Anesthetic management of three pediatric cases with Pena-Shokeir syndrome.

Journal of Anesthesia
Shogo TsujikawaYutaka Oda

Abstract

Pena-Shokeir syndrome is a rare, early lethal disease. It is characterized by fetal growth restriction; craniofacial deformities, for example micrognathia and microcephaly; multiple ankyloses; and pulmonary hypoplasia. For patients with this syndrome, maintenance of airway and control of perioperative respiratory complications are important for anesthetic management. We report 3 pediatric cases of Pena-Shokeir syndrome undergoing tracheostomy and arthrolysis under general anesthesia using sevoflurane, nitrous oxide, fentanyl, and vecuronium bromide. Anesthetic procedures including mask ventilation, tracheal intubation, and extubation were successfully performed without complications during and after surgery. In patients with Pena-Shokeir syndrome, inhalational anesthetics can be safely used for induction and maintenance of anesthesia, although it is important to assume that difficult airway management might be encountered.

References

Mar 1, 1990·American Journal of Medical Genetics·P MoermanJ M Lauweryns
Sep 1, 1986·American Journal of Medical Genetics·J G Hall
Jan 1, 1974·Clinical Genetics·S D Pena, M H Shokeir
Aug 2, 2002·Journal of Child Neurology·Nathaniel KhoStephen Coons
Jul 1, 1990·Journal of Anesthesia·Y OdaM Fujimori
Aug 1, 2009·Birth Defects Research. Part A, Clinical and Molecular Teratology·Judith G Hall
Mar 26, 2010·Fetal and Pediatric Pathology·Wendy L Coto-PuckettBahig M Shehata

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