RADIOLOGY
1. History: A 23-year-old female with a history of Kabuki syndrome presented with
umbilical pain that worsens with standing and lifting. A CT image was obtained. Click
to enlarge the image.
Which of the following is appropriate to include in the differential diagnosis for
umbilical pain?
A. Meckel’s diverticulum
B. Early appendicitis
C. Umbilical hernia
D. Abdominal abscess
E. Perforated bowel
F. Urachal duct cyst
G. All of the above
The patient has a recent history of drainage from the umbilicus. Given the image
above, what fluid is most likely draining?
A. Faecal matter
B. Blood
C. Ascitic fluid
D. Urine and pus
E. Lymphatic fluid
Urachal patency places patients at increased risk for adenocarcinoma of the urachal
remnant.
A. True
B. False
,Findings
Remnant urachus with a connection to a urachal duct cyst measuring 3 x 2 x 1.5 cm
decreased in size from a prior measurement of 3 x 2.5 x 2 cm. The urachal duct cyst no
longer appears as inflamed, with decreased fat stranding compared to prior imaging. No
suspicious features are present.
Differential diagnosis
● Umbilical hernia
● Urachal duct cyst
● Meckel's diverticulum
● Abdominal abscess
● Perforated bowel
, ● Early acute appendicitis
Diagnosis: Urachal duct cyst
Discussion
Urachal duct cyst
Epidemiology and pathogenesis
During embryologic development, the embryologic bladder and allantois are continuous with
a cannulized fibromuscular stalk that connects the fetal bladder to the umbilical cord for
drainage. The urachal canal normally obliterates as the bladder descends into the fetal
pelvis, forming a fibrous connection with the ventral abdominal wall known as the median
umbilical ligament.
Under normal circumstances, complete obliteration of the urachus typically occurs during
late fetal development or early infancy, typically by 6 months of age. When urachal
obliteration fails, there are multiple embryologic malformations that can result.
The four distinct embryologic malformations include a patent urachus, an umbilical urachal
sinus, a vesicourachal diverticulum, or a urachal cyst. When the urachal canal remains
patent, it drains the urinary bladder into the umbilicus. Roughly 80% of urachal defects
resolve after two years of age. Patients with signs and symptoms of infection typically
require surgery.
Clinical presentation
Symptomatic patients present with umbilical drainage, fever, and/or tender inferior umbilical
mass. Urachal cyst infections predispose patients to a number of acute complications,
including bladder fistula formation, cyst rupture, peritonitis, and sepsis.
Neoplasms arising from urachal remnants are infrequent, especially in the younger pediatric
population. Those who present from middle age and elderly populations are more affected.
Benign urachal neoplasms are extremely infrequent. Benign neoplasms that could occur
include adenomas, fibromas, fibroadenomas, fibroids, and hamartomas. The urachus is
mainly lined by transitional epithelium; however, the most common malignant manifestation
of the urachal remnant, occurring in 90% of cases, is adenocarcinoma.
Imaging
Ultrasound is the most common imaging modality in the diagnosis of patients presenting with
urachal abnormalities. Urachal cysts present as fluid collections in the abdominal midline
between the umbilicus and the bladder. CT is the preferred imaging modality for solidifying
diagnosis when ultrasound or fistulography are inconclusive. MRI may provide more detailed
soft tissue imaging that can detect a hollow urachal tube, neoplasm, cyst, or abscess.
Treatment
Surgical excision of the urachal remnant is the therapeutic mainstay for symptomatic urachal
disease. Often a two-stage management approach is used, first focusing on treating urachal
1. History: A 23-year-old female with a history of Kabuki syndrome presented with
umbilical pain that worsens with standing and lifting. A CT image was obtained. Click
to enlarge the image.
Which of the following is appropriate to include in the differential diagnosis for
umbilical pain?
A. Meckel’s diverticulum
B. Early appendicitis
C. Umbilical hernia
D. Abdominal abscess
E. Perforated bowel
F. Urachal duct cyst
G. All of the above
The patient has a recent history of drainage from the umbilicus. Given the image
above, what fluid is most likely draining?
A. Faecal matter
B. Blood
C. Ascitic fluid
D. Urine and pus
E. Lymphatic fluid
Urachal patency places patients at increased risk for adenocarcinoma of the urachal
remnant.
A. True
B. False
,Findings
Remnant urachus with a connection to a urachal duct cyst measuring 3 x 2 x 1.5 cm
decreased in size from a prior measurement of 3 x 2.5 x 2 cm. The urachal duct cyst no
longer appears as inflamed, with decreased fat stranding compared to prior imaging. No
suspicious features are present.
Differential diagnosis
● Umbilical hernia
● Urachal duct cyst
● Meckel's diverticulum
● Abdominal abscess
● Perforated bowel
, ● Early acute appendicitis
Diagnosis: Urachal duct cyst
Discussion
Urachal duct cyst
Epidemiology and pathogenesis
During embryologic development, the embryologic bladder and allantois are continuous with
a cannulized fibromuscular stalk that connects the fetal bladder to the umbilical cord for
drainage. The urachal canal normally obliterates as the bladder descends into the fetal
pelvis, forming a fibrous connection with the ventral abdominal wall known as the median
umbilical ligament.
Under normal circumstances, complete obliteration of the urachus typically occurs during
late fetal development or early infancy, typically by 6 months of age. When urachal
obliteration fails, there are multiple embryologic malformations that can result.
The four distinct embryologic malformations include a patent urachus, an umbilical urachal
sinus, a vesicourachal diverticulum, or a urachal cyst. When the urachal canal remains
patent, it drains the urinary bladder into the umbilicus. Roughly 80% of urachal defects
resolve after two years of age. Patients with signs and symptoms of infection typically
require surgery.
Clinical presentation
Symptomatic patients present with umbilical drainage, fever, and/or tender inferior umbilical
mass. Urachal cyst infections predispose patients to a number of acute complications,
including bladder fistula formation, cyst rupture, peritonitis, and sepsis.
Neoplasms arising from urachal remnants are infrequent, especially in the younger pediatric
population. Those who present from middle age and elderly populations are more affected.
Benign urachal neoplasms are extremely infrequent. Benign neoplasms that could occur
include adenomas, fibromas, fibroadenomas, fibroids, and hamartomas. The urachus is
mainly lined by transitional epithelium; however, the most common malignant manifestation
of the urachal remnant, occurring in 90% of cases, is adenocarcinoma.
Imaging
Ultrasound is the most common imaging modality in the diagnosis of patients presenting with
urachal abnormalities. Urachal cysts present as fluid collections in the abdominal midline
between the umbilicus and the bladder. CT is the preferred imaging modality for solidifying
diagnosis when ultrasound or fistulography are inconclusive. MRI may provide more detailed
soft tissue imaging that can detect a hollow urachal tube, neoplasm, cyst, or abscess.
Treatment
Surgical excision of the urachal remnant is the therapeutic mainstay for symptomatic urachal
disease. Often a two-stage management approach is used, first focusing on treating urachal
