Fitz-Hugh-Curtis syndrome resulting in nutmeg liver on computed tomography.

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Fitz-Hugh-Curtis syndrome resulting in nutmeg liver on computed tomography.

Boer, J.P.; Verpalen, Inez M.; Gabriels, Ruben; de Haan, H. ; Meijssen, M.A.C. ;

Bloembergen, P.; Meier, M.

Published in:

Radiology Case Reports

DOI:

10.1016/j.radcr.2019.04.008

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Citation for published version (APA):

Boer, J. P., Verpalen, I. M., Gabriels, R., de Haan, H., Meijssen, M. A. C., Bloembergen, P., & Meier, M.

(2019). Fitz-Hugh-Curtis syndrome resulting in nutmeg liver on computed tomography. Radiology Case

Reports, 14(8), 930-933. https://doi.org/10.1016/j.radcr.2019.04.008

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Case

Report

Fitz-Hugh-Curtis

syndrome

resulting

in

nutmeg

liver

on

computed

tomography

Jolien

P. de

Boer,

MD

a, ∗

_,

_Inez

_M.

_Verpalen,

_MD

b

_,

_Ruben

_Y.

_Gabriëls,

_MD

c

_,

_Harm

_de

Haan,

MD,

PhD

a

_,

_Maarten

_Meijssen,

_MD,

_PhD

c

_,

_Peter

_Bloembergen,

_MD,

_PhD

d

_,

Mark

Meier,

MD

b

a_Department_of_Gynaecology,_Isala_hospital,_Dokter_van_Heesweg_2,_Zwolle₈₀₂₅_AB,_the_Netherlands b_Department_of_Radiology,_Isala_hospital,_Zwolle,_the_Netherlands

c_Department_of_{Gastroenterology,}_Isala_hospital,_Zwolle,_the_Netherlands

d_Laboratory_of_Medical_Microbiology_and_Infectious_Diseases,_Isala_hospital,_Zwolle,_the_Netherlands

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received3March2019 Revised1April2019 Accepted7April2019 Availableonline24May2019

Keywords:

Fitz-Hugh-Curtissyndrome Perihepatitis

Hepatomegaly Nutmegliver

PelvicInflammatorydisease

Chlamydiatrachomatis

a

b

s

t

r

a

c

t

A34-year-oldwomanenteredtheemergencyroomwithabdominalpainintheright up-perquadrant.Computedtomographyscanshowedanutmegliversuspectedforincreased venouspressurebythrombosisoftheliverveins,Budd-Chiarimalformation,orright-sided heartfailure.Interestingly,thediagnosiswaspelvicinflammatorydiseasecomplicatedby theFitz-Hugh-Curtissyndrome(FHCS).

PelvicinflammatorydiseaseresultedfromanascendedinfectionbyChlamydia trachoma-tis.FHCSwascausedbyperihepatitisdefinedasinflammationoftheperitonealcapsuleof theliver.Fastdiagnosisandtreatmentiscrucial.Therefore,wereportacaseofFHCS char-acterizedbyanutmegliveroncomputedtomography.

Case

report

A34-year-oldfemalepresentedatouremergencyroomwith rightupperquadrantabdominalpainfor5daysandpainful respiration.Herhistoryreports2normalvaginalbirths(3and

Abbreviations:FHCS,Fitz-Hugh-Curtissyndrome;GP,generalpractitioner;IUD,intrauterinedevice;PID,pelvicinflammatorydisease. CompetingInterests:Nograntorfinancialsupportwasusedforthiscasereport.Noauthorhadanyfinancialinterestinthesubjectmatter

discussedinthesubmittedmanuscript.Noconflictofinterestneedstobedisclosed.Allauthorsstatethatthisstudycomplieswiththe DeclarationofHelsinki.

∗_{Corresponding}_author_.

E-mailaddress:j.deboer5@mzh.nl(J.P.deBoer).

