Title: Anaesthetic Management of a Patient with Hereditary Spherocytosis and Cholelithiasis for Splenectomy and open Cholecystectomy under General Anaesthesia – A Case Report

Authors: Dr Arun B V, Dr Lailu Mathews

 DOI: https://dx.doi.org/10.18535/jmscr/v9i1.09

Abstract

Hereditary spherocytosis (HS) is an autosomal dominant type of congenital haemolytic anaemia. The spherocytes are susceptible to osmotic lysis resulting in anaemia, jaundice, splenomegaly, cholelithiasis and altered the liver function. The management of hereditary spherocytosis is largely dependent on the severity of anaemia and degree of haemolysis. The anaesthetic management of a patient with HS and cholelithiasis for splenectomy and open cholecystectomy is discussed here.

Keywords: Hereditary Spherocytosis; Haemolysis; Splenomegaly; Splenectomy.

References

  1. Anuradha M, Sonal M, Rochana B et al., – Anesthetic considerations for patient of hereditary spherocytosis for splenectomy and cholecystectomy: A case report. Indian Journal of Basic and Applied Medical Research 2014;4(1):295-297.
  2. Sonal SK, Widya ST, Syed MK et al., – Anesthetic management of a case with hereditary spherocytosis for splenectomy and open cholecystectomy. Medical journal of Dr. D.Y.Patil University 2016; 9(2):267-270.
  3. Sidhesh B, Vilas G, Merlyn D - Anesthetic management of patient with hereditary spherocytosis for laparoscopic cholecystectomy and splenectomy. Saudi Journal of Anesthesia 2012;6(4):438-439.
  4. Kalpana K, Sunetra D, Shirish T – Anaesthetic management of a child with hereditary spherocytosis for splenectomy. EC Anaesthesia 2019; 5:14-19.
  5. Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ, et al., Guidelines for the diagnosis and management of hereditary spherocytosis. British Journal of Haematology 2004; 126:455-74.
  6. Olga C – old and new insights into the diagnosis of hereditary spherocytosis. Ann Transl Med 2018; 6(17):339.
  7. Eber SW, Armbrust R, Schröter W. Variable clinical severity of hereditary spherocytosis: relation to erythrocyticspectrin concentration, osmotic fragility, and autohemolysis. J Pediatr 1990;117(3):409-416.

Corresponding Author

Dr Arun B V

Final year postgraduate, Department of Anesthesiology, Chettinad Hospital and Research Institute