A case of Type 2 appendiceal diverticulum perforated and a review of the literature

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InternationalJournalofSurgeryCaseReports77(2020)450–453

ContentslistsavailableatScienceDirect

International

Journal

of

Surgery

Case

Reports

jo u r n al hom e p a g e :w w w . c a s e r e p o r t s . c o m

A

case

of

Type

2 appendiceal

diverticulum

perforated

and

a

review

of

the

literature

Michele

Fiordaliso

a,∗

_,

_Antonia

_Flavia

_De

_Marco

b

_,

_Raffaele

_Costantini

c a_Department_of_Surgery,_Erbach_General_Hospital,_Germany

b_Department_of_{Gastroenterology,}_Groß_Umstadt_General_Hospital,_Germany

c_Institute_of_Surgical_Pathology,_Department_of_Medical,_Oral_and_{Biotechnological}_Sciences,_“G._{D’Annunzio”}_University_of_Chieti,_Chieti,_Italy

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received15September2020

Receivedinrevisedform25October2020 Accepted25October2020

Availableonline10November2020

Keywords:

Appendicealdiverticulumperforated Appendicitis

a

b

s

t

r

a

c

t

BACKGROUND:Appendicealdiverticulosisdiseaseisarareentity.Anperforatedappendicealdiverticulosis mimickingacuteappendicitisisaextremelyunusualsurgicalﬁndingandthereportedprevalenceis between0.014and3.7%.

CASEREPORT:Wereportthecaseofanelderlyman,whopresentedwithatypicalclinicalimageof acuteappendicitisandunderwentlaparoscopicsurgery.Intraoperativeanacuteappendicitiswith local-izedperitonitiswasidentiﬁedandalaparoscopicappendectomywasperformed,butpathologicanalysis demonstratedatype2appendicealdiverticulitis.

CONCLUSION:Appendicealdiverticulosisdiseaseshouldbeincludedindifferentialdiagnosisofpatients presentingwithclinicalsignsofanacuteappendicitisandpromptsurgicaltreatmentisessentialinorder toavoidseverecomplications.

1. Introduction

Appendiceal diverticulosis disease is a rare entity. The ﬁrst description waspublishedbythe pathologistKelynackin 1893 [1]. The reported prevalence is between 0.014 and 3.7% [2,3]. The sizeof mostappendiceal diverticulosisisless than 0.5cm. Therefore,theycanbeeasilyneglectedduringmacroscopic exam-ination [4]. Acute appendicitis is the most common appendix disease.Bothacuteappendicitisandappendicealdiverticulosiscan presentwithpainintherightlowerquadrant,whichmakes dif-ferentialdiagnosisdifﬁcult.Thisconditionmaybeassociatedwith neuroendocrinetumours(carcinoids),mucinousadenomas, tubu-lar adenomas and adenocarcinomas[4]. Thereisnoassociation between colondiverticulosis and appendicealdiverticulosis [2]. A caseofperforatedappendiceal diverticulosismimicking acute appendicitisisreported,diagnosedonlyinpostoperativesurgery basedonhistopathologicalfeatures.

2. Casepresentation

Apreviouslyhealthy68-year-oldmanpresentedtothe emer-gencydepartmentbecauseofan5-dayhistoryofbandlikelower abdominalpain.Thepainwasprogressiveinseverityandwas

asso-∗ Correspondingauthorat:Dreikönigstarße47,60594Frankfurt,Germany. E-mailaddresses:michele.ﬁ[email protected](M.Fiordaliso),

ﬂ[email protected](A.F.DeMarco),[email protected](R.Costantini).

ciatedwithconstipationandfever.Physicalexaminationrevealed amanwithatoxicappearanceinmoderatedistresswitha temper-atureof38◦C.

Theabdomenwassoft withbilaterallowerquadrant tender-ness,worseontherightthanontheleftside.Theleukocytecount was14×109_/L_The_computed_tomography_CT_with_intravenous undrectalcontrastshowsthefulllengthofadistendedappendix, appendicealwallthickeningwithenhancement,periappendiceal freefluid,findingsthatindicateappendicitis(Fig.1Fig.1,2). Diver-ticulaofthesigmoidcolonwashighlighted,butwithoutsignsof inflammation.

