Anaesthetic Implications of the Mucopolysaccharidoses: A Fifteen-Year Experience in a Children's Hospital

Abstract

Patients suffering from the mucopolysaccharidoses, most commonly Hurler's syndrome, present special problems in anaesthesia. A retrospective review of the anaesthetic management of such patients over fifteen years revealed a high incidence of airway problems. Two case histories illustrating these difficulties are presented.

High rate of postoperative mortality in patients with mucopolysaccharidosis I: findings from the MPS I Registry

“…Numerous airway problems have been reported, including obscured airway landmarks owing to excess glycosaminoglycan deposition, copious thick secretions, narrow stiff airways, and difficulty oxygenating owing to glycosaminoglycan deposition within alveoli [26][27][28][29][30][31]. In an article by Herrick and Rhine [28], the incidence of airway-related problems in patients with MPS I and II was 54%.…”

Montgomery© T-tubes in the management of multilevel airway obstruction in mucopolysaccharidosis

“…The literature states that up to 16% of all children with MPS require a tracheostomy, which in the event of total airway obstruction or failed extubation following a general anaesthetic, can aid weaning and preserve life [4,11]. Tracheostomy insertion in patients with MPS can often be technically difficult, due to unfavourable anatomy (narrow trachea, short neck, disease of the cervical spine) and thickened soft-tissues in the anterior neck [12]. Likewise, a short neck and deep-seated trachea lead to an increased accidental decannulation rate with associated mortality [13].…”

Anesthesia for an adult with mucopolysaccharidosis I

“…As a consequence organ, skeletal and connective tissue involvement is diffuse and worsens with time [4]. Dwarfism, hypertelorism, progressive mental retardation, hepatosplenomegaly, kyphosis, cardiac valvular disease, cardiomyopathy, and coronary artery disease characterize Hurler syndrome [4,5]. Expected life span is 5 to 10 years, with death most commonly due to respiratory or cardiac failure [6][7][8] This is changing as bone marrow transplant and enzyme replacement therapy become more widely available [1,2].…”

“…The difficulty in taking care of these patients cannot be understated. Death from failed intubation [9], failed tracheostomy [4], and postoperative respiratory arrest [5] have all been reported.…”

“…For the anesthesiologist, the establishment and maintenance of an adequate airway represent the most commonly encountered problem in MPS I [5,9,10]. Several anatomical abnormalities predispose these patients to upper airway obstruction, sleep apnea, and difficult intubation; these include a short neck with limited mobility, relatively high epiglottis, a deep cranial fossa that narrows the nasopharynx, hypoplastic mandible, ankylosis of the temporomandibular joints, infiltration of the pharyngeal tissues and tracheal cartilages, intraluminal narrowing of the conductive airways, and excessive secretions.…”