Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the abdomen. Splenomegaly is usually associated with increased workload which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease that involves abnormal red blood cells being destroyed in the spleen. In terms of Biliary Atresia it is Portal Hypertension and backpressure on the spleen which results in its increased size.
A normal Spleen is about 12 cm long, 7 cm wide. Splenomegaly is defined as spleen size over 12 cm, as measured by ultrasound along its longer dimension.
• Moderate splenomegaly, if the largest dimension is between 11–20 cm
• Severe splenomegaly, if the largest dimension is greater than 20 cm
Normally, you have anywhere from 150,000 to 450,000 platelets per microlitre of circulating blood. Because each platelet lives only about 10 days, your body continually renews your platelet supply by producing new platelets in your bone marrow. When your spleen is enlarged your platelet count will usually be below normal range, it is not uncommon for platelets to drop and remain below 50,000, in some cases even 20,000 or less.
Your spleen works to fight infection and filter unwanted material from your blood. An enlarged spleen, therefore, ‘overworks’ at breaking down old blood cells causing a decrease in the number of platelets in circulation. This particularly effects the platelets that help with blood clotting. When the platelet count is low, bruising and nosebleeds are more likely.
Splenomegaly should not be confused with hypersplenism. The former is a statement about the size of the spleen, and the latter about the spleen’s function: these may coexist, or they may not.
Symptoms may include enlarged tummy; abdominal pain; chest pain; chest pain similar to pleuritic pain; pain similar to when stomach, bladder or bowels are full; back pain; a feeling of fullness after eating and feeling full quickly (early satiety) this is due to the spleen encroaching onto other organs in the abdominal space.
While the Spleen is usually protected by the ribs, the enlarged size makes it more vulnerable to knocks and bangs. Your child may be advised to avoid contact sport. For children with a very low platelet count, below about 50,000, it may be advisable to avoid any kind of activity where bumps and bruises are likely to occur. However, this should be discussed with your child’s specialist as it is important to continue with suitable activities and exercise.
The term burst spleen is a little misleading. If the spleen does rupture it is more like a wet paper tissue separating and breaking up. Nevertheless, like a plastic bag full of beads in water, once burst it is hard to stop its demise and because the Spleen has such a rich network of blood vessels rupture can result in death through blood loss. Any injury to the left abdomen needs immediate medical attention.