5yearsago),abdominalwallsurgery,andarecentknee opera-tion.Shewasanonsmokeranddrank2unitsalcoholaweek. Socialanamnesisreportsahighsocioeconomicstatus,a full-timejob,andamarriagefor14years.Amonthago,the gen-eralpractitionertriedtoplaceanintrauterinedevicefor con-traception,butwasunsuccessful.Shewastreatedforpossible

https://doi.org/10.1016/j.radcr.2019.04.008

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Fig.1– Contrast-enhancedCTscanoftheenlargedliverinaxial,coronal,andsagittaldimension(righttoleft)demonstrates hepaticparenchymalattenuationdifferences,alsocalleda“nutmegliver.”

cystitiswithnitrofurantoinfor4dayspriortoherpresenting symptoms.

Thepainwassharpandprogressivesincethefirstday, ag-gravated byrespirationand coughing.There were no com-plaintsofnausea,vomiting,dysuria,anddefecation.Before startingtheoralcontraceptive,shehadaregularcycleof28 dayswithamenstrualperiodof5days.Therewasno irregu-larbloodlossordyspareunia.

The patient was hemodynamically stable with a blood pressureofRR130/97 mmHg,temperature of37.7°C,and a pulseof74beats/min.Duringphysicalexamabdominal pal-pationwasextremelypainful,inparticularatthelevelofthe rightupperquadrant.Hepatomegalywasdiagnosedclinically bypercussion.Therewerenosignsofsplenomegaly.

Onthefirstdayofpresentation,laboratorydatasuggested aninflammatoryprocess.AnX-rayofthechestshowed nor-mallungs,anelevatedpositionofthediaphragm,and mini-malfreefluid.Anultrasoundoftheupperabdomenshowed hepatomegaly resulting ina displaced pancreas. The

diag-Fig.2– Earlyphasecontrast-enhancedCTscanoftheliver

showinghyperintenseenhancementoftheanteriorhepatic

capsuleoftherightlobe.

nosishepatitiswasconsidered,althoughliverfunctiontests were normal.ShewasadmittedtotheDepartmentof Gas-troenterology.

Onthesecondday,laboratoryresultsshowedanincreased levelofD-dimers(920μg/L).Thepatientwasalsoexperiencing chestpain,whilebreathingthereforeacomputedtomography (CT)pulmonaryangiographywasperformedtoexclude pul-monaryveinembolism.Noabnormalitieswereseenonthis CTpulmonaryangiography.Asecondultrasoundwasmade because symptomsworsened.Nonewfeatures werefound onthe ultrasound.Thegynecologist wasconsultedand ex-aminedthepatient.Thevaginalultrasoundshowedanormal uterusandadnexalregions.Avaginalswabwasperformedto testforbyChlamydiatrachomatisandNeisseriagonorrhoeae. Fur-therimagingwasnotindicatedatthattimeastheabdominal painhasreduced.Thepatientwasdischargedwith instruc-tionstoseekmedicaladviceifsymptomsworsened.

Shereturnedonday4becauseofthesevereprogressive pain in the right upperquadrant. Acontrast-enhanced CT scanoftheabdomenwasperformedbecauseofthe contin-uous increasedinfection parameters (C-reactive protein 65 mg/mL),feverof38.6°C,andthelackofadiagnosis.The pre-viously diagnosedhepatomegaly was confirmed. Second,it demonstrated reversibleintrahepaticparenchymal attenua-tiondifferences,alsocalledanutmegliver.Anutmeg appear-anceoftheliverisduetohepaticvenouscongestion.Contrast ispreventedfromdiffusingthroughtheliverinanormal man-ner,whichresultsinareticularormosaicpatternofcontrast enhancementintheearlyportalvenousphase.Thisspecific findingishighlysuspiciousforincreasedvenouspressureby thrombosisoftheliverveins,Budd-Chiarisyndrome,or right-sidedheartfailure(Fig.1).

Interestingly,laboratoryfindingsshowedanincreasedlevel ofN-terminalprob-typenatriureticpeptideof238pg/mL sus-pectedforright-sidedheartfailure.Thecardiologistwas con-sulted.Anelectrocardiogramwasconductedandnosignsof heartfailurewerefound.

