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LETTERS TO EDITOR
Year : 2019  |  Volume : 13  |  Issue : 4  |  Page : 384-385

Wilson disease – Challenge for safe anesthesia!


Department of Anaesthesiology and Intensive Care, GGS Medical College and Hospital, Faridkot, Punjab, India

Correspondence Address:
Dr. Kewal K Gupta
House No:-204, Medical Campus, Faridkot - 151 203, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sja.SJA_12_19

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Date of Web Publication5-Sep-2019
 


How to cite this article:
Kaur A, Gupta KK, Deep G, Thakur S. Wilson disease – Challenge for safe anesthesia!. Saudi J Anaesth 2019;13:384-5

How to cite this URL:
Kaur A, Gupta KK, Deep G, Thakur S. Wilson disease – Challenge for safe anesthesia!. Saudi J Anaesth [serial online] 2019 [cited 2019 Nov 18];13:384-5. Available from: http://www.saudija.org/text.asp?2019/13/4/384/265985



Sir,

Wilson disease (WD) is a rare hereditary disorder of human copper metabolism due to deficiency of ceruloplasmin protein. Clinical findings may vary from neuropsychiatric symptoms like tremor, dysarthria, seizures, and hepatic symptoms like acute liver failure, chronic hepatitis, and cirrhosis.[1] Due to this, anesthetic management here may be challenging. Hence, we report a case of successful anesthetic management of WD patient posted for orthopedic surgery.

A 40-year-old female posted for the right arm implant removal surgery due to chronic sinus discharge. She was a diagnosed with Wilson's disease since 8 years and had symptoms of depression, slurred speech, fine tremors of hands. She was on treatment with oral Zinc Acetate 50 mg BD, Trihexiphenidyl 2 mg BD, Propranolol 20 mg BD and Glycopyrrolate 2 mg BD. Her surgical history included total hip replacement 5 years ago and right humerus plating 8 months ago under uneventful regional anesthesia. Ophthalmology examination revealed bilateral  Kayser-Fleischer ring More Details on slit lamp examination. ENT examination for voice changes revealed phonatory gap with right vocal cord being less mobile than left. All routine preoperative investigations including Hemoglobin-11.0 g/dL, TLC-6500/mm 3, Platelets: 2.6 × 106/mm3, BUN- 16 mg/dL, Serum creatinine: 0.8 mg/dL, International normalized ratio (INR) 1.06 and AST- 32 IU/L, ALT: 26 IU/L were within normal limits owing to regular medication. After obtaining written informed consent, standard monitoring, and venous access secured. Under all aseptic precautions, an ultrasound guided interscalene brachial plexus block was performed and inj. levobupivacaine with dexamethasone (24 ml of 0.5% levobupivacaine and 4 mg of dexamethasone) was given. After confirming the adequate sensory and motor block, surgery started and it lasted for 90 minutes during which the patient remained haemodynamically stable. After completion of the surgery, the patient was shifted to the recovery room and postoperative period remain uneventful without any complication.

Wilson's disease (WD) is an autosomal recessive multisystem disorder with an estimated incidence of 1: 40,000.[2] Wilson's disease initially presents as a liver disease in children while patients with predominant neurological features tend to present late, frequently in third decade as seen in our patient. Although use of different anesthetic techniques including general anesthesia as well as regional anesthesia have been reported in patients of Wilson's disease but there is no clear consensus regarding the safest technique.[3],[4],[5] General anesthesia has certain disadvantages as it may aggravate the already impaired hepatic function due to reduction in total hepatic blood flow and toxic effects of anesthetic drugs on liver function. In other way, deranged hepatic function can adversely alter the whole pharmacokinetics of several anesthetics drugs including muscle relaxants, analgesics and sedatives. Hypnotic and sedative drugs significantly interfere with the neurological system and may, therefore, exacerbate neurological and psychiatric problems in the postoperative period. Furthermore, these patients may be more sensitive to muscle relaxants due to reduced muscle function secondary to use of D-penicillamine or elevated blood copper levels interfering with neuromuscular transmission. Even a case had been reported on occurrence of acute neuropsychiatric symptoms in postoperative period after general anesthesia which had led to the diagnosis of WD.[6] Despite all these drawbacks, few authors have reported smooth application of general anesthesia with specific anesthetic agents which have minimal effect on liver function.[2]

Hence, to avoid general anesthesia, we had planned and managed our case under ultrasound guided interscalene brachial plexus block without any complication. Regional anesthesia also reduces the requirements of systemic analgesics including opioids which may require hepatic metabolism and minimize the risk of neurologic deterioration and hemodynamic alterations. Moreover in WD, altered visual and auditory evoked potentials suggest cerebral damage but normal somatic-sensory evoked potential has made regional anesthesia as a better and safe anesthetic method in these patients.[5]

In conclusion, Wilson's disease patient must undergo preoperative comprehensive multisystem evaluation and regional anesthesia should be preferred over general anesthesia wherever possible, to avoid exacerbation of hepatic and neuropsychiatric dysfunction.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
De Souza Hobaika AB. Anesthesia for a patient with Wilson's disease--a case report. Middle East J Anesthesiol 2008;19:905-8.  Back to cited text no. 1
    
2.
Mohanty B. Anaesthetic management in a case of Wilson's disease in pregnancy. J Anesth Clin Res 2018;9:845.  Back to cited text no. 2
    
3.
Wan Y, Jiang X, Lin X. Anesthetic management of cesarean delivery for a parturient with Wilson's disease: A case report. Medicine (Baltimore) 2018;97:10454.  Back to cited text no. 3
    
4.
Gupta V, Kumar A, Kayarat B. Anaesthetic management of Scoliotic Deformity with Wilson's Disease. J Med Sci Clin Res 2018;6:1100-3.  Back to cited text no. 4
    
5.
Athar M, Ali S, Khurshid S, Mazahir R. Anesthetic management of a patient with Wilson's disease: Factors to be considered. Int J Health Allied Sci 2017;6:118-20.  Back to cited text no. 5
  [Full text]  
6.
Dikmen PY, Baybas S, Aydinlar EI, Kaymakoglu S, Onur E, Keskinbora K. A case of Wilson's disease with acute neuropsychiatric symptoms after general anesthesia. Arch Neuropsychiatry 2008;45:21-2.  Back to cited text no. 6
    




 

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