Mehmet ÇÖMEZ, Ahmet KACIROĞLU, Mehmet AKSOY, Ali AHISKALIOĞLU, İlker İNCE

Anaesthesia in Poland syndrome: A case report

Poland sendromu nadir görülen bir konjenital anomalidir. Kas iskelet sistemi, kalp, böbrek ve akciğerlerle ilgili birçok anomaliyi içerir. Bu sendrom sıklıkla göğüs deformitesi ile karakterizedir. İpsilateral el anomalileri en önemli özelliği olup sindaktili ve ektrodaktili gibi el anomalileri de beraberinde görülebilir. Hastalarda genel anestezi indüksiyonu ve idamesinde kas gevşetici kullanımına bağlı akciğer komplikasyonları gelişebilir. Ayrıca genel anestezi bu hastalarda malign hipertermi gelişme riskini arttırır. Malign hipertermi ve solunumsal komplikasyon gelişebilmesi riski nedeniyle genel anestezi dikkatli bir şekilde uygulanmalıdır. Biz Poland sendromlu, meme rekonstrüksiyon cerrahisi uygulanan 23 yaşında bayan hastanın anestezi yönetimini sunmayı amaçladık.

Poland sendromunda anestezi: Olgu Raporu

Poland syndrome is a rare congenital anomaly. It includes in different anomalies such as musculo-skeletal system, heart, kidneys and lungs. This syndrome is characterized by thorax deformity most commonly. Ipsilateral hand anomalies are most important features and can be seen as syndactyly or ectrodactyly. Patients may have lung complications due to the use of muscle relaxants, which is used induction and maintenance of general anesthesia. In addition, these patients show increased risk of malignant hyperthermia during general anesthesia. Because of malignant hyperthermia risk, general anesthesia should be provided carefully. In this report, we discuss the anesthetic concerns related to the management of a 23-yearold female patient with right-sided Poland syndrome undergoing breast reconstruction surgery.

PDF

___

Kevin P, David CS. Disorders of sternum and the tho- racic wall. In: Sabiston DC, Spencer FC (Eds.). Sur- gery of the Chest, 6th ed. Philadelphia: WB Saunders, 1995: 507-511.
Sethuraman R, Kannan S, Bala I, et al. Anaesthesia in Poland syndrome. Can J Anaesth 1998; 45 : 277-279.
Erden IA, Koseoglu A, Kurkcuoglu BS, et al. The anes- thetic management in the patient with Kabuki makeup syndrome. J Clin Exp Invest 2013;4:116-118.
Yılmaz A, Acar HV, Tezer E, et al. Anesthesia for sco- liosis surgery in a patient with arthrogryposis multiplex congenita. J Clin Exp Invest 2013;4:113-115.
Kupper HJ. Anaesthesia in Poland's syndrome. Can J Anaesth 1999;46:513-514.
Fokin AA, Robicsek F. Poland's syndrome revisited. Ann Thorac Surg 2002;74:2218-2225.
Kabukcu HK, Sahin N, Kanevetci BN, et al. Anaesthet- ic management of patient with Poland syndrome and rheumatic mitral valve stenosis: A case report. Ann Card Anaesth 2005; 8:145-147.
Bavinck JNB, Weaver DD. Subclavian artery supply disruption sequence: hypothesis of a vascular etiol- ogy for Poland, Klippel-Feil and Möbius anomalies. Am J Med Genet 1986;23:903-918.