management revolves primarily around proper assessment of clinical features leading to difficult airway and judicious use of available airway equipment. We want to review the literature as regards the clinical features and anaesthetic complications especially involving intubation difficulties and techniques and equipment used in managing these difficulties.We want to study the literature as regards the use of latest gadgets available for managing difficult intubation in these patients in a procedural manner so as to improve the quality of care and minimize anaesthetic complications when handling such kind of patients. Material Method: A thorough literature review was undertaken to locate all the relevant articles that described the clinical features and anaesthetic management of MPS. We searched the pub med EMBASETM , google scholar, Wiley online and mediline plus for relevant articles using the following search termsMucopolysaccharitosis, Anaesthesia, management, morquios, hurlers, huters syndrome. Conclusion: MPS disorders are characterized by progressive craniofacial, joint and skeletal deformities, progressive cardiac involvement and early death from pulmonary infection or cardiac failure Patients of MPS are short statured with skeletal deformities like short broad metacarpals and phalanges , kyphosis, scoliosis,immobile cervical and temporomandibular joint and coxa vara.. Children with MPS should be handled by anaesthesiologists and surgeons who are aware of the expected complications of this disease. Before subjecting such a patient to anaesthesia a proper informed consent should be obtained, difficult airway cart should be ready and available and even a surgeon standing by ready to do an emergency tracheostomy. Inhalational induction with maintenance of spontaneous ventilation is preferred but in mentally retarded and uncooperative patients intravenous induction is more satisfactory. A fibreoptic technique of intubation for children of MPS using laryngeal mask airway has been described15. LMA allows a good airway control without tracheal intubation. Anaesthesia should ideally be administered by experienced anaesthesiologist and in a set up which has facility for paediatric intensive care unit. Recovery after general anaesthesia in such cases may be accompanied by periods of breath holding, apnoea, bronchospasm,cyanosis and respiratory arrest.16 A fibreoptic technique of intubation for children of MPS using laryngeal mask airway has been described15. LMA allows a good airway control without tracheal intubation.

Authors and Affiliations

Dr Vandana chugh, Dr Nidhi P Sehgal

Keywords

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EP ID EP370350
DOI 10.9790/0853-1704023344.
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