P032: MANAGEMENT OF AN INFANT WITH CHARGE SYNDROME: "DIFFICULT....EVERYTHING"
Jeremiah Jeffers, Pediatric Anesthesiology Fellow1, Jason Mishal, Staff Anesthesiologist2; 1University of Miami/Jackson Memorial, 2Nicklaus Childrens' Hospital
Children with CHARGE syndrome may have a myriad of airway related anomalies including: choanal atresia, cleft lip/palate, cranial nerve palsy leading to laryngeal dysfunction, and the risk of aspiration. As these patients often present for surgery during the neonatal period, an understanding of the perioperative implications is of great significance to the pediatric anesthesiologist.
A 45 week post-gestational male presented to the operating room for repair of bilateral choanal atresia. The patient previously underwent successful ligation of a persistent PDA at 28 days of life. This child also had left hemifacial paralysis, micropenis, and bilateral cryptorchidism. The patient lost vascular access 2 days prior to presenting to the operating room for the scheduled procedure. Multiple attempts at intravenous catheters and PICC lines were made without success. The child did not show signs of dehydration, and was able to receive adequate oral intake so further attempts were abdandoned.
We describe the techniques used to secure vascular access prior to induction, airway management, and postoperative care of an infant with CHARGE syndrome undergoing upper airway surgery. We also discuss the implemented plan, as well as the secondary planning that was made in case of difficulty with either airway management or vascular access.
CHARGE syndrome, first described in the 1980s, is a an association of features that includes: Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of Growth and development, and Ear abnormalities and deafness. The estimated incidence is 1:8000-10,000 births. A mutation of the CHD7 gene leading to alteration of gene expression has been implicated in the majority of CHARGE sydrome diagnoses. Prognosis is highly variable from severe developmental delay to normally functioning adults. The highest rate of mortality occurs in the first year of life, as the child often receives treatment for the sequelae of CHARGE syndrome. An understanding of the perioperative implications and anesthetic considerations is of great significance to the pediatric anesthesiologist.
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