Surgical Science, 2010, 1, 20-23
doi:10.4236/ss.2010.11004 Published Online July 2010 (
Copyright © 2010 SciRes. SS
Isolated Mediastinal Lymphangioma: Pr enatal Diagnosis
and Thoracoscopic Treatmen t
Varlet F.1, Guye E.1, Varlet M. N.2, Tronchet M.2, Maria t G.3, Chene G.2
1Departments of Pediatric Surgery, Centre Hospitalier Universitaire, Saint-Etienne, France
2Obstetrics and Gynecol o gy, C ent re Hos pi ta l i e r Universit aire, Saint-Eti en ne, France
3Anesthesiology Centre Hospitalier Universitaire, Saint-Etien ne, France
E-mail: chenegautier@
Received June 12, 2010; accepted July 13, 2010
Isolated mediastinal lymphangiomas are uncommon. We report a case of a 14 × 8 mm right paracardiac cyst
diagnosed at 20 weeks’ gestation. The prenatal evolution was uneventful and a magnetic resonance im aging
at 31 weeks showed the limited extension of the cyst into the anterior mediastinum. At birth, the baby was
asymptomatic, but the size of the lesion increased steadily (48 × 29 mm). At 7 months of life, he underwent a
thoracoscopic resection of the cyst without intra or postoperative complications. Histological examination
showed a lymphangioma. This case is remarkable for its prenatal diagnosis, the thoracoscopic treatment and
the 8 years of follow-up without recurrence.
Keywords: Mediastinal Tumor; Mediastinal Lymphangioma; Thoracoscopic Treatment; Prenatal Diagnosis
1. Introduction
Isolated anterior mediastinal lymphangiomas (ML) are
uncommon, with an occurrence less than 1% of all the
lymphangiomas [1], and most of them are asymptomatic
during childhood. They can lead to compression of vital
structures, even life-threatening airway compromise. A
prenatal diagnosis is now possible, but several patholo-
gies can be evocated when a paracardial cystic lesion is
discovered. Once diagnosed, they should be resected,
typically by thoracotomy or median sternotomy. We re-
port a case of ML with prenatal diagnosis and thoraco-
scopic treatment.
2. Case Report
An 8 months old boy presented a 14 × 8 mm anecho-
genic right-sided anterior mediastinal cyst, which had
been diagnosed at 20 weeks’ gestation by ultrasono-
graphic examination (Figure 1) and confirmed by mag-
netic resonance imaging (MRI) at 31 weeks’ gestation
(Figure 2). At 34 weeks, the cyst was heterogeneous and
measured 27 × 23 mm (Figure 3), but no complications
were observed during the pregnancy and the baby was
delivered at 37 weeks, weighing 2870 g, without respi-
ratory distress. In the first week of life, sonography and
MRI showed a 33 × 26 mm cyst and it was decided to
delay resection for a few months. By 7 months, the cyst
had enlarged to 48 × 29 mm, without respiratory com-
plication, and the baby was operated on account of this
evolution. In the operating room, the patient was placed
in left lateral decubitus position and 4 ports were neces-
sary. The cyst was to the right of the thymus, close to the
phrenic nerve. The posterior parietal pleura was opened
over the cyst from its lower part and easily dissected off
the thymus; the dissection was performed cephalad along
the right phrenic nerve and the pedicle was ligated close
to the superior vena cava. Pathological examination
showed a typical lymphangioma. The postoperative course
was uneventful and no phrenic palsy or pleural effusion
was noted. The patient remains asymptomatic 8 years
after surgical excision, without recurrence of the lym-
3. Discussion
Lymphangiomas are beni gn hamartomatous tumors of
the lymphatic system and less than 1% of all cystic lym-
phangiomas are purely mediastinal in origin [1]. They
constitute about 3% of all mediastinal masses in children
[2]. A prenatal diagnosis has already been reported in 8
cases for an isolated ML [3-10]. It may be s uspected
when the sonographic examination shows a single or
multilocated paracardiac anterior mediastinal cystic
Figure 1. transverse sonography at 20 weeks’ gestation
showing a 14 × 8 mm right paracardiac anechogenic mass.
