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Diagnosis Deconstructed

Diagnosis Deconstructed: Crossing Lines a Long Way from Home

Morchi, Ravi MD

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doi: 10.1097/01.EEM.0000428248.86974.fa

    Seeing a straight vertical line cut through an island appears odd to me. It looks perfectly natural when dividing one state from another on a U.S. map, but my eye has trouble accepting it across a small island here in the Pacific. It does not seem right.

    This has to qualify as neglect. Why would anyone wait so long? I should have a talk with the father. It is up to me.

    “Tell me what happened. How did it start?”

    He begins, and I can make out only every other word. The woman standing between us converts his version of English to my version of English.

    Day 1. Father noticed a swelling of the lower lip. Maybe an ulcer. No matter though. Keep working. The fields need tending.

    Day 2. The patient is complaining of a headache, specifically pain behind his eyes. Both of them perhaps; father is not sure. No worries; keep working. Crops are livelihood.

    Day 3. The 8-year-old tells dad his vision is not normal. Something is wrong. Is it simply blurred? Double vision, a field cut, flashes, or floaters? Just what exactly is the vision disturbance? The language and our translation do not allow for these details.

    Day 4. Father notices that both of his son's eyes have turned red, and he can no longer work outside. His son remains in bed while the others care for the farm. By redness, he means conjunctival injection. I can see it at the bedside. Vision must have been severely diminished by this time. Were his eyes bulging out then? Father is not sure. I see bilateral proptosis as of now.

    Day 5. His son stopped responding normally. He would moan and speak nonsense, but that is all. He would no longer open his eyes. Father is now concerned, and brings his eldest to the hospital.

    We are east of the vertical line. It is a land of rugged terrain and steep ascents, mountainous topology peaking above cloud cover where native people have taken to using the fertile soil. They abandoned the hunter-gatherer lifestyle of those at lower altitudes thousands of years ago to work the fields, domesticate plants, and provide for their families. A fragmented people with hundreds of small communities separated by physical barriers littered through the highlands and speaking hundreds of different languages, the inhabitants of the mountains to the east of the line are at once fierce individualists and loyal tribesmen.

    I am standing at the bedside of an 8-year-old moaning in pain. He shakes his head every now and then, and I have to peel back his upper lids to get a glimpse of diffusely injected conjunctiva laced over bilateral proptotic globes. He holds a vacant stare straight ahead. This child is blind.

    I do not have an ophthalmoscope, but I suspect it would show me a fundus loaded with ketchup stains. He can rotate, but he resists when I try to flex his neck. Firm resistance with a scream. His knees and hips may bend a bit, but his neck will not flex. His lower lip is swollen with puruleant drainage from the inner mucosal surface.

    To the west of the line is a place rarely traveled to from the highlands. The inhabitants there bear no resemblance to our tribesmen. They are Southeast Asians in appearance and culture. Perhaps it is this fact that tells us the most about our patient population. The distinction of our highlanders is testimony to their power and independence throughout history. Strength in numbers, with an assist from geography, meant resistance to ancient Austronesian and Asian incorporation. And a culture, a diet, and a physical appearance markedly different from their neighbors just hundreds of kilometers away. Such is the character of a people who made their place in history to the east of the line and above the cloud cover.

    This is bad. Does this qualify as neglect? Why did he wait so long? Should I have a talk with the father? There is no agency to address the issue here. Is it up to me?

    I am called to see a new patient in the Casualty Area of the hospital. Walking back across the hallway, I notice a horizontal line etched into a wall in the clinic. It is here that the fate of young parous women is determined. Not by trial of labor with continuous external fetal monitoring because we do not have the resource or personnel for that, but rather by height. The highlanders are small. It has been discovered over the years, likely at the cost of many fetal lives, that women who fall short of the mark cannot delivery vaginally. Their pelvis, in proportion to their height, will not accommodate a term infant. We perform an elective C-section if they do not reach the mark on the wall. The mark has undergone some minor revisions over the life of the hospital, but it is still there, the main diagnostic modality for pelvic insufficiency.

