Umbilical Arteriovenous Malformation

Nick Kennedy

Loyola University Medical Center

Dr. F Prendergast

Children's Memorial Hospital

Abstract

2 week old boy born at 32 week gestation found to have a visible and palpable pulsatile umbilical hernia.

Keywords

1615 Arteriovenous Malformation Umbilical artery Umbilical vein


Publication Date: 2010-04-19

History

2 week old boy born at 32 week gestation found to have a visible and palpable pulsatile umbilical hernia.

Findings

Contrast enhanced CT demonstrates patent bilateral umbilical arteries leading to a tangled mass of enhancing vessels in an umbilical hernia with an enlarged patent draining umbilical vein.

The volume rendered images exquisitely depict the normal course of an umbilical venous catheter (umbilical vein - left portal vein - ductus venosus - IVC - right atrium) and an umbilical arterial catheter (umbilical artery - internal iliac artery - common iliac artery - descending aorta).

Diagnosis

Umbilical Arteriovenous Malformation

DDx

None

Discussion

Umbilical arteriovenous malformations are rare congenital or acquired abnormal connections between the umbilical arteries and vein. Acquired arteriovenous malformations can rarely be seen secondary to umbilical vessel catheterization. Additional feeding vessels may also arise from other intra-abdominal sources including visceral and inferior epigastric arteries.

Clinical presentation is usually with a visible or palpable pulsatile umbilical mass. An umbilical arteriovenous malformation is a rare cause of high output cardiac failure in a newborn secondary to high flow through a fistula. The brain (Vein of Galen Malformation) and liver (hemangioendothelioma) are much more common locations for such shunts. Consumptive coagulopathy and bowel ischemia can also occur.

Ultrasound should be the initial radiologic evaluation given the lack of radiation exposure and ability for real time tailoring of the examination. Doppler interrogation will demonstrate patent umbilical arteries and veins, an abnormal post-natal finding. There may also be abnormal arterial waveforms in the umbilical vein. Contrast enhanced cross sectional imaging is often times needed to evaluate the full extent of the malformation and determine if other critical vessels are involved, such as visceral arteries, which may result in bowel compromise during surgical resection.

Definitive treatment involves surgical ligation of all feeding arteries and draining veins with complete removal of the malformation nidus. Endovascular coiling can be attempted, but becomes technically challenging in the very tiny newborns.

References

  1. Shibata M, Kanehiro H, Shinkawa T, et al. A neonate with umbilical arteriovenous malformation showing hemorrhagic shock from massive umbilical hemorrhage. Am J Perinatol 2009; 26: 583-586.
  2. Cox TD, Winters WD, Holterman MJ, et al. Neonatal bowel ischemia attributable to an umbilical arteriovenous fistula: imaging findings. AJR 1995; 165: 940-942.
  3. Graham SM, Seashore JH, Markowitz RI, et al. Congenital umbilical arteriovenous malformation: a rare cause of congestive heart failure in the newborn. J Pediatr Surg 1989; 24:1144-5.
  4. Richards DS, Lutfi E, Mullins D, et al. Prenatal diagnosis of fetal disseminated intravascular coagulation associated with umbilical cord arteriovenous malformation. Obstetrics and Gynecology 1995; 85: 860-862.
  5. Schlesinger AE, Braverman RM, DiPietro MA. Neonates and Umbilical Venous Catheters: Normal Appearance, Anomalous Positions, Complications, and Potential Aid to Diagnosis. AJR 2003; 180: 1147-1153.

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