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 Table of Contents  
Year : 2019  |  Volume : 6  |  Issue : 2  |  Page : 249-251

Pheochromocytoma in pregnancy: a perilous coexistence

Department of Anaesthesiology, Pandit Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India

Date of Submission19-Apr-2018
Date of Acceptance12-Dec-2018
Date of Web Publication12-Jun-2019

Correspondence Address:
Renu Bala
Department of Anaesthesiology, Pandit Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/roaic.roaic_35_18

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Hypertension in pregnancy is most commonly attributed to preeclampsia; other potentially disastrous hypertensive disorders such as pheochromocytoma (PCC) remain understudied. PCC is a tumor of catecholamine producing cells of the adrenal medulla. Administration of anesthesia to patients with PCC is a conundrum. Presence of pregnancy further aggravates the difficulties. Safety of both mother and fetus is very crucial. We describe a pregnant patient diagnosed with PCC in mid-trimester who underwent successful tumor resection. Our case illustrates that with proper preoperative preparation, comprehensive teamwork, and painstaking anesthetic management, favourable outcome is possible even in such risky cases.

Keywords: hypertension, laparotomy, pheochromocytoma, pregnancy

How to cite this article:
Bala R, Seelwal D, Vashisht G, Kiran S. Pheochromocytoma in pregnancy: a perilous coexistence. Res Opin Anesth Intensive Care 2019;6:249-51

How to cite this URL:
Bala R, Seelwal D, Vashisht G, Kiran S. Pheochromocytoma in pregnancy: a perilous coexistence. Res Opin Anesth Intensive Care [serial online] 2019 [cited 2019 Oct 15];6:249-51. Available from: http://www.roaic.eg.net/text.asp?2019/6/2/249/260143

  Introduction Top

A pheochromocytoma (PCC) in a pregnant patient is one of the most life-threatening medical conditions. Fortunately, it is extremely rare, with an incidence of 0.2 per 10 000 pregnancies (approximately one in 54 000). However, their potential to cause severe cardiac problems makes them highly detrimental to both the fetus and the mother [1]. Its diagnosis is not so easy, as the features may mimic other disorders of pregnancy. If not diagnosed early and managed properly, there is high fetomaternal mortality [2],[3].

When such patients are posted for resection of tumor, which is the definitive treatment for these tumors, anesthetic management is very difficult and challenging even to the most experienced anesthesiologist [4]. The primary goal of an anesthetic is to provide stable hemodynamics in the face of catecholamine surges followed by the opposite scenario after tumor ligation. A teamwork of endocrinologist, anesthesiologist, surgeon, and obstetrician is of paramount importance for successful outcome. We report and describe anesthetic management of a 23-year-old primigravida posted for PCC resection. The patient underwent successful tumor resection in mid-trimester and uneventful cesarean delivery of a healthy baby at term.

  Case report Top

A 23-year-old, primigravida with 18 weeks of pregnancy had a history of headache, palpitation, dizziness, and pain in abdomen since 1 year. These symptoms got aggravated since pregnancy. Her blood pressure (BP) was 160/100 mmHg and pulse rate was 100/min. She was put on methyldopa 500 mg 12 hourly, but neither her symptoms nor hypertension resolved. There was no proteinuria or pedal edema. Ultrasonography of abdomen done for assessing fetal well-being revealed left adrenal mass. Biochemical tests showed raised plasma norepinephrine levels (2998 pg/ml); urine norepinephrine and metanephrine levels were also raised (1000 pg/ml and 7 mg/day, respectively). MRI scan of abdomen confirmed diagnosis of PCC; there was 4×4 cm left adrenal mass.

