Be careful what you wish for

A diagnostic delay of 60 years sets the scene for a Christmas surprise

I was a cranky baby, so my mother told me, but sympathy finally came my way at age 2 when I urinated blood. “Nephritis,” was our doctor’s diagnosis, “Post-strep, most likely.” This was back in the 1950s, when doctors were still diagnosticians, diagnoses were often clinical, and clinical management usually expectant. In hindsight, the decision to do nothing more proved to be a wise one.

I grew to be a cranky boy, so my father told me, perhaps explaining why I don’t recall ever being in the swing of things in the 60s. But in the 70s — as a medical student doing an elective at a snow-shrouded New York hospital in a deep and dark December — I first became aware of a dull gnawing sensation consistent with what my textbooks termed left loin pain. I tossed and turned throughout those painful nights, worrying if I had picked up some esoteric endemic American disease. I even remember one wintry evening when I longed to buy a ticket to Tchaikovsky’s Nutcracker ballet to distract myself, but just couldn’t afford it. My anxiety about the ache faded after returning to antipodean climes in the New Year, but I was left with the nagging doubt that maybe my distress had been caused as much by homesickness as by organic disease.

The 80s brought me and my family back to the northern hemisphere for a postdoctoral stint in Boston, where the recurring ache in my left back caused me to hypothesise that I had either unilateral vesicoureteral reflux or, by default, a too-fertile imagination. Come the 90s, we again flew west to make a new nest in the East for a dozen or so years. But the arrival of the homophonic “noughties” made the temptation of returning to Australia for unlimited alcohol, gambling and low wages too hard to resist. Sure enough, as soon as our plane hit the tarmac at Sydney Airport, I found myself begging the locals for permission to raise the largest mortgage debt possible, as one does, and the resulting stress persuaded me to unload whatever was left of our savings into the rock-solid investment vehicle of term life insurance. Yet on detecting microhaematuria, my prospective insurers — who until then had exuded only bliss about the prospect of purchasing peace of mind from them — became so agitated about the risk of my premature death that they requested a repeat test. Hydrating myself to the hilt so as to provide a clearer specimen appeased them enough to issue the first of many premium invoices — none of which, sadly, was ever to yield any returns. But the anxiety unleashed by my insurers’ doubts drove me to dipstick myself for years ahead, and there always seemed to remain that stubborn trace of blood — consistent with subclinical and hence benign damage from my childhood nephritis, or so I told myself.

It was not until last year, as if to herald the start of my seventh decade, that I first witnessed my own haematuria in real time. This occurred after an uncustomary attempt at sit-ups, an initiative I have since been careful not to repeat. But the shock of seeing port pouring on the porcelain did at least force me to think harder about enlisting help from colleagues. Could the problem, I kidded myself, be kidney cancer — an attractive diagnosis, since at least I had one of those organs to spare? Unlikely perhaps, given that I’d just had normal abdominal imaging as part of a health screen for worried-well neurotics. Bladder cancer? Well yes, that was a worry. But did I really want to rush into a cystoscopy with the sole aim of condemning my trusty hollow viscus to surgical ablation?

Now don’t get me wrong, some of my best friends are urologists, but after much thought I opted to ask a nephrology colleague to help me navigate through what I felt sure would prove a diagnostic tempest in a teacup. Sympathising with my aversion to surgery for some reason, he ordered the usual tests, all of which proved negative as intended; plus he arranged a multiphase computed tomography pyelogram. On the morn of Christmas Eve, I received a cheery call from a mutual friend in radiology who said she had diagnosed a circumaortic left renal vein with gross dilatation of the dorsal limb. Excuse me? “You’ve got nutcracker syndrome,” she giggled, as if some long-kept shameful secret was now finally out of the closet. “Merry Christmas.”

Getting a name for a subchondral pain that I’d always suspected was hypochondriac has helped me. Working backwards from the anatomy, I have since found that the only painless position at night is on my right side with the left hip extended. If I roll onto my left side during sleep, I then need to rid myself of the ensuing pain by flopping back over to the right and taking three deep breaths to speed venous return. Had I discovered this antidote earlier, I could possibly have saved myself entire decades of nocturnal fretting.

I now understand that nutcracker syndrome is better termed “left renal vein entrapment” syndrome, of which there are numerous variants and presentations. Opinion is split as to whether this is a rare or common cause of symptoms, although there is a consensus that the causal vasculopathies are underdiagnosed. Incidental diagnoses of nutcracker syndrome are increasing with the use of ever-more-sensitive scans, while the reported prevalence of nutcracker-like renal vein anomalies has now hit 5–10% in both imaging and cadaver studies.1-3

This high frequency of a hitherto uncommon condition raises challenges for scarce health resources. Treatments abound for nutcracker syndrome in scholarly journals — vein reanastomosis, stenting, embolisation — but we doctors also know that publication bias favours positive results. Now don’t get me wrong, some of my best friends are vascular surgeons; but one of my few remaining ambitions in life is to undergo as few operations as necessary to get me up to that great recovery room in the sky.

And with that calming thought, I switch off my bedside lamp and flip back over to my right side for perhaps the thousandth time this year, take three deep breaths, and reflect with respect: be careful for what you wish, and grateful for what you get.