On the table lays a young Asian male. He is cold and pale and staring blankly at the ceiling. His globes are deflated as if vitreous had been aspirated from them. His back is propped up on a block giving him an arch resembling opisthotonus from tetanus. His head and neck dangle backwards without any support. He is not alive. He has not been alive for four days, ever since being found dead in his bathtub. Discovered by a distraught family member who called 911, he was pronounced at the scene. We now gather to determine his cause of death.
Drug overdose is high on the differential anytime a young patient ends up on this table. I inspect his skin closely but find no track marks or evidence for venipuncture. Not in his antecubital fossa nor, as is often used to avoid being noticed, in the femoral area. No lower extremity scars indicative of injection into the subcutaneous space. Maybe he inhaled something? An oral ingestion of a sedative or narcotic? Found in a pool of warm bath water, probably an intentional rather than accidental overdose.
The abdomen. We open it, and find no hemoperitoneum. His stomach and intestines are clean. No bluish hue suggestive of intraluminal blood from a massive GI bleed. The liver appears healthy and normal. No portal triad lymphadenopathy and no splenomegaly indicative of intravenous drug use. My eyes scan a long way down to the pelvis; he does not have a distended bladder to corroborate sedative or narcotic overdose. The cause of death is not in the abdomen.
But before we move to the chest, I want to know something. I strain to reach into his right upper quadrant. Things seem a lot farther than they should be. Everything is just out of my reach. Is this table set high?
Opening the tissue to the right of the duodenum and the avascular peritoneal reflection lateral to the ascending colon, we can move the duodenum and large bowel medially. This exposes his inferior vena cava. It is not flat but distended. Very distended. His venous pressure was high at the time his heart stopped beating.
IVC. Visible to us by ultrasound in the living emergency department patient, the IVC can tell us a lot about the immediate cause of hypotension. Distended it indicates adequate or supranormal right heart pressures. The problem is not plasma depletion, hemorrhage, or vasodilation and venodilation redistributing blood to the periphery in these cases. Rather, a distended IVC means an inability to circulate that volume: RV infarction, obstruction to transpulmonary flow by pulmonary emboli, or poor left ventricular myocyte or valvular function resulting in a congested pulmonary circuit and right heart. The answer is in his chest.
The records. He was seen by a family physician for episodes of atypical chest pain two weeks prior. The practitioner, finding nothing, referred him to a cardiologist. This was a 25-year-old with no family history of cardiac disease and no history of diabetes, yet the primary care doctor was concerned enough to send him to a specialist. The cardiologist was impressed enough to do a stress test. Our patient reached his maximum heart rate, but the ST segments did not move. He was sent home without a diagnosis.
The chest. His chest wall caves inward naturally. Pectus excavatum pointing us toward the answer, we follow it with large bone cutters to remove the sternum and costal cartilage. I lean in and strain to peer into his chest, and find my eyes scanning up and down a long thoracic cavity. That is when we recognize that this is not an overdose.
The problem is that the pericardium is bulging and tense. Opening it, we find clotted blood. Tamponade is the reason his IVC was distended. Are we sure there was no trauma in this case? The pathologist and I meet eyes and nod. We know where this is going. The answer is close by.
Clearing the clotted blood and using fine forceps to tease at the aortic root within the pericardial sac, I find it. We have to look closely, but it is there. A hole three millimeters in size. Aortic rupture decompressed into the pericardial sac. One third of a centimeter sealed his fate at 25 years of age.
The aortic root at the site of rupture is dilated and floppy. But the wall does not feel firm like normal aorta. It is awfully thin and flimsy. It does not contain all three layers: adventitia, media, and intima are not represented in this wall. This is not an aneurysm. It is a pseudoaneurysm.
Pseudoaneurysmal dilation of the aortic root and ascending aorta: it means the intima is peeled from the other two layers. The wallpaper has been pulled off the wall, and blood is filling the false lumen. Aortic dissection.
We can follow the flap up into the right carotid as well as across the aortic arch down the descending thoracic aorta, passing below the diaphragm and entering the celiac artery. It had not ruptured into his pleural or peritoneal cavities. He had not lived long enough for that to happen.
Stepping back, the case takes form. His long legs approach the end of the metallic table. His knees have a genu valgus deformity. His chest wall caves inward, and his facial features are long and drawn out, with crowding of his teeth. When I take his hand in mine to passively oppose his thumb across his palm, it reaches well beyond the ulnar border.
Unnoticed by his primary care doctor, a cardiologist, a pathologist, and myself. We were all looking but too closely. Magnifying the complaint and the organ in our minds, we neglected to see our person as a whole.
Click and Connect! Access the links in this article by reading it on www.EM-News.com.
Dr. Morchi is the di...Image Tools
▪ Read more about Marfan syndrome at www.marfan.org.
▪ Read all of Dr. Morchi's past columns in the EM-News.com archive.
▪ Comments about this article? Write to EMN at email@example.com.
© 2012 Lippincott Williams & Wilkins, Inc.