The Urachus Revisited

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BJR © 2020 The Authors.

Published by the British Institute of Radiology


https://​doi.​org/​10.​1259/​bjr.​20190118
Received: Revised: Accepted:
31 January 2019 17 November 2019 10 February 2020

Cite this article as:


Das JP, Vargas HA, Lee A, Hutchinson B, O'Connor E, Kok HK, et al. The urachus revisited: multimodal imaging of benign & malignant
urachal pathology. Br J Radiol 2020; 93: 20190118.

Pictorial Review

The urachus revisited: multimodal imaging of benign &


malignant urachal pathology

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1,2,3
Jeeban Paul Das, MD, 2Hebert Alberto Vargas, MD, 3Aoife Lee, MD, 1Barry Hutchinson, MD,
4
Eabhann O'Connor, MD, 5Hong Kuan Kok, MD, 5William Torreggiani, MD, 1Joe Murphy, MD,
1
Clare Roche, MD, 1John Bruzzi, MD and 1,6Peter McCarthy, MD
1
Dept. of Radiology, Galway University Hospital, Co., Galway, Ireland
2
Dept. of Oncologic Imaging, Memorial Sloan Kettering Cancer Centre, New York, USA
3
Dept. of Radiology, Beaumont Hospital, Dublin 9, Ireland
4
Dept. of Urology, St. Vincent's University Hospital, Dublin, Ireland
5
Dept. of Radiology, Adelaide & Meath Hospital, Dublin, Ireland
6
School of Medicine, Clinical Science Institute, National University of Ireland, Galway, Ireland

Address correspondence to: Dr Jeeban Paul Das


E-mail: ​jeeban.​paul.​das@​gmail.​com

ABSTRACT:
The urachus is a fibrous tube extending from the umbilicus to the anterosuperior bladder dome that usually obliterates
at week 12 of gestation, becoming the median umbilical ligament. Urachal pathology occurs when there is incomplete
obliteration of this channel during foetal development, resulting in the formation of a urachal cyst, patent urachus,
urachal sinus or urachal diverticulum. Patients with persistent urachal remnants may be asymptomatic or present with
lower abdominal or urinary tract symptoms and can develop complications. The purpose of this review is to describe
imaging features of urachal remnant pathology and potential benign and malignant complications on ultrasound, CT,
positron emission tomography CT and MRI.

Introduction Imaging of urachal remnants


Urachal embryology Urachal cysts account for up to 54% of urachal remnant
A urachal remnant occurs due to maldevelopment of the anomalies, arising from the lower third of the urachus
allantois and cloaca. The allantois arises from the postero- and are often asymptomatic and incidentally discovered.
inferior yolk sac after the second week of embryonic devel- Sonography can demonstrate an extraluminal midline
opment. The urinary bladder initially extends to the level fluid-­filled structure that does not communicate with the
of the umbilicus descending into the pelvis by month 5 of umbilicus. CT following intravenous contrast usually reveal
gestation. The apical portion of the descending bladder, a fluid-­attenuation supravesical lesion (Figure 3). On MRI,
derived from the cloaca, degenerates into an extraperitoneal
urachal cysts usually demonstrate a thin, non-­enhancing
fibrous cord within the Retzius space, eventually becoming
wall with intrinsic T1 hypointense and T2 hyperintense
the median umbilical ligament. This structure extends from
the bladder dome to the umbilicus. The vestigial remnant of signal.1–4
this is known as the urachus1,2 (Figure 1).
A urachal sinus is a tubular dilatation of the umbil-
ical portion of the urachus that occurs three times more
Epidemiology of urachal anomalies
frequently in children than adults. Clinical presentation
A persistent urachus has an incidence of 1/5000 in the
is most commonly with periumbilical inflammation and
adult population, occurring more frequently in males. The
purulent discharge. On ultrasound, a urachal sinus may
most common urachal anomalies are urachal cysts, urachal
sinus, patent urachus and vesicourachal diverticulum appear tubular and hypoechoic communicating with the
(Figure 2). Infection is the most common benign compli- umbilicus only. Fluoroscopic sinography can show a tube-­
cation of urachal remnant pathology occurring in children like cul-­de-­sac. CT may better delineate infective complica-
and adults whereas malignant complications have been tions. MRI can better evaluate for superimposed infection
described exclusively in adults to date.1–3 or developing malignancy.1,3
BJR Das et al

Figure 1. Schematic diagram (a) showing a normal urachus. Saggital (b) and axial (c) CT images following intravenous contrast
demonstrating a normal urachus extending into Retzius space.

