Portal Pyaemia as a Presenting Feature of Paediatric Crohn Disease : Journal of Pediatric Gastroenterology and Nutrition

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Case Reports

Portal Pyaemia as a Presenting Feature of Paediatric Crohn Disease

El-Matary, W.; Jaffray, B.; Scott, J.; Hodges, S.

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Journal of Pediatric Gastroenterology and Nutrition 43(2):p 260-262, August 2006. | DOI: 10.1097/01.mpg.0000221900.38286.17
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INTRODUCTION

Thromboembolic complications of inflammatory bowel diseases (IBD) are well recognised and associated with a high mortality (1). Portal vein thrombosis is a rare and serious complication especially when associated with sepsis (portal pylephlebitis). Whilst it is a well-recognised complication of Crohn disease in adults, only in 1 case has it been reported as the presenting feature (2). In paediatric practice, there is only 1 case report of this complication in an 18-year-old patient 8 years after diagnosis.

We present a 14-year-old girl who presented to our unit with sepsis, jaundice and massive weight loss. Septic thrombophlebitis of the portal vein was diagnosed by abdominal computerised tomography (CT). Further investigation demonstrated she had Crohn disease. She was successfully treated with antibiotics, anticoagulants and surgical resection of affected bowel. To our knowledge, this is the first reported child who has Crohn disease presenting with portal vein pylephlebitis.

CASE REPORT

A 14-year-old girl presented having been unwell for 3 months. Her initial symptoms were those of depression with associated anorexia and weight loss. Three weeks before admission, she started having rigors (these were perceived by her family to be panic attacks because there was a history of school bullying); and 2 days before admission, she became jaundiced. She denied any bowel symptoms.

On examination, the patient was extremely unwell. Her weight was below 0.4th percentile and height on 9th percentile. She was jaundiced with loss of muscle bulk. She was covered with diffuse bruises and purpuric spots. There was clubbing of both fingers and toes, lower limb pitting oedema, gross ascites and hepatomegaly with a liver span of 23 cm. The spleen was not palpable.

Initial investigations demonstrated a hypochromic microcytic anaemia with an elevated white blood cell count and a low platelet count (haemoglobin 9.1 g/dL, white blood cell count 16.3 × 109/L, neutrophils 13.8 × 109/L, platelets 43 × 109/L). Her clotting was abnormal, with evidence of disseminated intravascular coagulation [prothrombin time 22 seconds, normal activated partial thromboplastin time, dimerized plasmin fragment D 3.3 mg/L (0-0.3) and fibrinogen 0.9 g/L (1.5-4)]. Her liver function tests were consistent with a sepsis-related cholangitis [total serum bilirubin 168 μmol/L (conjugated fraction 84 μmol/L), alanine transferase 97 IU/L, gamma-glutamyl transpeptidase 56 IU/L, normal alkaline phosphatase, low serum albumin 14 g/L]. C-reactive protein was elevated at 149 mg/L (normal value, less than 5 mg/L). Other investigations for liver disease excluded viral and autoimmune hepatitis, alpha 1 antitrypsin deficiency and Wilson disease. Results of investigations for a hypercoagulable state were negative. Escherichia coli, sensitive to meropenem, was isolated from blood cultures.

An ultrasound of her abdomen showed an enlarged liver with diffuse abnormal echogenicity but no evidence of a focal lesion. Initially, there was felt to be a pelvic mass, and therefore, an abdominal CT was performed. This showed a portal vein thrombosis that contained gas locules, the thrombus extending into the intrahepatic branches of the portal vein and the superior mesenteric vein. There was generalised thickening of the wall of the large and small bowel and dilatation of the caecum and ascending colon (Fig. 1a and b). There was no evidence of bowel perforation, infarction or abnormal abdominal masses or collections. The result of a white cell scan was negative. She was initially treated with intravenous meropenem and gentamicin, albumen infusions with diuretics and vitamin K. She was heparinised (target activated partial thromboplastin time twice normal) and had total parenteral nutrition via a central venous catheter. Her temperature settled quickly.

