WHAT IS POLYSPLENIA?

Polysplenia is a diverse disease that mainly affects the asymmetric organs. This may include the heart, lungs, bronchi, liver, intestines, gallbladder, portal vein, stomach and spleen. Crucial manifestations of this disease include Congenital Heart Disease (CHD), biliary atresia, intestinal malrotation, and Functional Asplenia.

Polysplenia is also known has bilateral left-sidedness. The left lung lobar anatomy is bilaterally/bilateral hyparterial bronchi (2 lobe lungs). The incidences of multiple small spleens are common in people with Polysplenia. People who have Polysplenia have bilateral SVCs, (about 50 to 70% percent have a) continuation of inferior vena cava (IVC). Chloe has an interrupted inferior vena cava, venous return for which occurs on the right-or left sided azygous system.

I have read and was told that Polysplenia occurs in an estimated 4 per 1 million live births. Approximately 90% of patients with Polysplenia have congenital heart disease (CHD). Chloe is lucky and falls in the 10% that never had heart surgery. Did you know that death can result from congenital heart disease (CHD), biliary atresia, intestinal malrotation, or sepsis? That is why early detection is so important.

I have also read that Functional Asplenia is overlooked in the Polysplenia community. Children who live past the age of 1 have a higher risk of dying from sepsis than from related CHD (especially if you have Asplenia). That is why it’s important to detect spleens that are not working at its full capacity in patients with Polysplenia. This will help prevent fatal bacterial sepsis. Patients with Functional Asplenia are vulnerable to streptococcus pneumococcal, Hamemophilus Influenza Type B, Niesseria Meningitidis and salmonella.

Two of Chloe’s doctors recommend that I keep Chloe away from certain animals. Such as; dogs, cats, rats, mice, hamsters, turtles, lizards, snakes, frogs and so on. In my case, I feel it’s important for friends, family or anyone who has an animal to fully understand this. Your child’s health may depend on it if they don’t. My daughter loves animals, it hard for her at times to understand why her mom is cautious with her around them.

The treatment for Funtional Asplenia and Asplenia involves four important things: antibiotic prophylaxis, appropriate immunization, aggressive management of suspected infection, and parent education.

It is also noted that, in one large showing assessment from Canada, the 1-year mortality rate was 32% and the 15-year mortality rate was 49%. I feel that we need more studies done on this rare syndrome. I have read that the frequency of Polysplenia has not been studied extensively, but it appears to be uncommon, comprising less than 1% of congenital heart defects. In a 26-year study done at the Hospital for Sick Children in Toronto Canada, only 91 cases were found. Yes, you heard me correctly. In only 26 years they only found 91 cases with Polysplenia.

For more information please visit the following links:

http://www.emedicine.com/ped/topic150.htm
http://www.emedicine.com/Radio/topic58.htm
http://www.emedicine.com/ped/topic2514.htm
http://www.emedicine.com/radio/topic639.htm