Guidelines for Postoperative Monitoring of Pediatric Patients
with Prader-Willi Syndrome
Winthrop University Hospital Cares for over 300 patients with Prader-Willi
syndrome. The Prader-Willi Center at Winthrop is a national referral resource
for these patients. Many of them have chosen to perform their emergency and
elective surgeries at our institution.
Background Information:
Patients with Prader-Willi syndrome are known to have increased morbidity
after surgery due to:
- Abnormal physiologic response to
hypercapnia and hypoxia
- Hypotonia
- Narrow oropharyngeal space
- High incidence of central, obstructive and
mixed apnea
- Thick secretions
- Obesity
- Increased incidence of scoliosis with
decreased pulmonary function
- Prolonged exaggerated response to
sedatives
- Increased risk for aspiration
- Decreased pain sensation is common in PWS
Recommendations:
I. Infants
and children with Prader-Willi syndrome who undergo deep sedation and general
anesthesia
should be recovered overnight in a monitored unit, either the Pediatric
Recovery Room or Pediatric Intensive Care Unit.
II. Continuous monitoring of Pulse oximetry for 24 hours postoperative is
strongly recommended with attention to airway and breathing
III. A conservative approach to pain management and use of narcotic agents is
recommended.
IV. Full assessment of return of GI motility prior to initiation of intake by
mouth because of the predisposition to ileus after surgery.
V. Direct supervision (1:1) to prevent foraging postoperatively.
VI. Monitor for picking of wounds.
Moris Angulo, MD – Director of Medical Genetics, Assistant Director of
Pediatric Endocrinology
Mary Cataletto, MD – Associate Director Pediatric Pulmonology
Maria Lyn Quintos-Alagheband MD – Associate Director Pediatric Critical Care
Department of Anesthesia
revised: 03/23/2010
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