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FIG. 1:
A, CT of the abdomen with intravenous contrast shows the superior mesenteric vein being partially thrombosed and containing gas locules (vertical arrow). The bowel wall is thickened and dilated (transverse arrow). B, CT of the abdomen with intravenous contrast shows thrombus and gas extending into the intrahepatic branches of the portal vein (transverse arrows).

The cause of her portal vein thrombosis, in view of the previous history, was considered to be either the consequence of an appendix abscess or Crohn disease. Three weeks after admission, it was therefore elected for her to undergo laparoscopy and proceed to a lapararotomy if necessary. At laparotomy, she was found to have a thickened appendix, fistulating into the duodenum. The terminal ileum was grossly thickened, with a tight fibrosing ileal stricture and upstream dilatation. There were areas of thickened jejunum. The diseased ileal loops and right colon were resected and a wedge liver biopsy was performed.

The histology of the resected bowel was entirely in keeping with Crohn disease. The liver biopsy showed mild chronic inflammation with ductular proliferation and moderate macrovesicular steatosis.

Once she had recovered from the laparotomy, she was started on nasogastric elemental feeds (EO28 Scientific Hospital Supply, SHS International Ltd., Liverpool, UK). Intravenous meropenem and gentamicin were continued for 6 weeks in total and then she had a further 6 weeks of oral amoxicillin. She was switched from intravenous heparin to warfarin (target international normalised ratio of 2) and this was continued for 6 months. Azothioprine 2 mg/kg/d was started.

Six months after her operation, she was well. Her weight was on the 25th percentile, her liver was not clinically enlarged and her inflammatory parameters and liver function tests were normal.

A follow-up magnetic resonance imaging of her abdomen with flow sensitive sequences demonstrated clearance of thrombus from the main portal vein. There was mild atrophy of the left lobe of the liver resulting from previous left portal vein thrombosis (Fig. 2).

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FIG. 2:
Axial fast imaging with steady-state precession flow sensitive magnetic resonance imaging image without intravenous contrast shows flow within main portal vein (arrow) and mild atrophy of the left lobe of liver.

DISCUSSION

Portal vein thrombosis may be triggered by a variety of conditions including cirrhosis of the liver, malignant tumours, coagulation disorders, myeloproliferative diseases and trauma (4). When combined with suppuration (ie, portal pylephlebitis), it normally results from ascending infection from the gastrointestinal tract into the portal vein. Although rare, this event may occur in association with diverticulitis, appendicitis, IBD, biliary tract infections, acute necrotising pancreatitis, infection after abdominal surgery and generalised sepsis, and from an unknown origin (2-9).

Whilst rare, portal pylephlebitis is often a fatal complication of IBD. It can be attributed to changes of coagulation factors induced by inflammation or steroid therapy (4,10).

Talbot et al. identified 3 risk factors to be associated with thrombotic tendency: active bowel disease, prior abdominal surgery and sepsis (1). A hypercoagulable state was excluded in the patient described and she was taking no steroids. As in previously described cases, E. coli was the causative organism (3,8). Infection with Streptococcus intermedius and S. milleri has also been described (4,11). These bacterial species are all normal inhabitants of the gastrointestinal tract and are presumably translocated through the damaged mucosa of the gut (in our case, terminal ileum) to the portal circulation, inducing thrombogenesis. This might explain why some of the reported cases responded to treatment with antibiotics only, anticoagulants and thrombolytic agents being contraindicated because of the risk of bleeding (3). Nevertheless, it has been suggested, even with the lack of controlled studies, that thrombolysis may be a valuable approach in life-threatening portal vein thrombosis because it is less traumatic than operative procedures and more effective than anticoagulant (4). In this case, we have demonstrated resolution of the thrombosis with a prolonged course of antibiotics and 6 months of anticoagulation.

It is difficult however to specify the duration of the antibiotic or anticoagulant therapy in view of lack of controlled studies. Reports have ranged from 3 weeks (3) to 6 months (2). The prolonged course of antibiotics was suggested on the assumption of difficult penetration of antibiotics into the infected thrombus (2).

In conclusion, we describe what we believe to be the first paediatric case of Crohn disease presenting with portal pylephlebitis. The patient was successfully treated with a combination of antibiotics, anticoagulants and surgical resection of the affected bowel.

REFERENCES

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© 2006 Lippincott Williams & Wilkins, Inc.