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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 8  |  Issue : 1  |  Page : 78-80

Perioperative management of a rare case of severe polycythemia vera with gout coming for an emergency minor surgery – A concern for the anesthesiologists


1 Department of Anaesthesiology and Critical Care, Pondicherry Institute of Medical Sciences, Puducherry, India
2 Department of Surgical Oncology, Sri Ramachandra Institute of Medical Sciences, Chennai, Tamil Nadu, India

Date of Submission03-Dec-2021
Date of Decision13-Mar-2022
Date of Acceptance14-Mar-2022
Date of Web Publication8-Jul-2022

Correspondence Address:
Dr. Amoolya Kamalnath
Department of Anesthesiology, Pondicherry Institute of Medical Sciences, Kalathumettupathai, Ganapathichettikulam, Village 20, Kalapet, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrsm.jcrsm_88_21

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  Abstract 


Polycythaemia Vera is a chronic myeloproliferative neoplasm with an unknown etiology and is a rare occurrence. The erythrocytosis and thrombocytosis along with the hypercoagulable state as a direct result of surgery predispose these patients to thrombosis and hence haemorrhage. Here, we report the anaesthetic issues concerning an elderly male patient presenting with complaints of difficulty in passing stools, fever, and cough with mucoid sputum and diagnosed to be having low anal fistula and perianal abscess with presence of haemorrhoids along with polycythaemia and gout, who was not on any treatment, and posted for an emergency perianal abscess drainage and fistulectomy. With a hematocrit of 63.3% and normal arterial oxygen saturation, the anesthetic management included a thorough preoperative evaluation of associated comorbidities, proper positioning, conducting regional anesthesia meticulously and prevention of acute thrombotic or hemorrhagic episodes. The uneventful perioperative period resulted in the smooth recovery of the patient.

Keywords: Early ambulation, polycythemia vera, primary polycythemia, gout, subarachnoid block


How to cite this article:
Manujkumar G, Kamalnath A, Ruthrendra E, Ranjan VR. Perioperative management of a rare case of severe polycythemia vera with gout coming for an emergency minor surgery – A concern for the anesthesiologists. J Curr Res Sci Med 2022;8:78-80

How to cite this URL:
Manujkumar G, Kamalnath A, Ruthrendra E, Ranjan VR. Perioperative management of a rare case of severe polycythemia vera with gout coming for an emergency minor surgery – A concern for the anesthesiologists. J Curr Res Sci Med [serial online] 2022 [cited 2022 Aug 19];8:78-80. Available from: https://www.jcrsmed.org/text.asp?2022/8/1/78/350143




  Introduction Top


Polycythemia vera (PV) is a hematopoietic stem cell disorder, Janus kinase-2 gene mutation being primarily responsible. The incidence is 0.7–2.6/100,000 persons per year.[1] We report a rare case of severe polycythemia with gout possessing substantial risk of spinal hematoma, acute cerebrovascular accidents, myocardial infarction due to dislodgments of blood clots,[2] posted for emergency surgery.


  Case Report Top


A 69-year-old male patient presented with a history of difficulty in passing stools and fever. On local examination, a perianal abscess with low anal fistula and hemorrhoids was revealed. Emergency drainage of perianal abscess and fistulectomy was planned. The preanesthetic evaluation revealed a thin-built man having pain and itching around his left ankle joint with no swelling. Local skin examination suggested Lichen simplex chronicus. The patient had taken allopurinol 300 mg (bis in die [BD]) and calcitriol (omne in die [OD]) for hyperuricemia 1 year before this episode, for 3 months, and then stopped treatment on his own. Investigations revealed hemoglobin of 21.7 g/dl, hematocrit 63.3%, total count 13,600/cubic mm, C-reactive protein 157, uric acid 7.4 mg/dl, urea 19 mg/dl, and creatinine 0.8 mg/dl and ultrasonography of the kidneys, ureters, and bladder was normal. All other investigations including platelet count and coagulation profiles were normal. The patient was started on antibiotics.

The adequacy of nil per oral status was confirmed and written informed consent was obtained. An 18 gauge (G) intravenous (IV) cannula was secured and the patient was hydrated with Ringer's lactate solution (RL) at the rate of 200 ml/hour for 1 h. Premedication with injection pantoprazole 0.6 mg/kg IV and injection metoclopramide 0.18 mg/kg IV was administered preoperatively. On connecting to standard monitors in the operation theater, all baseline parameters were normal including 100% saturation. RL infusion was continued at 100 ml/hr. Under strict aseptic precautions, with the patient in sitting position, 2 ml of 0.5% (Heavy) injection bupivacaine was administered at L3-L4 subarachnoid space using 26 G Quincke's needle after ensuring free flow of clear cerebrospinal fluid. The patient was made to sit until the patient achieved adequate subarachnoid saddle blockade. Pressure points were sufficiently padded during lithotomy positioning. Intraoperatively, 1000 ml RL was infused. The surgery was uneventful. Postoperative pain was managed with injection paracetamol 1 g IV BD.

