Pancreatic necrosis secondary to haemolytic uraemic syndrome

Case contributed by Dr Anne Marie McLellan

Presentation

An 18 month old male presented to the ER with hematochezia. He was diagnosed with thrombocytopenia and worsening renal failure. After two days he began having increased abdominal distention.

Patient Data

Age: 18 month old
Gender: Male
CT

Oral and IV Contrast CT Performed

The constellation of findings resulting from hemolytic uremic syndrome includes multiple organs with pleural effusions, peritoneal fluid, pancreatic necrosis,  renal cortical necrosis, and gastric and bowel wall hemorrhage/thickening.

Case Discussion

 

Hemolytic uremic syndrome (HUS) is an uncommon primarily pediatric disease most closely associated with Shigella toxin ecoli  0157:H7  (STEC)1,2 .  However non 0157:H7 types have been implicated in at least 43% of  394 patients with HUS from a prospective study by Gerber in 2002 from Germany and Austria 3.  Shigella has also been associated with HUS  4. Symptoms begin with diarrhea followed by anuria and hypertension from acute kidney injury (AKI) 1,2.  Proinflammatory cytokines such as Interluekin-1 beta (IL-1B) and tumor necrosis factor – alpha (TNF alpha) produce endothelial damage leading to a thrombotic microangiopathy (TMA). This causes endothelial swelling and end organ damage most frequently in the glomerulus resulting in AKI and within the bowel walls with resultant ischemic colitis and bowel wall edema2,5. End organ hypoxia and ischemia are reported as occurring in the liver (40%), pancreas and brain (20%), heart/lung/other (<1%)6. Despite this pancreatitis only occurs in approximately 2% of HUS cases7. The reported number cases of pancreatitis leading to necrosis with HUS is small8,9. Argyle in 1990 reported a case of Pancreatic Islet cell necrosis on autopsy 10. In 1997 Bendel-Stenzel reported two cases of Pancreatic Necrosis secondary to HUS requiring simultaneous pancreatic and kidney transplants (SPKT)11. In the opposite scenario there have been rare cases of SPKT who have then developed HUS and resultant pancreatitis successfully treated with Tacrolimus12.  Despite this reporting there are few if any documented images of pediatric pancreatic ischemia or necrosis secondary to HUS in our literature search.

We report a case of an 18-month-old male with a history of a gastrointestinal infection who went on to develop anuria and hypertension from AKI. He was diagnosed with HUS and required dialysis. Then on day five he began to develop worsening abdominal pain and thrombocytopenia. An oral and IV contrast chest abdomen and pelvis was performed demonstrating multisystem organ involvement with pleural effusions, pancreatic edema and hypoperfusion which appeared like necrosis, ascites, bowel wall thickening and hypoperfused renal cortices consistent with renal cortical necrosis (Figures 1 & 2).

His amylase and lipase were severely elevated.  He developed diabetes and required dialysis. He had severe thrombocytopenia requiring platelets. A CT one week later demonstrated the pleural effusions had increased, the pancreatic body necrosis had spread to involve the pancreatic head, he had worsening bilateral renal cortical necrosis and bowel wall hemorrhage/thickening (Figures 3,4,5). After supportive care at approximately one month, this patient made a complete recovery. His  renal function returned to normal and he did not require dialysis. His amylase and lipase normalized as did his blood sugars. He did not require treatment for diabetes.  The importance of this case is to remember the association of HUS with pancreatic necrosis and that in some cases the CT appearance of necrosis can resolve and the patient can have return of a normal appearance of the pancreas. On follow up this 18-month-old boy had an ultrasound at one month that demonstrated a normal sonographic appearance to the pancreas (not shown).

 

 

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Case information

rID: 32361
Case created: 24th Nov 2014
Last edited: 3rd Oct 2015
Inclusion in quiz mode: Included

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