ReevaluationoftheCTscanrevealedenhancementofthe hepaticcapsuleoftheleft lobe(Fig.2).Thisspecificfinding

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issuggestiveofFitz-Hugh-Curtissyndrome(FHCS),whichisa complicationofmoreadvancedpelvicinflammatorydisease (PID).Additionally,pelvicfatinflammationandfreefluidwas present.

A positive test for C trachomatis was found later as a causativeorganismforPIDcomplicatedbytheFHCS. Doxycy-cline100mgwasdirectlysubscribedtwiceadayfor14days. Thepatientwasdischargedand6weekslatershewasseenin agoodclinicalconditionattheoutpatientdepartment.

Discussion

AChlamydiainfectionisassociatedwithawidespectrumof uppergenitaltractpathologies,rangingfromasymptomatic endometritis to symptomatic salpingitis, tubo-ovarian ab-scess,peritonitis,andlong-termsequelaesuchasinfertility, ectopicpregnancy,andchronicpelvicpain[1–4].

FHCS ischaracterized byacuteperihepatitissecondarily toPIDand classicallypresentswith suddenonset ofsharp rightupperquadrant abdominalpain [5].Ctrachomatis and

NgonorrhoeaeinfectionsareusuallyresponsibleforFHCS, al-though30%-40% ofcasesarepolymicrobial[6].The inflam-mationmechanism oftheperitoneal capsuleoftheliveris believed toresultfromascendinginfection from thepelvic cavity.Bacteriaspreadbymeansofdirectextensionalongthe rightparacolicgutterorthroughthelymphaticsystem, caus-inginflammationoftherightupperquadrantperitoneal sur-facesoftheliver.

Irritationofthehepaticcapsuleorperitoneumbythe accu-mulatedinflammatoryexudatescausesthesharprightupper quadrantabdominalpain.Thispainisaggravatedwithdeep respirationorcoughingasaresultofnegativepressureduring respiratorymovements.Otherrelatedsymptomsarenausea, vomiting,fever,andmalaise.Fever,lowerabdominalpain,and changedvaginaldischargeareoftenseeninpatientswithPID and/orFHCS.

FHCScan bemisdiagnosedasit maypresent like many otherdisordersincludingpulmonaryembolism,pneumonia, cholecystitis,renalcolic,andperforatedulcer[5].Whena pa-tientpresentswithnonspecificsymptomsonly,thediagnosis isevenmoredifficult.

Inretrospect,uncertaintyinthediagnosticprocess com-binedwiththesevereabdominalpainofthepatientresulted inoverutilizationofmedicalstudies.Importantly,theoveruse ofdiagnosticimagingleadedtoanincreaseinthenumberof diagnosticerrorsandresultedinhighcosts.TheCTfindingof thenutmegliverwassuspiciousforotherdiseasesdiverging fromthecorrectfinaldiagnosis.FHCSisnotinthedifferential diagnosisofthisspecificimagingfinding.Therefore,wewill discusstheseimagingfindingsinfurtherdetail.

CTfindingsofearlyPIDaretypicallysubtleinthefemale re-productivetract.Oftentheonlyfindingsarefluidinthepouch ofDouglasandpelvicfatinflammation.Astheinfection pro-gresses,CTfeaturesofPIDincludefluidfilledfallopiantubes withthickwallsandenlargedovarieswithaheterogeneous orpartlycysticappearance[7](Fig.3).Atubo-ovarianabscess representsamoreadvancedstageofPID.

Fig.3– Contrast-enhancedCTscanofthepelvis,which

demonstratesenhancedandthickenedfallopiantubes

suspectedforsalpingitis.Additionally,theovariesare enlarged,inflamed,andhaveacysticappearancedueto earlyoophoritis.Moreover,pelvicfatinflammationandfree fluidisseen.

CTfindingsofperihepatitiswithPIDarerelativelywell de-scribed[8].However,thesestudiesfocusonthespecific find-ingofthelivercapsule,alsoknownasGlisson’scapsule.They reportcapsularenhancementalongthesurfaceoftheliver, mostcommonlyseen onthe right-anterior hepaticsurface

[9].Themechanismofcapsularenhancementseenon early-phaseimagesisthoughttobeduetoincreasedbloodflowat theinflamedhepaticcapsule.Onthecontrary,capsular en-hancementseenondelayedimagesmayreflectearly capsu-larfibrosis[10].Inourcase,hepaticcapsularenhancementon earlyphaseimageswithnormalizationofthecapsuleonthe delayedimagesreflectstheacutepresentationorearlyphase ofFHCS.