After the radiological diagnosis the patient was transferred totheoperatingroom.Intraoperativeanacuteappendicitiswith localizedperitonitiswasidentifiedandalaparoscopic appendec-tomy was performed, but pathologic analysis demonstrated a type 2appendiceal diverticulitis;histologically, a single appen-dicealdiverticulumwasidentified,withacuteinflammationand perforation.A neutrophilinfiltration was also identified in the appendicularmucosaasasignofacuteinflammation(Fig.3Fig. 3,4,5,6).Thepatientwastreatedfor6dayswithbroad-spectrum intravenousantibiotics,andherecoveredwithoutcomplications. 3. Discussion

Althoughacuteappendicitisisoneofthemostcommonacute abdominal conditions[12], diverticulosisof the appendix is an uncommonentity[13]. Similartodiverticula occurringinother parts of the intestine, these can be classiﬁed as congenital or

https://doi.org/10.1016/j.ijscr.2020.10.114

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M.Fiordalisoetal. InternationalJournalofSurgeryCaseReports77(2020)450–453

Fig.1,2.Abdominalcomputedtomographydemonstratingenlargedappendixindiameterandperiappendicealfreeﬂuid..

Figs.3,4,5,6.Histologicalsectionofappendicealdiverticulumatthetipoftheappendix.Herniationofappendicealmucosathroughmuscularispropriainanperforated diverticulum..

acquired.Congenitaldiverticulatendtobesingleandlocatedatthe antimesentericmarginoftheappendix,whileacquiredcasestend tobemultipleandlocatedatthemesentericborder,most com-monlyatthedistalthirdoftheappendixandareusuallysmallin size(2–5mm).Severalriskfactorsareassociatedwithacquired appendiceal diverticulosis.Theseincludemale sex,anageolder than30years,andadiagnosisofHirschsprung’sdiseaseorcystic ﬁbrosis.Patientsaretypicallydiagnosedwithcysticﬁbrosisat ado-lescence(onaverageat13years)andhaveuptoa14%incidence of acquired appendicealdiverticulosis [2]. Congenital diverticu-losishasalsobeenassociatedwithotherdiseasessuchasDown syndrome or Patau syndrome [5]. True diverticulosis, which is rarer(incidence0.014%),containsalllayersoftheintestinalwall whileinacquiredcasesitlacksamusclelayer[2,6].Theaverage ageofpatientswithcongenitaldiverticulosisis31yearsandthe averageageofpatientswithacquireddiverticulosisis37years.

Table1

Fourmorphologicaltypesofdiverticulardiseaseoftheappendix[2].

Type1 Appendicealdiverticulitis+normalappendix

Type2 Appendicealdiverticulitis+acuteappendicitis

Type3 Uncomplicateddiverticulum+acuteappendicitis

Type4 Uncomplicateddiverticulum+normalappendix

Multiplediverticulosisispresentinpatientswithacquired diver-ticulosis,whileonlyonediverticulumhasbeenidentifiedinpeople withcongenitaldiverticulosis[2].Appendicealdiverticulosiscan becomplicated by inflammationand morphologicallyclassified intofourtypesdependingonwhethertheinflammatoryprocess involvesthediverticulumand/orappendix(Table1).

In1989,Liptonetal.[8]reportedthemorphological classiﬁ-cationofdiverticulardiseaseofthecaecalappendix.Thereare4

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differenttypes,andtypeI(classical)isthemostfrequent,witha prevalenceofaround40–50%.

Theperforationofacquireddiverticulosisoccursquiteeasily(up to66%ofcases)duetothelackofalayerofthemuscularispropria. Congenitaldiverticulosishaveathickmusclelayer(i.e.muscularis propria)andthereforedonotperforateeasily(perforationoccurs onlyin6.6%ofcases).Increasedintraluminalpressureinan intra-abdominalappendixisbelievedtobethepathologicalmechanism underlyingthedevelopmentofacquireddiverticula[6].

