Korean J Anesthesiol related to genes located on q22-24 of chromosome 17 and p16 of chromosome 2. There is not much known about the 2p16, but over 70% of cases with typical phenotypes have PRKAR1A subunit mutation on 17q22-24. It encodes the protein kinase A (PKA) regulatory subunit and is the key component of the cAMP signaling pathway [1,7,8]. Patients with the PRKAR1A mutation compared to patients with no mutation have clinical patterns of myxomas, skin lesions, thyroid, and gonadal tumors at a younger age [1]. The two patients in present cases did not undergo chromosomal studies. Because of the endocrine dysfunction from multiple endo- crine tumors, there are many things to consider during pre- anesthetic evaluation and during anesthesia. For instance, growth hormone-producing pituitary adenoma occurs in 12% of the patients and is presented with acromegaly [1]. Acromegaly features include macroglossia, prognathism, enlargement and distortion (development of additional folds) of the glottis structure, hypertrophy of the soft tissues of the larynx and pharynx, and longer thyromental distance. The mask ventilation in patients with acromegaly compared to normal patients is known to be more difficult, and the rate of difficult intubation is known to be higher [3,4]. Therefore, preoperative airway evaluation is essential, such as the upper lip bite test, reported to be more accurate than the Mallampati classification predicting difficult intubation [4]. The patient in case 1 had findings of typical acromegaly, but endotracheal intubation was relatively easy. Primary pigmented nodular adrenocortical disease is the most common form of adrenal gland tumor occurring in 60% of patients, and 25-30% of these patients show features of Cushing syndrome [1]. Cushing’s syndrome due to primary pigmented adrenocortical disease appears to be adrenocorticotropic hormone-independent and its diagnosis is difficult because hypercortisolism progresses over several years [1,2]. Therefore, in diagnosing Carney complex, evaluation of the adrenal function and Cushing’s syndrome related features such as fluid- electrolyte imbalance hypertension, diastolic dysfunction, glucose intolerance, and thromboembolic risk is essential [2,6]. Cardiac myxoma, a common co-morbidity, may cause cardiac blood flow obstruction (decreased filling of the left and right ventricle) [9]. Especially if the myxoma is located in the right ventricular outflow tract as in case 1, it may cause congestive heart failure due to pulmonary blood flow obstruction and right ventricle filling defect that may lead to peripheral edema, hepatomegaly, ascites, dyspnea, frequent syncope, and even sudden death [10,11]. Physical examination reveals jugular venous dilatation, ejection systolic murmur adjacent to left sternal border, diastolic murmur, 3rd heart sound, and delayed 2nd heart sound [10]. To prevent life-threatening complications, it is very impor- www.ekja.org Kang and Kim tant to prevent pulmonary embolization and pulmonary blood flow obstruction during anesthetic induction and maintenance. Drugs causing hemodynamic instability and inconvenient manipulations of the tumor and heart should be avoided. The occurrence of intra-operative central pulmonary artery embolism must be monitored with a transesophageal echocardiography [12]. In addition pre-anesthetic placement of the arterial line is imperative, and a large bore IV line and transfusion should be prepared as precautions for massive bleeding. Because Carney complex is a multiple endocrine dysfunction comprising cardiac myxomas as well as other accompanying disorders, it requires thorough preparation by anesthesiologists. First, the pre-anesthetic evaluation of the presence of an accompanying disorder must be made. Second, an accurate evaluation of multiple endocrine function and management strategy are required. Thorough physical examination including that of the airway must be done and the location, size, clinical pattern of the myxoma must be checked. The cardiac function must also be evaluated. Third, the anesthesiologist must make careful preparations for events from difficult intubation to various hemodynamic changes due to adrenal gland tumor or cardiac myxoma at the time of anesthetic induction and maintenance. Finally, the anesthesiologist must be able to respond immediately and appropriately to sudden situations arising during anesthesia such as massive bleeding from repetitive surgeries. References 1. Almeida MQ, Stratakis CA. Carney complex and other conditions associated with micronodular adrenal hyperplasias. Best Pract Res Clin Endocrinol Metab 2010; 24: 907-14. 2. Rothenbuhler A, Stratakis CA. Clinical and molecular genetics of Carney complex. Best Pract Res Clin Endocrinol Metab 2010; 24: 389-99. 3. Khan ZH, Rasouli MR. Intubation in patients with acromegaly: experience in more than 800 patients. Eur J Anaesthesiol 2009; 26: 354-5. 4. Sharma D, Prabhakar H, Bithal PK, Ali Z, Singh GP, Rath GP, et al. Predicting difficult laryngoscopy in acromegaly: a comparison of upper lip bite test with modified Mallampati classification. J Neurosurg Anesthesiol 2010; 22: 138-43. 5. Graham GW, Unger BP, Coursin DB. Perioperative management of selected endocrine disorders. Int Anesthesiol Clin 2000; 38: 31-67. 6. Maciel RT, Fernandes FC, Pereira Ldos S. Anesthesia in a patient with multiple endocrine abnormalities. Case report. Rev Bras Anestesiol 2008; 58: 172-8. 7. Bireta C, Popov AF, Schotola H, Trethowan B, Friedrich M, El- Mehsen M, et al. Carney-Complex: multiple resections of recurrent cardiac myxoma. J Cardiothorac Surg 2011; 6: 12. 8. 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