Background and aims Krabbe disease is a severe neurological condition. Initial signs and symptoms include irritability, muscle weakness, feeding difficulties, stiff posture and delayed mental and physical development. As the disease progresses, muscles continue to weaken, affecting the infant’s ability to move, chew, swallow and breathe.1 Of concern to the anesthetist is the high prevalence of seizure disorders, gastroesophageal reflux with risk of aspiration and airway complications related to poor pharyngeal muscle control and copious oral secretions.2
Methods We present a case of a 9-month-old boy with Krabbe disease with nasogastric tube for feeding, proposed for laparoscopic Nissen fundoplication and surgical gastrostomy. Considering the weight of 7,5 kg, after standard ASA monitoring, patient was sedated with IV midazolam. In left lateral decubitus, we placed an epidural catheter at T7-T8 level under ultrasound control via caudal approach. Inhaled induction with sevoflurane was performed after epidural administration of 3 mg of ropivacaine (0.2%). General anesthesia was maintained with sevoflurane and a mixture of oxygen and air through a face mask, maintaining spontaneous ventilation. 20 minutes after induction, surgical incision was performed.
Results Patient remained hemodynamically stable during the surgery. Ketorolac and paracetamol were also provided. Surgery lasted approximately 2 hours and was uneventful. In the pediatric intensive care unit, bolus of ropivacaine 0.1% was given every 4 hours during 24h and the patient remained comfortable.
Conclusions General anesthesia with spontaneous ventilation combined with epidural anesthesia is an alternative in laparoscopic Nissen fundoplication, avoiding the need of orotracheal intubation and avoiding the airway complications related to poor pharyngeal muscle control.
Graziano AC, Cardile V. History, genetic, and recent advances on Krabbe disease. Gene. 2015;555(1):2–13.
Tobias JD. Anaesthetic considerations for the child with leukodystrophy. Can J Anaesth. 1992;39(4):394–7.
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