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A case of umbilical cord hemangioma: Doppler studies and review of the literature

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Abstract

Hemangiomas of the umbilical cord are extremely rare benign vascular tumors, not always detected prenatally. They have been associated with increased alpha-fetoprotein (AFP), hydramnios, congenital anomalies, and increased perinatal mortality. Impaired umbilical circulation has been proposed as the predisposing factor for fetal compromise. We report a case of an antenatally detected umbilical cord hemangioma with one artery crossing the tumor, and we reviewed the literature. Close surveillance with Doppler flow studies of the umbilical vessels were carried out throughout the pregnancy. All indices were normal, except from the intra-tumoral part of the umbilical artery under discussion that showed increasing resistance from 32 weeks onwards.

Our review confirmed the reported association with increased AFP and hydramnios. The placental end of the cord was the preferred site of location, and the umbilical artery the commonest vessel of origin. Association with cutaneous vascular malformations, and single umbilical artery were assessed.

Introduction

The true tumors of the umbilical cord consist of hemangiomas and teratomas [1]; the former are by far the more common, and the only one that has been identified prenatally thus far [2]. Hemangiomas have been associated with congenital anomalies, increased maternal serum alpha fetoprotein (MS-AFP), and increased perinatal mortality, in specific intrauterine deaths [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14]. Nevertheless, due to their rarity, with less than 50 cases being reported in the literature, their clinical importance remains uncertain [2], [3], [7], [15], [16].

Impaired umbilical circulation is considered as the predisposing factor for fetal demise [6], [7], [9], [10], [11], [14], and several authors speculated that Doppler flow studies of umbilical vessels can be helpful in managing these cases [10], [17]. However, to our knowledge, no reports with Doppler studies of each umbilical vessel separately have been conducted to show differences in flow resistance, and to confirm possible risks [10], [13], [17].

We present a case of a prenatally diagnosed umbilical cord haemangioma, in an otherwise uncomplicated pregnancy. Close follow up was carried out, with sequential scans to monitor the size and the morphology of the tumor. Furthermore, the umbilical cord circulation was monitored with Doppler flow studies of each umbilical vessel separately, with special interest in the part within the tumor (intra-tumoral). We present our data with review of the relevant literature.

Section snippets

Case report

A 27-year-old woman, gravida 2, para 1, with an otherwise uncomplicated pregnancy was referred to our Department for a routine anomaly scan, at 22 weeks. Her first pregnancy was uneventful, and ended in a vaginal delivery of a healthy female infant weighing 2970 g. Both parents had an unremarkable medical and family history. The first trimester scan was normal and the second trimester biochemical markers were normal as well. The MS-AFP was 68.9 IU/l (1.38 MoM).

The ultrasound machine used was a

Prevalence

The reports of umbilical cord hemangiomas in the English literature are scarce. We were able to identify 35 cases (including our own) [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29], [30], [31], [32], which are summarized (Table 2), and discussed herein. Fourteen of them were detected after delivery [3], [4], [5], [6], [7], [11], [17], [18], [19], [20], [21], either as a coincidental

Conclusion

Hemangiomas are extremely rare benign tumors of the umbilical cord. They are associated with increased MS-AFP and hydramnios, while a predisposition towards female gender might exist. A relationship with increased perinatal mortality does exist, although its power remains to be defined. Impaired umbilical circulation seems to be the contributing factor to fetal compromise. Close follow up is mandatory with Doppler flow studies of all umbilical vessels involved, with special attention to the

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