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The patent urachus is a fistulous communication occuring A urachal diverticulum is a rare clinical entity accounting for
between the urinary bladder and umbilicus, most commonly 3–5% of urachal remnant pathology occurring when the urachus
presenting with umbilical urine leak. Ultrasound may demon- communicates with the bladder dome only. Sonographic evalua-
strate a tube-­shaped anechoic structure communicating with tion of a urachal diverticulum shows a blind-­ending supravesical
the antero superior bladder and umbilicus. Diagnosis can be hypoechogenic structure arising from the bladder dome. CT can
confirmed on voiding cystourography or fluoroscopic sinogram demonstrate an out-­pouching from the superior aspect of the
with instilled contrast media defining the umbilical-­vesical tract. bladder (Figure 4) without umbilical communication, and may
CT and MRI can better demonstrate air, fluid or calculi within demonstrate intraluminal calcification, present in up to 50% of
the patent urachus, and evaluate for potential infective complica- cases. In children, VCUG can show contrast reflux into the diver-
tions on post-­contrast imaging.1–3 ticulum and help delineate the extent of the involvement of the

Figure 2. Schematic diagrams showing common urachal remnant pathology. (a) Urachal cyst; (b) Urachal sinus; (c) Patent ura-
chus; (d) Urachal diverticulum.

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Imaging of Urachal Pathology BJR

Figure 3. 38-­year-­old male with a lower urinary tract symp- Figure 5. 5-­ year-­
old child with fever and urinary retention.
toms. Axial (a) and saggital (b) CT images after intravenous Longitudinal (a) and transverse (b) greyscale ultrasound
contrast showing a supravesical cystic structure without defi- images demonstrating a lesion containing internal echoes
nite communication with the bladder or umbilicus confirmed (arrows) anterosuperior to the urinary bladder (*). Surgical
as a urachal cyst following image-­guided aspiration. excision following intravenous antibiotics confirmed diagnosis
of an infected urachal cyst.

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urachus. On MRI, a protruding midline soft-­tissue tract can be
seen, demonstrating variable signal intensity and post-­contrast cyst from adjacent pelvic structures and identify possible rupture
enhancement.1–3 and abscess formation.1,3–5

Imaging of benign urachal pathology Urachal abscess can develop if an infected cyst is left untreated.
Infection of a persistent urachus is the most common benign Infected urachal cysts usually discharge into the umbilicus or
complication occuring in patients with a urachal remnant, usually urinary bladder but may rarely rupture intraperitoneally from
due to Staphylococcus aureus. Patients with infected urachal cysts their extraperitoneal location causing sepsis and peritonitis.
may be asymptomatic with leukocytosis or present with recur- Given the lack of direct communication with either bladder
rent urinary tract infections, abdominal pain or fever.1,2,4 or umbilicus, clinical asessment may result in misdiagnosis of
more commonly encountered caused of acute abdomen, such
Infected urachal cysts may be difficult to differentiate from non-­ as appendicitis or bowel perforation.5,6 Ultrasound may show a
infected cysts or other pelvic pathology on initial sonographic complex cystic mass in the midline above the urinary bladder.
evaluation in the absence of clinical signs and symptoms.5 CT following intravenous contrast can demonstrate a supraves-
Ultrasound is often the first-­line modality for imaging a child ical heterogenous collection with a thick enhancing irregular
presenting with symptoms of abdominal pathology. Greyscale wall and central non-­ enhancing low attenuation (Figure 7),
ultrasound reveals a midline extraperitoneal structure extending that may abut or communicate with the abdominal wall.1,3,5,6
from the bladder dome to the umbilicus containing internal On MRI, a urachal abscess can appear as a thick-­walled, multi-
echoes (Figure 5) and may mimic other pelvic pathology, such loculated predominantly T2 hyperintense mass extending into
as haematoma. On CT, infected urachal cysts can appear thick-­ Retzius space demonstrating heterogenous enhancement on
walled and irregular with peripheral enhancment and underlying post-­contrast imaging. (Figure 8)
bladder wall thickening (Figure 6). On MRI, infected urachal
cysts can appear as irregular thick-­walled supravesical struc- Benign urachal tumours are rare clinical and radiological entities
tures demonstrating variable signal intensity and heterogenous and include fibroadenomatous lesions, lipoatous tumours and
contrast enhancement. MRI may delineate the infected urachal hamartomas.1,3

