Excision of pheochromocytoma

{{Infobox surgical case reference

| anesthesia_type = General

| airway = ETT

| lines_access = Large bore IV

Arterial line

± Central line

± PA Catheter

± Epidural

| monitors = Standard

5-lead ECG

Temperature

ABP

± CVP

± PAP

| considerations_preoperative = Preoperative alpha-blockade

| considerations_intraoperative = Rapid episodes of extreme hypertension

Severe hypotension after adrenal vein ligation

Cardiovascular collapse

Hyperglycemia

Hypovolemia

| considerations_postoperative = Residual hypertension

Prolonged hypotension (requiring vasopressors)

Hyper/hypoglycemia

}}The '''excision of a pheochromocytoma''' is a variant of an [[adrenalectomy]], which is the removal of one or both adrenal glands. When the tumor being removed is a [[pheochromocytoma]], careful preoperative optimization and intraoperative management are required to ensure hemodynamic stability during the procedure.

Surgical resection can be performed via open laparotomy, laparoscopic transabdominal, laparoscopic retroperitoneal, or single incision laparoscopic retroperitoneal approaches, each of which has different indications, advantages and disadvantages, as well as unique line and monitoring choices.

==Preoperative management==

===Patient evaluation<!-- Describe the unique and important aspects of preoperative evaluation. Add or remove rows from the systems table as needed. -->===

{| class="wikitable"

|+

!System

!Considerations

|-

|Neurologic

|

* Investigate headaches and fatigue

|-

|Cardiovascular

|

*Evaluate history of chest pain, palpitations, arrhythmia and signs of heart failure

*Patients may require EKG or echocardiography

*Patients may present with catecholamine-induced, Takotsubo, or dilated cardiomyopathy<ref>{{Cite journal|last=Prejbisz|first=Aleksander|last2=Lenders|first2=Jacques W.M.|last3=Eisenhofer|first3=Graeme|last4=Januszewicz|first4=Andrzej|date=2011|title=Cardiovascular manifestations of phaeochromocytoma|url=https://journals.lww.com/00004872-201111000-00001|journal=Journal of Hypertension|language=en|volume=29|issue=11|pages=2049–2060|doi=10.1097/HJH.0b013e32834a4ce9|issn=0263-6352|via=}}</ref><ref>{{Cite journal|last=Gu|first=Yu Wei|last2=Poste|first2=Jennifer|last3=Kunal|first3=Mehta|last4=Schwarcz|first4=Monica|last5=Weiss|first5=Irene|date=2017|title=Cardiovascular Manifestations of Pheochromocytoma|url=https://journals.lww.com/00045415-201709000-00004|journal=Cardiology in Review|language=en|volume=25|issue=5|pages=215–222|doi=10.1097/CRD.0000000000000141|issn=1061-5377|via=}}</ref>

|-

|Pulmonary

|

*Classify obstructive or restrictive lung disease

|-

|Renal

|

*Evaluate electrolyte disturbances<ref>{{Cite journal|last=Peramunage|first=Dasun|last2=Nikravan|first2=Sara|date=2020-03-01|title=Anesthesia for Endocrine Emergencies|url=https://www.anesthesiology.theclinics.com/article/S1932-2275(19)30088-6/abstract|journal=Anesthesiology Clinics|language=English|volume=38|issue=1|pages=149–163|doi=10.1016/j.anclin.2019.10.006|issn=1932-2275|pmid=32008649}}</ref>

*Evaluate fluid status as patients are often hypovolemic from catecholamine excess

|-

|Endocrine

|

*Patients may be functionally hyperglycemic due to excessive catecholamine release

|}

===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

*CBC to assess baseline hemoglobin and platelet values

*CMP to identify electrolyte abnormalities such as hypernatremia or hypokalemia

*Plasma metanephrines (Normetanephrine, Norepinephrine, Epinephrine , Dopamine)

===Operating room setup<!-- Describe any unique aspects of operating room preparation. Avoid excessively granular information. Use drug classes instead of specific drugs when appropriate. If none, this section may be removed. -->===

* 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.

