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

• CHUC (Congenital Hernia of Umbilical Cord)
• Umbilical Cord Hernia

What is Congenital Hernia of Umbilical Cord? (Definition/Background Information)

• Congenital Hernia of Umbilical Cord (CHUC) occurs when intestines remain in the umbilical cord during development of the baby. It presents as a cystic swelling of the umbilical cord. The cystic swelling consists of herniated bowel loops
• In the early fetal life, there is herniation (protrusion) of intestines into the umbilical cord cavity, known as extracelomic cavity, at about 10–12 weeks of gestation. This herniation noted earlier is part of a normal development. During this phase, there is bowel rotation occurring in the extracelomic cavity
• As part of normal fetal development, the umbilical ring closes after the bowel recedes back into the abdominal cavity and the extracelomic cavity in the umbilical cord disappears
• This means that normally there is no intestine persisting in the umbilical cord. Sometimes, due to an abnormal development, the umbilical ring and extracelomic cavity persists, and the intestines remain in the umbilical cord. This results in Congenital Hernia of Umbilical Cord
• With appropriate and early treatment that may involve a surgical correction of the defect, the prognosis of Congenital Hernia of Umbilical Cord is generally good

Who gets Congenital Hernia of Umbilical Cord? (Age and Sex Distribution)

• According to one medical report, the incidence of Congenital Hernia of Umbilical Cord is estimated to be about 1 in 5,000 pregnancies
• A male preponderance of 3:1 male-female ratio is observed
• There are no racial or ethnic preferences observed and the condition is observed worldwide

What are the Risk Factors for Congenital Hernia of Umbilical Cord? (Predisposing Factors)

There are no known risk factors for Congenital Hernia of Umbilical Cord (CHUC), as it is commonly an isolated finding.

• However, a few cases have been reported wherein the condition is associated with persistent vitellointestinal duct (PVID), cloacal anomaly, and abnormal insertion of the umbilical cord
• CHUC has been associated with prematurity (preterm delivery) in many case series
• An association with malrotation of intestines, umbilical cord cysts, and ascites is also sometimes reported

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 Congenital Hernia of Umbilical Cord? (Etiology)

The exact cause of development of Congenital Hernia of Umbilical Cord is unknown.

• The persistence of umbilical ring and extracelomic cavity causes the intestines to remain in the umbilical cord even after 10-12 weeks of gestation
• Unlike omphalocele, CHUC is not associated with any chromosomal anomalies

What are the Signs and Symptoms of Congenital Hernia of Umbilical Cord?

Typically, during the development of Congenital Hernia of Umbilical Cord, there is no significant signs and symptoms noted.

• In Congenital Hernia of Umbilical Cord, the herniated portion may be a small part of the small intestine or large intestine (colon)
• Sometimes, it may be free floating bowel in the amniotic fluid which may get twisted resulting in volvulus with bowel obstruction and intestinal atresia

How is Congenital Hernia of Umbilical Cord Diagnosed?

Congenital Hernia of Umbilical Cord (CHUC) is often a misdiagnosed and under-reported condition. It can be confused for a small omphalocele (or omphalocele minor). It is important to note that CHUC is not the same as umbilical hernia that is diagnosed after birth. The awareness of CHUC by the healthcare professional prevents misdiagnosis and undue stress in the expectant parents.

The diagnosis of Congenital Hernia of Umbilical Cord may involve:

• Physical examination along with an evaluation of one’s medical history: The healthcare provider may check for any signs of fetal distress (such as non-reassuring fetal heart rate)
• Prenatal abdominal ultrasound may reveal the condition and other associated fetal abnormalities, as the condition occurs during early gestation and can be detected by USG in around early second trimester; this may show certain characteristic features of the condition

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 Congenital Hernia of Umbilical Cord?

The complications of Congenital Hernia of Umbilical Cord may include:

• The condition can cause anxiety in the parents
• Bowel obstruction due to volvulus
• Bowel perforation, which can occur antenatally of after delivery
• Adherent perforated Meckel’s diverticulum
• Peritonitis or inflammation of the peritoneum
• Cord hematoma with increased risk of fetal death
• Clamping of the umbilical cord during delivery may cause damage to the herniated bowel within the umbilical cord

How is Congenital Hernia of Umbilical Cord Treated?

• Close fetal monitoring is necessary during pregnancy and is very important following a diagnosis of Congenital Hernia of Umbilical Cord
• After delivery, the newborn infant may be placed in an intensive care; a surgery to correct the defect may also be planned
• Surgical repair of hernia termed umbilicoplasty: The surgical procedure may be performed as early as possible, when the infant is in a stable condition

How can Congenital Hernia of Umbilical Cord be Prevented?

• Currently, there are no definitive methods available to prevent Congenital Hernia of Umbilical Cord
• A careful and periodic monitoring of the pregnancy is advised and recommended

What is the Prognosis of Congenital Hernia of Umbilical Cord? (Outcomes/Resolutions)

• The outcome of Congenital Hernia of Umbilical Cord is excellent in most cases, as there is seldom any association with other congenital or chromosomal anomalies. However, this also depends on the presence of associated complications and their management
• In a majority, the condition is benign, and the prognosis is very good with treatment (usually uncomplicated with no significant issues being noted)

Additional and Relevant Useful Information for Congenital Hernia of Umbilical Cord:

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

https://www.dovemed.com/diseases-conditions/pregnancy-related-disorders/