    Passing through the clinic, I arrive in Casualty to greet a victim with multiple machete wounds. It is election time, and this seems to be the fallout for independent voting. Attacks and intimidation, and a judicial system with no teeth, mean no legal recourse for the victims. My review of the patient is followed by a discussion with the native doctor who called me. “Democracy? Democracy does not work here,” he says.

    I am a long way from the United States. I am reminded again of a viscerally unpleasant lesson particular to the developing world. Is this my home? Are these my people? I did not grow up in the highlands. I am an outsider to their culture and to their ways. I only have peripheral knowledge of their lives. My education in the United States, my experiences in a well differentiated medical setting, all the resources and moral structure gifted by our environment of plenty back home cannot make me the judge of the height of a horizontal line here above the cloud cover or the best method of leadership for a country of fragmented tribesmen. It cannot make me the judge of a universal right and wrong. Of a why-did-you-come-so-late father or a why-did-you-not-do-more sister. This is the most difficult lesson of the developing world, and I find myself forgetting and relearning it multiple times over, locale by locale, culture by culture.

    I greet the father again, with no lecture in mind this time. I choose to focus instead on the immediate problem and thank him for his history. He has chronicled the pathophysiology well. Step by step, he has taken us to the diagnosis.

    Day 1. An injury to the lower lip that became secondarily infected.

    Day 2. Bacteria traverse the venous drainage of the lower lip. Instead of migrating south through lingual and facial veins to jugulars down to the SVC, pathogens have chosen another route. To take the path less traveled … going west and north, crossing the border into a pterygoid plexus of veins draining into the retro-orbital space.

    Day 3. One component of Virchow's triad manifests. Endothelial damage from bacterial invasion has resulted in thrombosis. Thrombosis in the pool of valveless veins behind the bony orbits: the cavernous sinus.

    Day 4. Venous drainage from conjunctiva and the fundus is impaired secondary to the thrombosis. Venous insufficiency results in conjunctival injection and exudation of red blood cells into the fundus. Retinal congestion and visual disturbance set in.

    Day 5. Bacteria do not stop here. They have moved just millimeters cephalad and posterior to cross the meninges and blood brain barrier. This line is normally difficult to cross; this must be an aggressive assault. They take residence in CSF and neighboring brain parenchyma. They form communities and reproduce.

    Diagnosis deconstructed? Bacterial meningoencephalitis propagated from cavernous sinus thrombosis secondary to a lower lip abscess. No CT or MRI is here in these highlands to confirm my suspicion. If I am correct, what can I do about it here, a place where Chloramphenicol has historically been the best bet against meningitis? This child needs something more. The lip abscess is already draining, but I bluntly explore the cavity to ensure adequate decompression. An inquiry to our pharmacist produces a few doses of Ceftriaxone. This will have to do as a start. New volunteers are arriving from nearby Australia. We have asked them to bring Vancomycin and something more.

    Anticoagulation is not normally an option in the rural resource-poor district hospital. Suspected extremity venous thrombosis and their emboli are treated simply with fluids and aspirin. I am not entirely sure if anticoagulation will alter the course for cavernous sinus thrombosis, but I feel it is one of only a few paths available to us. Our volunteers bring enoxaparin on request, and we will cross our fingers.

    We may be in a foreign world where four days of puruleance and blindness alone are not enough to seek medical attention, treating a boy whose main value lies in his ability to do his part for the family by working in fields, with older relatives who own and use machetes on crops as well as other human beings who happen to disagree with a political agenda and whose mother may have had a C-section because she was too short. But his cavernous sinus and his meninges, they do not know that. Few things I have found retain value across oceans and continents like the anatomy and fundamentals of pathophysiology. They are good in any culture, in any time period in human history, beyond any border, and on the other side of any line.

    Five days into his treatment with vancomycin and enoxaparin he is awake and interactive. His proptosis and ocular motion have improved dramatically. He still has a significant residual visual deficit, but he can nonetheless walk and perform basic activities of daily living. He has been discharged, but will not leave us. Like many other patients on the ward, he sits waiting patiently for two more days as his father and uncle go to gather the money to pay for his care.

    I am a long way from home.

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