25 year old patient for laparoscopic cholecystectomy and splenectomy due to hereditary spherocytosis.
- Hereditary spherocytosis
- Pigment gallstones and biliary colic
- Symptomatic splenomegaly
- Hb drop from 132 to 107 over last 3 weeks
- Recurrent severe haemolytic crises – Admission this year with nil precipitant, Hb drop to 40g/L
- Pre-splenectomy vaccinations up to date
- Anti-E antibodies (due to recurrent blood transfusion)
- Current URTI
- COVID negative
- Improving last 2 days, almost normal
- What is hereditary spherocytosis?
- Autosomal dominant, abnormality of red cell membrane structural proteins
- Classically diagnosed in children with haemolytic anaemia after Parvovirus infection, but may be asymptomatic
- Variable severity.
- Precipitated by infection
- Splenic red cells sequestered in spleen, shortening T1/2 to as little as 10d.
- Anaemia, splenomegaly, jaundice, increased reticulocyte count and spherocytes on peripheral blood smear.
- Gallstones common, occurring in 50% of HS patients by 50yo.
- Treated symptomatically, with folic acid, transfusions, vaccinations (same as for splenectomy), cholecystectomy and splenectomy, as needed.
- Risk of overwhelming post-splenectomy infection (OPSI). Pneumococcal vaccination required preop. Prophylactic antibiotics used for at least 3 years, lifelong in some cases. Patients always carry emergency antibiotics with them, due to risk of rapid progression of sepsis.
- Splenectomy also carries a long-term thrombosis risk.
- Spherocytes persist in the blood after splenectomy, but their lifespan is normalised.
- Should surgery proceed given current URTI?
- Frequent URTIs in the community at present, may cause repeated delays to surgery.
- ARISCAT scoring (assuming conversion to open abdominal procedure) suggests proceeding within 1 month of URTI in this patient would be associated with high risk (~44%) risk of POPC, dropping to moderate risk (~13%) thereafter.
- Severity of haemolytic crisis this year is motivation to proceed.
- Proceed with surgery, as discussed with the treating surgeon.
- Phone call to procedural anaesthetist