Figure 4. 70-­year-­old male with back pain. Saggital (a) and Figure 6. 49-­year-­old male with recurrent urinary tract infec-
axial (b) CT images following intravenous contrast showing a tions. Saggital (a) and axial (b) CT images following intra-
urachal diverticulum (arrows) detected as an incidental find- venous contrast demonstrating an irregular cystic structure
ing. anterosuperior to the urinary bladder demonstrating periph-
eral enhancement, confirmed as an infected urachal cyst fol-
lowing surgical excision.

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Figure 7. 21-­year-­old male with 2-­week history of fever and Figure 9. 32-­year-­old female with abdominal swelling and
tender suprapubic mass. Axial CT image after intravenous lower urinary tract symptoms. Axial CT image following
contrast showing an irregular, mixed attenuation lesion intravenous contrast showing a solid-­ cystic mass (arrow)
anterosuperior to the bladder dome (arrow) with surrounding containing a focus of curvlinear calcification (arrowhead)
inflammatory change. Image-­guided aspiration yielded puru- anterosuperior to the urinary bladder (*). Histopathology
lent material. Subsequent surgical excision confirmed diagno- confirmed the diagnosis of urachal adenocarcinoma, follow-
sis of a urachal abscess. ing surgical excision.

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anterior abdominal wall demonstrating internal vascularity
Imaging of malignant urachal pathology on colour doppler imaging. Urachal cancer can demonstrate a
Malignant transformation of a urachal remnant is rare, variable appearance on cross-­sectional imaging. On CT urachal
accounting for <0.5% of bladder cancer and develops twice as malignancy can appear as a solid-­cystic mass extending toward
often in males, most commonly between the ages of 46 and 56. the umbilicus with an irregular, enhancing wall (Figure 9). Up
Adenocarcinoma accounts for 80–90% of cases with up to 75% to 90% of cases arise from the juxtavesical urachus, extending
producing mucin. Sarcomatous neoplasms, squamous cell and cranially into the space of Retzius. Urachal cancer may also
transitional cell urachal cancers have also been reported.1,3,7,8 appear as a predominantly cystic mass (Figure 10) or as a
midline or paramidline enhancing nodule arising from the
Clinical presentation is variable and patients may be asymptom- anterosuperior aspect of the urinary bladder (Figure 11).
atic until local spread or development of metastases. Haematuria Urachal cancer appears low attenuation in 60% of cases due to
is the most common symptom, present in up to 80% of cases. the presence of mucin or necrosis. CT can also identify central
Mucinuria can occur in up to 25% of patients, a rare symptom or peripheral calcification, present in almost 75% of urachal
that should raise suspicion and prompt further investigation for cancers.1,3,8–10 Urachal malignancies may be predominantly
a urachal cancer.3,7–9
Figure 10. 39-­year-­old female with abdominal bloating and
On ultrasound, urachal malignancy can appear as a hypere- mucosuria. Axial CT image demonstrating a well-­ defined
chogenic soft-­tissue lesion between the urinary bladder and cystic structure with mural thickening and peripheral calcifi-
cation (arrowhead) anterosuperior to the urinary bladder (*).
Image-­guided aspiration and histopathology confirmed muci-
Figure 8. 28-­year-­old female with pelvic pain and fever. Axial
nous urachal adenocarcinoma.
(a) and sagittal (b) T2 weighted images showing a multiloc-
ulated heterogenously T2 hyperintense collection antero-
superior to the bladder (*), confirmed as a urachal abscess
following surgical intervention.