*IV phentolamine

===Patient preparation and premedication<!-- Describe any unique considerations for patient preparation and premedication. If none, this section may be removed. -->===

'''<u>Perioperative α-blockade</u>''': 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<ref>{{Cite journal|last=Roizen|first=M.F.|last2=Horrigan|first2=R.W.|last3=Koike|first3=M.|last4=Eger|first4=E.I.|last5=Mulroy|first5=M.F.|last6=Frazer|first6=B.|last7=Simmons|first7=A.|last8=Hunt|first8=T.K.|last9=Thomas|first9=C.|last10=Tyrell|first10=B.|date=1982-09-01|title=A PROSPECTIVE RANDOMIZED TRIAL OF FOUR ANESTHETIC TECHNIQUES FOR RESECTION OF PHEOCHROMOCYTOMA|url=https://doi.org/10.1097/00000542-198209001-00043|journal=Anesthesiology|volume=57|issue=3|pages=A43–A43|doi=10.1097/00000542-198209001-00043|issn=0003-3022|via=}}</ref>:

*#Blood pressures < 160/90 for 24 hours prior to surgery

*#Absence of orthostatic hypotension

*#Absence of ST or T-wave changes prior to surgery

*#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.

'''<u>Pre-Operative anxiolysis and analgesia</u>''':

*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.

'''<u>Early Fluid Resuscitation:</u>'''

*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<!-- Describe any potential regional and/or neuraxial techniques which may be used for this case. If none, this section may be removed. -->===

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

==Intraoperative management==

===Monitoring and access<!-- List and/or describe monitors and access typically needed for this case. Please describe rationale for any special monitors or 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<!-- Describe the important considerations and general approach to the induction of anesthesia and how the airway is typically managed for this case. -->===

*Endotracheal tube (consider armored ETT for prone position)

===Positioning<!-- Describe any unique positioning considerations, including potential intraoperative position changes. If none, this section may be removed. -->===

* 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<!-- Describe the important considerations and general approach to the maintenance of anesthesia, including potential complications. Be sure to include any steps to the surgical procedure that have anesthetic implications. -->===

*Consider pre-induction arterial line as catecholamine surge can occur during mask ventilation and intubation.

**Some centers describe a conducting a phenylephrine titration prior to induction to test the adequacy of α-blockade<ref>{{Cite journal|last=Saksa|first=Dane|last2=Shuch|first2=Brian|last3=Donahue|first3=Timothy|last4=Cusumano|first4=Lucas|last5=Yu|first5=Run|last6=Alapag|first6=Catharina|last7=Kamdar|first7=Nirav|date=2021-01-14|title=Telemedicine-Based Perioperative Management of Pheochromocytoma in a Patient With Von Hippel Lindau Disease: A Case Report|url=https://pubmed.ncbi.nlm.nih.gov/33512909|journal=A&A Practice|volume=15|issue=1|pages=e01378|doi=10.1213/XAA.0000000000001378|issn=2575-3126|pmid=33512909}}</ref>

*Catecholamine surges can occur during the following intraoperative periods<ref>{{Cite journal|last=Joris|first=J. L.|last2=Hamoir|first2=E. E.|last3=Hartstein|first3=G. M.|last4=Meurisse|first4=M. R.|last5=Hubert|first5=B. M.|last6=Charlier|first6=C. J.|last7=Lamy|first7=M. L.|date=1999|title=Hemodynamic changes and catecholamine release during laparoscopic adrenalectomy for pheochromocytoma|url=https://pubmed.ncbi.nlm.nih.gov/9895059|journal=Anesthesia and Analgesia|volume=88|issue=1|pages=16–21|doi=10.1097/00000539-199901000-00004|issn=0003-2999|pmid=9895059|via=}}</ref>: intubation < Positioning < Insufflation < tumor manipulation

*Treat and control hypertension prior to adrenal vein ligation.

**Start with vasodilators (nitroprusside, nitroglycerine, nicardipine, clevidipine) and then supplement with short-acting beta-blockade (esmolol)

**Consider phentolamine IV (1 mg push at a time)

*Surgery team should communicate with anesthesia team when the adrenal vein has been identified and prior to clamping

**Anesthesia team should load patients with fluid prior to adrenal vein identification

**Increase vasopressor support (i.e. vasopressin) to prevent sudden loss of blood pressure after ligation of adrenal vein. Titrate down vasodilators at this time.

*Anticipate sudden drops of blood pressure after adrenal vein clamping. Such changes can induce cardiac collapse.

**Support blood pressure with vasopressors (i.e. vasopressin)

===Emergence<!-- List and/or describe any important considerations related to the emergence from anesthesia for this case. -->===

*Extubation after case completion is customary

==Postoperative management==

===Disposition<!-- List and/or describe the postoperative disposition and any special considerations for transport of patients for this case. -->===

*Many centers still admit all pheochromocytoma patients to the intensive care units.

**A percentage of patients will require vasopressor support after surgical completion until fluid shifts and physiology equilibrates

**A small population of patients will continue to have circulating catecholamines for several hours and may require a few hours of vasodilation

*Specialty centers are able to titrate all vasopressors off by the end of the case and patients can be admitted into the PACU

*With diabetics, the sudden withdrawal of catecholamines can precipitate sudden hypoglycemia - particularly in patients on insulin drips intraoperatively

===Pain management<!-- Describe the expected level of postoperative pain and approaches to pain management for this case. -->===

*Open laparotomy may require epidural pain control. Extreme caution must be utilized in epidural dosing as hemodynamic changes can occur rapidly during surgery. Frequently the epidural is placed preoperatively and not utilized until hemodynamic stability with the tumor removed is achieved.

*Laparoscopic and particularly single-incision retroperitoneal support rarely require epidural pain management. Pain can be controlled using IV and PO pain medications.

===Potential complications<!-- List and/or describe any potential postoperative complications for this case. -->===

*Sudden, wide, blood pressure changes can cause cardiac ischemia, cardiovascular collapse and ischemic or hemorrhagic stroke

*With diabetics, the sudden withdrawal of catecholamines can precipitate sudden <u>hypoglycemia</u> - particularly in patients on intraoperative insulin drips. Monitor post-operative glucose carefully.

==Procedure variants<!-- This section should only be used for cases with multiple approaches (e.g. Laparoscopic vs. open appendectomy). Otherwise, remove this section. Use this table to very briefly compare and contrast various aspects between approaches. Add or remove rows as needed to maximize relevance. Consider using symbols rather than words when possible (e.g. +, –, additional symbols such as ↑ and ↓ are available using the "Ω" tool in the editor). -->==

{| class="wikitable"

|+

!

!Open Laparotomy

!Transabdominal

Laparoscopic

!Retroperitoneal

Laparoscopic

!Single-Incision Retroperitoneal

Laparoscopic<ref>{{Cite journal|last=Sho|first=Shonan|last2=Yeh|first2=Michael W.|last3=Li|first3=Ning|last4=Livhits|first4=Masha J.|date=2017|title=Single-incision retroperitoneoscopic adrenalectomy: a North American experience|url=http://link.springer.com/10.1007/s00464-016-5325-8|journal=Surgical Endoscopy|language=en|volume=31|issue=7|pages=3014–3019|doi=10.1007/s00464-016-5325-8|issn=0930-2794|via=}}</ref>

|-

|'''Position'''

|Supine

| Lateral

|Prone

|Prone and half jackknife 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<ref>{{Cite journal|last=Sho|first=Shonan|last2=Yeh|first2=Michael W.|last3=Li|first3=Ning|last4=Livhits|first4=Masha J.|date=2017|title=Single-incision retroperitoneoscopic adrenalectomy: a North American experience|url=http://link.springer.com/10.1007/s00464-016-5325-8|journal=Surgical Endoscopy|language=en|volume=31|issue=7|pages=3014–3019|doi=10.1007/s00464-016-5325-8|issn=0930-2794|via=}}</ref>

|-

|'''Other considerations'''

|

|

|

|High Insufflation pressures (20-30mmHg)

|}

==Outcomes==

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. 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<ref>{{Cite journal|last=Shen|first=Wen T.|last2=Grogan|first2=Raymon|last3=Vriens|first3=Menno|last4=Clark|first4=Orlo H.|last5=Duh|first5=Quan-Yang|date=2010|title=One hundred two patients with pheochromocytoma treated at a single institution since the introduction of laparoscopic adrenalectomy|url=https://pubmed.ncbi.nlm.nih.gov/20855761|journal=Archives of Surgery (Chicago, Ill.: 1960)|volume=145|issue=9|pages=893–897|doi=10.1001/archsurg.2010.159|issn=1538-3644|pmid=20855761|via=}}</ref>.

==References==

[[Category:Surgical procedures]]

<references />

[[Category:General surgery]]

[[Category:Endocrine surgery]]