Abstract | BACKGROUND: METHODS: A literature search was carried out using PubMed, Medline, and the Cochrane Library, and using the MESH search terms detailed below. Patients were identified by formal request to the department of pediatric endocrinology at Great Ormond Street Hospital. Each patient's notes were searched manually and electronically for both clinical presentation and outcome, and anesthesia records. DISCUSSION: There is currently no published literature relating to anesthetic management of Donohue syndrome. We report a case series of 5 patients with Donohue syndrome who have presented to our institution. This small series of children with this complex disorder demonstrates a clearly increased risk of general anesthesia. Many of the risks relate to restrictive lung disease and abdominal distension which worsens with bag valve mask ventilation and limited respiratory reserve which leads to precipitous desaturation. During induction, a spontaneously breathing technique is recommended. If required, bag valve mask ventilation should be accompanied by constant gastric aspiration. Intubation is challenging, and a difficult airway plan, including a second experienced anesthetist and ENT support, should be in place. These children are predisposed to developing cardiomyopathy and therefore at risk of cardiovascular collapse under anesthesia.
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Authors | Alana Kirkwood, Grant Stuart, Louise Harding |
Journal | Paediatric anaesthesia
(Paediatr Anaesth)
Vol. 28
Issue 1
Pg. 23-27
(Jan 2018)
ISSN: 1460-9592 [Electronic] France |
PMID | 29148123
(Publication Type: Journal Article, Meta-Analysis, Review)
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Copyright | © 2017 John Wiley & Sons Ltd. |
Topics |
- Anesthesia
- Cardiomyopathy, Hypertrophic
(therapy)
- Donohue Syndrome
(epidemiology, physiopathology, therapy)
- Humans
- Infant
- Infant, Newborn
- Insulin Resistance
- Sepsis
(therapy)
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