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 Table of Contents  
Year : 2023  |  Volume : 13  |  Issue : 1  |  Page : 116-118

Anesthetic management of an emergency cesarean section in a parturient with inoperable paraganglioma: A challenging case report

Department of Anesthesiology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia

Date of Submission21-Jul-2022
Date of Acceptance03-Oct-2022
Date of Web Publication09-Mar-2023

Correspondence Address:
Dr. Samahir AlJubairy
Department of Anesthesiology, King Faisal Specialist Hospital and Research Center, Almather, Makka Almukarama Road, Riyadh - 11564
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JOACC.JOACC_45_22

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Paraganglioma is a neuroendocrine tumor characterized by small organs comprising neuroendocrine cells that can produce catecholamines. Because of the nature of such tumors, hemodynamic instability can pose a management challenge. This condition can influence the provision of medical care across different clinical settings. The aim of this case report is to highlight anesthesia management in parturient patients with abdominopelvic paraganglioma, particularly during an emergency cesarean section. At 34 weeks of gestation, the 33-year-old female patient with three pregnancies and two past deliveries had a known case of inoperable large pelvic and lower abdominal retroperitoneal paraganglioma and poor radiotherapy response. The 15.5 × 4.2 × 22 cm tumor encased the aorta, inferior vena cava, and bilateral iliac vessels, compressing the lumbar vertebrae. The patient had an uneventful and successful cesarean delivery with a multidisciplinary team effort. Paragangliomas are rarely reported in a parturient and its optimization course. Despite being interrupted by an emergency cesarean section, which was handled safely and effectively, both mother and fetus had a safe delivery and postoperative recovery without complications.

Keywords: Abdominopelvic paraganglioma, cesarean section, hypertension, pheochromocytoma, pregnancy

How to cite this article:
AlJubairy S, Alwatban SJ, AlRaffa A. Anesthetic management of an emergency cesarean section in a parturient with inoperable paraganglioma: A challenging case report. J Obstet Anaesth Crit Care 2023;13:116-8

How to cite this URL:
AlJubairy S, Alwatban SJ, AlRaffa A. Anesthetic management of an emergency cesarean section in a parturient with inoperable paraganglioma: A challenging case report. J Obstet Anaesth Crit Care [serial online] 2023 [cited 2023 Apr 1];13:116-8. Available from: https://www.joacc.com/text.asp?2023/13/1/116/371317

Pheochromocytoma and paraganglioma (PPGLs) encase 1 in 54,000 pregnancies, posing an elevated risk to both the mother and child. Timely intervention is of immense importance.[1] Paraganglioma is characterized by catecholamines in the sympathetic paraspinal region. Usually, these tumors appear in the abdomen, pelvis, and thorax.[2] A neuroendocrine tumor known as paraganglioma is characterized by small organs comprising neuroendocrine cells that can produce catecholamines.[1],[2] Paragangliomas can cause high blood pressure, headaches, and sweating. Although PPGLs are neuroendocrine tumors, diagnosing such a disease in pregnancy poses a challenge due to the similarities in signs and symptoms.[2]

  Case Presentation Top

The patient was a 33-year-old female with three pregnancies and two past deliveries. Her family history was negative for neurofibromatosis, Von Hippel–Lindau syndrome, paraganglioma syndrome, and malignancy. She was a known case of inoperable large pelvic and lower abdominal retroperitoneal paraganglioma with catecholamine hypersecretion. In addition, she had type 2 diabetes and was on insulin, taking 100 mg of labetalol twice daily.

In April 2015, the patient had a complicated road traffic accident, causing a transection of an aneurysm in the descending aorta (5 cm), and underwent thoracic endovascular aortic repair. She had stent grafting of the distal arch and descending aorta through the right common femoral artery. The patient followed up with her primary team annually since her repair.

The patient was referred from a local hospital to King Faisal Specialist Hospital and Research Center because of vague back pain, occasional headaches, and hypertension. Magnetic resonance imaging (MRI) of her spine revealed an incidental finding of a mass, which was biopsied. The results indicated a maturing ganglioneuroma, and she was referred to a specialized clinic. In February 2011, the initial MRI revealed a tumor measuring around 20 × 14 cm in the lumbar retroperitoneal space encased by the abdominal aorta and psoas muscles.

