An Angiomyxoma Involving the Whole Length of the Umbilical Cord Prenatal Diagnosis by Ultrasonography Eric Jauniaux, MD, Gonzalo Moscoso, MD, Lyn Chitty, MD, PhD, MRCOG, Donald Gibb, BSc, ChB, MRCOG, Marie Driver, FRCPath, Stuart Campbell, FRCOG The discovery of an umbilical cord angiomyxoma or hemangioma is a rare event. Heifetz and Rueda·Pedraya have found 19 pathologic descriptions of this tumor published in the world literature since the beginning of this century. 1 Improvement and wide use of ultrasound techniques in antenatal care have made the prenatal diagnosis of this lesion possible. 2 This report describes an unusually extended angio- myxoma with progressive shortening of the umbilical cord associated with raised maternal serum alpha-feto- protein and superficial angiomas of the infant's skin. The lesion was followed by ultrasound from 20 weeks gestation until delivery. CASE REPORT A 38-year-old white primigravida was referred at 20 weeks of gestation for a level- 11 sonographic examination after ele- vated maternal serum alpha-fetoprotein levels (3.3 and 3.1 multiples of the median at 16 and 18 weeks gestation, respec- tively). Detailed ultrasound examination showed a single, live, anatomically normal fetus. A heterogeneous mass composed Received August 28, 1989, from the Department of Obstetrics and Gynecology and Fetal and Perinatal Pathology Unit, King's College Hospital, King's College School of Medicine and Dentistry, Denmark Hill, London, United Kingdom. Revised manuscript accepted for pub- lication November 16, 1989. Address correspondence and reprint requests to Dr. Jauniaux: De- partment of Obstetrics and Gynecology, Academic Hospital Erasme, 808 route de Lennik, 1070 Brussels, Belgium. of a central echodense zone surrounded by a peripheral hypo- echoic multicystic area was observed at the placental insertion of the umbilical cord. Sequential ultrasound examinations from 22 weeks to the end of pregnancy demonstrated progres- sive involvement of the rest of the cord by the same process and the development of a second hypoechoic multicystic area (Figs. 1 and 2). The fetal growth pattern and blood flow veloc- ity waveforms of both the umbilical and uteroplacental circu- lations remained normal. The fetus showed no evidence of hydrops., and the amniotic fluid volume remained within nor• mal limits. At 34 weeks gestation the length of the cord was estimated by ultrasound to be less than 30 em. In view of these findings vaginal delivery was considered hazardous. At 37 weeks gestation a 3,620 g male infant was delivered by cesar· ean section without difficulty. The Apgar scores were 7 and 9 at 1 and 5 minutes of life, respectively. The maternal postoper- ative course was uneventful. Examination of the neonate showed portwine, flat superfi- cial hemangiomas on the abdomen, the legs, and a few areas of the face. His physical examination was otherwise normal and his hematological constants were within normal limits. Spon- taneous dropping of the 2-em length of fetal umbilical cord did not occur until39 days after delivery. Abdominal ultrasound of the infant performed at that time was unremarkable. On gross examination, the placenta was oval, measuring 22 X 23 X 4 em and weighing 885 g. The cord measured 26 em in length, varying between 2 and 8 em in diameter, and it weighed 540 g (Fig. 3). At 4 em from its placental insertion and at 2 em from its fetal insertion, two fusiform swellings of 10 X 12 X 13 em and 6 X 6 X 8 em, respectively, were observed (Fig. 3). These two cord expansions contained a gelatinous mucoid substance (pseudocyst). Histologically, they corre· sponded to areas of edematous Wharton's jelly. Microscopic examination of serial sections of the cord showed a rich prolif· © 1990 by the American Institute of Ultrasound in Medicine • J Ultrasound Med 9:419· 422, 1990 • 0278-429ij'90f$3.50