Onexaminingthepathogenesisofcongenitaldiverticula,Favara et al. foundthat trisomy 13 e 21 affected seven of eight con-genital diverticulumpatients[13].Thissuggeststheimportance of genetic or chromosomal factors. Otherpossible mechanisms includefailedrecanalizationoftheappendiceallumen,duplication oftheappendix,remnantsofepithelialinclusioncystsinthe appen-dicealwall,failedobliterationofthevitellineduct,andwalltraction causedbyadhesions[14].

Hypothesesonthedevelopmentofacquireddiverticula advo-cate either inflammatory causes or advocate noninflammatory causes.Theinflammationhypothesisstatesthatseveralepisodes of inflammation or infection lead to atrophy of lymphoid tis-sues, resulting in a weaker and thinner residual wall [2]. The noninflammation hypothesis holds that increased intraluminal pressurecausesacquireddiverticulatodevelop[2].The combina-tionofluminalobstructionandmuscularcontractionsdrivethis development. Secondary obstructions after inflammation, stric-ture,fecaliths,andtumorscauseincreasesinmuscularactivityand luminalpressure[2].Nearly60%ofdiverticulaarelocatedinthe distalthirdoftheappendix[4].

Diverticules accompanying epithelial neoplasms have been reportedinliterature.Dupreetal.[4]demonstratedastatistically signiﬁcantassociationbetweenthepresenceofdiverticulosisofthe vermiformappendixandneoplasms(47.8%),especiallycarcinoid tumoursandmucinousadenomas[8].IntheMedlicottand Urban-skiseries,aprimaryappendicealneoplasmwasdetectedin30% of acquireddiverticulosiscases[7]. InthestudyofLampset al. diverticules weredeterminedin8 outof19 patientswith low-grademucinousneoplasm(LGMN)(42%)[9].Marcacuzcoetal.in theirstudyreportedappendicealneoplasmin7.1%[11]ofatotalof 42(0.59%)patientswithappendix-associateddiverticulum.Inthe studyPasaglouetal.[10]theyfoundaccompanyingdiverticulain 23outof38casesofLGMN(60.5%).Thediverticulawerecoated withneoplasticepitheliuminallcases.Oneofthereasonsforthis coexistencecouldbetheincreasedintraluminalpressurecausedby theproductionofmucin,whichthinnedthemusculatureitselfand causedthemucinousepitheliumtoproliferatethroughweakpoints wherevesselspenetrate.Theotherpossiblecauseisthe develop-mentofLGMNinthepre-existingdiverticulum.AlsointhePasaglou study[10]poolsofserosaland/ormesoappendicularmucinwere detectedin78.3%ofcaseswithdiverticulumand33.3%ofNGMLs without diverticula, andthis differencewas statistically signiﬁ-cant.Detectingtheaccumulationofmucinonthemesoappendix incasesofLGMNwithdiverticulummorefrequently,hasledtothe hypothesisthatdiverticulosismayplayaroleinthepathogenesis ofperiappendicularmucindepositionandperitoneal pseudomyx-oma.Theruptureofadiverticulummaycausemucinlossesinthe intraabdominalspaceandmaybethebasisforthedevelopmentof apseudomyxoma.

Thereareveryfewstudiesthatstudytherelationshipbetween diverticulum rupturein LGMNsand pseudomyxomaperitoneas. InthestudybyLampsetal.theaccumulationofacellularmucin wasdetectedaroundtheinﬂamedandperforateddiverticulumin threeoutofeightLGMNs.However,noaccumulationofmucinwas observedontheserosalsurfaceormesoappendix[9]. Ofthe13 casesreportedbyPasaglou,whichshowedmucinaccumulationon themesoappendixandmonitoredoveraperiodofeightmonths