Upon regular visits to medical oncology, the patient started chemotherapy and radiotherapy. The tumor responded poorly to both therapies for 5 to 6 years. The debulking decision was taken after a tumor board meeting. The last MRI in 2021 presented a 9.5 × 15.5 × 22 cm tumor, primarily in the lumbar retroperitoneal space, encasing the abdominal aorta, inferior vena cava, and psoas muscle. The mass extended caudally from the second lumbar vertebrae to the second sacral vertebrae level. Upon a regular visit to the hospital, she presented to the endocrine clinic at 15 weeks of gestation. The plan was to have the patient undergo debulking, but because of the unplanned conception, debulking was postponed.

Her obstetric history included two uneventful previous cesarean deliveries. In August 2013, the first cesarean section was conducted because of placenta abruption. In January 2015, the second cesarean section was due to an abnormal Doppler. At her obstetric and gynecologic appointment, her vital signs were a heart rate of 80 bpm, blood pressure of 170/90 mmHg, and O2 saturation of 99% of the ambient atmosphere. Further investigation revealed significant findings, including glycated hemoglobin levels of 8.9% and a fasting blood sugar of 240 mg\dL. In addition, urine chemistry revealed urinary normetanephrine and 3-Methoxytyramine excretion at twice that of her baseline.

After a multidisciplinary team meeting, she was started on 100 mg of metoprolol with 10 mg of prazosin. She started taking prophylactic aspirin. After a family meeting with the couple regarding the patient's condition and risks, she insisted on continuing her pregnancy. She was scheduled for an elective cesarean section under general anesthesia at 37 weeks. The patient had been discharged on 20 units of insulin detemir at bedtime, 18 units of insulin aspart, 100 mg of labetalol twice daily, and 10 mg of nifedipine once daily. The patient was initially planned to be admitted and start an alpha-blocker at 33 weeks of gestation; however, she skipped her appointment to start a preoperative medication.

She presented later at 34 weeks of gestation to the emergency department complaining of severe palpitations and sweating with shortness of breath. She had uncontrolled blood pressure of 190/120 mmHg, mean arterial pressure of 130 mmHg, a heart rate of 110 bpm, and a respiratory rate of 25 breaths per min, with an oxygen saturation level of 98% on room air. She later informed the team of her lack of compliance with the medication.

The team agreed to do an emergency cesarean section. We anticipated hemodynamic instability during surgery. The patient was connected to Standard American Society of Anesthesia monitors and invasive monitors, and two large intravenous cannulas were inserted. Instrumental delivery was planned to decrease pressure on the mother's abdomen. The patient was induced with 70 mcg of remifentanil, 150 mg of propofol, and 50 mg of rocuronium, supported with 100 mcg/min of phenylephrine infusion. The endotracheal tube was secured with right uterine displacement.

The baby was delivered using forceps. Before delivering the placenta, the hemodynamic values fluctuated, with blood pressure readings ranging from 220/96 to 180/80 mmHg with fundal pressure, which was managed by boluses of labetalol and remifentanil. In addition, 100 mcg of the uterotonic carbetocin was administered over 5 min, and 15 mg of intravenous morphine was titrated throughout the surgery. After extubating, the patients' blood pressure started to rise again, and nitroglycerin infusion was initiated at 0.3 mcg/kg/min. The patient was extubated, fully awake, oriented, responding to commands, and pain-free but complaining of headaches and dizziness. An incremental infusion of up to 0.5 mcg/kg/min of nitroglycerin was continued. Once her symptoms subsided, the patient was shifted to the intensive care unit, and intravenous patient-controlled analgesia morphine was started.

The patient consented to the publication of her case.

