Case of the Week #579

Parker S. Sheldon; Roxanna Twedt; Garrett Lam; David N. Jackson
High Risk Pregnancy Center, Hera Women's Health (Las Vegas and Reno, Nevada USA)

Posting Dates: Mar 31, 2023 - Apr 14, 2023

Case report: A 35-year-old multiparous patient was referred for a consultative ultrasound. Serial ultrasounds were performed between 23 and 38 weeks gestation. The following images were obtained.

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We present a case of Umbilical Cord Hemangioma

Ultrasound identified an echogenic mass at the umbilical cord insertion, extending up the cord toward the fetus measuring 7.6cm x 7.1cm x 4cm in diameter. Three-dimensional imaging confirmed a central solid tissue nodule and multiple pseudocysts. Doppler indices were without increased resistance. A small umbilical varix was seen. Serial weekly imaging (with biophysical profile and Doppler of the umbilical artery) identified a progressive mass expansion and polyhydramnios development. At 31 weeks, the fetus developed moderate cardiomegaly, mild pericardial effusion, and an altered E/A ratio. At that point, the mass diameter was 12.9cm x 9.6cm x 5cm. Monitoring was increased to twice a week. The mass and cardiac findings stabilized. There were no abnormalities on monitoring or Doppler. She was delivered vaginally at 38 weeks. Mother and baby did well. The child did not require any surgical interventions.

On pathologic examination, the placenta weighed 1187g with a 15.0 x 10.0 x 5.0cm tumor near the umbilical cord insertion at the placenta. The gross pathologic evaluation revealed a dense mass with rubber-like consistency and pseudocyst formation. Histologically there were thin-walled, dilated vascular channels distributed in a myxoid matrix, characteristic of a true hemangioma of the umbilical cord.

**Image 1** Cord insertion with pseudocyst and tumor with firm, rubber consistency at the base.

**Image 3** Central dense mass with thin-walled, dilated vascular channels distributed in a myxoid matrix on histology.

Discussion

Umbilical cord hemangiomas are rare tumors of the umbilical cord that require serial evaluation and monitoring when detected on prenatal ultrasound. Since 1951, 56 cases of umbilical cord hemangiomas have been reported in the literature. While most cases result in a live birth, evidence suggests that at least a quarter end in demise and approximately 20% in preterm birth. Reports vary regarding the incidence of maternal complications, growth of the mass, integrity of cord and placental vasculature, amniotic fluid indices, as well as the presence of fetal cardiac dysfunction and other associated anomalies.

While hemangiomas of the placenta are well known, identified in an estimated 1% of pregnancies [1], hemangiomas arising within the umbilical cord are far less common, with only 40 cases published since 1990, 33 of which were identified prenatally. These vascular neoplasms typically originate from the umbilical artery. Histologically, they are characterized by endothelial proliferation and present with a distinct pattern of capillaries embedded within myxoid stroma [2,3]. On examination, the lesion presents as a fusiform swelling comprised of vascularized nodules and pseudocysts that cause expansion of Wharton’s jelly manifesting as cord edema [2,4,5]. Progressive cord edema and pseudocyst degeneration may cause mechanical compression of cord vessels, compromising fetal circulation, and leading to associated complications which include fetal cardiac dysfunction, non-immune fetal hydrops, intravascular thrombosis, fetal hemorrhage, and intrauterine fetal demise [3,5]. Furthermore, excessive, closely approximated, low resistance vasculature can lead to arteriovenous malformation, which can further contribute to the development of hydropic changes and cardiac dysfunction [3]. Umbilical cord hemangiomas have also been associated, to varying degrees, with increased maternal serum alpha-fetoprotein levels, other vascular anomalies, polyhydramnios, and preterm birth [6].

Umbilical cord hemangiomas should be suspected when antenatal sonography reveals a hyperechoic mass with or without cystic features, often containing branching, low-resistance vessels [2]. Differential diagnoses include hematomas, varicose veins, aneurysms, thrombosis, abdominal wall hernia, omphalomesenteric duct cysts, allantoic cysts, and tumors such as teratoma and metastatic neuroblastoma [7]. The presence of blood flow on color Doppler with associated uterine artery hypoplasia or the presence of calcifications within the mass can help differentiate hemangioma from teratomas [4,5]. Three-dimensional imaging may improve the spatial orientation of the size, location, and internal characteristics of cord swelling [8].