The patient was put on prazocin 5 mg 12 hourly, and within 7–10 days, her BP was controlled. There was no tachycardia or dysrhythmia, thus ruling out requirement of β-blocker. At 20 weeks of pregnancy, she was posted for surgical resection of adrenal tumor. Preoperative investigations like complete hemogram, liver function tests, renal function tests, coagulation profile, ECG, and echocardiograph were conducted, and all were within normal limits. Obstetrician opinion was sought, and patient was put on tocolytics (oral nifedepine 20 mg, 8 hourly) a day before surgery and which continued 3 days following surgery. Premedication in the form of tablet ranitidine 150 mg and diazepam 5 mg was given at night and 2 h before surgery. Baseline vitals were BP − 110/70 mmHg and pulse − 78/min. All routine monitors were attached, intravenous access was obtained, and preoxygenation was done for 3 min. Induction of anesthesia was done with injectable thiopentone 275 mg, fentanyl 100 mcg, and vecuronium 6 mg. Injectable lignocaine 80 mg was given 90 s before intubation; modified rapid sequence induction [5] was done, and endotracheal intubation was carried out. Under all aseptic precautions, left radial artery and right internal jugular vein were cannulated. Infusion of nitroglycerine (NTG) and labetalol was started and titrated according to BP and pulse. Maintenance of anesthesia was done with oxygen (40%) and nitrous oxide (60%) in sevoflurane (0.5–1.5%) to maintain adequate depth of anesthesia. Hourly supplementation of fentanyl (50 mcg) and vecuronium (1 mg) was done. Blood sugar charting was done hourly with the help of glucometer. BP remained in the range of 140–170/80–96 mmHg and pulse rate 80–100/min. Once tumor was isolated and draining vein ligated, infusion of NTG and labetalol was stopped. Although the BP decreased to 90/40 mmHg, it responded to noradrenaline infusion. The surgery took 3 h during which blood loss was 400 ml, which was calculated by counting the number of sponges and blood in suction bottle (the amount of saline used was deducted from it). Intravenous fluid infused was 2 l crystalloid and 500 ml colloid. She was extubated successfully and shifted to ICU. After a couple of hours, ionotrope infusion was stopped. Postoperative catecholamine levels were obtained, which showed decreasing trend. She was discharged home on sixth postoperative day. The patient was readmitted to hospital at term and underwent uneventful cesarean section under spinal anesthesia; a healthy male child was delivered with Apgar score of 9/10.

  Discussion Top

PCC is a neuroendocrine tumor of the chromaffin cells of the adrenal medulla that secrete catecholamines. They are more frequent in third to fifth decade, and there is no sex preponderance [1],[6],[7]. In pregnancy, they are quite rare and their diagnosis is quite difficult. These tumors have varied presentations, which may be attributed to preeclampsia. One should keep high index of suspicion if hypertension does not resolve with routine antihypertensives, and there are nonspecific features such as sweating and palpitation. Furthermore, features of preeclampsia like proteinuria and pedal edema are absent in PCC [2],[8]. Our patient possibly had tumor before conception, but the features got aggravated during pregnancy. This is owing to increased vascularity of tumors causing more catecholamine secretion and increased pressure on PCC because of increased intra-abdominal pressure owing to either growing fetus or fetal movements [4].

The diagnosis is based on biochemical tests, ultrasonography, and MRI. The use of other imaging modalities such as computed tomography scan is limited in pregnancy [8]. Following the confirmation of diagnosis, next line of management is to achieve hemodynamic stability and proceed for surgical resection of tumor, which is the definitive treatment for this pathology [2]. The decision is guided by multiple factors, and they are location of tumor, gestational age of pregnancy, adequacy of BP control, multiple or malignant tumors, and accessibility of the lesion to surgery [4]. Second trimester is considered to be the best period for any nonobstetric surgery in pregnant patient as chances of teratogenicity and miscarriage are minimal [9]. In our patient, the presentation was in second trimester and after achieving cardiovascular stability surgery was planned. We preferred prazocin − an α-blocker which is short acting and its effect can be titrated easily, although other drugs such as phenoxybenzamine have also been used. After α-blockade, if there is tachycardia or arrhythmia, β-blockers may be administered, but we did not require them [6],[10]. Proper preoperative preparation of patient decreases intraoperative hemodynamic fluctuations [11].

