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Case Report

Submitted: 24 Oct 2012Accepted: 7 Jan 2013

Hepatomegaly and Periportal Oedema of the Liver in a Patient with Eosinophilic Gastroenteritis

Chee-Kin Hui1,2

1 Centre for Alimentary Studies, Hong Kong SAR, China

2 Quality Healthcare Medical Services, Rm 601-605, HK Pacific Centre, 28 Hankow Road, Tsim Sha Tsui, Hong Kong SAR, China

Abstract Periportal halos are an uncommon finding on computerised tomography (CT) of theliver.Here, reported a case of periportal halos and hepatomegaly in a patientwith eosinophilicgastroenteritis.A49-year-oldmalepresentedwithasixweekhistoryofrightlowerquadrantpainand diarrhoea. A CT of the abdomen showed hepatomegaly and multiple hypodense periportalhalos around the patent portal veins consistent with periportal oedema. A colonoscopy showednormal looking mucosa in the colon and terminal ileum. Blind biopsies taken throughout theterminalileumandcolonshowedincreasednumbersofeosinophils(morethan25perhigh-powerfield) consistent with eosinophilic gastroenteritis. A liver biopsy showed minimal non-specificchronicinflammatoryinfiltratesandeosinophilsintheportaltractswithductularproliferation.Inconclusion,eosinophilicgastroenteritisshouldbeconsideredinpatientspresentingwithperiportalhalos,hepatomegaly,anddiarrhoea.

Keywords: periportal, oedema, hepatomegaly, eosinophilic gastroenteritis, diarrhoea, corticosteroid

Introduction

Periportalhalosareanuncommonfindingoncomputerised tomography (CT)of the liver.Thecauseofthesehalosisprobablyanaccumulationoffluidaroundthesurroundingperiportalregions.These periportal halos are typical of periportaloedemaandcanbeseeninpatientswithtrauma,congestive heart failure, pericardial tamponade,venous occlusion after haemotopoietic stemcell transplantation, acute hepatitis, primarysclerosing cholangitis or tumours in the portahepatis resulting in obstruction of lymphaticdrainage(1,2).Acasereportofperiportalhalosinapatientwitheosinophilicgastroenteritis.

Case Report

A 49-year-old male presented with a sixweek history of right lower quadrant pain anddiarrhoea 10 times a day. He had good pasthealth, was a non-smoker, non-alcoholic, andhad not taken any drugs, Western or herbal,in the last six months. Physical examinationshowed tenderness over the right lowerquadrant, but there was no evidence ofrebound or rigidity. Liver biochemistry showedelevated serum alanine aminotransaminase

110 U/L (normal range 5–53 U/L), aspartateaminotransaminase 235 U/L (normal range14–64 U/L), alkaline phosphatase 155 U/L(normalrange30–90U/L),andgammaglutamyltranspeptidase180U/L(normalrange<84U/L).The patient’s albumin, globulin, and bilirubinwerenormal.Acompletebloodcount,erythrocytesedimentation rate, C-reactive protein,immunoglobulinE, peripheral eosinophil count,andrenalbiochemistrywereallnormal.HepatitisA,B,C,andEmarkerswereallnegative.Repeatedstoolsamplesforparasiteswereallnegative. ACToftheabdomenshowedhepatomegalywith the liver measuring 14.8 cm in length atthe mid-clavicular line. Multiple hypodenseperiportal halos were noted around the patentportal veins consistent with periportal oedema(Figure1a,1b).Thehepaticandportalveinswerepatent.Nofocalmasswasseenintheliverorintherestoftheabdomen. A colonoscopy showed normal lookingmucosa in the colon and terminal ileum. Blindbiopsies taken throughout the terminal ileumandcolonshowednormalglandulararchitecture.However, the lamina propria and submucosalregionsattheterminalileum,caecum,ascendingcolon, transverse colon, and descending colonshowed increasednumbersofeosinophils (more

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than 25 per high-power field) consistent witheosinophilicgastroenteritis. Magnetic resonance cholangiopancrea-tography (MRCP) four days after the CT wasnormal, and there was no evidence of primarysclerosing cholangitis or ductal obstruction. Aliver biopsy was performed to determine thecause of the hepatomegaly and periportalhalos. It revealed minimal non-specific chronicinflammatory infiltrates in the portal tracts,with ductular proliferation. Eosinophils wereidentified,andthe limitingplatewas intact.Thehepatic lobules showed mild macrovesicularfatty change and glycogenation of nuclei.Occasional foci of mild intrahepatic cholestasisand haemosiderosis were noted. There wasalso no evidence of liver cell apoptosis,granulomatous inflammation, dysplasia, ormalignancy. No malignancy was found on CT of theabdomen and the pelvis, the MRCP, upperendoscopy, colonoscopy, or a chest X-ray.Therewasnoevidenceofparasite infestation inrepeated stool samples. Serological tests forTrichinella spiralis, Wuchereria bancrofti, Toxocara canis, Schistosoma,andEchinococcus wereallnegative.

