Adrenalectomy/Excision of pheochromocytoma

From WikiAnesthesia

Pheochromocytoma is a rare neuroendocrine disease of the adrenal gland where catecholamine-secreting tumors cause hypertension. Pheochromocytoma is present in 0.05%[1] - 0.2%[2] of hypertensive individuals, the incidence of the disease presents equally between men and women with a distribution across age groups but peaks in between 40 and 50 years of age. The classic presentation of the disease is a triad of symptoms including headache, palpitations, diaphoresis with a documented clinical sign of hypertension (present in 90% of patients with pheochromocytoma)[3]. However, patients can often present with less definitive symptoms such as tremor, anxiety, flushing, weight loss, and hyperglycemia. While the majority of pheochromocytoma emerges from adrenal tumors, roughly 15-20% can be extra-adrenal in etiology.

During the early part of the 20th century, the perioperative mortality of this disease ranged between 26-50%. As surgery is curative in about 90% of presenting cases, the mortality has decreased to roughly 1% in specialized centers. Surgical resection can be approached in a variety of ways: (1) open laparotomy; (2) laparoscopic transabdominal; (3) laparoscopic retroperitoneal; and (4) single incision laparoscopic retroperitoneal. Each approach has different indications, advantages and disadvantages, as well as unique line and monitoring choices. The largest North American series published about pheochromocytoma excision described 108 cases, where 90% were conducted laparoscopically, and the perioperative morbidity rate was 13% without a single mortality[4].

Adrenalectomy/Excision of pheochromocytoma
Anesthesia type General
Airway ETT
Lines and access Arterial line

Additional large-bore PIVs
± Central venous catheter
± Pulmonary Artery Catheter

± Epidural catheter (dependant upon approach)
Monitors Standard Monitors

Invasive blood pressure monitor
± Central venous catheter

± Pulmonary Artery Catheter
Primary anesthetic considerations
Preoperative Preoperative alpha-blockade
Intraoperative Rapid episodes of extreme hypertension

Severe hypotension after adrenal vein ligation Cardiovascular collapse Hyperglycemia

Hypovolemia
Postoperative Residual hypertension

Prolonged hypotension (requiring vasopressors)

Hyperglycemia/Hypoglycemia


Preoperative management

Patient evaluation

System Considerations
Neurologic
  • Investigate headaches and fatigue
Cardiovascular
  • Evaluate history of chest pain, palpitations, arrythmia and signs of heart failure
  • Patients may require EKG or echocardiography
  • Patients may present with catecholamine-induced, Takotsubo, or dilated cardiomyopathy[5][6]
Respiratory
  • Classify obstructive or restrictive lung disease
Gastrointestinal
Hematologic
Renal
  • Evaluate electrolyte disturbances[7]
  • Evaluate fluid status as patients are often hypovolemic from catecholamine excess
Endocrine
  • Patients may be functionally hyperglycemic due to excessive catacholamine release
Other

Labs and studies

  • ± electrocardiogram to investigate palpitations, arrhythmia, cardiac ischemia, bundle branch block, or left ventricular hypertrophy
  • ± echocardiogram to assess signs of heart failure, Takotsubo cardiomyopathy, or to diagnose cardiac paragangliomas
  • Capillary glucose to test temporary insulin resistance

Operating room setup

  • Infusion manifold with high rate carrier
  • Vasopressor infusion (typically non-direct sympathomimetics such as vasopressin)
  • Direct vasodilator infusion
  • ± insulin infusion to treat hyperglycemia
  • Diluted push syringes of vasodilators and vasopressors to adjust blood pressure with sudden changes to blood pressure during catecholamine surges during induction and tumor manipulation.

Patient preparation and premedication

Perioperative α-blockade: α-blockade continues to be a staple medication therapy for 10-14 days prior to pheochromocytoma excision

  • Choice between Irreversible non-selective α-blockade (Phenoxybenzamine) or non-selective α-blockade (Doxazosin, Prazosin, and Terazosin)
  • α-blockade blockade adequacy was originally described by the Roizen Criteria[8]:
    1. Blood pressures < 160/90 for 24 hours prior to surgery
    2. Absence of orthostatic hypotension
    3. Absence of ST or T-wave changes prior to surgery
    4. No more than 5 premature ventricular contractions in a minute.
  • However, several centers have recently abandoned these strict criteria, given it's largely inpatient applications, and use a combination of symptoms of orthostatic hypotension, blood pressures, and duration of α-blockade to guide block adequacy.
  • As titration of α-blockade increases prior to surgery, patients will typically exhibit tachycardia. At this time β-blockade or calcium-channel blockade may be introduced. It is still recommended to introduce β-blockade only after several days of α-blockade titration to avoid unopposed α-agonism from the circulating catecholamines, which may cause extreme hypertensive episodes for the patient.

Pre-Operative anxiolysis and analgesia:

  • Catecholamine surges can occur with any noxious stimuli such as laryngoscopy, positive pressure ventilation, or abdominal insufflation. Preoperative anxiolysis and analgesia is useful to prevent catecholamine surges during these episodes.


Early Fluid Resuscitation:

  • Patients are often intravascularly dry due to excessive catecholamines. Early infusion of fluid to establish euvolemia prior to the clamp of the adrenal vein is advisable.
  • Without proper resuscitation during the day-of-surgery or early intraoperative period, patients may exhibit drastic hypotension once the pheochromocytoma is removed.

