Unique anatomy continued: Polysplenia

Two weeks ago, I wrote about my daughter Lexie having situs inverses (organ positions reversed right to left) and an interrupted inferior vena cava. Both conditions were diagnosed at 16-weeks gestation.

Because she had these two conditions, my fetal-maternal specialist was worried Lexie might have asplenia (no spleen) or polysplenia (multiple small accessory spleens). Of the two, asplenia is worse as without a spleen to filter your blood, you are more likely to get infections. People can live without a spleen as sometimes theirs is damaged by disease or in an accident. But for infants, asplenia comes with a high infant mortality rate. Talk about a scary diagnosis to have for the remaining 21 weeks of pregnancy. And the hardest part is they couldn’t diagnose either of these until Lexie was born.

A sonogram after her birth did show Lexie had multiple spleens. The next step was to find out if one of these spleens was functioning. They ran a blood test which indicated at least one of the spleens was doing its job.

Because of her situs inversus, they did a whole bunch of other tests to make sure things were working properly. One test was a barium swallow study to verify her digestive track while backwards didn’t have any other problems or blockage. She actually wasn’t allowed to eat anything until they did this test which took place when she was two days old. They did another test which required them to stop her heart so they could verify everything was correct there.

We actually already knew she had a small hole between the top two chambers of her heart (Atrial septal defect). This had been diagnosed at the same prenatal appointment with the pediatric cardiologist who did the fetal echocardiogram to check out her interrupted IVC. Congential heart defects such as this one are common with polysplenia, occurring in 90% of the cases. Luckily, this hole filled in on its own by the time Lexie was six months old and never required any surgery.

So what has all this meant for Lexie? So far, it hasn’t meant anything. Since her spleen is functioning, we have not had any problems with infections other than some bacterial infections due to her scratching because of her eczema.

Unfortunately, this is not the case for many children born with polysplenia or asplenia. It also isn’t the case with children born with heterotaxy. I realize now that my husband and I did not ask a lot of the questions we should have asked when she was first diagnosed. We were in too much shock and overwhelmed. Even later as we prepared for her birth, we didn’t know what to ask. We knew Lexie would be taken to the NICU, but we never met with the staff that would take care of her or asked what tests and procedures would be done. I recently found this website that has a good list of question that one might ask in this situation though all the questions may not apply.

Again, just let me say that if you have a child with polysplenia, situs inversus or an interrupted IVC, feel free to contact me. I am not a doctor and have no medical training, but I have been there when you are feeling overwhelmed by everything your doctor is telling you. I can only hope that by discussing this that I can help or even reassure someone out there.

One thought on “Unique anatomy continued: Polysplenia

  1. Alexandra says:

    Thank you for sharing ❤ Wonderful post!

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