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Figure 11. 41-­year-­old male with haematuria. Axial CT image Figure 13. 45-­year-­
old female with haemturia and abdom-
showing an enhancing soft-­tissue nodule (arrow) arising from inal bloating. Axial (a) and sagittal (b) T2 weighted images
left anterosuperior wall of the bladder (*). Histopathology fol- demonstrate a complex, multilobulated mass involving the
lowing cystoscopy and biopsy confirmed diagnosis of urachal dome of the urinary bladder (*) with intravesical extension
adenocarcinoma. (arrowhead). Histopathology confirmed mucinous urachal
adenocarcinoma.

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also be seen in ~20% of urachal cysts.2,3 Fludeoxyglucose posi-
tron emission tomography (PET) CT (Figure 14, 14,15) has been
demonstrated as useful in the assessment of urachal cancer14
However, due to lack of avid fludeoxyglucose uptake in over
half of mucinous tumours, diagnostic performance of PET/CT
cystic demonstrating high T2 signal (Figure 12) or multilobu- may be unreliable in excluding urachal malignancy, given that
lated with solid and cystic components demonstrating interme- up to three quarters urachal tumours produce mucin. Imaging
diate and high T2 signal representing mucin, cystic change and/ may identify a urachal remnant complication but ultimately
or necrosis (Figure 13). On post-­contrast T1 weighted imaging, cystoscopy and biopsy may be required to confirm the diag-
urachal cancers may demonstrate inhomogenous enhancement. nosis of a benign or malignant lesion.2,7,8,15
MRI may provide addional value by defining local invasion of
adjacent soft-­tissue and visceral structures, as well as identifying Treatment of urachal pathology
local nodal metastases.1,11,12 Controversy regarding optimal treatment of urachal remnants
has become more apparent in recent years. Management strat-
Assessment of urachal pathology: the egies also differ between adult and paediatric patients. While
role of imaging surgery is required for most symptomatic patients, sponta-
Accurate diagnosis of benign from malignant urachal pathology neous regression of urachal anomalies has also been reported.
on imaging is imprecise. Meeks et al found that pre-­operative Current series demonstrate a 10-­year survival rate of only 49%
imaging with CT had a low specificity and negative predictive with surgery for urachal cancer while salvage chemotherapy
value of 21 and 43% respectively.13 In addition, although the is effective in less than 10% of patients with metastases,
majority of malignant urachal tumours are solid, up to 27% can suggesting that the risk of delaying the urachal remnant resec-
demonstrate a cystic component.2 Similarly, although intrinsic tion in older adults may exceed the risk of surgery.2,7,8
calcification is considered 'pathognomic' of urachal cancer
seen in up to 70% of cases on CT, 'egg-­shell' calcification can
Figure 14. 47-­year-­old female with haematuria. Axial (a) and
sagittal (b) 18-­
fluorine FDG PET CT showing an FDG-­ avid
Figure 12. 39-­year-­old female with abdominal bloating. Sag- supravesical mass (arrows) containg punctate calcifications
gital (a) and coronal (b) T2 weighted magnetic resonance demonstrating a maximum SUV of 6.8. Biopsy and subse-
images show a paramidline cystic mass arising from the dome quent resection confirmed the diagnosis of urachal adeno-
of the bladder (*). Histopathology confirmed mucinous ura- carconoma. FDG, fludeoxyglucose; PET, positron emission
chal adenocarcinoma. tomography; SUV, standardized uptake value.

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BJR Das et al

Whereas earlier recommendations favoured surgical interven- disease can potentially result in delayed diagnosis and treatment.
tion for in the majority of paediatric patients with a urachal Multimodal imaging with ultrasound, CT, PET/CT and MRI can
remnant (due to the risk of recurrent infection and urachal identify and characterise potential complications related to the
cancer in later life), more recent reports have suggested that urachal remnant, however differentiating benign from malignant
between 50 and 80% resolve non-­ operatively. In addition, urachal complications is imprecise with imaging alone and histo-
almost 15% of paediatric patients develop post-­ operative
pathological analysis is usually required to confidently diagnose
complications including iatrogenic bladder injury requiring
reoperation.2,13,16 urachal cancer from an infectious/inflammatory process.

Conclusion Funding
Urachal pathology, although rare, can cause significant morbidity This research was funded in part through the NIH/NCI Cancer
and mortality. Non-­specific pelvic symptomatology of urachal Center Support Grant P30 CA008748

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