NM-217
Anaesthetic management of an infant with Nager syndrome and a complex congenital heart defect
Bolivar Trujillo M, Amezquita Trujillo A, Reyes R
Fundacion Hospital la Misericordia, Bogota, Cundinamar, Colombia
Introduction/Study Question
The main Nager syndrome clinical features include craniofacial, limb and musculoskeletal malformations. Complex congenital cardiac defects are rare. The objective is to present the first case of anesthetic management of a patient with Nager syndrome in Colombia, highlighting the difficult airway approach with an associated congenital heart defect.
Methods
The clinical case is about a one month old infant, diagnosed with Nager syndrome due to micrognathia, cleft palate, hipoplastic thumbs. Transthoracic echocardiography revealed patent ductus arteriosus (PDA), ostium secundum atrial septal defect, perimembranous ventricular septal defect, leading to severe pulmonary hypertension. A multidisciplinary assessment concluded that the patient was candidate to patent ductus arteriosus ligation and, according to the evolution, to undergo primary mandibular distraction osteogenesis. On physical examination, the infant weighed 4.6 kg, with hypoplastic mandible and glossoptosis, and signs of decompensated heart failure due to increased pulmonary blood flow. The preoperative tests were acceptable, although a persistent respiratory acidosis was noted. Before the surgical procedure, an axillary central venous catheter was inserted in the neonatal intensive care unit (NICU). The patient fasted 6 h. Inhalatory anesthetic induction with sevoflurane was performed, a fiberoptic bronchoscope (2.8 mm) was introduced orally and tracheal intubation with a 4.0 uncuffed endotracheal tube proceeded without difficulty. Balanced general anesthesia with fentanyl 5 μg/kg and sevoflurane 0.8 MAC was maintained. Dopamine infusion was required during the procedure. Patent ductus arteriosus ligation was uneventful. The infant was transferred to the NICU. The patient remained intubated and a primary mandibular distraction osteogenesis was scheduled for a week later. Hence, inhalational induction with sevoflurane was done, a 4.0 uncuffed endotracheal tube was introduced nasally including the fiberoptic bronchoscope with adequate visualization of the glottis was accomplished. The procedure lasted 100 min. Ten days later, the patient was extubated, after reaching a mandibular distraction of 10 mm. The patient was supported with nasopharyngeal cannula, and a tracheostomy was performed after 15 days.
Results
Acrofacial dysostosis represents a rare malformation of unknown etiology. The anesthetic management of Nager syndrome represents a challenge as a result of the predicted difficult intubation and ventilation. Administering adequate anesthesia, so that these patients will tolerate the stimulation from bronchoscopy and intubation while maintaining spontaneous ventilation is a demanding task, being more critical in the context of increased pulmonary blood flow, where hypoxia, hypercarbia, acidosis, hypothermia and pain could increase pulmonary hypertension.
Conclusion/Discussion
Nager syndrome airway is difficult. Fiberoptic intubation is the preferred mean to secure the airway; however, tracheostomy remains the standard for long-term management of severe airway obstruction, even though mandibular distraction osteogenesis is a surgical alternative. A multidisciplinary team should be available in the care of this patients
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