Managing the difficult airway in the syndromic child
2014年4月3日 · MPS I (Hurler syndrome), IV (Morquio syndrome), and VI (Maroteaux–Lamy syndrome) are associated with potential odontoid hypoplasia and may exhibit radiological signs of atlanto-axial subluxation, which may lead to anterior dislocation and spinal cord compression.
Airway Anatomy of an Adult with Hurler’s Syndrome
These videos are from a 19-year-old patient with Hurler’s Syndrome presenting for surgery whereby a “cannot intubate” scenario occurred requiring urgent supraglottic airway placement and intubation using a flexible bronchoscope through the supraglottic airway.
Airway management in Hurler's syndrome: A persistent …
The difficulty in airway management of Hurler's syndrome or mucopolysaccharidosis (MPS) type I patients has been described as the worst. These patients may develop serious complications under anaesthesia including airway obstruction leading to severe hypoxaemia, inability to ventilate or intubate and post-extubation problems.[ 2 ]
Society for Pediatric Anesthesia - SPA News
Hurler syndrome (MPS I) has been called “the worst airway problem in pediatric anesthesia.”(4) This review will provide an overview of pertinent clinical information for anesthesiologists caring for MPS patients.
Hurler syndrome or MPS 1 H is the prototype of MPS and is the most severe form of it3. In Hurler’s syndrome airway problem has been described as the worst in pediatric anesthesia4. Perioperative mortality rates averaging 20% have been reported for patients with this disease, with failure to control the airway as the
Airway management in Hurler's syndrome - journals.lww.com
We at our centre decided to insert a Proseal laryngeal mask airway (PLMA) in awake state in a patient of Hurler syndrome to circumvent the problem of the difficult airway. A 15-year-old …
Journal of Anaesthesiology Clinical Pharmacology - LWW
We encountered difficult mask ventilation and endotracheal intubation was not possible and finally ventilated with laryngeal mask airway in a known case of Hurler syndrome posted for umbilical hernia repair. Hurler syndrome is a subtype of mucopolysaccharidosis (MPS Type I) named after a pediatrician Gerturd Hurler in 1919. [ 1] .
Hurler Syndrome | Syndromes: Rapid Recognition and …
Children with Hurler syndrome frequently present difficulties with airway management that increases with age. About half of direct laryngoscopy and tracheal intubations are very difficult, and securing the airway may fail in 10%. The airway is compromised by accumulation of mucopolysaccharides in the soft tissues of the head and neck.
Management of an anticipated difficult airway in Hurler's syndrome
Management of an anticipated difficult airway in Hurler syndrome. Hurler syndrome is a subtype of Mucopolysaccharidosis (MPS) type 1. Mucopolysaccharidosis (lysosomal storage diseases)...
The Difficult Paediatric Airway : Virtual Library - WFSA Resource …
2012年2月13日 · Conversely, children with Hunter or Hurler’s syndrome have a difficult mask airway AND difficult intubation – a careful inhalational induction is the wisest course. Patient Preparation. Starvation times must be strictly adhered to prior to elective surgery. A variety of premedication drugs are available: