Download Anesthetic Management for Bilateral Paraganglioma Removal in a

PBLD Table #13
Anesthetic Management for Bilateral Paraganglioma Removal in a Malignant
Hyperthermia Susceptible Teenager
SPA 2011, San Diego, CA
Moderators:
Pilar Castro, MD. Staff Anesthesiologist, Department of Pediatric Anesthesia,
Cleveland
Clinic, Cleveland, OH.
Melanie Ward, MD. Pediatric Anesthesia Fellow, Children’s Hospital of Los Angeles,
Los Angeles, CA.
Goals/Objectives:
1. Review the preoperative evaluation and management of a patient presenting
for resection of a paraganglioma or pheochromocytoma.
2. Discuss the options for the anesthetic management of a patient coming for
resection of paragangliomas who is susceptible to Malignant Hyperthermia.
3. Compare and contrast the clinical presentations of Malignant Hyperthermia
and pheochromocytoma.
4. Review the diagnosis and anesthetic management of Malignant Hyperthermia
susceptibility.
5. Discuss the postoperative pain management options for this type of case and
its implications.
Stem Case:
A 15 year old male presents for a scheduled resection of bilateral intra-abdominal
paragangliomas. He has a 4 year history of hypertension which was diagnosed
incidentally after a sports injury at 11 years old. The original work up for a secondary
cause of the hypertension was negative.
Questions:
1. What are the common causes of hypertension in children?
2. What labs or imaging would be diagnostically useful?
The patient’s hypertension has worsened over the last year, accompanied by
headaches and mood fluctuations; new renal arteriograms demonstrated bilateral
masses. Plasma catecholamine/metanephrine studies were:
o Dopamine: 471 (nml:<20 pg/mL)
o Norepinephrine: 2424 (< 520 pg/mL)
o Epinephrine (PLCAT): <10 (10-200 pg/mL)
o Normetanephrine,Free,Plasma: 6.44 (<0.90 nmol/L)
o Chromogranin A: 42.9 (<36.5 ng/mL)
A CT of the abdomen revealed a left paraaortic mass (4.6cm x 4.3cm x 2.7cm) inferior
to the renal vessels and a right interaortocaval mass (4.8cm x 2.5cm x 3.2cm) inferior to
the right renal artery. The proposed surgery was exploratory laparotomy with bilateral
PBLD Table #13
paraganglioma resection, renal vessel reconstruction, possible left renal autotransplant
and iliac lymph node dissection.
Questions:
1. Would the metanephrine results alter your anesthetic plan? If so, how?
2. What concerns do the CT results raise?
3. What additional information would you like to get?
Currently, the patient’s blood pressure has been well controlled as an outpatient, SBP
110-130s, DBP 40-60s, with mild orthostatic hypotension noted in clinic notes. A
transthoracic echocardiogram revealed normal ventricular function and size with
intermittent Wenkebach seen throughout exam. He was being medically managed with
an unchanged regimen of:
o Doxazosin 6mg qday
o Candesartan 8mg qday
o Amlodipine 10mg qday
The patient has no other medical history except for a misdiagnosis of Attention Deficit
and Hyperactivity Disorder and his anesthetic history included a general anesthetic in
an outside hospital at the time of the arteriograms. CBC, CMP were within normal limits.
On exam, pt was well-appearing, comfortable, 70kg pleasant but nervous young man.
Patient was breathing at normal respiratory rate with 98% O2 saturation on room air.
His pulse was regular with a rate in the 60s.
Questions:
1. Why does the patient have orthostatic hypertension and what is the significance?
2. What is the typical medical management of hypertension secondary to
pheochromocytoma?
A first cousin had a history of masseter spasm during general anesthesia with
succhinylcholine administration with subsequent elevated creatine phosphokinase
levels and was diagnosed with malignant hyperthermia by an MH expert upon case
review. There was also an unconfirmed report of a grandfather with a possible
malignant hyperthermia episode.
Questions:
1. What are the diagnostic tests for MH?
2. What are the clinical signs and symptoms of MH?
a. What is the differential diagnosis?
b. How do you prevent MH?
3. What is your anesthetic plan?
a. Does this family history alter your anesthetic management?
b. Would you attempt an epidural preoperatively?
c. How would you induce him?
d. What monitors would you use and when would you place them?
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Prior to the start of the case, the anesthesia machine was prepared to minimize the risk
of MH. After securing a large bore peripheral IV, the patient was premedicated with
midazolam, fentanyl and a dexmedetomidine infusion is started. A right radial arterial
line was placed. A thoracic epidural was attempted, but aborted after a few attempts
due to patient discomfort and worsening hypertension despite additional medication
administration. The patient was induced with propofol, lidocaine, fentanyl and
rocuronium and developed a junctional bradyarrythmia that subsequently resolved.
