An Interesting Case Of Atrial Septal Defect, Patent Urachus And … · 2012. 1. 2. · Keywords:Patent urachus, Valvular anus, ASD, Low Lying Umbilicus and Ammonical Dermatitis How

Article ID: WMC001502 2046-1690

An Interesting Case Of Atrial Septal Defect, PatentUrachus And Valvular Anus Presenting InAdulthoodCorresponding Author:Dr. Abhishek Shah,Resident, KMC Mangalore Manipal University, Dept of General Surgery, 575001 - India

Submitting Author:Dr. Abhishek Shah,Resident, KMC Mangalore Manipal University, Dept of General Surgery , 575001 - India

Article ID: WMC001502

Article Type: Case Report

Submitted on:23-Jan-2011, 03:23:21 PM GMT Published on: 24-Jan-2011, 08:25:01 PM GMT

Article URL: http://www.webmedcentral.com/article_view/1502

Subject Categories:UROLOGY

Keywords:Patent urachus, Valvular anus, ASD, Low Lying Umbilicus and Ammonical Dermatitis

How to cite the article:Shah A , Prabhu L . An Interesting Case Of Atrial Septal Defect, Patent Urachus AndValvular Anus Presenting In Adulthood . WebmedCentral UROLOGY 2011;2(1):WMC001502

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An Interesting Case Of Atrial Septal Defect, PatentUrachus And Valvular Anus Presenting InAdulthoodAuthor(s): Shah A , Prabhu L

Abstract

Patent urachus presenting in adulthood is extremelyrare. Majority of these would have sought medicalattention in their infancy or early childhood. This reportdescr ibes a female pat ient wi th weepingumbilicusbrought by a social worker.There wereadditional anomalies,namely: anorectal malformation(ARM) and atrial septal defect (ASD). This is a rarity ofa trinity of genitourinary, anorectal and cardiacanomalies.

Introduction

The urachus is a fibrous cord located in theextraperitoneal tissues of the anterior abdominal wall.Whenit fails to get obliterated, four distinct types ofanomalies arise. In the order of frequency, they are 1)patent urachus (50%), 2) urachal cyst (30%), 3)u m b i l i c a l u r a c h a l s i n u s ( 1 5 % ) , a n d4)vesicourachaldiverticulum (3% -5%) (1, 2).ARM occursmore frequently in boys than girls. The sex ratio variesfrom 55% to 70% in favor of boys(3, 4). The cardiacanomaly is associated most commonly with anorectalmalformation but not with patent urachus. But,combination these three congenital anomaliesareextremely rare.

Case Report(s)

The patient was a middle aged female brought to usby a social worker with history of long standingweeping umbilicus. She waswell preserved with anormal mental status. There was no fecal incontinence.She complained of chest pain occasionally. The mostconspicuous point in the historywas absence ofrepeated episodes of urinary tract infection in thepast.She had normal menstrual cycles and had notconceived after 15 yrs of marriage.Examination showed aleaking opening in the lower lipof a low placed umbilicus. There was periumbilicalammonical dermatitis.Genital examination showed the urethral, vaginal and

anal openings were within the vestibule.2D ECHOof heart showed atrial septal defectmeasuring 39 mmin diameter with right to leftshunt.Ultrasound of the abdomen revealed a smalluterus and a small sized right kidney.Urinalysis showed 5-7 WBC/HPF. Hemoglobin was10Gms/dl. Blood Urea was 40mg/dl and Serumcreatinine was 1.3mg/dl. No organism was grown inurine cultured.A short channel connecting the domeof the bladderwith an external opening close to the umbilicus wasnoted at Cystoscopy. Ureteral orifices were orthotopic.This tubular connection was then excised intoto with acuff of bladder and the defect in bladder was repaired.The wound healed well and she could void wellwithout incontinence or discomfort. Histopathologyconfirmed that the excised tract was urachus. Nointervention was done for ARM and ASD as she wasnot willing for the same.

Discussion

A valvular anus as in this case results because theanus lies within the vestibule as a result off very littledevelopment of perineum. In this anomaly, the analorifice is usually small but not stenotic and issurrounded by wet vestibular epithelium.This patient had a low translevator ARM in which allthe 3 openings were within the vestibule i.e.urethral,vaginal and the anus.Patent urachus is explained bynon-descent of the bladder or, more commonly, failureof the epithelial-lined urachal canal to obliterate (5).Bladder obstruction during fetal development has beenblamed for the urachus remaining patent.It is seen thaturachal patency is often absent in severely obstructedbladders in utero as in a case of posterior urethralvalves. So the obliteration of the urachus may beindependent from the level of bladder distention (6, 7).In our case the bladder was well within the pelvis witha normal outlet and orthotopic ureteric orifices.Therefore re-tubularization, rather than primarypatency, might be the cause for urinary drainage fromthe umbilicus(8, 9).Delayed presentation and absence of repeatedurinary tract infections despite the fact that the



urethra, vagina and anus being within thevestibule facilitating colonization by bacteria areunique features in this report. Combination ofcardiac, urinary and anorectal anomalies makes itextremely rare.

References

1. Walsh PC, Retik AB, Vaughan ED, et al (eds):Campbell's Urology, s8th ed. Philadelphia: W.B.Saunders, 2002. (s)2. Diehl K. A rare case of urachal calculus.Britishjournal of urology 1991; 67: 327-8. (s)3. Tong MC (1981) Anorectal anomalies: a review of49 cases. Ann Acad Med Singapore 10:479–4844. Otte JB (1983) Imperforate anus.Various Belgianepidemiologic data.ActaChirBelg 82:158–1625.Gearhart, 2002. Gearhart JP: Exstrophy, epispadias,and other bladder anomalies. In: Walsh PC, et al ed.Campbell's Urology, 8th ed.. Philadelphia: WBSaunders; 2002:2136-2196.6. Schreck and Campbell, 1972. Schreck WR,Campbell 3rd WA: The relation of bladder outletobstruction to urinary umbilical fistula. J Urol 1972;108:641.7. Mesrobian et al., 1997. Mesrobian HG, Zacharias A,Balcom AH, Cohen RD: Ten years of experi-ence withisolated urachal anomalies in children. J Urol 1997;158:1316-1318.8. Schubert et al., 1983. Schubert GE, Pavkovic MB,Bethke-Bedurftig BA: Tubular urachal remnants inadult bladders. J Urol 1983; 127:40-42.9. Berman et al., 1988. Berman SM, Tolia BM, Laor E,et al: Urachal remnants in adults. Urology 1988;31:17-21.



Fig 1:- Low placed umbilicus

Fig 2:- Catheter entering the bladder through the umbilical end of patent urachus.

Illustrations

Illustration 1

Clinical Photographs



Fig 3:- All the 3 openings within the vestibule

Fig 4:- urethral, vaginal and anal openings within the vestibule



Fig 5:- chest X ray showing increased pulmonary vascularity with prominent hilar markings andcardiomegaly

Fig 6:- Post op picture after repair of umbilicus



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An Interesting Case Of Atrial Septal Defect, Patent Urachus And … · 2012. 1. 2. · Keywords:Patent urachus, Valvular anus, ASD, Low Lying Umbilicus and Ammonical Dermatitis How

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