Umbilical cord hemangioma associated with multiple cutaneous hemangiomas in a newborn

Abstract

Umbilical cord hemangiomas are rare with fewer than 50 cases reported in the English literature. Associated fetal cutaneous hemangiomas are exceptional. A 39 year old G3P1021 underwent ultrasound at 12 weeks. No abnormalities were detected. She underwent another ultrasound at 19+ weeks. A 1.8×1.9 cm homogeneous echogenic mass was noted at the base of the cord where it inserted into the placenta, interpreted as a hemangioma. Adjacent to the mass were two cystic structures, possibly pseudocysts, measuring 8.9×6.6 cm and 8.7×5.6 cm. Amniocentesis showed normal male karyotype. Serial ultrasounds revealed polyhydramnios and persistence of the mass. She presented at 37 weeks, in labor and a low transverse cesarean section was performed. A live 3455 gram male was delivered with Apgar scores of 9.9. The newborn had numerous port-wine stains involving the face bilaterally including the eyelids, neck, posterior occiput, superior chest, left shoulder, buttocks, and bilateral extremities. At 2 days old, the baby experienced mild tremors which resolved. Brain imaging revealed no evidence of brain parenchymal, leptomeningeal, or vascular abnormality, although the extradural space in the area of the confluence of sinuses showed a heterogeneous prominence, possibly a hemangioma. The child was discharged 5 days after delivery for follow-up. The placenta weighed 420 g. The trivascular umbilical cord measured 15 cm in length and showed fusiform dilation up to 7.3 cm in maximum diameter, over a length of 8 cm, beginning 1.2 cm from the site of placental insertion. A separate multicystic myxoid area was present adjacent to the maximally dilated cord on the fetal side (Fig. 1). Histologically, the lesion was consistent with hemangioma. Cord hemangioma, although rare, is the most common umbilical cord tumor. There is a significant morbidity and mortality association, including prematurity and fetal death [1]. Associated fetal anomalies include cutaneous and systemic hemangiomas [2,3]. Cutaneous hemangiomas are the most common benign tumor of infancy. Although most remain asymptomatic, some can cause significant morbidity. Our case shares some features with Sturge–Weber and Klippel–Trenaunay–Weber Syndromes but does not quite fit either. Interestingly, this case showed polyhydramnios which is infrequently seen in association with umbilical cord hemangioma [2]. Patients should be counseled of the risk for neonatal mortality and morbidity, including cutaneous or visceral hemangioma when umbilical cord hemangioma is diagnosed prenatally. Antepartum fetal surveillance may be of benefit.