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CLOACALEXSTROPHYINIDENTICALTWINS JOHNF .REDMAN,M .D . JOANNAJ .SEIBERT,M .D . B .C .PAGE,M .D . FromtheDepartmentsofUrologyandRadiology, UniversityofArkansasCollegeofMedicineand ArkansasChildren'sHospital,LittleRock,Arkansas ABSTRACT - Cloacalexstrophyoccurringinidenticaltwinsisreported. Cloacalexstrophyorvesicointestinalfissureisa rareanomaly .Theincidencehasbeenestimated tobeapproximately1 :200,000to1 :250,000 births .'Wecouldfindnoreportofthisanomaly occurringintwins .Identicaltwinswithcloacal exstrophyarethebasisforthisreport . CaseReports Twingirls,weighing1,840Gm .and1,980 Gm .,werebornbycesareansectiontoa UROLOGY / JANUARY1981 / VOLUMEXVII,NUMBERI FIGURE 1 . (A)TwinA,and (B) Twin B . twenty-year-oldprimigravidawoman .The placentawasmonochorionicandmonoamniotic . Eachtwinhadthesameanomaliesconsistingof exstrophyofthebladderandbowel,aterminal ileostomy,bisectionoftheexternalgenitalia, imperforateanus,andasacralmyelocystocele . TwinAwasdeadonarrivaltoArkansasChil- dren'sHospital(Fig .1A) .Apostmortemexami- nationwasrefused .TwinBdiedatagetwenty daysofsepsis(Fig .1B) .Apostmortemexamina- tionwasallowedanddisclosedinadditiontothe .3

Cloacal exstrophy in identical twins

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Page 1: Cloacal exstrophy in identical twins

CLOACAL EXSTROPHY IN IDENTICAL TWINS

JOHN F. REDMAN, M .D .JOANNA J. SEIBERT, M.D.B .C. PAGE, M .D .

From the Departments of Urology and Radiology,University of Arkansas College of Medicine andArkansas Children's Hospital, Little Rock, Arkansas

ABSTRACT - Cloacal exstrophy occurring in identical twins is reported.

Cloacal exstrophy or vesicointestinal fissure is arare anomaly . The incidence has been estimatedto be approximately 1 :200,000 to 1 :250,000births.' We could find no report of this anomalyoccurring in twins. Identical twins with cloacalexstrophy are the basis for this report .

Case Reports

Twin girls, weighing 1,840 Gm . and 1,980Gm ., were born by cesarean section to a

UROLOGY / JANUARY 1981 / VOLUME XVII, NUMBER I

FIGURE 1 . (A) Twin A, and (B) Twin B .

twenty-year-old primigravida woman . Theplacenta was monochorionic and monoamniotic .Each twin had the same anomalies consisting ofexstrophy of the bladder and bowel, a terminalileostomy, bisection of the external genitalia,imperforate anus, and a sacral myelocystocele .

Twin A was dead on arrival to Arkansas Chil-dren's Hospital (Fig. 1A). A postmortem exami-nation was refused . Twin B died at age twentydays of sepsis (Fig . 1B) . A postmortem examina-tion was allowed and disclosed in addition to the

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Page 2: Cloacal exstrophy in identical twins

external anomalies as described that the uretersbilaterally terminated in a persistent meso-nephric duct bilaterally . The right kidney wassituated in the pelvis . The left kidney was or-thotopic, but the ureter was greatly dilatedbeing obstructed at its terminal end .

Comment

Although cloacal exstrophy has not been pre-viously reported in twins, the related anomalyexstrophy of the bladder has been so noted .Higgins reported exstrophy of the bladder inidentical twin boys .' Marshall and Mueckc 3 re-ported vesical exstrophy in a twin boy whosetwin sister was normal . Exstrophy of the blad-der has also been noted in siblings.",'

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The genitourinary anomalies and other associ-ated anomalies in children with cloacalexstrophy have been well reviewed previouslyby Soper and Kilger .'

Little Rock, Arkansas 72201(DR. REDMAN)

References

1 . Soper RT, and Kilger K : Vesico-intestinal fissure, J . Urol .92 : 490 (1964) .2. Higgins CC : Exstrophy of the bladder in twins, Cleve . Clin .

Quart . 10 : 138 (1943) .3. Marshall VF, and Mueckc EC : Variations in exstrophy of the

bladder, J . Urol . 88 : 766 (1962) .4. Glaser LH, and Lewis APR: A case of familial incidence of

ectopia vesical, Br . Med . J . 2 : 1333 (1961) .5 . Chishohn TC : Exstrophv of the bladder, in Mustard WT, ei

al. (Eds): Pediatric Surgery, 2nd cd ., Chicago, Year Book MedicalPublishers Inc ., 1969 .

UROLOGY

JANUARY 1931 1 VOLUME XV71, NUMBER I