tosevenyears, nonedeveloped pseudomyxomaperitoneum. In thestudyby Lamps et al.,diverticulum rupturewasrelated to pseudomyxomaperitoneumin onlyonecase [9]. Theincidence ofneoplasmsassociatedwithdiverticulardiseasewas7.1%inthe studybyMarcacuzoetal.[11].Moreover,therearestudies,such asthearticlebyStockletal.,thatreporta42%associationbetween thepresenceofSchwanncellproliferationinthemucosaandthe presenceofappendicealdiverticulitis[20].Distinguishing appen-diculardiverticulitisfromacuteappendicitisisdifficult;however, somedifferenceshavebeenobserved.Comparedtoappendicitis symptoms,symptomatic appendicitis diverticulitishasa longer durationofpain(1–14days);itdevelopsmainlyinadults(over 30yearsofage);ithasalowerfrequencyofaccompanying abdom-inalpain,nauseaandvomiting;andithasagreaterpresenceofpain intherightlowerabdominalquadrant[2,15].Thecomplicationsof appendicealdiverticulitisvaryfromchronicpain,acute inflamma-tionandperforationtotheriskofdevelopingneoplasms[7].Itis curiousthattheageandincidenceofperforationofaninflamed diverticulumaregreater intype Ithanin therest.42%of low-grademucinousneoplasms oftheappendixareassociatedwith thisdisease,soitisthereforerecommendedthatallappendectomy specimenssthat presentdiverticula bethoroughlyexaminedto excludeconcomitantneoplasticdisease[16–18].Imagestudiescan facilitatepreoperativediagnosis.However,CTimagingresults(e.g., appendecularthickening,pericardialinflammation,abscess, phleg-moneandincreasedpericardialfatdensity)havenotsufficiently distinguishedappendeculardiverticulitisfromcecaldiverticulitis orappendicitis[13].ManyauthorsbelievethatmultidetectorCT canbeveryusefulbecauseithasdetectedacuteappendiceal diver-ticulitisin86%ofcaseswithpathologicallyconfirmedappendiceal diverticulitis[19,20].Accordingtootherauthors[21,22], preoper-ativeimagingtestsdonotcontributetothedefinitivediagnosis becausethediagnosis ismainly determinedby thehistological study.

4. Conclusion

Inconclusion,appendicealdiverticulosisdiseaseisgenerallyan accidentalﬁnding.Appendicealdiverticulosisdebutswith abdom-inalpainintherightiliacfossa,withsymptomsintheacutephase thatareindistinguishablefromthoseofacuteappendicitis.

The preferredtreatment is appendectomy. For symptomatic appendicealdiverticulosis,anappendectomyistheoptimal treat-ment.Regardlessofwhetherornotpatientshavesymptoms,most surgeons suggest prophylactic appendectomy because, even in patientswithoutsymptoms,theriskofperforationandmortalityis higherinthesepatientsthaninthegeneralpopulation[2].The per-forationrateofappendicitisdiverticulitisisfourtimeshigherthan theperforationrateofacuteappendicitis[2].Themortalityrate is30timeshigherinpatientswithperforatedappendicitis com-paredtopatientswithuncomplicatedappendicitis[2].Inaddition, theremaybea riskofdevelopingperitonealpseudomyxomain somepatientswithappendicealdiverticulosisastheyhaveahigher incidenceofappendicealmucinoustumors.Laparoscopic appen-dectomyisconsideredasafeandappropriatetreatmentforsimple appendicealdiverticulitis.

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TheworkhasbeenreportedinlinewiththeSCAREchecklist [23].

DeclarationofCompetingInterest

Theauthorsreportnodeclarationsofinterest.

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Sourcesoffunding

Nosourceoffundingorsponsors. Ethicalapproval

Thisisa CaseReportforwhich thepatientprovidedwritten informedconsent.

Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages. Authorscontribution

MicheleFiordaliso:Studydesign,datacollection,writing. FlaviaAntoniaDeMarco:Datacollection,writing. RaffaeleCostantini:Datacollection,writing.

MicheleFiordaliso:Writing, studydesign, Surgeon,who per-formedtheoperation.

Allauthorshaveapprovedtheﬁnalarticle. Registrationofresearchstudies

N/A. Guarantor

MicheleFiordaliso. Provenanceandpeerreview

Notcommissioned,externallypeer-reviewed. References

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