Tetra Amelili Hastada Santral Venöz Kateter Yerleştirilmesine Bağlı Şilotoraks

Öz

Tetra-ameli, tüm ekstremitelerin yokluğu ile karakterize nadir bir genetik bozukluktur. Hastalar akciğer enfeksiyonu veya ek anomalileri nedeni ile sık sık hastaneye yatmak zorunda kalırlar. Bu hastalarda kafatası ve boyundan periferik intravenöz girişimle veya internal juguler venden santral venöz kateter yerleştirilmesi (SVKY) ile damaryolu bulunabilir. SVKY sonrası damar ve sinir yaralanmaları, pnömotoraks, hemotoraks, kardiyak tamponad, pulmoner emboli, aritmi, sepsis ve giriş yerinde enfeksiyon gibi komplikasyonlar bildirilmiştir. Şilotoraks nadir bir komplikasyondur. SVKY gereken olgularda uygun kalınlık ve uzunlukta kateterin seçilmesi ve sağ internal juguler venin tercih edilmesi şilotoraksı azaltacaktır. Bu yazıda, tetra-amelili hastada damar yolu uygulamaları ve SVKY ile ilişkili şilotoraksın tanı ve tedavisinin tartışılması amaçlanmaktadır.

Anahtar Kelimeler

• Ameli, Sanral venöz kateterizasyon, Şilotoraks, Total parenteral nutrisyon

Chylothorax Following Central Venous Catheterization in a Patient with Tetra-Amelia

Öz

Tetra-amelia is a very rare genetic disorder, characterized by the complete absence of all extremities. Patients often have to be hospitalized because of pulmonary infection and associated abnormalities. In this patient group, venous access is possible by the insertion of either a peripheral intracath at the scalp/neck or a central venous catheter (CVC) at the internal jugular vein. Following central venous catheterization, many complications such as vessel and nerve injury, pneumothorax, hemothorax, cardiac tamponade, pulmonary embolism, arrhythmia, sepsis, and infection at the insertion site have been reported in the English literature. Chylothorax, a rare complication following central venous catheterization, can be decreased by preferring insertion of the CVC to the right internal jugular vein and using a flexible CVC of an appropriate length and gauge. Herein, insertion of CVC in a case with tetra-amelia and diagnosis and treatment of CVC-related chylothorax were summarized

Anahtar Kelimeler

• Amelia, Central venous catheterization, Chylothorax, Total Parenteral Nutrition

Kaynakça

1. Niemann S, Zhao C, Pascu F, Stahl U, Aulepp U, Niswander L, et al. Homozygous WNT3 mutation causes tetra-amelia in a large consanguineous family. Am J Hum Genet 2004;74:558-63.
2. Ohdo S, Madokoro H, Sonoda T, Takei M, Yasuda H, Mori N. Association of tetra-amelia, ectodermal dysplasia, hypoplastic lacrimal ducts and sacs opening towards the exterior, peculiar face, and developmental retardation. J Med Genet 1987;24:609- 12.
3. Sousa SB, Pina R, Ramos L, Pereira N, Krahn M, Borozdin W, et al. Tetra-amelia and lung hypo/aplasia syndrome: New case report and review. Am J Med Genet A 2008;146A:2799-803.
4. Williams AR, Bailey MK. Anesthetic management of a patient with tetra-amelia. South Med J 1999;92:325-7.
5. Taylor RW, Palagiri AV. Central venous catheterization. Crit Care Med 2007;35:1390-6.
6. Gullu AU, Senay S. Central venous catheter related stenosis and thrombosis of Superior Vena Cava: An update of treatment strategies. Turkiye Klinikleri J Cardiovasc Sci 2010;22:344-51.
7. Le Coultre C. Chylothorax. In: Ziegler MM, Azizkhan RG, Weber TR(eds). Operative Pediatric Surgery. 1 st ed. New York: McGraw- Hill, 2003:465-74.
8. Beghetti M, La Scala G, Belli D, Bugmann P, Kalangos A, Le Coultre C. Etiology and management of pediatric chylothorax. J Pediatr 2000;136:653-8.

9. Ross P Jr, Seashore JH. Bilateral hydrothorax complicating central venous catheterization in a child: Case report. J Pediatr Surg 1989;24:263-4.
10. Flatley ME, Schapira RM. Hydropneumomediastinum and bilateral hydropneumothorax as delayed complications of central venous catheterization. Chest 1993;103:1914-6.
11. Ulíbarri JI, Sanz Y, Fuentes C, Mancha A, Aramendia M, Sánchez S. Reduction of lymphorrhagia from ruptured thoracic duct by somatostatin. Lancet 1990;336:258.
12. Binkert CA, Yucel EK, Davison BD, Sugarbaker DJ, Baum RA. Percutaneous treatment of high-output chylothorax with embolization or needle disruption technique. J Vasc Interv Radiol 2005;16:1257-62.