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Anesth Essays Res. 2017 Jul-Sep; 11(3): 806–807.
PMCID: PMC5594817

Pregnancy with Wilson's Disease: An Anesthetic Experience with “Ketofol”

Sir,

Wilson's disease is a rare autosomal recessive disorder characterized by a reduction in the synthesis of the copper transporter protein ceruloplasmin. Copper from the liver cannot be exported into the bile and copper accumulates in the liver, brain, kidneys, and cornea.[1] The worldwide prevalence of this genetic disorder is 30 per million.[2] Very few anesthetic experiences with Wilson's disease have been reported in world literature.[1,3,4] We recently encountered a 33-year-old primigravida with Wilson's disease and missed abortion at 14 weeks gestation. We had to anesthetize her for an elective suction evacuation. Nine months back, she had been diagnosed as a case of Wilson's disease with portal hypertension and esophageal varices. Her serum ceruloplasmin level was low, and the 24 h urinary copper excretion was 4527 μg. She was started on oral propranolol, zinc, and frusemide which she stopped taking after 5 months. She weighed 45 kg, had pallor, pedal edema, icterus, facial puffiness, and a moderately distended abdomen. Abnormal laboratory parameters included a hemoglobin of 5.1 g%, total bilirubin 3.4 mg/dl, direct bilirubin 1.4 mg/dl, and serum aspartate transaminase 69U/L. An ultrasound and computed tomography of the abdomen revealed a dilated portal vein, mild hepatosplenomegaly, and mild ascites. Ophthalmic examination revealed limbal hyperpigmentation. She was given oral propranolol 20 mg on the morning of surgery. She was premedicated on table with intravenous glycopyrrolate 0.2 mg, fentanyl 50 μg, induced with intravenous ketamine 40 mg and propofol 50 mg and maintained on spontaneous ventilation on oxygen and nitrous oxide. The procedure was short. She recovered normally and was observed closely for signs of worsening of jaundice, renal dysfunction, hypoglycemia, and neuropsychiatric symptoms none of which occurred.

Patients with Wilson's disease may be asymptomatic or present with acute liver failure, chronic hepatitis, cirrhosis, neurological manifestations such as tremors, dysarthria, dystonia, seizures, and Kayser-Fleischer (KF) corneal rings.[3] Neurological manifestations typically present later than the liver disease, mostly in the third decade of life. In a patient series, KF rings were present in only 44%–62% with mainly hepatic presentation.[5] Our patient too was in her third decade of life and did not have KF rings. Excessive copper accumulation can lead to infertility, abortions, intrauterine growth restriction, pregnancy-induced hypertension, placental abruption, coomb's negative hemolytic anemia and postpartum depression.[3] Incidentally, our patient too was a case of missed abortion with anemia. Although pregnancy does not aggravate Wilson's disease, it is said that there is an increased risk of ascites, gastrointestinal bleed, and variceal rupture in pregnant women with preexisting cirrhosis like in our case. Very few anesthetic experiences in pregnant women with Wilson's disease have been described.[3] Decreased pseudocholinesterase levels can slow the metabolism of succinylcholine. Furthermore, decreased hepatic metabolism, decreased excretion, decreased muscle power, and altered neuromuscular transmission in Wilson's disease can lead to increased sensitivity to nondepolarizing muscle relaxants.[6] Our patient had liver involvement and was posted for a short surgical procedure. Hence, we totally avoided muscle relaxants. Neuropsychiatric symptoms can get aggravated due to delayed metabolism of sedative drugs. Reduced mean arterial pressure can aggravate hepatic dysfunction.[6] Furthermore, it is better to use the least hepatotoxic agents.[1] Keeping this in mind, we successfully used a combination of low-dose ketamine and propofol “Ketofol” in our case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

REFERENCES

1. Baykal M, Karapolat S. Anesthetic management of a pediatric patient with Wilsons disease. J Clin Med Res. 2010;2:99–101. [PMC free article] [PubMed]
2. Sheikh SM, Khair H. Pregnancy with Wilson's disease a case report. ISRA Med J. 2013;5:151–2.
3. Chakravarthy K, Nalagandla SR, Cherukuri SK, Konda RR. Anaesthetic management of a patient with Wilson's disease posted for emergency LSCS: A case report. J Evol Med Dent Sci. 2015;4:8229–32.
4. De Souza Hobaika AB. Anesthesia for a patient with Wilson's disease – A case report. Middle East J Anaesthesiol. 2008;19:905–8. [PubMed]
5. Roberts EA, Schilsky ML. American Association for Study of Liver Diseases (AASLD). Diagnosis and treatment of Wilson disease: An update. Hepatology. 2008;47:2089–111. [PubMed]
6. Langley A, Dameron CT. Copper and anesthesia: Clinical relevance and management of copper related disorders. Anesthesiol Res Pract 2013. 2013:750901. [PMC free article] [PubMed]

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