Laparoscopic Splenectomy in Children with Sickle Cell Disease

AORN Journal, March, 1999 by Margaret L. Anthony, Elise M. Hardee

Sickle cell disease is a group of genetic disorders that are characterized by the production of hemoglobin S, anemia, and acute and chronic tissue damage secondary to blockages caused by abnormally shaped (ie, sickle-shaped) red blood cells (Figure 1). Sickle cell anemia is the most common form of these disorders, and affects approximately 1 in every 375 African-American infants. In the United States, sickle cell disease primarily affects the African-American population; however, it has been found in other populations (eg, in individuals from the Mediterranean basin, Middle East, India).(1) A person who carries one sickle hemoglobin-producing and one normal hemoglobin-producing gene is described as having the sickle cell trait.

[Figure 1 ILLUSTRATION OMITTED]

There are three different types of sickle cell disease in the United States (ie, hemoglobin SS or sickle cell anemia, hemoglobin SC disease, hemoglobin sickle beta thalassemia), and some are more common than others. Sickle cell anemia has the highest occurrence, with one in every 625 live births in African Americans; hemoglobin SC occurs in one in every 833 live births to African Americans; and hemoglobin sickle beta thalassemia occurs in one in every 1,667 live births to African Americans.(2) Each type can cause sickle pain episodes and complications. Complications arise when the sickle-shaped blood cells block blood flow and degenerate. This degeneration can result in

* episodes of pain,

* stroke,

* infections,

* leg ulcers,

* bone damage,

* jaundice,

* lung blockage,

* kidney damage,

* circulation blockage in the spleen or liver, and

* low red blood cell counts.

Prevention of these complications is necessary to maintain the health of the sickle cell patient. To accomplish this, patients and their families are taught how to monitor for conditions that would encourage urgent medical evaluation. For example, patients and their family members should watch for fever, chest pain, shortness of breath, increasing fatigue, abdominal swelling, and unusual headaches, all of which would indicate a need for medical evaluation. In addition, they should be taught to watch for signs of anemia (eg, pallor, lethargy, poor appetite, increased jaundice).(3)

THE ROLE OF THE SPLEEN IN SICKLE CELL DISEASE

The spleen lies in the upper left abdominal cavity, protected by the 10th, 11th, and 12th ribs. It is covered with peritoneum that forms its supporting ligaments. The splenic artery, a branch of the celiac axis, provides the arterial blood supply, and the splenic vein, which drains into the portal system, provides the venous drainage. The spleen is responsible for defending the body from infection, through the process of phagocytosis, the formation of leukocytes and plasma cells, and the phagocytosis of damaged red blood cells.(4)

Much of the morbidity and mortality associated with sickle cell anemia in the first few years of life is a consequence of acute sequestration crises. These crises are characterized by severe anemia, splenomegaly, hypovolemic shock, and sudden death. Acute splenic sequestration results from rapid entrapment of red blood cells in the spleen. Microinfarcts are caused by the inundation of sickled cells in the spleen with the resulting slower blood flow. This increases the larger fast-flow circulation of the spleen, and a large amount of the circulating blood volume pools in the spleen, depriving the peripheral circulation of its regular blood volume.(5) The first documentation of this was in 1945. Eleven fatal cases were described, and it was suggested at the time that the patients had "bled into their spleens."(6)

During severe splenic sequestration, the spleen becomes enormous, and can distend into the pelvis. Minor or subacute episodes also are common and are characterized by a moderate increase in spleen size and a fall in the baseline hemoglobin of 2 to 3 g/dL. Treatment of acute splenic sequestration involves correction of the patient's hypovolemia with plasma expanders, followed by blood transfusions. Dramatic regression of the splenomegaly is usually seen after transfusion.

In children with splenomegaly, acute splenic infarction can occur, causing severe upper left quadrant pain accompanied by a "splenic friction rub" that is audible with inspiration in the patient's left upper quadrant. Because of the mortality associated with severe splenic sequestration, splenectomy should be considered if a child has two or more sequestration episodes. In children with sickle cell disease, the spleen is frequently dysfunctional after six to nine months of age; therefore, the risk of postsplenectomy infection is not greatly increased.

After an initial sequestration episode in children less than five years of age, a program of chronic transfusion therapy is begun and, because of the increased mortality associated with severe splenic sequestration, caregivers are taught about splenic palpation and asked to report any change in spleen size to their health care provider for evaluation. Health care professionals may recommend splenectomy for children older than age five who have experienced only one sequestration episode because of its high recurrence rate. Some patients with sickle cell disease develop chronic, massive splenomegaly with associated hypersplenism. Splenectomy is indicated for these patients when the degree of anemia, neutropenia, or thrombocytopenia is severe, or when the child is experiencing pain and discomfort due to the size of his or her spleen.(7)