In addition to sedation and anxiolysis, tocolysis and aspiration prophylaxis are very crucial in antenatal patient. Various noxious stimuli such as patient movement, laryngoscopy and intubation, and surgical handling can cause catecholamine surge and hypertensive crisis. Thus, the main goal of anesthesia is to avoid drugs and events that may lead to sympathetic stimulation [6],[10]. In our patient, NTG and labetalol infusion sufficed to control BP. Sodium nitroprusside has been used by few but cyanide toxicity of fetus may occur. Anesthetic drugs that do not lead to sympathetic stimulation should be preferred. Among inhalational agents, sevoflurane is considered as the best agent. Halothane for its arrhythmogenic potentials and desflurane for its sympathetic stimulation should be avoided. Neuromuscular blockers like atracurium and succinylcholine should not be used. Short-acting opioids (fentanyl and remifentanil) are indicated. Above all, adequate depth of anesthesia should be maintained. Once the tumor is isolated, hypotension occurs and inotropes are administered [6],[10],[12]. We used noradrenaline, though phenylephrine can also be used. Blood glucose monitoring is very important in these cases because fluctuating levels of catecholamines may lead to hypoglycemia or hyperglycemia.

Laparoscopic resection of PCC is preferred nowadays; however, in our patient, laparotomy was opted by surgeons [4]. The choice of technique does not alter patient outcome, and it should be left to the discretion of the surgeon. Recent review of PCC cases has shown that there is dramatic improvement in outcome of these patients. This is probably owing to advanced surgical and anesthetic techniques, monitoring equipment, and short-acting safe drugs [2]. Despite these, the perioperative period carries significant risks for major adverse cardiac complications. Both maternal and fetal aspects need to be considered, and a multidisciplinary team is very essential for careful planning. Our case highlights that proper preoperative optimization, meticulous intraoperative planning, and hemodynamic management can improve maternal and fetal outcome.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Sarathi V, Lila AR, Bangdar TR, Menon PS, Shah NS. Pheochromocytoma and pregnancy: a rare but dangerous combination. Endocr Pract 2010; 16:300–309.  Back to cited text no. 1
Yulia A, Seetho IW, Ramineni A, Jaiyesimi RAK. Pheochromocytoma in pregnancy: a review of literature. Obstet Gynecol Cases Rev 2016; 3:096.  Back to cited text no. 2
Oliva R, Angelos P, Kaplan E, George B. Pheochromocytoma in pregnancy: a case series and review. Hypertension 2010; 55:600–606.  Back to cited text no. 3
Doo AR, Kim D, Cha KN, Han YJ, Kim DC. Anesthetic management of a pregnant woman undergoing laparoscopic surgery for pheochromocytoma − a case report. Korean J Anesthesiol 2013; 64:373–375.  Back to cited text no. 4
Ehrenfeld J, Cassedy EA, Forbes VE, Mercaldo ND, Sandberg WS. Modified rapid sequence induction and intubation: a survey of United States current practice. Anesth Analg 2012; 115:95–101.  Back to cited text no. 5
Ramakrishna H. Pheochromocytomaresection: current concepts in anesthetic management. J Anaesthesiol Clin Pharmacol 2015; 31:317–323.  Back to cited text no. 6
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Ahlawat SK, Jain S, Kumari S, Varma S, Sharma BK. Pheochromocytoma associated with pregnancy: case report and review of literature. Obstet Gynecol Surv 1999; 54:728–737.  Back to cited text no. 9
Dugas G, Fuller J, Singh S, Watson J. Pheochromocytoma and pregnancy: a case report and review ofanesthetic management. Can J Anaesth 2004; 51:134–138.  Back to cited text no. 10
Bruynzeel H, Feelders RA, Groenland TH, van den Meiracker AH, van Eijck CH, Lange JF et al. Risk factors for hemodynamic instability during surgery for pheochromocytoma. J Clin Endocrinol Metab 2010; 95:678–685.  Back to cited text no. 11
Kulkarni S, Kulkarni S, Futane S, Pachore P. Anesthesia for combined caesarean section and pheochromocytoma resection. J Obstet Anaesth Crit Care 2017; 7:97–99.  Back to cited text no. 12
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