Case: Forest vs. Trees

I was a fourth-year medical student by the time I finally rotated through anesthesiology.  Students who suspected they might be interested in entering this specialty scheduled their rotations early in the third year, but crunchy family practice types like me let it slide until fourth year, when you have a bit of medicine under your belt and can kick back and have some fun on a procedure-rich rotation.  Yet there are primary-care teaching moments on every rotation, even ones which take place largely in the operating room rather than the clinic.  Here's one example:

I'd been on the rotation for a couple of weeks, mainly at an outpatient surgery center where the pace was mellow and the attendings did a lot of teaching because they were never knee-deep in a heart-lung transplant or any other edge-of-your-seat cases.  The students got to do a lot of intubations and laryngeal mask airways (LMAs) because the case turnover was rapid and the patients were in pretty good health overall.  I'd seen adult cases primarily so I jumped at the chance to ride shotgun in the pediatric rooms.

Most of the cases were for simple procedures requiring sedation in pediatric patients, such as lumbar punctures and stereoscopic eye exams.  I got to put in a few pediatric LMAs but there weren't any intubations and I found myself observing the procedures to pick up tips on how to do them on kids who were awake rather than sedated.  It's a lot easier to observe technique on a patient who is not moving.  Most of the kids were cancer patients and it was poignant to see them interact with their parents, who tried to minimize the threatening environment of the procedure room by reading picture books until their child was comfortably asleep.

After so many cancer cases, it was a relief to hear we were going to have a nice, routine, happy case.  A five year-old girl had been born with severe polycystic kidney disease and had undergone a successful kidney transplant a couple of months earlier.  Her mother was the donor and was still careful picking up her daughter because of slow wound healing at the donor site.  The little girl was coming in for a stereoscopic eye exam because her mother had noticed a white spot in one pupil whenever her daughter was in direct sunlight.  Apparently she'd raised this issue with her daughter's many doctors but the eye exam had always been deferred in favor of moving forward with the kidney transplant.  Now that things were going well on the kidney front, they were finally in for an ophthalmic exam.

The girl was easily sedated and the pediatric ophthalmologist began the exam.  She looked into the girl's eyes carefully for a few moments, saying little, and then she said "Oh no."

This is not the kind of thing you like to hear from a doctor, and even though the parents were not in the procedure room, I had a sudden feeling of misgiving.  The anesthesiologist asked what was wrong.

"Yeah, I think she's got it," said the ophthalmologist, speaking elliptically because she was examining the girl's eyes with a hand-held stereoscopic lens.  "It looks like a piece of cauliflower in the right eye.  Damn!"

One of the many random facts I'd crammed into my brain during second year popped into the forefront of my mind.  "You mean she has a retinoblastoma?" I asked.

"Yes, and it's advanced," said the ophthalmologist.  She looked up from her lens.  "Are you a student?  Come take a look."

I did.  Even to my unskilled eye the mass was unmistakable.  The ophthalmologist was right; the right eye looked like there was a tiny cauliflower encased in lucite.  

Retinoblastoma is a malignant cancer of the eye.  It can be inherited or arise from a genetic mutation.  It is usually detected in kids around eighteen months of age, and the usual symptom is a white spot in one eye.  If detected early, the tumor can be treated with radiotherapy and efforts made to preserve as much vision as possible.  If detected late, the eye must be removed and the tumor can metastasize to the central nervous system.  This girl was already five years old, and she had another significant disease (polycystic kidneys), and I think everyone in the procedure room shared the sinking feeling that This Was Not Good for this girl and her family.

The ophthalmologist asked the girl's mother to come back into the room and showed her the tumor.  She explained what it was and what the next steps in the work-up would be.  The mother was quiet initially, then she asked in a shaky voice:  "Can she see?"

The ophthalmologist chose her words carefully.  "This tumor covers her retina, so I don't believe she can see through her right eye."

This was devastating to hear.  The girl's mother ran sobbing from the room.  Suddenly I felt clammy and tight-chested and tears stung the corners of my eyes.  The mood in the room was somber as the anesthesiologist quietly lightened the patient's sedation.

My medical school was one of the top five medical centers in the U.S. at the time, and there are few places better for a child with a major medical problem to go for specialty care.  What this case illustrates is the downside of highly specialized care.  Any healthy child gets serial screening eye exams from birth until they register to vote.  Of course, these are not comprehensive, dilated, stereoscopic exams but at least a primary care doctor shines a light into the pupil specifically in search of an abnormal flash of white.  I suspect this part of her exam had not been done for years, or done only perfunctorily, by the specialists who were so involved in her kidney disease.  Please understand, I'm not blaming them for missing this diagnosis.  I just wish she'd had regular contact with a primary care pediatrician who might be rusty on anti-rejection protocols for transplant patients but who would have reached for the ophthalmoscope out of years of habit and care.

Image in the public domain.

Comments

[AnnR]

Or, it might underscore the idea that even though you've been seeing a million fancy-pants specialists you still need to be dragging yourself or your loved ones into your PCP for boring and routine exams.

If you have a special problem it can seem like all you ever do it go or take your loved one to the doctor. Who wants to add more visits in when it seems like every last hour of vacation time is spent attending appointments?

I think your story makes a good point any way you choose to look at it.

[QuietusLeo]

This sad case underscores that all doctors, specialists and primary care physicians alike must first of all be caretakers for our patients and examine them fully, just like we were taught in med school.

[Bianca Castafiore]

You're so right about this being a down side of specialized medicine. Imagine being told, after asking what needs to be done next to address a growing aortic arch aneurysm and being told: "Don't worry about that... There are more pressing matters." Honestly, what can be more pressing than waiting for your chest to explode?

Thanks for sharing this story.

[Mommy michael]

My best friend's daughter has this in her right eye. Luckily it's benign. Only thing they can do is fix the muscles in her so she doesn't look like she has a lazy eye.
Her mother is putting that particular procedure off until her daughter says she wants it done. if it's not going to help her see, then it's not worth putting her through surgery.

[rlbates]

So sad that no one had noticed it earlier.