Table 1. Congenital umbilical AVMs reported until 2016.

Report	Sex, GA	DOL	Principal sign	Size (mm)	Complementary tests	Surgical treatment	Postoperative course
Gregorio, 2016, Spain	F, 34 wk	4	Abdominal murmur	36.6 × 16.6 × 22.3	Chest X-ray: cardiomegaly Echocardiography: volume overload, biventricular dilatation Angio-CT, Doppler US: multiple feeding arteries coming from external iliac, hypogastric, epigastric, and mammary arteries and a dilated umbilical vein	Laparotomy; ligation of umbilical vein and upper feeding arteries from subxiphoid up to supraumbilical level	Uneventful
Gozar, 2014, Germany	F	540	Umbilical pulsatile murmur	45 × 20	Echocardiography: mildly dilated left cardiac chambers and a normal left ventricular function Angio-CT, Doppler US: umbilical arteries, emerging from the hypogastric artery, ascend to the umbilicus and after a tortuous trajectory flow into the umbilical vein	Laparotomy; all vessels and the urachus were ligated and divided. Excision of the tumor	Uneventful
Boglione, 2013, Argentina	M	20	Wet umbilicus and hernia	20	Doppler US: a mass at the end of the umbilical cord harboring dilated blood vessels with turbulent blood flow which continued to the dilated umbilical vein and flowed into the liver. Angio-CT: umbilical arteries coming from both internal iliac arteries, into the mass directly at the end of the umbilical cord, and the dilated umbilical vein flowed out portal vein in the liver	Ligation of umbilical arteries and umbilical vein and excision of the mass	Uneventful
Meyer, 2013, United States	M, 32 wk		Pulsatile umbilical stump	Data not shown	Echocardiography: normal global cardiac function Angio-CT, Doppler US: both umbilical arteries feeding a large cluster of vessels in the umbilical stump, with a widely patent umbilical vein draining the cluster	Laparoscopy, ligation of feeding arteries and draining vein and excision of remaining malformation	Uneventful
Shibata, 2009, Japan	M, 38 + 3 wk	3	Umbilical hemorrhage causing shock and cardiopulmonary arrest	Data not shown	Coagulopathy exams: normal Angio-CT: both umbilical arteries, which were patent from both internal iliac arteries, flowed into the mass directly under the umbilicus, and the enormously dilated umbilical vein flowed out from the mass to the portal vein	Ligation of feeding arteries (two umbilical and one from left abdominal rectus)	No surgical complications. Psychomotor retardation has a consequence of cardiopulmonary arrest
Graham, 1989	M, 35 wk	2	Heart failure	15	Chest X-ray: cardiomegaly Echocardiography: dilated cardiac chambers Cardiac catheterization: structurally normal with pulmonary hypertension Distal aortography: large right umbilical artery, complex AVM, and umbilical vein to patent ductus venosus to IVC	Laparotomy, ligation of arteries and veins and en bloc excision of the entire AVM	Unremarkable
Murray, 1969, United States	M, term	0 (birth)	Heart failure, dilated veins in the abdomen, close to the umbilicus	Data not shown	Chest X-ray: cardiomegaly Aortogram: early venous filling, dilatation of left inferior epigastric artery, and enormously dilated umbilical vein	Ligation of three feeding arteries (two coming from inferior and superior epigastric arteries) and excision of umbilicus and surrounding vessels	Right bundle branch block, feeding intolerance, suspected surgical wound infection

Abbreviations: Angio-CT, computed tomography angiogram; AVM, arteriovenous malformation; DOL, days of life; GA, gestational age; IVC, inferior vena cava.