Only1casereportdescribesthefindingoftransienthepatic attenuationdifferenceduetohepaticvenousoutflow obstruc-tion[11].Thiscanbecategorizedinto3categoriesaccording tothelevelofobstruction:(1)atthelevelofsinusoidsand ter-minalvenules,(2)fromhepaticveinstothesuperiorendof in-feriorvenacava,and(3)venousobstructionatthelevelofthe heart.Hepaticvenousoutflowobstructionisusuallycaused byhepaticveno-occlusive diseaseafterhematopoieticstem celltransplantation,Budd-Chiarisyndrome,orcardiovascular conditionleadingtoanincreasedcentralvenouspressure[11]. However,inourcase,thesediagnoseswereunlikelybecause therewasnohistoryoftransplant,therewerenosignsof in-creasedcentralvenouspressureandultrasoundshowedflow intheportalvein.

TheCToftheabdomeninourcaseshowedhepatomegaly andmultiplehypodenseperiportalhalosaroundthepatent portal veins consistent with periportal edema (Fig. 4). Al-thoughthe portalvein did notappear tobe compromised inourcase,hepaticvenousoutflowobstructionwasbelieved tobe due to the inflammatory process.The pathophysiol-ogyisprobablyperihepatic andcapsularinflammationthat mayhaveprogressedtoperiportalinfiltrationof inflamma-torycells.DilatedlymphaticsandlymphedemaonCTscans havebeendescribedinbothintrahepaticandextrahepatic

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dis-Fig.4– Azoneoflowattenuationaroundthecentralportal veinsisseen,whichindicatesthatthereisperiportal edemapresent,alsoreferredtoasperiportalhalosign.

eases[12].Theinflammatoryprocessleadstotheformationof periportaledema.Thereisfluidaccumulationordilatationof lymphaticsinthelooseareolartissuearoundtheportal tri-adsorsubsegmentalportalvenousbranches.Thismaycause partialhepaticvenousoutflowobstructionbynarrowingthe hepaticveins,whichinducescongestionatthelevelofthe si-nusoids.Localinflammationisaless-commoncauseof tran-sienthepaticattenuationdifferences;thishowevercancause decreasedparenchymalattenuationoftheliverparenchyma. Therefore,thefindingofanutmegliverinourcasewas believedtobeduetotheinflammationoftheperiportal re-gionssecondarytothe perihepaticandcapsular inflamma-toryprocess.TheCT finding oftransienthepatic perfusion abnormalities in patients withFHCS can bereferred to as the Budd-Chiari phenomenon [11]. Additionally, we found hepatomegalyonultrasoundandCTimaging.Hepatomegaly withoutelevatedliverfunctiontestsisanonspecificsignand hasmultiplecausesincludinginfection,fatinfiltration, hep-aticveinocclusion,andhepaticcongestion.

Conclusion

Insummary,severeabdominalpainintherightupper quad-rantinawomanofreproductiveageshouldraisesuspicion forprogressivePIDknownastheFHCS.Timelydiagnosisand treatment,especiallytoretainfertility,isimportant.

Characteristicfindings onCTscan regardingtothe liver arehepatomegaly,intraparenchymalreversibledynamic per-fusionabnormalitiesandcapsularenhancement.Wereporta caseofFHCSandtheBudd-Chiariphenomenon.This impres-siveCTpresentationshowsanutmegliverappearance,which

wasbelievedtobeduetolocalinflammationofthe peripor-talregionssecondarytotheperihepaticandcapsular inflam-matoryprocess.Hopefully,thiscaseallowsforaccurateand promptdiagnosisinfuturecases.

Supplementary

materials

Supplementary materialassociatedwiththis articlecanbe found,intheonlineversion,atdoi:10.1016/j.radcr.2019.04.008.

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