Prolonged Compilation Due to Rett Syndrome: Case Report

Abstract

Rett Sendromu, zeka geriliği, nöbetler, hareket bozukluğu, otistik davranış ve anormal solunum yolu örneği gibi nörolojik belirtilere eşlik eden X bağlantılı bir kalıtsal hastalıktır. Anesteziyologlar için perioperatif dönemde skolyoz mikrognati ve buna bağlı solunum problemleri nedeniyle entübasyon zorluğu ve anormal solunum yolu paternine bağlı önemli bir klinik tablo. Bu olgu sunumunda, Rett Sendromu ve anestezik yaklaşımdan dolayı bu vakalarda uzun süreli derlemeyi sunmak istiyoruz.

Keywords

• RETT sendromu,genişletilmiş derleme,genel anestezi

Prolonged Compilation Due to Rett Syndrome: Case Report

Abstract

Rett Syndrome is a X-linked hereditary disease, accompanying neurological manifestations such as mental retardation, seizures, movement disorder, autistic behavior and abnormal respiratory pattern. It is an important clinical picture for anesthesiologists during perioperative period due to scoliosis and related respiratory problems, intubation diffi culty due to micrognathia and abnormal respiratory pattern. In this case report we would like to present prolonged compilation due to Rett Syndrome and anesthetic approach in these cases.

Keywords

• RETT syndrome,extended compilation,general anesthesia

References

1. 1. Tuba Berra Sarıtaş, Kevser Babacan, Gamze Sarkılar, Selmin Ökesli. Rett Sendromlu Bir Olguda Anestezik Yaklaşım. Türk Anest Rean Der Dergisi 2011; 39(3):153-158
2. 2. Weaving LS1, Ellaway CJ, Gécz J, Christodoulou J. Rett syndrome: clinical review and genetic update. J Med Genet. 2005 Jan;42(1):1-7.
3. 3. Huppke P, Laccone F, Kramer N, Engel W, Hanefeld F, Rett Syndrome: analysis of MECPP2 and clinical characterization of 31 patients. Hum Mol Genet 2000;9:1369-75.
4. 4. Hiromi Kako,1 David P Martin,1,2 Richard Cartabuke,1,2 Allan Beebe,3 Jan Klamar,3 and Joseph D Tobias. Perioperative management of a patient with Rett syndromeInt J Clin Exp Med. 2013; 6(5): 393–403
5. 5. Cardiac disease and Rett syndrome. M Acampa, F Guideri. Arch Dis Child 2006;91:440–443.
6. 6. Sekul EA1, Moak JP, Schultz RJ, Glaze DG, Dunn JK, Percy AK. Electrocardiographic fi ndings in Rett syndrome: an explanation for sudden death? J Pediatr. 1994 Jul;125(1):80-2.
7. 7. Kerr AM. A review of the respiratory disorder in the Rett Syndrome. Brain Dev 1992;14:543-5.
8. 8. Cirignotta F, Lugaresi E, Montagna P. Breathing impairment in Rett syndrome. Am J Med Genet Suppl.1986;1:167–73.

9. 9. Konarzewski WH, Misso S. Rett syndrome and delayed recovery from anaesthesia. Anaesthesia.1994;49:357.