mass. Sometimes, the lymphangioma was described in
the pos- terior mediastinum [4,10,11]. In our case, a fetal
MRI was also performed and showed the exact location
of the lesion and its extension. As the intracystic septa-
tions are not always visible on fetal ultrasonography,
other diagnoses have to be proposed: pericardial cyst,
bronchogenic cyst, thymic cyst, teratoma, esophageal
duplication and neurenteric cyst [8,12,13]. A poor out-
come is possible with fetal hydrops and hypoplastic
lungs, and prenatal thoracocentese may be discussed [3].
For the 8 cases with prenatal diagnosis, the evolution
during the pregnancy was variable with 1 spontaneous
disappearance, 3 stable lesions and 4 increases of the ML.
Among the 4 last cases, 3 fetal hydrops occurred with 1
neonatal death [3], 1 prematurity at 35 weeks’ gestation
[7] and the third underwent drainage of the cyst at 24
weeks with success [5]. The ML may be associated with
a cervical cyst [9,14] and sometimes with an abdominal
extension [15]. A termination of the pregnancy was per-
formed for one fetus presented a cervico-mediastino-
retroperitoneal lymphangioma [11].
After birth, most ML are not diagnosed because they
are asymptomatic; among the patients presenting symp-
toms, the most common are respiratory, cough or stridor
by extrinsic compression of the airway as a result of
hemorrhage or inflammation, sometimes with acute res-
piratory distress [15,16]. Less common symptoms are
dysphagia, superior vena cava syndrome, dysarythmia,
Horners syndrome or phrenic nerve paresis [17]; a fatal
outcome in a 12 year-old boy has been described [18].
Chest radiograph may show an anterior mediastinal mass
and sonography may establish its cystic aspect with sep-
tations; however computerized tomography and espe-
cially MRI are useful for the diagnosis and the extension
of the lesion [14-16]. Calcifications have been described
in ML, although this is more characteristic of teratomas
Mediastinal lymphangiomas, as other mediastinal ma-
sses, must be removed to avoid complications. Among the
Figure 2. (a) fetal MRI at 31 weeks with the right mediasti-
nal cyst. Coronal view; (b) fetal MRI at 31 weeks with the
right mediastinal cyst. Sagittal view.
Copyright © 2010 SciRes. SS
Figure 3. Sonography at 34 weeks’ gestation with a 27 × 23
mm heterogeneous right anterior mediastinal cyst.
children with a prenatal diagnosis, 3 thora cotomies were
performed at birth for t he 2 fetal hydrops and 1 huge
lymphangioma; one of them underwent a second thora-
cotomy at 19 months for recurrence [5]. One 51 × 24 mm
ML at 31 weeks’ gestation disappeared spontaneously at
6 months of life [6]. The last 3 ML were respected and
overseen after birth, but the ML increased , as in our case,
and a thoracotomy was decided at 6 weeks of life for 2
and 19 months for one. Usually, thoracotomy or median
sternotomy are performed, but the thoracoscopic treat-
ment of such lesion is now possible [13,20]; this proce-
dure has been shown to be safe in a series of 22 medi-
astinal cysts in children, one of which being a ML [13],
and in 2 other cases at 19 months and 7 years old [8,16].
Our child was 7 m onths old when the thoracoscopic
treatment was performed. Nevertheless, postoperative
complications can arise after the treatment of ML; the
surgical resection may be incomplete because of adhe-
sions with the great vessels or pericardium and recur-
rences are possible [1,5,9,21]; a few patients displayed
temporary or definitive phrenic nerve palsy or Horner’s
syndrome as well [1,21,22]. In our case, a complete re-
section was performed and the phrenic nerve was seen
during the entire procedure, without postoperative com-
plication. We have 8 years of follow-up and we can con-
sider that the recovery is obtained now.
