Article ID: WMC001502 2046-1690

An Interesting Case Of Atrial Septal Defect, Patent
Urachus And Valvular Anus Presenting In
Adulthood
Corresponding 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 And
Valvular Anus Presenting In Adulthood . WebmedCentral UROLOGY 2011;2(1):WMC001502
Source(s) of Funding:
Hospital and Patient
Additional Files:
case report
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An Interesting Case Of Atrial Septal Defect, Patent
Urachus And Valvular Anus Presenting In
Adulthood
Author(s): Shah A , Prabhu L
Abstract
Patent urachus presenting in adulthood is extremely
rare. Majority of these would have sought medical
attention in their infancy or early childhood. This report
descr i bes a f emal e pat i ent wi t h weepi ng
umbilicusbrought by a social worker.There were
additional anomalies,namely: anorectal malformation
(ARM) and atrial septal defect (ASD). This is a rarity of
a trinity of genitourinary, anorectal and cardiac
anomalies.
Introduction
The urachus i s a fi brous cord l ocated i n the
extraperitoneal tissues of the anterior abdominal wall.
Whenit fails to get obliterated, four distinct types of
anomalies arise. In the order of frequency, they are 1)
patent urachus (50%), 2) urachal cyst (30%), 3)
umbi l i c al ur ac hal s i nus ( 15%) , and
4)vesicourachaldiverticulum (3% -5%)
(1, 2).
ARM occurs
more frequently in boys than girls. The sex ratio varies
from 55% to 70% in favor of boys
(3, 4).
The cardiac
anomaly is associated most commonly with anorectal
malformation but not with patent urachus. But,
combination these three congenital anomaliesare
extremely rare.
Case Report(s)
The patient was a middle aged female brought to us
by a social worker with history of long standing
weeping umbilicus. She waswell preserved with a
normal mental status. There was no fecal incontinence.
She complained of chest pain occasionally. The most
conspicuous point in the historywas absence of
repeated episodes of urinary tract infection in the
past.She had normal menstrual cycles and had not
conceived after 15 yrs of marriage.
Examination showed aleaking opening in the lower lip
of a low placed umbilicus. There was periumbilical
ammonical dermatitis.
Genital examination showed the urethral, vaginal and
anal openings were within the vestibule.
2D ECHOof heart showed atrial septal defect
measuring 39 mmin diameter with right to left
shunt.Ultrasound of the abdomen revealed a small
uterus and a small sized right kidney.
Urinalysis showed 5-7 WBC/HPF. Hemoglobin was
10Gms/dl. Blood Urea was 40mg/dl and Serum
creatinine was 1.3mg/dl. No organism was grown in
urine cultured.
A short channel connecting the domeof the bladder
with an external opening close to the umbilicus was
noted at Cystoscopy. Ureteral orifices were orthotopic.
This tubular connection was then excised intoto with a
cuff of bladder and the defect in bladder was repaired.
The wound healed well and she could void well
without incontinence or discomfort. Histopathology
confirmed that the excised tract was urachus. No
intervention was done for ARM and ASD as she was
not willing for the same.
Discussion
A valvular anus as in this case results because the
anus lies within the vestibule as a result off very little
development of perineum. In this anomaly, the anal
orifice is usually small but not stenotic and is
surrounded by wet vestibular epithelium.
This patient had a low translevator ARM in which all
the 3 openings were within the vestibule i.e.urethral,
vaginal and the anus.Patent urachus is explained by
non-descent of the bladder or, more commonly, failure
of the epithelial-lined urachal canal to obliterate (5).
Bladder obstruction during fetal development has been
blamed for the urachus remaining patent.It is seen that
urachal patency is often absent in severely obstructed
bladders in utero as in a case of posterior urethral
valves. So the obliteration of the urachus may be
independent from the level of bladder distention (6, 7).
In our case the bladder was well within the pelvis with
a normal outlet and orthotopic ureteric orifices.
Therefore re-tubularization, rather than primary
patency, might be the cause for urinary drainage from
the umbilicus(8, 9).
Delayed presentation and absence of repeated
urinary tract infections despite the fact that the
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urethra, vagina and anus being within the
vestibule facilitating colonization by bacteria are
unique features in this report. Combination of
cardiac, urinary and anorectal anomalies makes it
extremely 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.British
journal of urology 1991; 67: 327-8. (s)
3. Tong MC (1981) Anorectal anomalies: a review of
49 cases. Ann Acad Med Singapore 10:479–484
4. Otte JB (1983) Imperforate anus.Various Belgian
epidemiologic data.ActaChirBelg 82:158–162
5.Gearhart, 2002. Gearhart JP: Exstrophy, epispadias,
and other bladder anomalies. In: Walsh PC, et al ed.
Campbell's Urology, 8th ed.. Philadelphia: WB
Saunders; 2002:2136-2196.
6. Schreck and Campbell, 1972. Schreck WR,
Campbell 3rd WA: The relation of bladder outlet
obstruction 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 with
isolated 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 in
adult 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.
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Fig 1:- Low placed umbilicus
Fig 2:- Catheter entering the bladder through the umbilical end of patent urachus.
Illustrations
Illustration 1
Clinical Photographs
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Fig 3:- All the 3 openings within the vestibule
Fig 4:- urethral, vaginal and anal openings within the vestibule
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Fig 5:- chest X ray showing increased pulmonary vascularity with prominent hilar markings and
cardiomegaly
Fig 6:- Post op picture after repair of umbilicus
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