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| He is usually a neat eater, but he was obviously not feeling well. Note the hyphema on the right lateral iris and the hyperemia. |
Past Medical History: 6 month old full term male. Fraternal twin A. Delivered C-section. Breast fed and recently started on baby foods. Vaccines up to date. Mild upper respiratory infection 2 weeks previous to current illness which resolved with supportive treatment. Normal newborn screening labs. Taking vitamin D, no other medications.
History of Present Illness: Giles was in beautiful Beaufort, SC visiting his Grandparents for Thanksgiving. He was having a great time and for the first time ate a little mashed potatoes. Nothing seemed amiss until the day after Thanksgiving when we noticed he had a pink right eye. We assumed it was conjunctivitis and we assumed it was viral. We were wrong.
We drove home on Saturday and noticed that Mr.Giles was not feeling like himself. He was grumpy and his eye looked like "let's take him to our clinic and have a pediatrician check him out." He felt it was likely conjunctivitis and likely a virus. It wasn't, but we primary care people all think alike. We were wrong.
The next day we went to see a local ophthalmologist, Dr.Nothing-is-Wrong, where he was diagnosed with a grade 1 hyphema (blood in the anterior chamber, where the iris is). It was theorized that he hit himself or his twin hit him in the eye. Dr.Nothing-is-Wrong felt that the odds of another cause would be very rare and he would have other symptoms. He prescribed prednisone drops (for inflammation) and atropine drops (to keep the eye dilated). No pressure of the eye was taken.
Giles wasn't feeling any better. He continued to sleep either all morning or all afternoon and woke throughout the night. His eye looked about the same. He was acting weird. We started to entertain all kinds of horrific scenarios and it was scaring us.
We followed up with Dr. Nothing-is-Wrong who stated that his eye was looking good, that he had a hyphema that was resolving and to continue the treatment plan. He tested his pressure and it was normal but, as we now know, this was probably false. We asked if his lethargy, somnolence, and irritability could be contributed to the hyphema and Dr.Nothing-is-Wrong said, "It is not his eye giving him those symptoms." It is at this point that Cymande almost demanded we go straight to the ER, but I felt that if Dr. Nothing-is-Wrong said nothing was wrong then nothing was wrong. He was wrong.
(Note: We had read in several pediatric ophthalmology textbooks that somnolence is often associated with hyphema and that it often results in a rule-out sepsis work-ups (foreshadow). This was mentioned to Dr.Nothing-is-Wrong and he seemed underwhelmed.)
Over the next 3 days Giles got worse. He was more somnolent, more irritable and acting strange. It was rare to get a smile out him. Finally, he refused to wake up in the morning. So, off to the University of Florida ER...
Upon arrival we were put in a private room where the ER resident, Dr. Something-is-Wrong, took the history and looked very worried. First, she called for an ophthalmology consult and the resident adult ophthalmologist arrived and was soon followed by her attending. They examined him and confirmed that he indeed had a hyphema, but they were unaware of it creating symptoms like his. Then the ER attending arrived and said 'We are worried about him.' Which made us even more worried. The work up: Head CT: no brain tumor or eye tumor. Labs: no obvious bleeding disorder, no obvious infection, no obvious metabolic disorder. Urine: clean. Diagnosis: Hyphema and Suspected Afebrile Sepsis.
At 2pm and Giles was still sleeping and not really feeding well. The ER work-up revealed no significant illness, but there was no lumbar puncture done. The great unknowns remained: afebrile meninigitis vs afebrile encephalitis. It was decided that he needed to be in the PICU. The PICU team sat us down and went through their thinking process. We felt nervous, but safe. They were thorough, thoughtful and they listened to the entire history. They called back the ophthalmologists and examined him under conscious sedation where his eye pressure was noted to be over 3 times the normal pressure. They stopped any further workup and started him on pressure drops that miraculously brought him out of his stupor. He laughed and squealed for the first time in a week. We squealed with him.
The next day he was transfered to the general pediatric floor and a pediatric ophthalmologist, Dr. I'm-Going-to-Figure-Out-What-The-F%@#-Is-Wrong-With-Him (DR.WTF) examined him. His pressure was coming down and we were discharged. She later examined him under anesthesia and she ruled out all the bad stuff. The working diagnosis is juvenile xanthogranuloma (JXG) of the iris, but we are not 100% on that.
So, the likely scenario is this: JXG caused a friable lesion of the iris to bleed creating a spontaneous hyphema. The hyphema and cellular debris clogged the angle (drainage system of the eye) and caused uveitis (inflammation) which led to acute glaucoma (increased intraocular pressure). This caused a severe headache which led to his somnolence, irritability and near toxic appearance.
