Here we report the case of a 4-year-old male with severe acute pancreatitis due to hyperlipidemia, who presented with abdominal pain, metabolic abnormalities, and colonic necrosis. This colonic complication was secondary to the extension of a large peripancreatic fluid collection causing direct serosal autodigestion by pancreatic enzymes. Two weeks following the initial presentation, the peripancreatic fluid collection developed into a mature pancreatic pseudocyst, which was percutaneously drained. To our knowledge, this is the youngest documented pediatric case of colonic necrosis due to severe pancreatitis and the first descriptive pediatric case of a colonic complication due to hyperlipidemia-induced acute pancreatitis.
Colonic complications of pancreatitis, that is, necrosis, fistulae, and strictures, are uncommon in adults but are rarely reported in children [
A 4-year-old male with no known medical problems presented to a local emergency room with a 3-day history of decreased oral intake, nausea, nonbloody, nonbilious emesis, and abdominal pain. Laboratory assessment revealed lipemic serum with triglycerides of 7449 mg/dL (normal 30–149 mg/dL), anemia (hematocrit 19%), hyponatremia (sodium 129 mEq/L), metabolic acidosis (carbon dioxide 12 mEq/L), azotemia (blood urea nitrogen 73 mg/dL, creatinine 1.8 mg/dL), amylase 248 IU/L (normal 28–100 IU/L), and lipase 360 U/L (normal 13–60 U/L). A contrast abdominal CT scan demonstrated a large retroperitoneal mass appearing to arise from the left adrenal gland with normal pancreatic appearance. The patient was transferred to the University of Chicago Pediatric Intensive Care Unit for further management and diagnostic workup.
On admission the patient was febrile (39.4°C) and tachycardic (132 beats/min) with weight, height, and BMI at the 83rd, 33rd, and 97th percentiles, respectively. On physical exam his abdomen was distended with hypoactive bowel sounds and a palpable mass in the left upper quadrant; no xanthomata or lipemia retinalis was noted. The laboratory findings were comparable to the previous with the addition of amylase 79 U/L (normal 28–100 U/L), lipase 99 U/L (normal 11–65 U/L), and fasting triglycerides 6060 mg/dL.
Due to concern for malignancy, the patient underwent an extensive workup to rule out solid tumors. On repeat chest/abdominal CT scan, a diffusely enlarged pancreas without areas of necrosis or calcifications was noted. Additionally, the mass was described as a large infiltrative low density lesion extending from the left retroperitoneum around the pancreas, bilateral renal spaces, down to the perirectal area without obvious metastatic lesions within the lungs or abdomen (Figure
Abdominal CT (coronal view): 10 cm × 8.4 cm left-sided peritoneal mass (broad arrow) with significant inflammation of the pancreas (thin arrow).
We concluded that the patient had undiagnosed type I familial hypertriglyceridemia, presenting initially as chylomicronemia syndrome, resulting in acute pancreatitis with a large peripancreatic fluid accumulation causing colonic necrosis due to direct pancreatic enzyme serosal digestion. The patient was initially managed with total parenteral nutrition and kept NPO (nil per os) with subsequent advancement to a low-fat diet and colonic reanastomosis following a 3-month period. To date his hypertriglyceridemia has been controlled with a low-fat diet supplemented with omega-3 fatty acid ethyl esters.
Colonic complications, such as necrosis, fistulae, and strictures, have been reported in 15% of adults with severe pancreatitis [
In adults, pancreatitis associated colonic necrosis may be diagnosed at a median of 25 (range 1–55) days following symptomatic onset and has a mortality rate of 54% [
The association of hyperlipidemia and acute pancreatitis has been well described and accounts for ~6% of acute pancreatitis episodes [
Chylomicronemia syndrome, as identified in our patient, is defined as a plasma triglyceride level > 1000 mg/dL accompanied by one or more of the following: eruptive xanthoma, lipemia retinalis, abdominal pain, acute pancreatitis, and/or hepatosplenomegaly [
Although colonic complications are far more prevalent in the adult population, pediatricians need to be aware that they can occur in children with pancreatitis. Additionally, since the association between hyperlipidemia and pancreatitis is well established, it is important for clinicians to consider hyperlipidemic pancreatitis and its complications when faced with lactescent serum samples (and/or fasting serum triglyceride levels > 1000–2000 mg/dL) and abdominal pain even in the presence of normal or mildly elevated serum amylase and lipase. It is known that lipemia commonly interferes with the processing methods of various laboratory tests, potentially rendering test results inaccurate and difficult to interpret [
To our knowledge, this is the first and youngest pediatric report of colonic necrosis due to direct enzymatic contact with a peripancreatic fluid collection in acute pancreatitis secondary to hyperlipidemia. Several key features of this case should be noted. First, the patient presented with extremely high triglyceride levels although there was no significant history of hyperlipidemic symptoms. Second, absence of significantly elevated serum amylase and lipase in the setting of hyperlipemic serum may obscure the diagnosis of pancreatitis [
All stated authors have no financial relationships relevant to this paper to disclose.
All stated authors have no conflict of interests to disclose.