As a part of the work-up for polycythemia, abdominal ultrasonography and serum vitamin B-12 were done and found to be normal, antinuclear antibody was negative. Phlebotomy was done twice after surgery and hemoglobin and hematocrit were reduced to 19 g/dl and 55.3%, respectively. He was discharged with tablet aspirin 75 mg OD and halobetasol propionate and salicyclic acid oinments for his skin changes.


  Discussion Top


PV is a chronic progressive myeloproliferative disorder. Our patient was suspected to have PV as he did not present with any causes of secondary polycythemia. The two types of polycythemia and their causes are illustrated in [Figure 1]. PV is predominant in elderly males, is insidious in onset and symptoms include headache, pruritus, angina pectoris, intermittent claudication, etc., Our patient was an elderly male. He had pruritus around the lower limb joints. The Polycythemia Vera Study Group and comprehensive diagnostic criteria by the WHO[3] have classified these patients. This patient could not be evaluated for most diagnostic tests as they are not being done in our institution and were unaffordable for the poor fisherman.
Figure 1: Classification of polycythemia vera

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Acute normovolemic hemodilution (ANH)[4] involves a perioperative controlled removal of whole blood. The patient's intravascular volume is maintained with nonred cell solutions. The operative procedure is conducted with a normal blood volume but with reduced blood cell mass. This prevents hyperviscosity of red blood cells (RBCs). Due to the simple and emergent nature of the surgery, ANH was not done in our patient.

Anesthetic implications of perioperative thrombotic complications in patients with PV include increased hyperviscosity of blood impairing microcirculation leading to an increased risk of hypoxia and arrhythmia, hypercoagulability resulting in thrombosis/hemorrhage.[5]

Preoperatively, we hydrated our patient with 200 ml/hour RL to avoid dehydration further preventing hyperviscosity of RBCs. Subarachnoid block was administered in a single attempt using a 26G spinal needle by a senior anesthesiologist preventing spinal hematoma. Intraoperatively, continuous monitoring was done to recognize any acute cardiac events. Drugs that could trigger thrombotic events and aggravate platelet dysfunction complications such as nonsteroidal anti-inflammatory drugs and tranexamic acid were avoided. Careful padding of the joints was done after lithotomy positioning. Postoperatively, the patient was carefully monitored in the postanesthesia care unit.

Phlebotomy has been the treatment of choice, gradually lowering the hematocrit to about 45%. Our patient underwent phlebotomy postoperatively achieving a hematocrit of 57%. He was not phlebotomized presurgery as it was a minor emergency surgery. He was advised aspirin tablet 75 mg OD on discharge as advocated by the European Collaboration on Low-dose Aspirin in Polycythemia.[6] An exaggerated turnover of nucleic acids and an excess release of purine metabolites including uric acid leads to occasional symptomatic gout. Our patient showed signs of gout. The association of gout with PV is coincidental but has a high incidence.[7] However, as in our case, the order of precedence of both the conditions cannot be ascertained. In addition, severe persistent itchiness may be treated with antihistamines and/or ultraviolet light therapy.[8] Both these were prescribed for our patient on discharge.

Early ambulation should be instituted in these patients by a vigorous use of analgesics, compression stockings, and administration of peripheral nerve blocks. Nonopioid analgesics and compression stockings were administered to our patient.


  Conclusion Top


Patients with PV are at an increased perioperative risk of thrombosis/embolism or hemorrhage. Hence, PV requires meticulous perioperative assessment and subsequently tailored treatment strategies to avoid substantial morbidity and mortality.

Declaration of patient consent

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

Financial support and sponsorship:

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Singhal S, Saini S, Wason R. Polycythemia vera: Perioperative anesthetic challenges and review of literature. Indian Anaesth Forum 2019;20:322.  Back to cited text no. 1
  [Full text]  
2.
Im H, Min JJ, Yang J, Lee SM, Lee JH. Anesthetic management of a patient with polycythemia vera undergoing emergency repair of a type-A aortic dissection and concomitant coronary artery bypass grafting: A case report. Korean J Anesthesiol 2015;68:608-12.  Back to cited text no. 2
    
3.
Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016;127:2391-405.  Back to cited text no. 3
    
4.
Murray D. Acute normovolemic hemodilution. Eur Spine J 2004;13 Suppl 1:S72-5.  Back to cited text no. 4
    
5.
Ruggeri M, Rodeghiero F, Tosetto A, Castaman G, Scognamiglio F, Finazzi G, et al. Postsurgery outcomes in patients with polycythemia vera and essential thrombocythemia: A retrospective survey. Blood 2008;111:666-71.  Back to cited text no. 5
    
6.
Landolfi R, Marchioli R, Kutti J, Gisslinger H, Tognoni G, Patrono C, et al. Efficacy and safety of low-dose aspirin in polycythemia vera. N Engl J Med 2004;350:114-24.  Back to cited text no. 6
    
7.
Yü TF. Secondary gout associated with myeloproliferative diseases. Arthritis Rheum 1965;8:765-71.  Back to cited text no. 7
    
8.
Tefferi A. Rare Diseases. Danbury CT: National Organization for Rare Disorders (NORD); 1986. Available from: https://rarediseases.org/rare-diseases/polycythemia-vera/. [Last accessed on 2021 Jul 20; Last updated on 2018].  Back to cited text no. 8
    


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