CASE REPORT Open Access

Perioperative anesthetic management ofintestinal pseudo-obstruction as acomplication of pheochromocytomaSaki Okumura1, Makoto Sumie2* and Yuji Karashima3

Abstract

Background: Intestinal pseudo-obstruction, which is a rare complication of pheochromocytoma, can be caused byhypersecreted catecholamines.

Case presentation: A 45-year-old woman was admitted for local recurrence of pheochromocytoma complicatedby intestinal pseudo-obstruction. The intestinal pseudo-obstruction showed poor response to α-adrenergic receptorblocker and she was scheduled for surgical resection of pheochromocytoma. The surgery was uneventfullyaccomplished with general anesthesia combined with epidural anesthesia. The latter was performed with the aimof not only perioperative pain management but also of promoting intestinal peristalsis. The anticipated effect forintestinal peristalsis was not apparent in the early postoperative phase. The abdominal symptoms were graduallyrelieved over the course of about 1 month.

Conclusions: For intestinal pseudo-obstruction induced by pheochromocytoma, although inhibition of thesympathetic system by epidural infusion of local anesthetics may be promising, short-term usage of epidural localanesthetics infusion did not provide a quick recovery after pheochromocytoma removal surgery.

Keywords: Pheochromocytoma, Intestinal pseudo-obstruction, α-Adrenergic receptor blocker, Phentolamine,Epidural anesthesia

IntroductionGastrointestinal pseudo-obstruction, or paralytic ileus,can be caused by pheochromocytoma with hypersecre-tion of catecholamines, which act on α2-adrenergic re-ceptors of intestinal smooth muscle cells to decreaseintestinal peristalsis [1, 2]. Although several cases havebeen reported, the literature contains few descriptions ofperioperative anesthetic management for these patients[3–6]. Because of the absence of any obstructive structurallesions in the intestinal tract, we presumed that a strategyfor promoting intestinal peristalsis postoperatively wouldbe advantageous. In this report, we focus on perioperativeanesthetic management of intestinal pseudo-obstructioncomplicated with pheochromocytoma.

CaseA 45-year-old-woman (height 162.9 cm, weight 44.5 kg)was admitted with complaints of twice-daily headaches,abdominal pain, nausea, obstipation over several days,and excretion of only a small amount of aqueous stoolin spite of enema administrations. She had a medicalhistory of lip mucosal neuroma resection at the age of 6,total thyroidectomy for medullary carcinoma at the ageof 14, and right and left adrenalectomy for pheochromo-cytoma at the ages of 25 and 27, respectively. She hadreceived follow-up on suspicion of multiple endocrineneoplasia type 2B. Physical examination revealed thatthe abdomen was soft but bulging, with weakened intes-tinal peristaltic sound. Blood pressure was 110–150/60–90mmHg, heart rate was 90–120 beats/min in sinusrhythm, and body temperature was 36.8 °C. Subsequent24-h urine collection for catecholamines revealed ele-vated metanephrine levels of 0.4 [normal range (NR)0.04–0.19] μg/day, normetanephrine levels of 0.4 (NR0.09–0.33) μg/day, dopamine levels of 3241.8 (NR

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* Correspondence: sumimako@kuaccm.med.kyushu-u.ac.jp2Operating Rooms, Kyushu University Hospital, Fukuoka, JapanFull list of author information is available at the end of the article

Okumura et al. JA Clinical Reports (2019) 5:35 https://doi.org/10.1186/s40981-019-0255-9

365.0–961.5) μg/day, adrenaline levels of 40.7 (NR 3.4–26.9) mg/day, and noradrenaline levels of 253.1 (NR48.6–168.4) mg/day. 123I-metaiodobenzylguanidine(MIBG) scintigraphy showed a 17 × 15mm mass accu-mulation on the left side of the inferior vena cava (Fig. 1).X-ray and computed tomography of the abdomenshowed remarkably distended colon with the maximaldiameter of 12.6 cm and colon gas, which were consistentwith the patient’s symptoms (Fig. 2a). She was diagnosedwith local recurrence of pheochromocytoma and sched-uled for laparoscopic tumor resection. For preoperativepreparation, several strategies for reduction of intra-intestinal pressure were initiated, which included fasting,2000ml/day of fluid replacement, oral administration ofα1-adrenergic receptor blocker, doxazosin (1 mg/day),and intravenous administration of the nonselectiveα-adrenergic receptor blocker phentolamine (1.5 mg/h).Furthermore, lower intestinal endoscopy showed a largeamount of fecal mass and enema were performed.However, despite all those interventions, defecation wasdifficult and neither intra-intestinal pressure reductionnor symptomatic relief was achieved.We selected general anesthesia combined with epi-

