Day 0. He presents as a transfer from a nearby urgent care with the chief complaint of turning yellow. He had no history of being yellow. He did drink, perhaps too much. No pain or discomfort. No fever.
His vitals were normal, and he had tenderness in his right upper quadrant, not focally over what would be the fundus of his gallbladder. Not deep and localized over what could be his portal triad or the first part of the duodenum. Instead his tenderness ran below and parallel to his costal margin, extending across the RUQ to the high epigastrium, just under his xiphoid, an area corresponding to the left lobe of the liver. He did not complain of pain, but had hepatic tenderness.
His total bilirubin was 9 and predominantly conjugated, not suggestive of hemolysis. His transaminases and INR were normal. He denied any Tylenol ingestion.
Bedside ultrasound showed no evidence of extra-hepatic biliary obstruction but rather a loss of parenchymal detail and increased echogenicity suggestive of an intra-hepatic problem. Early cirrhosis or fatty transformation? This patient seemed to have baseline liver disease.
He appeared well and had no acute change in his mentation or level of function. His discharge diagnosis at the time was decompensation of chronic liver disease, possibly in association with cirrhosis from alcohol use. A brisk closure with its own problems.
No ascites. Cirrhotics who are currently jaundiced often have ascites, transudation from elevated portal pressures due to fibrotic venous sinusoidal crimping. It can be absent, however, in cases of recent therapeutic paracentesis, intravascular volume depletion, a prior TIPS procedure, or when the cause of liver dysfunction is acute rather than chronic.
Normal transaminases. Given that fibrosis has not yet set in, transudative ascites is often absent in acute hepatic failure provided it is not from an upstream hepatic vein thrombosis. But transaminases are usually elevated. Some evidence of recent hepatic inflammation or necrosis is expected.
Our patient does not fit nicely into either category. Maybe this was just the beginning of his liver's demise and we had yet to capture a transaminitis? Or maybe the diagnosis was not within hepatocytes themselves? He was out of the department now, and we had missed key information.
What we did not know. He was seen in a clinic two days before, and based on a few white cells in his urine, was diagnosed with a UTI. He was started on ciprofloxacin at the time, and his total bilirubin level that day was 5.
What we did not know was that in our triage area he complained of low back pain, central and referred to the midline of his sacrum. It was the only symptom that could have directed us to his primary problem.
What we did not know was that the nursing notes said he had persistent trouble urinating while in our ED. The notes specifically said, “Patient becomes diaphoretic when urinating.”
All of this information sat in a stack of papers that constitute his medical record, dutifully recorded by non-MD providers and no longer considered relevant by the patient.
Day 6. The diagnosis was made — sort of — on his second ED visit. He returned febrile and weak with a total bilirubin of 18 and a moderate transaminitis. He would receive a CT scan for vague, nonlocalizable abdominal pain. The radiologist determined he had pneumobilia. This, combined with his elevated conjugated bilirubin, led the ED team to diagnose him with cholangitis. He was admitted pending an ERCP. That is, until moments before the procedure, when his CT was overread as portal venous gas, not pneumobilia.
Air was then deemed to reside millimeters away within intrahepatic portal venous tributaries. The differential shifts from obstruction and infection of the biliary system to the intestinal wall. Necrosis or inflammation here results in leakage of luminal gas into neighboring venules. Anaerobic bacteria within the necrotic wall can pour more gas into the venous system. It rises along the mesenteric veins, through the portal vein, and pixelizes as speckles of air within the liver on CT scan. Air is trapped between hepatocytes, unable to transgress to the hepatic veins and into the IVC, as the intrahepatic diameter of these portal tributaries narrows.
So where is it coming from? The small bowel on CT appears normal. No ileus pattern. The cecum and ascending colon appear unimpressive. The SMV draining these areas is without intraluminal gas.
But as you round the hepatic flexure, float across the transverse colon, turn sharply down the splenic flexure, and enter the sigmoid colon you notice something: diverticula. Pulsion of mucosa in between the incomplete outer longitudinal layer of colonic muscle known as tenia coli, diverticula actually do not contain all layers of the bowel wall. The diverticula we see, the ones we familiarize ourselves with, are actually pseudodiverticula.
The rectosigmoid junction is quite thick. With stranding of the perirectal fat here, it is presumed that rectosigmoid diverticulitis is the source of portal venous air. You can see a distended IMV draining this area with islands of air sprinkled throughout its course. It merges with the splenic vein and finally the portal vein. Vasculature is grossly enlarged here, and a sharp filling defect seems to be at this point in the portal vein.
Thrombus. Our patient does not just have portal venous gas; he has portal venous thrombus as well. The thrombus must be infected. His diverticulitis did not evolve by perforating into the peritoneal cavity freely or by being walled off as a pool of pus in a pericolonic abscess. Instead, infection in this case decompressed in a third, less common route: into the draining veins. The endothelium of these veins injured by migrating bacteria and our own immune response, we see secondary thrombosis. Diverticulitis resulting in infected thrombus of the inferior mesenteric, splenic, and extra-hepatic portal veins. This is pylephlebitis.
He is moved from the ERCP suite to the operating room where he undergoes resection of a densely adherent, chronically inflamed conglomerate of diverticula at the rectosigmoid junction. The proximal portion of sigmoid colon is brought out as an end colostomy and the distal remaining rectum oversewn as a Hartmann's pouch. His IMV is ligated at a proximal rostral site as a roadblock to future bacterial migration. He does well postoperatively, and is discharged days later on Coumadin.
What did he have on day 0 to protect against early closure and guide us to the rectovesicular pouch? Clues missed by physicians but not nurses: external irritation of his bladder wall presenting as pain with urination and pyuria without organisms. Originally declared by urgent care as having bladder mucosal inflammation, this patient did not actually have infectious cystitis but an adjacent colonic diverticulitis.
Low back pain. Midline, sacral, low back pain was a visceral symptom whose origins were not the musculoskeletal system but the proximal rectum and rectosigmoid junction, both tucked far enough posteriorly and inferiorly that his anterior abdominal exam remained benign and high enough along his rectum that digital rectal exam was equally unimpressive.
Conjugated hyperbilirubinemia without transaminitis or ascites would turn out to be his warning sign. Faltering of his weakest baseline system: subpar functioning hepatocytes not up to the challenge of transferring conjugated bilirubin into neighboring canaliculi in the face of a new catabolic, infectious stimulus.
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