P083: ANESTHETIC MANAGEMENT IN A PATIENT WITH PRADER WILLI SYNDROME AND ORTHOPEDIC INJURY
Carmen D Manresa, MD1; Justin Shang2; Luis I Rodriguez, MD1; 1Jackson Memorial Hospital/University of Miami; 2Nova Southeastern University Dr. Kiran C. Patel College of Osteopathic Medicine
Introduction: Prader-Willi Syndrome (PWS) is a congenital disease primarily resulting from paternal chromosomal deletion at 15q11.2-13. In infancy, patients display hypotonia and developmental delay; during childhood, they display hypothalamic dysfunction, resulting in hyperphagia and obesity (1). In this case report, we discuss an 11 year-old PWS patient with morbid obesity and developmental delay who presented with a closed right tibial fracture and underwent ORIF under general anesthesia.
Case Report: 11 year old, ASA3, 134.5kg, BMI 48.4, female with PMH of PWS, OSA, morbid obesity, and developmental delay who sustained a right closed tibia fracture after a fall, presented for ORIF of right tibia. History was negative for prior anesthetics, surgeries, or family history of anesthesia complications. Airway exam was notable for MP III, short TMD, redundant neck tissue, normal mouth opening, and FROM of neck. Due to her risk of obesity hypoventilation syndrome and pulmonary hypertension, a 2D Echo was included in workup, which was negative. Given the characteristic hyperphagia of PWS, staff and family were instructed to remove all food items from patient’s room during NPO time. GETA was planned with expected transfer to the PICU postoperatively for respiratory monitoring. In the OR, patient was ramped and arranged in sniffing position. Standard ASA monitors were placed and patient was preoxygenated for 5 minutes. Afterwards, 2mg Midazolam IV was given for anxiolysis, and she was induced with 100mcg Fentanyl IV, 0.3mg Glycopyrrolate IV, 200mg Propofol IV, and 50mg Rocuronium IV. She required two provider mask ventilation and an oral airway. Direct laryngoscopy was performed with a MAC 4, 2a view was obtained, and she was intubated in 1 attempt with a styleted 6.5 cuffed OETT. For PONV prophylaxis, she received 8mg Dexamethasone IV after intubation and 4mg Ondansetron IV at end of case. She was given an additional 25mcg Fentanyl IV, 1000mg Ofirmev IV, and Dexmedetomidine drip at 0.1 mcg/kg/hr throughout the case. Inhaled Sevoflurane was used for maintenance. A nerve stimulator placed over the left ulnar nerve at the wrist was used for TOF monitoring. No additional NMBs were given after induction and TOF was 4/4 at end of case. She was reversed with 0.8mg Glycopyrrolate IV and 4mg Neostigmine IV (0.03 mg/kg) and extubated without any complications.
Discussion: Children with PWS present an anesthetic challenge; in this case report, we discuss a successful anesthetic for a patient with PWS. Although hypotonia can be an early presenting symptom in PWS, NDNMBs have been successfully used without evidence of residual paralysis or delayed return of spontaneous breathing (2). We assessed muscle relaxation and chose reversal dosing objectively based on TOF. Airway management can be problematic due to features including ineffective clearance of thick secretions, morbid obesity, poor dentition, micrognathia, limited neck mobility, OSA, and restrictive lung disease (2,3). Hypothalamic abnormalities can cause difficulties with thermoregulation, making core temperature monitoring essential. Furthermore, neurodevelopmental delay can make cooperation a challenge and limit anesthetic options.