What are the other Names for this Condition? (Also known as/Synonyms)

• Congenital Cyst of Urachus
• Congenital Urachal Cyst

What is Urachal Cyst? (Definition/Background Information)

• The urachus is a tubular structure that forms during early fetal development. This structure connects the anterior portion (anterior bone) of the urinary bladder to the umbilicus. Usually, the urachus closes by week 12 of gestation, leaving behind a thick fibrous band. However, rarely, some portions of the urachus may not close completely, resulting in a variety of urachal abnormalities
• A Urachal Cyst forms occurs when the urachus closes near the bladder and umbilicus, leaving the middle portion of the urachus patent (or open). There is no communication at either end. A cyst usually develops in the lower one-third of the urachus
• The cysts are usually small and asymptomatic; but, occasionally, they may become infected. When a Urachal Cyst becomes infected, the communication to umbilicus or urinary bladder that was previously closed may open up
• Even though Urachal Cyst is a congenital occurrence; in many cases, it may remain symptomless and thus, may be undiagnosed throughout one’s life. When symptoms develop, they are known to do so frequently in later childhood and adulthood
• The signs and symptoms of Urachal Cyst often arise due to infection and may include fluid discharge from the umbilicus, fever, and abdominal pain. There is an elevated risk for recurrent urinary tract infections
• Symptomatic Urachal Cyst may be surgically resected, either through an open approach or a laparoscopy. The prognosis of isolated urachal anomalies (i.e., not associated with any other congenital conditions) is typically good with adequate treatment

There are five forms of congenital urachal remnant abnormalities, and these include:

• Alternating sinus of urachus (some experts recognize this as a type of umbilical-urachal sinus)
• Patent urachus
• Umbilical-urachal sinus
• Urachal Cyst
• Vesicourachal diverticulum

Among these urachal abnormalities, patent urachus is the only condition that predominantly presents symptoms following birth of the child (early infancy).

Who gets Urachal Cyst? (Age and Sex Distribution)

• According to literature, Urachal Cyst constitutes between 31 to 43% of all congenital urachal abnormalities
• The condition is more often seen in slightly older children and adults
• It can occur both in males and females
• No specific racial or ethnic group predilection is noted

What are the Risk Factors for Urachal Cyst? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for the formation of Urachal Cyst

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Urachal Cyst? (Etiology)

Before birth, the urachus is a primitive structure that connects the umbilical cord to the bladder in the developing baby. The urachus helps in removal of nitrogenous wastes from the fetus through the placenta via the umbilical cord. The remnant fibrous band of urachus is called the medial umbilical ligament.

• The urachus is generally between 3-10 cm in length and 1 cm in diameter. When the urachus disappears normally around the 12^th week of fetal development, a small fibrous cord (median umbilical ligament) remains between the bladder and the umbilicus
• The urachus normally disappears before birth; but occasionally, it remains even after the child is born. This can lead to urachal abnormalities such as Urachal Cyst. The exact reason why this occurs is unknown
• Urachal Cyst may be described as an abnormal focal dilatation of the urachus (i.e., the urachus becoming fluid-filled and large in size), when both ends of the urachus are closed or sealed

What are the Signs and Symptoms of Urachal Cyst?

Urachal Cyst usually does not present any signs and symptoms in babies and young children. However, in older children and adults, infected Urachal Cysts may present associated signs and symptoms that may include:

• Abdominal pain
• Presence of an abdominal mass, particularly when the cysts are large in size
• Discharge of fluid, blood, or infected material from the umbilicus; wetness around the umbilicus
• Blood in urine
• Pyuria, or pus in the urine
• Pain while urinating
• Fever, due to infection
• Rarely, if it is associated with urinary tract infections, then the following signs and symptoms may be noted:
  • Changes in urination frequency
  • Burning sensation or pain during urination
  • Fever and chills
  • Changes in urine smell and color
  • Sometimes, pain during sexual intercourse

How is Urachal Cyst Diagnosed?

When Urachal Cyst is asymptomatic, it may be detected incidentally on ultrasound scans that are performed for other medical conditions. In other cases, the following tests and exams may be ordered to diagnose the condition:

• Physical examination and evaluation of medical history
• Ultrasound studies: Prenatal abdominal ultrasound may reveal associated fetal abnormalities, if any
• A voiding cystourethrogram (VCUG) is performed to look for urinary tract abnormalities
• Fistulogram: A special X-ray procedure using a contrast dye to analyse cysts and fistulas
• Computed tomography (CT scan) or magnetic resonance imaging (MRI scan) of the affected region, if necessary

The healthcare provider may undertake a differential diagnosis to eliminate other conditions or tumor types, before arriving at a definitive diagnosis. This may include the following:

• Bladder diverticulum
• Meckel diverticulum
• Mesenteric cyst
• Umbilical hernia
• Urinary bladder adenocarcinoma
• Vitelline cyst

According to a particular case study (among pediatric autopsies), an incidence of 1 in 5,000 was reported for Urachal Cyst.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Urachal Cyst?

The complications of Urachal Cyst may include:

• Swelling, pain, and discharge from the umbilicus (belly button)
• An establishment of abnormal connection between the cyst with other tissues, such as the urinary bladder or colon
• Inflammation of urinary bladder (cystitis), causing severe pain
• There is a small risk for urachal cancer; and in such cases, the entire urachus may be surgically removed

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Urachal Cyst Treated?

The healthcare provider may recommend a ‘wait and watch’ approach for asymptomatic cases of Urachal Cyst. In some cases, the urachal abnormalities are known to resolve on their own, in the absence of any treatment.

• In case of symptoms, surgical resection and removal of the urachal remnants, by either open surgery or a laparoscopic approach may be considered
• If there is an infection associated with the Urachal Cyst, then the infection is treated prior to any surgical corrections are undertaken
• A drainage of the cysts may result in its recurrence; hence, the symptomatic cysts are typically removed following drainage of its contents

Children below 12 months or age are usually treated conservatively; surgical procedures are typically considered in older children and adults.

How can Urachal Cyst be Prevented?

Presently, Urachal Cyst may not be preventable, since it is a congenital condition.

• Active research is currently being performed to explore the possibilities for treatment and prevention of such conditions
• A careful and periodic monitoring of the pregnancy is advised and recommended

What is the Prognosis of Urachal Cyst? (Outcomes/Resolutions)

• The prognosis for Urachal Cyst in isolation is generally good with appropriate treatment
• In many individuals, no treatment may be needed, since the cysts may not be associated with any symptoms or complications
• A surgical removal of the urachal remnant in symptomatic cases can result in a cure

Additional and Relevant Useful Information for Urachal Cyst:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/kidney-health/