|Year : 2017 | Volume
| Issue : 2 | Page : 118-120
Anesthetic management of a patient with Wilson's disease: Factors to be considered
Manazir Athar1, Shahna Ali1, Sana Khurshid1, Rufaida Mazahir2
1 Department of Anaesthesiology and Critical Care, Jawaharlal Nehru Medical College and Hospital, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
2 Department of Paediatrics, Jawaharlal Nehru Medical College and Hospital, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
|Date of Web Publication||18-May-2017|
Department of Anaesthesiology and Critical Care, Jawaharlal Nehru Medical College and Hospital, Aligarh Muslim University, Aligarh - 202 002, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Wilson's disease is an autosomal recessive, multisystem disorder particularly involving hepatic and neuropsychiatric systems. The primary pathology in these cases is the reduction in the synthesis of copper transporter protein leading to accumulation of copper in various organs. Excessive deposition in liver leads to chronic liver disease and cirrhosis that may alter the metabolism and excretion of anesthetic agents. Providing anesthesia can be a challenging task in these cases. Hence, a thorough workup for the severity and extent of the disease and optimal planning is essential for the successful management of these cases. We, hereby, describe a case of a 23-year-old male with Wilson's disease posted for bilateral contracture release of the knee.
Keywords: General anesthesia, subarachnoid block, Wilson's disease
|How to cite this article:|
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
|How to cite this URL:|
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 [serial online] 2017 [cited 2020 Nov 27];6:118-20. Available from: https://www.ijhas.in/text.asp?2017/6/2/118/206422
| Introduction|| |
Wilson's disease or hepatolenticular degeneration is a rare hereditary disease of copper metabolism with a prevalence of 1 in 30,000 in most populations. It is an autosomal recessive genetic disorder in which accumulation of copper takes place in the tissues. This is due to mutation in ATP7B gene. There is a loss of ability to export copper from liver into bile and to incorporate copper into hepatic ceruloplasmin.
The clinical manifestations of this disease range from hepatic to neurological or psychiatric symptoms. Due to the existence of liver disease, the anesthetic management of such patients presents a challenge to anesthetic techniques and drugs to be used. Although liver dysfunction is the presenting feature in majority of the patients, late manifestations in the form of dystonia, spasticity and rigidity, grand mal seizures, and multiple flexion deformities may complicate the disease [Table 1]., It has been observed that, in patients with Wilson's disease, there is an increased risk of cirrhosis and consequent liver failure that naturally affect the drug metabolism. Persistent neurological problems, renal stones, and a variety of psychiatric illnesses are other important concerns during anesthetic planning.
Despite several anesthetic problems, there is a paucity of literature on different aspects of anesthetic practice in these patients. Hence, we report a case of Wilson's disease with a brief literature review on anesthetic management.
| Case Report|| |
A 23-year-old male with Wilson's disease on D-penicillamine was posted for elective contracture release of both the knees [Figure 1]. Investigations showed hemoglobin - 10.3 g/dl, total leukocyte count - 6200/mm 3, differential leukocyte count - P60 L35E4M1, platelets - 200 × 103/mm 3, erythrocyte sedimentation rate - 10 mm/h in the 1st h, prothrombin time - 16 s, international normalized ratio - 1.2, total serum protein - 6.4 g/dl, albumin - 3.5 g/dl, globulin - 2.9 g/dl, albumin/globulin ratio - 1.2:1, serum bilirubin (total) - 0.8 mg/dl, serum glutamic pyruvic transaminase - 28.5 U/L, serum glutamic-oxaloacetic transaminase - 22.1 U/L, and serum alkaline phosphatase - 170.4 U/L. Considering the disease and patient's status, he was planned for contracture release under subarachnoid block. Informed consent for the same was obtained. Under strict aseptic precautions, the skin was infiltrated with lidocaine 2%, and lumbar puncture was performed in the sitting position with a 25-gauge Quincke spinal needle (Becton Dickinson, Madrid, Spain). Following this, 3 mL of 0.5% bupivacaine (heavy) was administered intrathecally. Surgery was allowed to proceed after confirming the adequate sensory and motor block. During the operation, the contracture was released and the patient was hemodynamically stable throughout the operation. There was no postoperative deterioration of renal function or hepatic function. The patient was shifted to ward and eventually discharged from the hospital in a satisfactory condition after a week.
| Discussion|| |
Wilson's disease is a congenital, multisystem disease that requires the concerned anesthesiologist to have a clear idea of the manifestations and the problems posed by this disease and should have a planned anesthetic approach. The pathology here mainly is copper accumulation which is due to deficiency in ceruloplasmin which is necessary for the excretion of copper from liver to bile. This excessive copper causes damage mainly to the liver and the brain. Signs and symptoms ranging from neurological (69%), hepatic (15%), psychiatric (2%), and osteomuscular (2%) may occur. The osteomuscular problems are generally diagnosed during the second decade of life and are less severe compared to other symptoms. If a clinical suspicion of Wilson's disease is made, investigations such as urinary copper, blood ceruloplasmin, and slit-lamp Kayser–Fleischer rings should be carried out to confirm the diagnosis. Beside these, cardiac manifestations in the form of rhythm abnormalities and renal involvement in the form of hematuria, urolithiasis, and fulminant renal failure may also complicate the disease.
There are few case reports describing the administration of general anesthesia (GA), as well as central neuraxial blocks., However, GA has certain drawbacks in cases of Wilson's disease as it can aggravate the already impaired liver function and there might arise a problem in the metabolism and elimination of drugs. In case GA is planned, care is to be taken in terms of type and dosage as hepatic involvement may alter the drug metabolism. It is seen that inducing agents and inhalational agents interfere with central nervous system function and exacerbate neurological and behavioral problems postoperatively. Furthermore, an increased sensitivity to muscle relaxants has been observed which might be due to reduced muscle function because of disease or from the use of D-penicillamine. Regional anesthesia is better for these patients as we use lesser amount of drugs and the effect on vital organs is lesser as compared to GA. It has the added advantage of opioid-sparing effect along with decreasing the risk of deep vein thrombosis that may expedite the recovery., Further, the awake and conscious state of the patient leads to early detection of intraoperative complications. However, deranged coagulation profile may be an issue and should be ruled out prior to any neuraxial block.
Despite these benefits, some researchers have observed altered visual- and auditory-evoked potentials in these patients, suggesting cerebral damage. However, peripheral nerve conduction studies and the somatic sensory-evoked potentials are normal, suggesting that the regional administration of local anesthetics may be safe in Wilson's disease.
| Conclusion|| |
Patients with Wilson's disease require a comprehensive multisystem evaluation with a primary emphasis on neurological and hepatic involvement. The technique of anesthesia should be tailored according to the patient's profile, site, duration and extent of surgery along with the provision of careful perioperative monitoring.
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Conflicts of interest
There are no conflicts of interest.
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