Another episode of House where diagnosis after diagnosis is discovered, but the whole was ultimately less than the sum of its parts. Frankly, I found the medicine in this episode to be weak and confusing, and the melodrama too schmaltzy for my taste.
Emma is a 41 year-old photographer who happens to be 21 weeks pregnant. At a photo shoot, she suffers a stroke. Luckily, she recognizes the signs and has an ambulance called. She is admitted to the hospital under the care of her physician. Even though her doctor is ready to discharge Emma, Cuddy asks House to examine her. He notes a pronator drift (a sign of muscle weakness or upper motor neuron disease), weakness in some of the mouth muscles, as well as some microaneurysms in her retinas (generally a sign of diabetic retinopathy). He also notes a copious amount of blood in her urine. Her discharge is canceled and many tests are ordered.
A urinalysis reveals protein and blood in the urine. Blood studies show an elevated creatinine, suggesting that Emma has developed kidney failure. An ultrasound of the kidneys is normal. Her blood pressure is good and there are no signs of pre-eclampsia (Also known as “toxemia of pregnancy”. Classic signs are high blood pressure, water retention, and protein in the urine). An echocardiogram is also normal. Despite this, House is suspicious that the problem is her heart. Her medical records show evidence of past Strep throats, and he suspects she must have gone on to develop rheumatic heart disease, specifically mitral valve stenosis, as a consequence of the Strep infections. A heart MRI does reveal a slightly calcified mitral valve. This is opened up with a balloon, but her kidneys do not improve (and frankly, why should they? A slightly stenotic mitral valve has no effect on the kidneys, unless the valve threw a clot to the kidneys, in which case the damage has already been done).
The differential diagnosis now includes pre-eclampsia, hypoperfusion (blood is going to the fetus preferentially instead of the mother), infection, HUS/TTP (hemolytic uremic syndrome/thrombotic thrombocytopenic purpura — diseases of the capillaries that can affect the kidneys), or a cholesterol embolus (a clump of cholesterol that breaks off the wall of a large vessel and proceeds to block off a small vessel), but tests for these are all normal. House believes that Emma has a rare condition known Maternal Mirror Syndrome (also called Ballantine Syndrome, or Maternal Hydrops). This is a maternal condition caused by an abnormality in the fetus (hence the term “mirror.”) House’s next step is to find the fetal abnormality. To get a good image of the fetus, House uses a paralytic agent to temporarily paralyze it while an MRI is obtained. The heart looks good, but the bladder is greatly enlarged, crushing the lungs. This bladder is enlarged because of a urinary obstruction. A stent is placed into the fetal bladder to relieve the obstruction. The surgery seems to go well, but now Emma is complaining of right upper quadrant pain (the location of the liver) and her sclera are icteric (the whites of her eyes are yellow), both signs of jaundice. Sure enough, lab tests show that she’s gone into liver failure.
House believes the only way to save Emma’s life is to terminate the pregnancy, but she refuses. Cuddy takes over the case. She slams Emma with high doses of steroids in an attempt to mature the fetal lungs. She suspects the liver failure may be unrelated to the Mirror Syndrome. This leaves a differential diagnoses of acute fatty liver of pregnancy, viral hepatitis, and HELPP (Hemolysis, Elevated Liver enzymes, Low Platelets) Syndrome. Cuddy wants a liver biopsy performed, but due to the risk of bleeding from low platelets, they’ll have to sample the liver from within the venous system itself.
The liver biopsy results are normal, but the procedure induced pre-term labor. Emma is given tocolytics to stop the contractions. Cuddy is now convinced that the Mirror Syndrome is the cause of everything and the problem must remain in the one area they haven’t been able to get a good look at: the fetus’s lungs — but because they are so underdeveloped, there’s no way to get a good look. Cuddy gives more steroids to Emma even though she is warned she could cause pulmonary edema — and (of course) she does. Emma is intubated and placed on a respirator and Cuddy keeps giving her steroids. Her plan works, the lungs open up enough that a scan shows that there is some sort of malformation there.
The only way to diagnose and treat for sure is to perform open surgery on the fetus. The surgery is rough, and Emma slips into ventricular fibrillation during the procedure. House wants to cut the umbilical cord (and kill the fetus) to save Emma, but Cuddy is able to shock her back into a normal rhythm and the surgery continues. The surgeon notes that the baby has CCAM (Congenital Cystic Adenomatoid Malformation) and he will be able to remove the lesions. With the baby’s lungs treated, Emma’s symptoms resolve and we learn in a closing scene that she delivered a healthy baby.
Let me state up front that I am no expert in maternal/fetal medicine and high-risk obstetrics. That being said, I still had some significant problems with the medicine in this episode. To save time (and my typing fingers and your eyeballs), I’ll just mention what I thought were the four biggest problems (I’ll leave the rest, including the surgery scene, up to everyone else — though if you have specific questions, I’ll do my best to answer them):
1. Why didn’t the mitral valve problem show up on echocardiogram? And if the mitral valve did cause the stroke, it’s because there would have been a clot or vegetation growing on it, which would have shown up on the echocardiogram. And if there was a clot, the last thing you would want to do is force the valve open.
2. Speaking of stroke, I’m not clear on what caused the stroke that set off the whole chain of events. I can find no connection between strokes and Mirror Syndrome. There has been one recorded case of a seizure and Mirror Syndrome, but that’s the closest I could find. Nor can I find any connection between blood in the urine and Mirror Syndrome. Protein in the urine – yes. Blood – no. And what was with eye exam showing diabetic retinopathy?
3. Mirror Syndrome occurs when there is fetal hydrops (a serious condition of severe edema in the fetus — which this fetus did not have), and the mother develops pre-eclampsia like symptoms including edema, liver problems, high blood pressure and proteinuria. There are also other key findings including anemia and elevated uric acid that were never mentioned. Mirror Syndrome can be caused by all the fetal conditions mentioned in the episode, but there is significant debate over whether surgery is good treatment. One reference I read went so far as to state that Mirror Syndrome was a contraindication for surgery (that is, a reason not to do it) because removing the lesions in the fetal lung did not help the mother at all.
As far as this episode goes, Emma had some symptoms of Mirror Syndrome, but was missing common ones, and had other symptoms that didn’t match (blood in the urine, stroke). Her baby had CCAM, which has been known to cause Mirror Syndrome, but did not show the fetal or placental edema seen with the condition. Surgery may not have been the best choice.
4. Mature lungs produce a chemical known as surfactant. Surfactant helps with the surface tension within the lung and keeps it from collapsing. Premature babies who are born without the ability to make surfactant can develop severe lung problems including respiratory distress syndrome. A dose or two of corticosteroids given 1-7 days before delivery will help the lungs create surfactant. Steroids do not otherwise help the lungs mature. Giving repeated doses of corticosteroids to a woman who is only 21 weeks pregnant (a long way from needing surfactant) will accomplish nothing useful.
I give this episode a
B for the mystery, because it was intriguing; and another
B for the solution, because it did ultimately fit. Unfortunately all the medicine in between was sub-par and earns a weak
D. The soap opera had some good moments, but seemed to waste a lot of potential, so it also earns a
B.
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Tags: television medicine house kidney failure liver failure pregnancy