SURGERY IN SICKLE CELL DISEASE

                   Surgery in Sickle Cell Disease

Sickle cell disease (SCD) is the result of sickling of Red Blood Cells (RBC) when deoxygenated.

This occurs in homozygote state is of haemoglobin-S gene (sickle cell anaemia), haemoglobin SC, haemoglobin-SD and haemoglobin-S beta thalasaemia states.

Sickle cell anaemia  HbS is the most severe sickle cell disorder.

Hemoglobin SD is the mildest.

Haemoglobin-SC and haemoglobin-S beta thalasaemia are in between.

Epidemiology:

SCD is prevalent in tropical Africa, the middle East and certain areas of India.

With ever increasing medical tourism, Indian surgeons need to be aware of role of surgery in SCD.

Histological:

Diagramatic picture of blood smear

Actual Blood Smear Picture with Sickle Cells

Clinical:

Episodes occur due to sickling, vaso spastic episodes, anemia, hemolytic, aplastic occurrences.

Presentation depends on:

SPLEEN	Sequestration in spleen
	Infarct in Spleen
	Splenic Abscess
LIVER	Hepatic Crisis
	Acute Liver Failure
BILIARY	Gall Stones
	CBD stones
GI	Peptic Ulcers
	Ischemic Colitis
	Miscell- Pancretitis, Appendicitis
Skin	Leg ulcers

Surgery in SCD:

Splenectomy is reserved for a small fraction of acute episodes associated with hypersplenism and pancytopoenia.

During surgery, correction of anemia should be done, Hb S should be kept below 40% levels, and hemoglobin near normal to prevent acute crisis.

Splenic percutaneous drainage may be attempted for splenic abscess and when this is not possible, splenectomy should be considered.

Vaccination against pneumococcal and hemophilus influenza group B should be carried out pre operatively. 

Overwhelming post splenectomy sepsis (OPSI) can occur up to 2 years after splenectomy.

Laparoscopic cholecystecomy should be done for gall stones with symptoms.

CBD stones can be treated similar to other situations.

Laparoscopy has made surgery  safe without complications so that even asymptomatic gall stones can be taken up for surgery.

Care must be taken to avoid hypotension, hypothermia and hypoxia during surgery which should be covered with antibiotics as there is some level of immunocompromised state due to auto splenectomy in the advanced cases.

Conclusions:

Though SCD is a medical disease, surgery is required for a large number of complications. 

Laparoscopy has made surgery safer and surgeon has to be aware of when to operate and  how to take care of prophylaxis against infection and post operative crisis.