Case Presentation: A 75-year-old female with a history of Crohn’s disease with known sigmoid colon stricture presented to the Emergency Department with severe abdominal pain; CT abdomen pelvis without contrast revealed pneumoperitoneum, ascites, and a likely perforated colon. She was emergently taken to the OR and subsequently underwent exploratory laparotomy with rectosigmoid colon resection, end colostomy, and Hartmann’s procedure. Surgery also included left salpingo-oophorectomy and pelvic washout due to extent of abscess formation. One unit of packed red blood cells was administered during surgery, and empiric ertapenem was started. Sterile cultures collected during surgery isolated E. coli, bacteroides, and C. clostridiforme; antibiotic regimen was deescalated to ceftriaxone and metronidazole was added. Blood cultures remained without growth. Septic shock worsened despite ongoing fluid resuscitation, and the patient acutely declined on post-operative day five. CT abdomen pelvis without contrast revealed bilateral adrenal hyperdensity indicating Waterhouse-Friderichsen Syndrome. Morning cortisol level was decreased at 3.6 ug/dL; hydrocortisone therapy was immediately initiated and anticoagulation was discontinued. Fludrocortisone was initiated although discontinued due to worsening hypokalemia. Hydrocortisone was increased to 15 mg daily and 10 mg nightly; the patient rapidly improved, bowel function returned, and the patient was transferred out of the ICU. She remained stable throughout the remainder of her hospitalization and was subsequently discharged home.
Discussion: Waterhouse-Friderichsen Syndrome was first described in 1911 by Rubert Waterhouse and remains a rare, often fatal, diagnosis characterized by bilateral adrenal hemorrhage most commonly observed at autopsy. Typical symptoms include fever, purpura, and shock associated with meningococcal infections usually seen in the pediatric population, however, infections with streptococcus and other bacteria have been evaluated as well. Non-infectious causes are increasingly rare and include trauma, anticoagulant therapy, anti-phospholipid syndrome, and postoperative adrenal hemorrhage. Mortality associated with Waterhouse-Friderichsen Syndrome ranges from 20-50% depending on the clinical picture and presence of shock.
Conclusions: This clinically significant case demonstrates a positive outcome of a rare, potentially fatal diagnosis of Waterhouse-Friderichsen Syndrome with an atypical presentation of hypoperfusion in the post-surgical patient with septic shock and perioperative heparin use. This patient’s septic shock initially responded to fluid resuscitation and empiric antibiotics; however, her clinical status rapidly deteriorated, prompting further investigation. Abdominal imaging is of utmost importance to ensure establishment of a diagnosis and initiation of appropriate treatment including cessation of anticoagulation and initiation of hydrocortisone. Delay in diagnosis or treatment can result in fatality of the patient. In typical cases of Waterhouse-Friderichsen Syndrome, antibiotic initiation is imperative; however, this report demonstrates a case that is multifactorial in origin, emphasizing the importance of prioritizing steroid administration. When this diagnosis is suspected or confirmed, regardless if the typical symptoms of rash and fever are present, treatment should not be delayed due to the rapid progression and often morbid outcome.