Questions:
1. What is your differential for this arrhythmia?
2. What kind of medication is dexmedetomidine and what are its potential side
effects?
3. Would you perform a post-induction thoracic epidural in this patient?
4. What are the main anesthetic goals for intraoperative management of
paraganglioma resections?
5. How would you treat MH if you suspected the patient was having an episode?
6. What medications would you use for maintenance throughout the case?
The patient was maintained throughout the case with total IV anesthesia without further
major complications; however, here were significant fluctuations in blood pressure and
heart rate, especially with tumor manipulation. The surgical resection was challenging
due to the proximity to big vascular structures; it lasted approximately 10 hours where
the EBL was approximately 1500 ml.
Questions:
1. What vasopressors and antihypertensives would you use to try to control the
fluctuations in blood pressure?
2. Does phenoxybenzamine play any role in the intraoperative control of
hypertension in patients with pheochromocytoma?
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Discussion Outline:
Thankfully, pheochromocytomas and malignant hyperthermia are uncommon, but both
present challenges to an anesthesia provider. In both cases, a patient may present for a
surgical procedure without either diagnosis and the anesthesiologist will be left with the
unexpected task of managing the patient in crisis under anesthesia. Because they can
both present during anesthesia in similar manners, it can also be difficult to discern what
the correct diagnosis is.
Luckily, in pediatrics hypertension is uncommon, and if discovered it should be
thoroughly investigated. Often, the pediatric patient will be presenting for surgical
resection of the known pheochromocytoma or paraganglioma. With a known
pheochromocytoma, it is imperative that pre-operative hemodynamics be optimized
prior to the operation. The anesthesia provider should have an understanding of how
and why the patient is being medically managed in addition to the pathophysiological
impact of the oversecreted catecholamines.
Malignant hyperthermia is an anesthesia provider’s nightmare, and so we are always
trying to find ways to minimize the risk of an episode by predetermining who is
susceptible. However, the current tests are not easy, not fast, and not inexpensive.
Therefore, we attempt to evaluate for risk, present the patient with the anesthesia risks
and options, and hope that we have given enough information for the patient to make an
informed decision. In this case scenario, we are presented with the difficulty of
recognizing an MH crisis under anesthesia during a paraganglioma resection and the
implications of not having a true MH diagnosis.
1. Discuss the common secondary causes of hypertension in children and the tools
used to diagnose them.
2. Review the preoperative evaluation and management of a patient with a
paraganglioma.
3. Review the signs and symptoms of malignant hyperthermia (MH), the
susceptibility criteria, the diagnostic tests available, and the differential diagnosis.
4. Discuss the prevention and treatment of MH.
5. Discuss the goals of intraoperative management of paraganglioma resection and
the anesthetic options.
6. Discuss the postoperative pain management options for this type of case and
their implications.
PBLD Table #13
References:
1. Allen, Gregory, Rosenberg, H. Phaeochromocytoma presenting as acute
malignant hyperthermia- a diagnostic challenge. Can J Anaesth 1990, 37:5, 5935
2. Bakan, Mefkur. Anesthesia management with short acting agents for bilateral
pheochromocytoma removal in a 12-year-old boy. Pediatric anesthesia 2006,
16:1184-1188
3. Bryskin, Robert. Dexmedetomidine and magnesium sulfate in the perioperative
management of a child undergoing laparoscopic resection of bilateral
pheochromocytomas. J of Clinical Anesthesia, 2010, 22:126-129
4. Hack, HA. The perioperative management of children with phaeochromocytoma.
Paediatric Anaesthesia 2000, 10, 463-476
5. Kinney, Michelle A. O., Warner, M, vanHeerden, J, et al. Perianesthetic Risks
and Outcomes of Pheochromocytoma and Paraganglioma Resection. Anesthesia
& Analgesia, 2000, 91, 5, 1118-1123
6. Larach, Marilyn G., Gronert, G, Allen, G, Brandom, B and Lehman, E. Clinical
Presentation, Treatment, and Complications of Malignant Hyperthermia in North
America from 1987 to 2006. Anesthesia & Analgesia, 2010, 110, 2, 498-507
7. Larach, Marilyn et al. A clinical Grading Scale to Predict Malignant Hyperthermia
Susceptibility. Anesthesiology, 1994, 80:771-779
8. Maddali, Madan and Prakash Matreja. Bispectral index monitored balanced
anesthesia technique for pheochromocytoma resection. Pediatric Anesthesia,
2007, 17:688-692