A 42-year-old man (175 cm in height, 61.8 kg in weight), with no medical and family history, experienced a temporal headache and nausea. He began to experience excessive sweating after meals and subsequently developed high blood pressure with the systolic pressure reaching 180 mmHg. Further examination revealed an elevated level of urinary noradrenaline excretion of 6460 μg/day (approximately 40 times above the upper limit of normal) and an increased serum concentration of interleukin-6 (IL-6) (74 ng/L, normally < 4.0 ng/L). Computed tomography (CT) images showed a 4.6-cm round mass on his right adrenal gland. Increased uptake of iodine-123-metaiodobenzylguanidine (MIBG) in both adrenal glands observed during iodine-123-MIBG scintigraphy and positron emission tomography (Fig. 1) led to the diagnosis of bilateral pheochromocytomas.
The patient was scheduled for surgical resection and was treated with a combination of bunazosin hydrochloride, phentolamine mesylate, and carvedilol to achieve prophylactic cardiovascular stabilization. Approximately 3 weeks later, he suddenly experienced dyspnea with a persistent fever peaking at 38.9 °C, and systemic administration of broad spectrum antibiotics was immediately initiated for a suspected bacterial pneumonia. However, the antibiotics were ineffective, and his respiratory condition deteriorated with PaO2 at 69 mmHg while receiving 5 L/min of oxygen via a face mask. Chest radiographs and CT images showed bilateral infiltration and air bronchograms, indicating pulmonary edema (Fig. 2). We found increased serum concentration of liver transaminases, suggesting hepatic insufficiency. Transthoracic echocardiography showed no signs of cardiac failure. The patient was suspected of developing pheochromocytoma multisystem crisis, a life-threatening condition that could cause permanent organ damage in a short span of time, and we thus decided to perform an emergency bilateral total adrenalectomy.
On arrival in the operating room, the vital signs were as follows: ABP 144/78 mmHg, heart rate 109 beats/min (sinus rhythm), SpO2 95% (under 5 L/min of oxygen via a face mask), and body temperature 38.7 °C. General anesthesia was induced with target-controlled infusion (target-controlled infusion pump: TE-371 Telfusion ® TERUMO Co.Tokyo, Japan) of an effect site concentration of 2.5 μg/mL propofol, 200 μg of fentanyl, and 40 mg of rocuronium bromide to facilitate endotracheal intubation. Epidural anesthesia was waived because of his respiratory discomfort. General anesthesia was maintained with continuous infusion of propofol (constantly set at 2.6 μg/mL during the surgery with the bispectral index 45–63) and remifentanil (constantly set at 0.25 μg/kg/min) and intermittent supplementation of rocuronium bromide. The PaO2/FiO2 ratio was 72 at the beginning of the surgery.
The radial artery catheter was connected to the FloTrac™ sensor (third generation: version 3.06, Edwards Life Science, Irvine, CA, USA) to measure APCO along with arterial waveform and stroke volume variation (SVV). The pulmonary artery catheter, which was placed in the right jugular vein, was connected to the Vigilance™ monitor (Edwards Life Science, Irvine, CA, USA) to measure PACO using the thermodilution method. Phentolamine mesylate, landiolol hydrochloride, and prostaglandin E1 were continuously infused to stabilize blood pressure and heart rate. When the surgeons started to operate the right adrenal gland, ABP surged to 218/113 mmHg, which required an immediate bolus injection of phentolamine mesylate. Once the right adrenal gland vessels were ligated, ABP dropped to 70/30 mmHg and SVV jumped from 8 to 9% to 17%, probably as a result of systemic vasodilation and hypovolemia. Administration of a continuous infusion of norepinephrine (0.05–0.2 μg/kg/min) in combination with a rapid infusion of 1000 mL of albumin solution stabilized hemodynamics with ABP raising to 91/50 mmHg and SVV returning to less than 10%.
While the right adrenal gland was being operated, APCO fluctuated markedly in more or less inverse proportion to the change in ABP, whereas PACO shifted rather smoothly (Fig. 3). However, there was no appreciable difference between APCO and PACO while the left adrenal gland was being resected, the histological examination of which revealed no abnormal findings. After bilateral resection of the adrenal glands, ABP remained around 80/40 mmHg with a continuous infusion of norepinephrine (0.1 μg/kg/min). The total time required for surgery and general anesthesia were 214 and 308 min, respectively. The intraoperative fluid balance was + 1575 mL (5.1 mL/kg/h) with an estimated blood loss of 610 mL and urine output of 415 mL. The patient did not receive blood transfusions.
After surgery, he remained ventilated for 3 days in the intensive care unit (ICU). The PaO2/FiO2 ratio, which was initially 214, improved gradually over the next few days. Norepinephrine was tapered off, and the circulatory hemodynamics was stable thereafter. After being extubated on postoperative day (POD) 4, he was transferred to the ward on POD 5 and was uneventfully discharged on POD 14.