Prenatal ultrasonographic diagnosis of abdominal cysticlymphangioma: A case report

SUSANA SANTO, JOAO PAULO MARQUES, PATRIZIA VECA, ANTONIETA MELO, &

LUIS MENDES DA GRACA

Ultrasound Unit of the Department of Obstetrics, Gynecology and Reproductive Medicine, Hospital Santa Maria, Lisbon,

Portugal

(Received 6 February 2008; accepted 13 April 2008)

Abstract

Abdominal lymphangioma is a rare tumor of the lymphatic vessels. A case of an abdominal cystic lymphangioma identified at22 weeks of gestation is reported. Ultrasonographic monitoring showed a progressive increase of mass size during thegestation. Pregnancy was terminated at 38 weeks and the newborn was submitted to a laparotomy with resection of all cysticstructures. At the present time the infant is three years old and is doing well.

Keywords: Abdominal lymphangioma, prenatal diagnosis

Introduction

Abdominal lymphangioma is a very rare tumor of the

lymphatic vessels that can occur in the mesentery of

the small bowel, in the omentum, or in the retro-

peritoneum [1]. They are commonly diagnosed in

the neonatal period or in early infancy [2].

Case report

A 26-year-old woman had an unremarkable

pregnancy until 22 weeks, when an ultrasound

(US) scan revealed a right-sided abdominal fetal

mass of 23 6 18 mm. It had an anechogenic and

septate appearance with thin and irregular borders,

suggesting the diagnosis of an abdominal cystic

lymphangioma. There was no evidence of other

anomalies. The fetal karyotype was 46,XX.

Serial US scans showed an increasing mass size

and an increasing number of septa. At 37 weeks the

multilocular and multiseptate mass (Figure 1)

measured 78 6 58 6 77 mm extending from the

infrahepatic region to the vesical area. Magnetic

resonance imaging (MRI) findings supported the

ultrasonographic ones. After discussion with the

perinatology committee, an elective cesarean section

was performed at 38 weeks and a female infant was

delivered weighing 3520 g, with Apgar scores of 9

and 10 at 1 and 5 minutes, respectively.

Neonatal US scan confirmed the prenatal findings.

On day six the newborn was submitted to a laparotomy

with resection of all cystic structures (Figure 2). The

postoperative period was unremarkable.

Histopathologic examination confirmed the diag-

nosis of mesenteric cystic lymphangioma. At the

present time the infant is three years old and is doing

well.

Discussion

Although considered benign tumors of the lymphatic

vessels, lymphangiomas have the potential to infil-

trate surrounding structures [2]. It is thought that

they result from an abnormal development of the

lymphatic system, by the sixth week, which prevents

the drainage of the lymphatic fluid and leads to its

collection in cysts [1,2].

Lymphangiomas are diagnosed by ultrasonogra-

phy and they present as a hypoechogenic, cystic, uni-

or multiloculated mass, well delineated and with fine

septations [3]. It is estimated that 50% to 65% of the

lymphangiomas are present at birth with 90% of

Correspondence: Susana Santo, Rua Camilo Pessanha n811 r/c esq, 1700-084 Lisbon, Portugal. Tel: þ351 966507377. E-mail: sf_santo@sapo.pt

The Journal of Maternal-Fetal and Neonatal Medicine, August 2008; 21(8): 565–566

ISSN 1476-7058 print/ISSN 1476-4954 online � 2008 Informa UK Ltd.

DOI: 10.1080/14767050802165927

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them manifesting before the second year of life [4],

so it is possible to make the prenatal diagnosis in a

considerable number of cases. MRI can be per-

formed to define more accurately the mass size and

localization, and to allow the differential diagnosis

with intestinal anomalies, ovarian cysts, hydrome-

trocolpos, urachal cysts, obstructive uropathy, anor-

ectal atresia, persistent cloaca, and mesenteric or

retroperitoneal cysts [3]. Lymphangiomas may be

associated with chromosomal abnormalities and fetal

karyotyping is recommended [3].

Ultrasonography had an important role in the

prenatal diagnosis and management of the abdom-

inal lymphangioma of our case. In fact, it allowed not

only the identification of the mass but also the

definition of its localization and extent. US monitor-

ing provided crucial information for perinatal

management, delivery decision, and parental coun-

seling. The treatment of lymphangiomas is surgical.

Our case highlights the good prognosis of large

lymphangiomas that are circumscribed to the abdo-

men. However they can recur and long-term follow-

up is always advisable [3].

Deshpande et al. [2] reviewed six cases of

abdominal lymphangiomas described in the litera-

ture until 2001: three of them presented as masses

confined to the abdomen and all had a favorable

outcome; the other three invaded the lower limbs

and pregnancy was terminated at the beginning of

the second trimester. Therefore we believe that

therapeutic abortion should not be proposed for

congenital abdominal lymphangiomas, particularly if

they remain confined to the abdomen.

Declaration of interest: The authors report no

conflicts of interest. The authors alone are respon-

sible for the content and writing of the paper.

References

1. Salvador A, Rosenberg HK, Horrow MM, Kumar SL.

Abdominal lymphangioma in a preterm Infant. J Perinatol

1996;16:305–308.

2. Deshpande P, Twining P, Neil O. Prenatal diagnosis of fetal

abdominal lymphangioma by ultrasonography. Ultrasound

Obstet Gynecol 2001;17:445–448.

3. Devesa R, Munoz A, Torrents M, Carrera JM. Prenatal

ultrasonographic findings of intra-abdominal cystic lymphan-

gioma: a case report. J Clin Ultrasound 1997;25:330–332.

4. Bill AH, Summer DS. A unified concept of lymphangioma and

cystic hygroma. Surg Gynecol Obstet 1965;120:79–84.

Figure 1. Transverse section of the abdomen showing a

hypoechogenic, multiloculate, and septate mass.

Figure 2. Abdominal mass at surgery.

566 S. Santo et al.

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