dural anesthesia for perioperative management of thispatient. An epidural catheter was placed between theninth and tenth thoracic vertebra, and its efficacy wasconfirmed by administration of a combination of 8 ml of2% mepivacaine and 100 μg of fentanyl before inductionof anesthesia. In addition to standard monitors, invasivearterial pressure via radial artery and stroke volumeindex (SVI) and stroke volume variation (SVV) with theuse of the FloTrac™-EV1000™ system (Edwards Life-sciences, Irvine, CA, USA) were monitored. The induc-tion was uneventful with 2 μg/kg of fentanyl, 2 mg/kg of

propofol, and 0.6 mg/kg of rocuronium under reverseTrendelenburg position and pressured cricothyroid car-tilage. Anesthesia was maintained with desflurane (0.5–0.7 MAC), remifentanil (0.15–0.4 μg/kg/min), and 0.25%levobupivacaine (5 ml/h) via epidural catheter. Althoughhemodynamic fluctuations occurred during manipula-tion of the tumor, the patient was successfully managedwith bolus infusions of phenylephrine (0.1 mg), nicardi-pine (0.2 mg), and landiolol (2.5 mg); neither of continu-ous infusion of vasodilators nor catecholamines wasneeded. Intraoperative fluid infusion was titrated tomaintain SVI values between 40 and 80ml/m2/beat andSVV between 5 and 10%. For postoperative analgesia,intravenous acetaminophen (700 mg) was administeredand continuous epidural infusion of 0.125% levobupiva-caine (5 ml/h) was initiated at the end of surgery.Postoperative pain was well controlled with continu-

ous epidural infusion of local anesthetics and regular ad-ministration of acetaminophen. On the secondpostoperative day, the numerical pain rating scale wasalmost 1 and the epidural catheter was removed. Acet-aminophen was continued postoperatively for 1 week.Regarding the patient’s postoperative symptoms, al-though headache and nausea were gradually relieved, in-testinal pseudo-obstruction and defecation difficultiescontinued over several weeks postoperatively (Fig. 2b),which required several stool suction treatments by lowergastrointestinal endoscopy. Finally, the patient was ableto resume oral intake on the 22nd postoperative day.

DiscussionPheochromocytomas are rare neuroendocrine tumorssecreting high levels of catecholamines that producehighly variable clinical signs and symptoms. Although

Fig. 1 123I-metaiodobenzylguanidine scintigraphy shows a 17 × 15 mm mass accumulation on the left of the inferior vena cava (white arrows)

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hypertension, tachycardia, pallor, headache, and anxietyusually dominate the clinical presentations, gastrointes-tinal manifestations, such as nausea, abdominal pain,and chronic constipation, are also reported in 20–40% ofpatients with pheochromocytoma [1, 2]. Gastrointestinalpseudo-obstruction, which differs from mechanical ileusin that there is no demonstrable blocking lesion, is a rareand relatively unknown but potentially life-threateningcomplication of pheochromocytoma. Osinga et al. re-port, in a review that describes 34 cases of pseudo-obstruction associated with pheochromocytoma andparaganglioma in 32 publications, that 5 patients (15%)developed bowel perforation and 16 patients (47%) diedwithin 1 year after the development of the condition [7].Its pathophysiology can be a direct consequence of theelevated catecholamine levels that reduce the peristalsisof the gastrointestinal tract. Gastrointestinal activity iscontrolled by the parasympathetic and sympathetic ner-vous systems. The activation of the parasympathetic ner-vous system causes acetylcholine release, which stimulatesmuscarinic receptors and leads to bowel movement. Incontrast, activation of the sympathetic nervous system re-leases noradrenaline, which inhibits bowel motilitythrough activation of the α1-, α2-, and β2-adrenergic re-ceptors. The activity of the sympathetic nervous system isfurther augmented by a presynaptic noradrenaline-mediated inhibition of parasympathetic acetylcholine re-lease. In addition, circulating catecholamines can not onlyinhibit gastrointestinal peristaltic activity through stimula-tion of β2 receptors but also cause intestinal vasoconstric-tion and ischemia through stimulation of α1 and α2receptors [4, 8, 9].The first-line treatment for pheochromocytoma-

