Anesthetic management of three pediatric cases with Pena-Shokeir syndrome - PubMed
Case Reports
Anesthetic management of three pediatric cases with Pena-Shokeir syndrome
Shogo Tsujikawa et al. J Anesth. 2012 Jun.
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.
Similar articles
-
[Bronchospasm during anesthesia in a patient with Pena-Shokeir syndrome].
Nakamura A, Kawahito S, Katayama T, Kawano T, Nitta K, Inui D, Kitahata H, Oshita S. Nakamura A, et al. Masui. 2005 Oct;54(10):1146-8. Masui. 2005. PMID: 16231771 Japanese.
-
Acute respiratory distress in Pena-Shokeir syndrome.
Boesen PV, French CE. Boesen PV, et al. Ear Nose Throat J. 2004 Nov;83(11):772-3. Ear Nose Throat J. 2004. PMID: 15628635
-
[Pena-Shokeir phenotype (fetal akinesia/hypokinesia sequence)].
Dzinović A, Heljić S. Dzinović A, et al. Med Arh. 2006;60(6):383-5. Med Arh. 2006. PMID: 17297855 Bosnian.
-
Khanna P, Ray BR, Govindrajan SR, Sinha R, Chandralekha, Talawar P. Khanna P, et al. J Anesth. 2015 Dec;29(6):857-61. doi: 10.1007/s00540-015-2042-8. Epub 2015 Jul 21. J Anesth. 2015. PMID: 26194909 Review.
-
Deli T, Kovács T. Deli T, et al. Orv Hetil. 2010 Jun 13;151(24):990-3. doi: 10.1556/OH.2010.28871. Orv Hetil. 2010. PMID: 20519182 Review. Hungarian.
Cited by
-
Pena-Shokeir syndrome: current management strategies and palliative care.
Adam S, Coetzee M, Honey EM. Adam S, et al. Appl Clin Genet. 2018 Oct 25;11:111-120. doi: 10.2147/TACG.S154643. eCollection 2018. Appl Clin Genet. 2018. PMID: 30498368 Free PMC article. Review.
References
Publication types
MeSH terms
LinkOut - more resources
Full Text Sources
Medical