1 - Interno de Anestesiologia, Centro Hospitalar de Lisboa Central
2 - Assistente Hospitalar, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central
3 - Chefe de Serviço, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central
- Euroanaesthesia 2015 (Berlin, Germany)
Background: Kartagener syndrome is a subset of primary ciliary dyskinesia, a rare autosomal recessive condition in which ciliary structure and/or function is abnormal; it is characterized by the clinical triad of situs inversus, chronic sinusitis and bronchiectasis. Clinical manifestations are more severe during the first decade of life and include chronic upper and lower respiratory tract disease due to impaired mucociliary clearance. Recurrent otitis media is a common manifestation. Even though surgical treatment is often needed, this syndrome is rarely encountered in paediatric anaesthesia practice. Case report: A 9-year-old male child with history of situs inversus with dextrocardia and hypoplasia of paranasal sinuses was scheduled for adenoidectomy and bilateral myringotomy tube placement due to chronic adenoiditis, sleep-disordered breathing and chronic otitis media. He presented frequent episodes of breathlessness and mucopurulent productive cough often needing postural drainage associated with antibiotic and bronchodilator therapy. Pre-operatively, postural drainage and chest physiotherapy were performed. Antibiotics and steroids were administred before induction. Anesthesia was induced with midazolam, propofol and alfentanyl and maintained with sevoflurane. The patient was ventilated with a humidified mixture of oxygen and air in the pressure-controlled mode. Intraoperatively, despite remaining hemodynamically stable, gentle endotracheal tube suction due do accumulated secretions with associated decrease of achieved tidal volumes was needed on three occasions. Bronchodilators were administered prior to deep extubation but mild bronchospasm still followed, requiring additional bronchodilators and assisted mask ventilation. Administration of supplemental oxygen and epinephrine nebulization were maintained during the immediate post-operative period, which was otherwise uneventful.
Discussion: Patients with Kartagener syndrome are prone to airway obstruction, air trapping and hypoxemia. If airway management is required, there may be an increased risk of complications such as bronchospasm or acute respiratory failure. Careful clinical evaluation and pre-operative measures such as chest physiotherapy and administration of steroids and bronchodilators are crucial.
Palavras Chave: Kartagener syndrome