associated gastrointestinal pseudo-obstruction is a con-servative approach, which includes fasting, nasogastricsuction, and intravenous replacement of fluids and

electrolytes. If initial treatment is not successful, endo-scopic desufflation and pharmacological treatmentshould be considered. Some reports suggest the use of non-selective α blockers, such as phentolamine or phenoxyben-zamine, for the treatment of gastrointestinal complicationsof pheochromocytoma [7]. However, neither phentolaminenor doxazosin was effective for our patient. The ultimatetherapy is surgical removal of the pheochromocytoma. Al-though there are some reports about surgical treatment,anesthetic considerations are scarcely described, and as faras we know, this is the first description of the anestheticmanagement of gastrointestinal pseudo-obstruction as acomplication of pheochromocytoma.From the pathophysiological characteristics of gastro-

intestinal pseudo-obstruction, we reasoned that inhibitionof the gastrointestinal sympathetic system perioperativelyby epidural infusion of local anesthetics would be advanta-geous for the patient to promote intestinal peristalsis. Inaddition, epidural analgesia would be beneficial in termsof avoiding postoperative opioid administration, which in-hibits intestinal peristalsis by activating intestinal μ recep-tors [9]. Although the postoperative pain of our patientwas well-controlled without using opioids, the anticipatedeffect of improved bowel movement was not apparent.One of the reasons for this could be attributed to the veryshort duration of epidural infusion of local anestheticspostoperatively. However, on the grounds that many fac-tors, e.g., perioperative fluid infusion and inflammation,can influence bowel movement in an early postoperativeperiod, it is impossible to determine whether the epiduralinfusion had little effect on our patient or the other factorsoverwhelmed it. Longer use of epidural infusion mighthave had an effect on intestinal peristalsis; however, wehave no evidence to prove it. Another possibility can bemaldistribution of adrenergic receptors, which should beaffected by over-secreted catecholamines due to

Fig. 2 Abdominal radiograph on admission (a) and on postoperative day 30 (b). Remarkable distended bowel and gas on admission was relievedon postoperative day 30

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pheochromocytoma and would recover gradually postop-eratively, which might be consistent with our patient’s re-covery time course. But again, we have no way to prove it.In conclusion, this case report illustrates the anesthetic

consideration of intestinal pseudo-obstruction inducedby pheochromocytoma. From a pathophysiological pointof view, epidural anesthesia may be promising in termsof promoting intestinal peristalsis postoperatively byinhibiting the sympathetic system; however, in our pa-tient, a beneficial effect was not apparent.

AbbreviationsMIBG: 123I-metaiodobenzylguanidine; NR: Normal range; SVI: Stroke volumeindex; SVV: Stroke volume variation

AcknowledgementsNot applicable

Authors’ contributionsSO conducted the anesthetic management. SO, MS, and YK wrote themanuscript. All authors have approved the final manuscript.

FundingNot applicable

Availability of data and materialsPlease contact the corresponding author for data requests.

Ethics approval and consent to participateNot applicable

Consent for publicationWritten informed consent was obtained from the patient for the publicationof this case report and any accompanying images.

Competing interestsThe authors declare that they have no competing interests.

Author details1Department of Anesthesiology and Critical Care Medicine, Kyushu UniversityHospital, Fukuoka, Japan. 2Operating Rooms, Kyushu University Hospital,Fukuoka, Japan. 3Department of Anesthesiology and Critical Care Medicine,Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

Received: 26 December 2018 Accepted: 17 January 2019

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