What Evaluation Should be Done for a Child with a Single Umbilical Artery?

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Patient Presentation
A newborn male was in the newborn nursery. He was born full-term to a healthy 26-year-old gravida 1 parity 0 female. There were no complications in the pregnancy or delivery.
His pertinent physical exam shows him to be an average for gestational age male. His examination is normal except that a single umbilical artery is noted.
The diagnosis of a healthy full-term infant with a single umbilical artery was made. He was discharged to home on day 2 of life. A renal ultrasound was scheduled for the following week.

Discussion
The umbilical cord usually has 2 arteries, 1 vein, the allantois, the omphalomesenteric duct remnant and Wharton’s jelly. It is spiraled (usually to the left), and is approximately 55 centimeters in a term infant. It usually detaches around 1 week of life and is entirely healed by about 2 weeks of life.
The arteries and vein still have some patentcy until ~1 month of life when they become the lateral umbilical ligaments and liamentum teres.

There are several common umbilical cord problems:

  • Congenital omphalocoele – a herniated peritoneal sac at the base of the umbilicus. It is often associated with Tristomy 13, 18, cardiac anomalies and Beckwith-Wiedemann syndrome (which also has macrosomia and hypoglycemia). Immediate treatment includes covering the omphalocoele with saline gauze or other wet protectant and a surgical consultation. A glucose and chromosomal analysis are also indicated.
  • Delayed separation of the cord – persistence of the cord more than 4-6 weeks may indicate a congenital leukocyte adherence defect (an autosomal recessive disorder of neutrophil adherence). Delayed separation is also associated with a higher risk of infection including Staphlococcus, Escherichia coli, Aspergillus and Candida. Treatment of infections and an evaluation for leukocyte adherence defect are indicated.
  • Granuloma – persistence of granulation tissue after 2 weeks. This is associated with bacterial invasion of the tissue, inadequate epithelialization and formation of granular, erythematous, seropurulent tissue. Treatment with silver nitrate cauterization and frequent alcohol applications is recommended.
  • Hemorrhage – usually due to inadequate ligation, but also due to sepsis, coagulation defects, or hemorrhagic disease of the newborn. Treatment depends on the cause.
  • Hernia – caused by incomplete closure or laxity of the umbilical ring. This is more common in dark-skinned individuals. Most resolve by 6 -12 months of age. They can spontaneously close by up to 6 years of age, but the older the child or the greater the hernia size, the less likely spontaneous closure will occur. Treatment includes observation and surgery if strangulation occurs which is rare. Surgery may also be indicated if the hernia is enlarging or the child is more than 5-6 years old.
  • Omphalitis – usually this is due to Staphlococcus aureas and Escherichia coli. The localized infection can quickly spread to the peritoneum and blood stream. Treatment includes antibiotics and possibly surgical drainage of abscesses.
  • Persistent omphalomesenteric duct – a lesion associated with an intestinal fistula and Meckel’s diverticulum. A Meckel’s scan or fistulogram may be indicated for diagnosis
  • Persistent urachus – a lesion resulting from inappropriate closure of the allantoic duct that is associated with bladder communication. Urinary fluid drainage is seen. A bladder and/or renal ultrasound often demonstrates the lesion.
  • Polyp – partial or complete present of omphalomesenteric duct or urachus. This presents as a pedunculated firm, red mass often with a mucoid secretion. This requires surgical excision.
  • Tumors – these are rare and most are benign. The differential diagnosis includes urachal cyst, omphalomesenterid duct cyst, angioma, dermoid cyst or myxosarcoma.

Learning Point
About 1% of all infants have a single umbilical artery and of those, ~15% have one or more congenital anomalies. These often involve the gastrointestinal, genitourinary, pulmonary, cardiovascular or nervous systems.
Occult renal abnormalities are common in children with single umbilical arteries and a significant proportion have Grade II or higher vesicoureteral reflux. Therefore, a renal ultrasound is a recommended screening procedure. A voiding cystourethrogram is sometimes also ordered. If other anomalies are found on careful physical examination, a chromosomal analysis should be considered.
This patient did not have a chromosomal analysis at this time as he did not have any other signs of congenital anomalies.

Questions for Further Discussion
1. What are the potential problems of a short umbilical cord?
2. What are the potential problems of a long umbilical cord?

Related Cases

    Symptom/Presentation

      Abnormal Physical Finding

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Information prescriptions for patients can be found at Pediatric Common Questions, Quick Answers for this topic: Birth Defects.

Clemente CD. Anatomy. 2nd Edit. Urban&Schwarzenberg Baltimore. 1981.

Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:62,89-90, 1553.

Srinivasan R, Arora RS. Do well infants born with an isolated single umbilical artery need investigation? Arch Dis Child. 2005;90:100-1.

Author
Donna M. D’Alessandro, MD
Associate Professor of Pediatrics, Children’s Hospital of Iowa

Date
May 16, 2005

Published