A Rare Cause of Rapid Death in Patients With Crohn's Disease.

Question: A 35-year-old male patient was hospitalized due to intermittent low back pain, abdominal pain, and fever. He had a 1-year history of suspected ankylosing spondylitis (AS) and had short-term nonsteroidal anti-inflammatory drug use for about 1 month. He was diagnosed as having Crohn’s disease (CD) and AS 1 month before admission. Computed tomography enterography revealed transmural ileocolitis without obvious fistula or abscess and segmental small intestinal lesions (Figures A, B, C). Meanwhile, the patient had bilateral sacroiliitis of grade 2 on computed tomography (CT) scan with no peripheral joint involvement. Anti–tumor necrosis factor therapy was recommended, however, he refused because of the cost and inconvenience. Oral methylprednisolone (0.75 mg/kg/d) and sulphasalazine were effective in improving his abdominal and joint symptoms in the first month after treatment. He continued tapering glucocorticoids until developing fever 4 days before admission. At admission, there was no abdominal tenderness and distension or signs of peritoneal irritation. He presented a body temperature of 38.4°C, blood pressure of 74/44 mm Hg, and heart rate of 84 beats per minute. His white blood cell count was 8.64 × 109/L. The C-reactive protein and procalcitonin levels were elevated (116 mg/L and 61 ng/L, respectively). A higher level of D-dimer was observed (13.76 μg/mL). The levels of serum alanine transaminase and aspartate aminotransferase were also elevated (91.17 U/L and 223.33 U/L, respectively). CT of the chest revealed normal findings. The intestinal ultrasound did not suggest intra-abdominal abscess or disease progression. The patient had no previous history of cardiovascular disease. Due to suspected severe infection and hypotension, the patient was given adequate intravenous rehydration and intravenous anti-infection therapy of cefoperazone-sulbactam. The blood pressure did return to 90–105/60–70 mm Hg and the body temperature fluctuated between 37.8°C and 38.4°C. On the second day of admission, the patient suddenly developed a high fever, convulsions, loss of consciousness, and coma. Endotracheal intubation and ventilator-assisted respiration were given immediately. His anion gap and lactate were elevated (10.8 mmol/L), whereas his arterial blood pH was 7.17. Brain and skull CT revealed normal findings. Chest CT revealed new-onset bilateral pneumonitis and pleural effusion. Abdominal CT revealed a small amount of thrombosis in the ileocolonic vein, submesenteric vein, portal vein, and perivascular inflammation (Figures E, F). Air bubbles in the superior mesenteric vein and swelling of the liver parenchyma were also noted (Figures D, F). At the same time, ileocecal bowel inflammation did not significantly worsen, and was even a bit better than before (Figure E). Surgery was considered but this critically ill patient could not tolerate surgery. Despite adequate treatment for septic shock with fluid resuscitation, intensive anti-infection therapy, and cardiovascular support, the patient died just 10 hours later. A positive blood culture result was confirmed 5 days later. Blood culture yielded Escherichia coli. What is the cause leading to rapid death? Look on page 547 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting to Gastro Curbside Consult. . Based on all the clinical features, histopathology, and imaging findings, we diagnosed the case to be CD complicated with enterovenous fistulization. This can be confirmed by thrombosis, perivasculitis, and intravascular gas in the mesenteric venous system corresponding to ileocecal bowel lesions. Meanwhile, AS was diagnosed based on clinical manifestations and imaging. The current case is an extremely rare clinical condition. Only 3 cases were previously reported, and all these patients underwent emergency ileostomy or ileocecal resection. Two patients had an uneventful recovery after surgery. One patient died of uncontrollable sepsis and hepatic failure.1Reiner L. Freed A. Bloom A. Enterovenous fistulization in Crohn’s disease.JAMA. 1978; 239: 130-132Crossref PubMed Scopus (10) Google Scholar, 2Ajzen S.A. Gibney R.G. Cooperberg P.L. et al.Enterovenous fistula: unusual complication of Crohn disease.Radiology. 1988; 166: 745-746Crossref PubMed Scopus (22) Google Scholar, 3Lim J.W. Kim K.J. Ye B.D. et al.Enterovenous fistulization: a rare complication of Crohn’s disease.World J Gastroenterol. 2011; 17: 5227-5230Crossref PubMed Scopus (5) Google Scholar As in our case, enterovenous fistulization formed on the basis of regional enteritis will cause a septic invasion of the mesenteric venous system, with consequent pylephlebitis and hepatic abscess formation. Most patients experienced abdominal pains, fever, and chills. Their clinical course deteriorated rapidly with progression due to systemic bloodstream infection. This is a rare case of CD complicated with enterovenous fistula, leading to septic shock and death. CD complicated with enterovenous fistula could cause rapidly progressive septic shock with an occult presentation. This dangerous complication requires early diagnosis and urgent surgical treatment to save lives. Thus, we report the rare condition and remind doctors of patients with inflammatory bowel disease to better diagnose and manage these patients.