  Discussion Top

Paragangliomas are catecholamine-producing tumors that usually extend in growth from the sympathetic chain. Clinical findings can assist in diagnosing PPGLs in pregnancy. There can be hypertension (61.7%), sweating (11.7%), and palpitations (16.7%).[1] Moreover, PPGLs can often be misdiagnosed as preeclampsia in pregnancies because 9.8% of patients with PPGLs may live asymptomatically.[1] Pheochromocytomas share a similar histopathology and are often confused with paragangliomas because these tumors share many features. One of the distinguishing factors is where they arise.[3]

Especially for parturient early diagnosis and treatment are essential to improve maternal and fetal outcomes. According to the literature, maternal and fetal/neonatal mortalities were 9.0% and 14.2%, respectively.[1] Optimization in these challenging patients includes optimizing the blood pressure. According to the Roizen criteria, preoperative alpha-blockers can reduce mortality rates as the primary concern in these patients is to avoid a hypertensive crisis due to excess catecholamine release, which can be a life-threatening condition with an 80% death rate.[4] Beta- and alpha-blockers are used in a 1:7 ratio to optimize the blood pressure in these patients and prevent end-organ dysfunction—most significantly cardiomyopathy.[5] Patients with chronic paragangliomas may develop severe dilated cardiomyopathy with varying degrees of heart failure, increasing the overall perioperative risk.[6]

One of the goals was to minimize the pressure exerted by the gravid uterus on the tumor and avoid any exacerbation of the hemodynamics, which can affect the Apgar score and stent grafting in the patient. Neuraxial anesthesia is not an option because of tumor invasion in the lumbar vertebrae. The risk of fundal pressure can release more catecholamines. Therefore, active labor during vaginal delivery can increase secretions.[7]

In this case, the limitations included the patient's noncompliance and incomplete optimization due to the limited time. Because of her tumor presentation in [Figure 1], neuraxial anesthesia was discouraged. General anesthesia was selected to avoid manipulating the paraganglioma. Because of the insufficient preparation time, tumor prognosis, and coexisting comorbidities, it was a challenge to manage this patient.
Figure 1: Paraganglioma in the lumbar retroperitoneal space extending the second intervertebral space

Click here to view

  Conclusion Top

Paraganglioma presenting in pregnancy poses a challenging case to manage. Despite the inadequate optimization conditions and emergency situation, this case was managed in an optimal manner, considering the vascular history and other comorbidities of the patient. Addressing multiple anesthetic concerns, both mother and fetus had a safe delivery and uneventful postoperative recovery.

Ethical approval

This research case report has been approved by the research advisory council and the ethical committee at King Faisal Specialist Hospital and Research Center (RAC# 2225177).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Langton K, Tufton N, Akker S, Deinum J, Eisenhofer G, Timmers HJLM, et al. Pregnancy and phaeochromocytoma/paraganglioma: Clinical clues affecting diagnosis and outcome-a systematic review. BJOG 2021;128:1264-1272.  Back to cited text no. 1
UpToDate. Alphen aan den Rihn, Netherlands: Wolters Kluwer N.V.; c2022. Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology. Available from: https://www.uptodate.com/contents/paragangliomas-epidemiology-clinical-presentation-diagnosis-and-histology?source=bookmarks_widget. [Last accessed on 2022 Jun 4].  Back to cited text no. 2
Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med 2019;381:552-65.  Back to cited text no. 3
O'Riordan JA. Pheochromocytomas and anesthesia. Int Anesthesiol Clin 1997;35:99-127.  Back to cited text no. 4
Richards DA, Prichard BN, Boakes AJ, Tuckman J, Knight EJ. Pharmacological basis for antihypertensive effects of intravenous labetalol. Br Heart J 1977;39:99-106.  Back to cited text no. 5
Kassim TA, Clarke DD, Mai VQ, Clyde PW, Mohamed Shakir KM. Catecholamine-induced cardiomyopathy. Endocr Pract 2008;14:1137-49.  Back to cited text no. 6
Wing LA, Conaglen JV, Meyer-Rochow GY, Elston MS. Paraganglioma in pregnancy: A case series and review of the literature. J Clin Endocrinol Metab 2015;100:3202-9.  Back to cited text no. 7


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