Regional and neuraxial techniques

  • Epidural analgesia may be useful for patients undergoing open laparotomy approach for pheochromocytoma excision

Intraoperative management

Monitoring and access

  • Standard monitors
  • Arterial line for immediate blood pressure management and electrolyte sampling
  • +/- Central Venous Catheter (CVC) for vasoactive drug infusions (some specialized centers are moving away from CVC insertion)
  • +/- Pulmonary artery catheter for severe heart failure or pulmonary hypertension
  • Foley catheter to monitor fluid status

Induction and airway management

  • Endotracheal tube (consider armored ETT for prone position)

Positioning

  • Supine for open laparotomy or combined procedures for multiple endocrine neoplasia presentations
  • Lateral for transabdominal laparoscopic approach
  • Prone for retroperitoneal laparoscopic approach

Maintenance and surgical considerations

Emergence

Postoperative management

Disposition

Pain management

Potential complications

Procedure variants

Open Laparotomy Transabdominal

Laparoscopic

Retroperitoneal

Laparoscopic

Single-Incision Retroperitoneal

Laparoscopic[9]

Unique considerations High Insufflation pressures (20-30mmHg)
Position Supine Lateral Prone Prone and half jacknife position (praying position)
Surgical time 4-6 hrs 3-5 hrs 1.5 hrs 1.6 hrs
EBL 5 mL 5 mL
Postoperative disposition PACU or ICU PACU or ICU PACU PACU
Pain management Epidural Oral and IV pain medications Oral pain medications Oral pain medications (76%)
Potential complications Subcutaneous emphysema Subcutaneous emphysema
Length of Stay 1.4 days 1.1 days[10]

References

  1. Lo, Chung-Yau; Lam, King-Yin; Wat, Ming-Sun; Lam, Karen S. (2000-03-01). "Adrenal pheochromocytoma remains a frequently overlooked diagnosis". The American Journal of Surgery. 179 (3): 212–215. doi:10.1016/S0002-9610(00)00296-8. ISSN 0002-9610. PMID 10827323.
  2. Yeh, Michael; Livhits, Masha; Duh, Quan-Yang (2022). "The Adrenal Glands". Sabiston textbook of surgery : the biological basis of modern surgical practice. Courtney M., Jr. Townsend, R. Daniel Beauchamp, B. Mark Evers, Kenneth L. Mattox, David C. Sabiston (21st ed.). St. Louis, Missour. ISBN 978-0-323-64064-0. OCLC 1235959889.
  3. Peramunage, Dasun; Nikravan, Sara (2020-03-01). "Anesthesia for Endocrine Emergencies". Anesthesiology Clinics. 38 (1): 149–163. doi:10.1016/j.anclin.2019.10.006. ISSN 1932-2275. PMID 32008649.
  4. Shen, Wen T.; Grogan, Raymon; Vriens, Menno; Clark, Orlo H.; Duh, Quan-Yang (2010). "One hundred two patients with pheochromocytoma treated at a single institution since the introduction of laparoscopic adrenalectomy". Archives of Surgery (Chicago, Ill.: 1960). 145 (9): 893–897. doi:10.1001/archsurg.2010.159. ISSN 1538-3644. PMID 20855761.
  5. Prejbisz, Aleksander; Lenders, Jacques W.M.; Eisenhofer, Graeme; Januszewicz, Andrzej (2011). "Cardiovascular manifestations of phaeochromocytoma". Journal of Hypertension. 29 (11): 2049–2060. doi:10.1097/HJH.0b013e32834a4ce9. ISSN 0263-6352.
  6. Gu, Yu Wei; Poste, Jennifer; Kunal, Mehta; Schwarcz, Monica; Weiss, Irene (2017). "Cardiovascular Manifestations of Pheochromocytoma". Cardiology in Review. 25 (5): 215–222. doi:10.1097/CRD.0000000000000141. ISSN 1061-5377.
  7. Peramunage, Dasun; Nikravan, Sara (2020-03-01). "Anesthesia for Endocrine Emergencies". Anesthesiology Clinics. 38 (1): 149–163. doi:10.1016/j.anclin.2019.10.006. ISSN 1932-2275. PMID 32008649.
  8. Roizen, M.F.; Horrigan, R.W.; Koike, M.; Eger, E.I.; Mulroy, M.F.; Frazer, B.; Simmons, A.; Hunt, T.K.; Thomas, C.; Tyrell, B. (1982-09-01). "A PROSPECTIVE RANDOMIZED TRIAL OF FOUR ANESTHETIC TECHNIQUES FOR RESECTION OF PHEOCHROMOCYTOMA". Anesthesiology. 57 (3): A43–A43. doi:10.1097/00000542-198209001-00043. ISSN 0003-3022.
  9. Sho, Shonan; Yeh, Michael W.; Li, Ning; Livhits, Masha J. (2017). "Single-incision retroperitoneoscopic adrenalectomy: a North American experience". Surgical Endoscopy. 31 (7): 3014–3019. doi:10.1007/s00464-016-5325-8. ISSN 0930-2794.
  10. Sho, Shonan; Yeh, Michael W.; Li, Ning; Livhits, Masha J. (2017). "Single-incision retroperitoneoscopic adrenalectomy: a North American experience". Surgical Endoscopy. 31 (7): 3014–3019. doi:10.1007/s00464-016-5325-8. ISSN 0930-2794.