In view of the severity of the patient’sdiarrhoeaandtheextra-intestinalinvolvementoftheeosinophilicgastroenteritis,anelementaldiet,systemiccorticosteroid(0.5mg/kg/day),ketotifen,cetrizine, and monteleukast were prescribedfor the eosinophilic gastroenteritis. Resolutionof the patient’s diarrhoea and abdominal painwas achieved after one week of therapy. Thepatient’s alanine aminotransaminase, aspartateaminotransaminase, and alkaline phosphatasenormalised after two weeks of the therapy. Arepeat CT of the abdomen three weeks afterthe initiation of the therapy showed completeresolution of the periportal halos, and the liverspanhadalsoreturnedtonormal. The systemic corticosteroid was slowlydecreased by 5 mg every two weeks after therepeat CT scan confirmed the resolution ofthe periportal halos. The patient received thesystemicsteroidforatotalof13weeks.Cetrizineand ketotifen were discontinued after the CTscan confirmed the resolution of the periportalhalos,andthepatientwasmaintainedthereafteron monteleukast only and an elemental diet.In 18 month follow-up, the patient showed norecurrence of diarrhoea or symptoms while onmonteleukastandtheelementaldiet.

Figure 1: (a) Computerised tomography of the liver showing periportal halos typical ofperiportal oedema without contrast enhancement, and (b) periportal halos oncomputerisedtomographyaftercontrastinjection.

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Discussion

Eosinophilic gastroenteritis was firstdescribed in the 1930s (3). Eosinophilicgastroenteritis isanuncommondisease,withanestimatedincidencearound28per105/year(4).Itcanaffectanypartofthegastrointestinaltract. Eosinophilic gastroenteritis can be dividedinto three types: mucosal, subserosal, andmuscular. Mucosal disease usually results inprotein-losing enteropathy or malabsorption;thesubserosalformcangiverisetoperitonitisorascites. Themuscular form, which is associatedwith involvementof themuscle layer,canresultin thickening of the bowel wall and intestinalobstruction(5). Thecolon(88%)andtheileum(72%)arethecommonest sites of involvement in eosinophilicgastroenteritis (5). Seventy percent of thosewith eosinophilic gastroenteritis will have atleast two segments of the intestine affected(5). Eosinophilic gastroenteritis has also beenreported to be associated with extra-intestinalinvolvement such as transmural eosinophiliccholecystocholangitis and acute pancreatitis(6,7).However,tothebestofourknowledge,thisis the first report of eosinophilic gastroenteritiswithhepaticinvolvement. The eosinophilic gastroenteritis in thispatient was complicated by hepatitis, periportalhalosandhepatomegaly.Thehepatitiswasmostlikely due to an inflammatory response in theliversecondarytotheeosinophilicgastroenteritis.Theeosinophilicgastroenteritismayhavecausedinflammatory cell infiltration into the portalareas and ductular proliferation, with resultantperiportalhalos. DilatedlymphaticsandlymphoedemaonCTscans have beendescribed in both intra-hepaticand extra-hepatic diseases (1,2). These dilatedlymphaticsandlymphoedemaareduetoalteredhepatic lymphatics and appear as periportalhalosonCT.Theseperiportalhalosmayrepresenttheaccumulationofdilatedlymphaticsorfluidinthe interstitial space around the portal vein oraroundtheportaltriads(1,2). Periportal halos on CT scans have alsobeen reported in patients with acute hepatitis(8,9).InastudybyCakiretal.(9),patientswithacute hepatitis with periportal halos on CT hadinflammatory infiltrates in theportal tractswithductular proliferation and periportal halos inliver biopsy. These findings are similar to thehistological findings in this case report. Similartothecasesofacutehepatitis(9), theperiportalhalos resolved completely in this patient

following the successful treatment of theeosinophilicgastroenteritis. Theincidenceofeosinophilicgastroenteritishas been increasing in the last 16 years (4).However, due to its low incidence, there is adearth of large prospective randomised trialson the treatment of this condition (4,5,10).Therefore,atpresent,thereisalackofconsensuson the optimal treatment of eosinophilicgastroenteritis. Currently, treatment for thisconditionincludesallergyavoidance,anelementaldiet, topical corticosteroids and/or systemicglucocorticoids(10). Someexpertshavesuggestedthatcliniciansshould initially consider allergy avoidance andthat topical glucocorticoid should be initiatedif allergy avoidance fails to improve symptoms(10). If there is no response to the topicalglucocorticoid, then systemic glucocorticoidshould be considered (10). They have alsorecommendedanelementaldietwith theaimofavoidingproteinantigenexposure(10). In view of the extra-intestinal involvementof the eosinophilic gastroenteritis and theseverity of the patient’s diarrhoea, systemicsteroid, monteleukast, ketotifen and cetrizinewere prescribed with the objective of inducingremission as soon as possible. Systemiccorticosteroid was commenced upon thediagnosis because its’ use has been found torapidly improve eosinophilic gastroenteritiswithextra-intestinalinvolvement(6,7). Therewasnorecurrenceoftheeosinophilicgastroenteritis in the 18 month follow-up. It isuncertainwhetherthiswasduetotheelementaldiet, monteleukast or the natural history ofeosinophilic gastroenteritis. As reported earlier,42% of those with eosinophilic gastroenteritiswill experience only one single episode withoutanyrecurrence (5).More randomisedcontrolledstudies on the use of an elemental diet inmanaging eosinophilic gastroenteritis should beconductedtodetermineitsefficacy.

Conclusion

In conclusion, eosinophilic gastroenteritisshouldbeconsideredinpatientspresentingwithperiportal halos, hepatomegaly and diarrhoea.The periportal oedema and hepatomegaly mayresolve following successful treatment of theeosinophilicgastroenteritis.

Acknowledgement

None.

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Conflict of Interest

None.

Funds

None.

Correspondence

DrChee-KinHuiMD(HK),MBBS(HK),MRCP(UK),FHKCP,FHKAM(Medicine)Rm601-605HKPacificCentre28HankowRoadTsimShaTsuiHongKongSAR,ChinaTel:+852-27231183Fax:+852-27236620E-mail:bckhui@gmail.com

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