Case Report

Anesthetic Challenges in Pregnancy with Wilson’s Disease Associated with Massive Splenomegaly Complicated with Intramyometrial Carboprost: A Case Report

Abstract

Wilson’s disease is a rare autosomal recessive condition, that affects the liver and brain mainly. Pregnancy in these patients is of high risk due to involvement of liver and high incidence of abortion, preeclampsia, HELLP syndrome. We report a case of a 24year old G2A1 (35+2 weeks) diagnosed with wilson’s disease 6 years ago, currently on tab zinc. She presented with thrombocytopenia, anemia, extrahepatic portal vein occlusion with splenomegaly and mild ascites. She was taken up for elective section at 35+2 weeks, under general anesthesia. With intramyometrial injection of carboprost, there was sudden desaturation and bronchospasm. However, we managed the case successfully with safe outcome of both the mother and child. There are only very few case reports of Wilson’s disease in pregnancy undergoing C-section under general anesthesia in the presence of hepatic dysfunction.

[1] Malik A, Khawaja A, Sheikh L. Wilson's disease in pregnancy: case series and review of literature. BMC Res Notes. 2013; 6:421.
[2] Chang IJ, Hahn SH. The genetics of Wilson disease. Handb Clin Neurol. 2017; 142:19-34.
[3] G J Brewer Wilson DiseaseNORD Guide to Rare Disorders Lippincott Williams & WilkinsPhiladelphia, PA2003506
[4] Mohanty B (2018) Anaesthetic Management in a Case of Wilson’s Disease in Pregnancy. J Anesth Clin Res 9: 845.
[5] Acharya N, Samal S, Kumar S, Acharya S, Shukla S. Pregnancy with Wilson’s disease complicated with thrombocytopenia: a case report. Int J Adv Med. 2014; 1: 155-157.
[6] Ala A, Walker AP, Ashkan K, Dooley JS, Schilsky ML. Wilson's disease. The Lancet. 2007; 369: 397-408.
[7] Brewer GJ. Recognition, diagnosis, and management of Wilson's disease. Proc Soc Exp Biol Med. 2000; 223: 39-46.
[8] Baykal M, Karapolat S. Anesthetic management of a pediatricpatient with Wilsons disease. J Clin Med Res. 2010; 2: 99-101.
[9] Vaja R, McNicol L, Sisley I. Anaesthesia for patients with liverdisease. Cont Educ Anaesth Crit Care Pain. 2009; 10: 15-19.
[10] De Souza, Hobaika AB. Anesthesia for a patient with Wilson's disease—a case report. Middle East J Anesthesiol. 2008; 19(4): 905-8.
[11] Wiklund RA. Preoperative preparation of patients with advanced liver disease. Crit Care Med. 2004;32(4 Suppl):S106–15.
[12] Bosilkovska M, Walder B, Besson M, Daali Y, Desmeules J. Analgesics in patients with hepatic impairment: pharmacology and clinical implications. Drugs. 2012; 72: 1645-69.
[13] Garg G, KumarS, Mahajan S. is intramyometrial carboprost troublesome? J Obstet Anesth Crit Care 2020; 10:150-1
[14] 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.
Files
IssueVol 9 No 2 (2023): Spring QRcode
SectionCase Report(s)
DOI https://doi.org/10.18502/aacc.v9i2.12529
Keywords
Carboprost caeserian section massive splenomegaly Wilson’s disease

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Deshpande J, Jacob M. Anesthetic Challenges in Pregnancy with Wilson’s Disease Associated with Massive Splenomegaly Complicated with Intramyometrial Carboprost: A Case Report. Arch Anesth & Crit Care. 2023;9(2):185-187.