In our literature review, 33 cases were identified prenatally at an average of 23.7 weeks gestation (Table 1) [2,4,5,7-36]. In the 28 cases that specified tumor location, umbilical cord hemangiomas were found within 10cm of the placenta in 50% of cases, within 10cm of the fetal insertion in approximately 40% of cases, and the remainder were in the mid-cord. These tumors were found to enlarge in nearly 40% of cases, and cystic structures were present in almost half. Umbilical cord hemangiomas are usually an isolated lesion, without major structural anomalies, however, they have been associated with vascular malformations. In our literature review, nearly 25% of umbilical cord hemangiomas were found in patients with cutaneous hemangiomas, visceral hemangiomas, and even Klippel-Trenaunay-Weber syndrome [9,11,18,20,22,30,32,34]. While umbilical cord hemangiomas have been associated with alterations in amniotic fluid, this was not often documented in the reports, and we identified oligohydramnios in 9%, and polyhydramnios in 15%. In cases identified prenatally, 40% resulted in preterm birth and 18% were found to have cardiac failure or dysfunction. In all, fetal survival was approximately 80%.

Table 1Literature review includes 33 prenatally diagnosed cases of umbilical cord hemangioma since 1990.
Study	GA	Location	Growth	Cysts	AFP	Amniotic fluid	Preterm birth	Hydrops / heart failure	Survival
GA: gestational age at diagnosis; AFP: maternal alpha fetoprotein; Y: yes; N: no; NA: not available/applicable; oligo: oligohydramnios; poly: polyhydramnios; IUFD: intrauterine fetal demise; TOP: termination of pregnancy
Jauniaux 1990	20w	placenta	Y	Y		normal	N	none	Y
Ghidini 1990	34w	10cm from fetal	N	Y	normal		N		Y
Gramellini 1993	26w	placenta	N			poly	36w	none	Y
Weyerts 1993	33w	fetal	N		normal		N		Y
Wilson 1994	17w	NA	Y	Y		oligo	36w		Y
Armes 1994	32w	9cm from placenta				poly	34w		N (perinatal)
Shipp 1995	33w	placenta				oligo	36w		Y
Miller 1997	15w	fetal			↑		N		Y
Tennsteadt 1998	18w	fetal	Y	Y	normal		36w		Y
Kamitomo 2000	16w	placenta		Y	↑		NA		IUFD 17w
Sahinoglu 2003	24w	NA				normal	26w		N (perinatal)
Caldarella 2003	32w	placenta			normal		32w	hydrops, cardiomegaly	Y
Daniel-Spiegel 2005	27w	10cm from placenta	Y	Y	normal	oligo	N		Y
Cheng 2006	19w	placenta	Y		↑		N		Y
Iyoob 2006	30w	placenta		Y		poly	30w	heart failure	Y
Natalucci 2006	33w	fetal			↑		N		Y
Malliah 2007	19w	placenta		Y		poly	N		Y
Papadopoulos 2009	22w	22cm from placenta	Y		normal		37w		Y
Gouin 2009	25w	NA				normal	N		Y
Sathiyathasan 2010	22w	NA					NA		IUFD 26w
Matsuda 2011	18w	fetal	Y	Y			N		Y
Hara 2015	26w	placenta	Y		↑		29w	heart failure	Y
Smulian 2016	25w	fetal					36w	hydrops	Y
Akiba 2017	19w	fetal	N	Y			N		Y
Rocha 2017	23w	NA				normal	N		Y
Schwickert 2018	38w	placenta	N				N		Y
Angelico 2019	34w	20cm from placenta	Y	Y			36w		Y
Yu 2020	17w	fetal	N	Y		normal	NA	pericardial effusion	TOP
Zhang 2021	22w	placenta	Y	Y			34w		Y
Hadravská 2021	22w	placenta	N		normal	poly	NA	hydrops	IUFD 28w
Kaur 2022	13w	fetal	Y	Y			33w		Y
Lok 2022	21w	fetal	Y				NA		IUFD 21w
Lisovaja 2022	24w	mid-cord	Y	Y			N		Y

The optimal management of a patient with cord swelling or mass is not clearly defined. Since 1990, serial surveillance has been suggested once a suspected umbilical cord hemangioma is detected on ultrasound [4]. Three decades later, there is no standard for frequency or type of antepartum assessment. Our patient underwent weekly ultrasound examinations and monitoring with biophysical profile. Despite progressive mass enlargement and development of polyhydramnios, there were no concerning findings in the cord vessel dynamics and no fetal distress. Upon detection of fetal cardiac changes at 31 weeks, we increased monitoring to twice weekly with non-stress testing and umbilical artery Doppler assessment. No flow abnormalities were detected and fetal cardiac findings stabilized. Despite a large mass confirmed at delivery, there were no variable decelerations in labor.

Umbilical cord hemangiomas should be suspected when a hyperechoic mass is found along the fetal umbilical cord. As a rare tumor with solid and pseudocystic changes, serious maternal and fetal complications may occur, including preterm birth and fetal demise. While labor may be tolerated, we recommend at least weekly ultrasound surveillance from the time of initial detection to monitor for enlargement, cord vessel compression, thrombus, hemorrhage, polyhydramnios, as well as fetal hydrops and cardiac dysfunction.

References

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