Rüveyda İrem DEMİRCİOĞL, Hüseyin SERT, KAVUN Nuran ÇİMEN, Burhanettin USTA

Vacterl asosiasyonu olgusunda anestezi yönetimi

VACTERL assosiasyonu vertebral anomaliler (V), anal atrezi veya imperfore anüs (A), kardiyovasküler defekt (sıklıkla ventriküler septal defekt) (C), trakeal ve özefagial anomaliler (TE), renal agenezi (R), parmak anomalileri (L) ile kendini gösteren otozomal resesif veya X kromozomuna bağlı bir assosiasyondur. Bu olgu sunusunda skolyoz, tortikolis, anal atrezi, sekundum tipi atrial septal defekt, gastro özefagial reflü, renal agenezi, veziko Ureteral reflü, parmak ve ekstremite anomalileri olan 6 yaşındaki VACTERL sendromlu erkek hastada polizasyon (başparmak yapımı) operasyonu esnasındaki anestezi yönetiminde anestezistin dikkat etmesi gereken hususlar sunulmuştur.

Anesthesia management of vacterl asosiation rüveyda

VACTERL association is an X linked, autosomal recessive disorder with vertebral anomalies (V), anal atresia or imperforated anus (A), cardiovascular defect (most often ventricular septal defect) (C), tracheal and esophageal malformation (TE), renal agenesis (R), limb anomalies (L). In this case report, we described anesthetic management of 6 years old VACTERL syndrome male patient with scoliosis, torticollis, anal atresia, secundum-type atrial septal defects, gastro esophageal reflux, renal agenesis, VUR, finger and limb abnormalities who undergone polization (finger-made) operation.

PDF

___

1. Khatavkar SS, Jaqtap SR. Anaesthetic management for cataract surgery in VACTERL syndrome case report. Indian J Anaesth 2009;53(l):94-97.
2. Froster UG, Wallner SJ, Reusche E, Schwinger E, Rehder H. VACTERL with hydrocephalus and branchial arch defects: prenatal, clinical, and autopsy findings in two brothers. Am J Med Genet 1996;62(2): 169-172.
3. Kaufman RL. Birth defects and oral contraceptives. Lancet 1973; 1(7816):1396.
4. Rittler M, Paz JE, Castilla EE. VACTERL association, epidemiologic definition and delineation. Am J Med Genet 1996;63(4): 529-536.
5. Quan L, Smith DW. The VATER association. Vertebral defects, anal atresia, TE fistula with oesophagal atresia, radial and renal dysplasia: a spectrum of associated defects. J Pediatr 1973;82(1): 104-107.
6. Benn A, Warburton D, Byrne JM et al. A fetus with a chromosome 13 ring and placenta with chromosome 13 rod/ring mosaicism. Prenat Diagn 1983;3(4):297-302.
7. Goldsmith CL, Tawagi GF, Carpenter BF, Speevak MD, Hunter AG. Mosaic r(13) in an infant with aprosencephaly. Am J Med Genet 1993;47(4):531-533.
8. Biles AR Jr, Luers T, Sperling K. D1 ring chromosome in newborn withpeculiar face, Polydactyly, imperforate anus, arrhinencephaly, and other malformations. J Med Genet 1970;7(4):399-401.
9. Shah D, Sharma S, Faridi MM, Mishra K. VACTERL Association with Prune-Belly Syndrome. Indian Pediatr 2004;41(8):845-847.
10. Cruysberg JR, Draaijer RW, Pinckers A, Brunner HG, Congenital corneal anesthesia in children with the VACTERL association. Am J Ophtalmology 1998;125(l):96-98.
11. Weaver DD, Mapstone CL, Yu PL. The VATER association. Analysis of 46 patients. Am J Dis Child 1986;140(3):225-229.
12. Leeners B, Sauer I, Schefels J, Cotarelo CL, Funk A. Prune-belly syndrome: therapeutic options including in utero placement of a vesicoamniotic shunt. J Clin Ultrasound 2000;28(9):500-507.
13. Kiran PS, Dutta S, Naranq A, Mukhopadhnyay K. Unusual manifestations of VACTERL association. Indian Pediatr 2003; 40(2): 162-165.
14. Shaw-Smith C. Oesophageal atresia, tracheo-oesophageal fistula, and the VACTERL association: review of genetics and epidemiology. J Med Genet 2006;43(7):545-554.
15. Hatemi AC, Gursoy M, Ceviker K et al. Ventricular septal defect closure in a patient with VACTERL syndrome: anticipating sequelae in a rare genetic disorder. Tex Heart Inst J 2008;35(2):203-205
16. Delius RE, Wheatley MJ, Coran AG. Etiology and management of respiratory complications after repair of esophageal atresia with tracheoesophageal fistula. Surgery 1992;112(3):527-532.
17. Luce V, Mercier FJ, Benhamou D. Anesthetic management for a parturient affected by the VACTERL association. Anesth Analg 2004;98(3):870-878.
18. Mariano ER, Chu LF, Albanese CT, Ramamoorthy C. Successful thorascopic repair of esophageal atresia with tracheoesophageal fistula in a new born with single ventricle physiology. Anesth Analg 2005;101(4):1000-1002.