4. Conclusions
Isolated mediastinal lymphangiomas must be suspected
when a cystic mass is noted on prenatal sonography in
the anterior mediastinum, differential diagnosis including
especially pericardial cyst or thymic cyst. The evolution
is variable from the spontaneous disappearance to fetal
hydrops or life-threatening complications. A thoraco-
scopic approach is now possible, even in infant.
Author Disclosure Statement: there is no conflict of
interest for each author.
5. References
[1] C. C. Wright, D. M. Cohen, R. K. Vegunta, J. T. Davis
and D. R. King, “Intrathoracic Cystic Hygroma: A Report
of Three Cases,” Journal of Pediatric Surgery, Vol. 31,
No. 10, 1996, pp. 1430-1432.
[2] A. I. Philippart and D. L. Farmer, “Benign Mediastinal
Cysts and Tumors,” In: J. A. O’Neill, M. I. Rowe, J. L.
Grosfeld, E. W. Fonkalsrud and A. G. Coran, Eds. Pedi-
atric Surgery, 5th Edition, St. Louis, 1998, pp. 839-851.
[3] A. Lindinger, R. Boos, K. Pistorius, H. G. Limbach, H. J.
Jesberger and W. Hoffmann, “Mediastinal Cystic Lym-
phangioma as a Cause of Hydrops Fetalis,” Klin Pediatr,
Vol. 204, No. 2, 1992, pp. 118-122.
[4] Y. Zalel, E. Shalev, M. Ben-Ami, G. Mogilner and E.
Weiner, “Ultrasonic Diagnosis of Mediastinal Cystic Hy-
groma,” Prenatal Diagnosis, Vol. 12, No. 3, 1992, pp.
[5] J. K. Muraskas, J. G. Gianopoulos, A. Husain and P. R.
Black, “Mediastinal Cystic Hygroma: Prenatal Decom-
Pression with Neonatal Resection and Recurrence at 19
Months of Age,” Journal of Perinatology, Vol. 13, 1993,
pp. 381-384.
[6] M. P. Wu, R. C. Wu, J. S. Lee, W. J. Yao and P. L. Kuo,
“Spontaneous Resolution of Fetal Mediastinal Cystic
Hygroma,” International Journal of Gynecology & Ob-
stetrics, Vol. 48, No. 3, 1995, pp. 295-298.
[7] E. Jung, H. S. Won, P. R. Lee, I. S. Lee, A. Kim and J. H.
Nam, “The Progression of Mediastinal Lymphangiomas
in Utero,” Ultrasound in Obstetrics and Gynecology, Vol.
16, No. 7, 2000, pp. 663-666.
[8] A. Bernasconi, S. J. Yoo, F. Golding, J. C. Langer and E.
T. Jaeggi, “Etiology and Outcome of Prenatally Detected
Paracardial Cystic Lesions: A Case Series and Review of
the Literature,” Ultrasound in Obstetrics and Gynecology,
Vol. 29, No. 4, 2007, pp. 388-394.
[9] C. H. Comstock, W. Lee, R. A. Bronsteen, I. Vettraino and
D. Wechter, “Fetal Mediastinal Lymphangiomas,” Jour-
nal of Ultrasound in Medicine, Vol. 27, No. 1, 2008, pp.
[10] R. Ruano, E. Takashi, R. Schultz and M. Zugaib, “Prena-
tal Diagnosis of Posterior Mediastinal Lymphangioma by
Two- and Three-Dimensional Ultrasonography,” Ultra-
sound in Obstetrics and Gynecology, Vol. 31 , No. 6,
2008, pp. 697-700.
[11] P. L. Giacalone, P. Boulot, P. Deschamps, et al., “Prena-
tal Diagnosis of a Multifocal Lymphangioma,” Prenatal
Diagnosis, Vol. 13, No. 12, 1993, pp. 1133-1137.
[12] M. Hendrickson, K. Azarow, S . Ein, B. Shandling, P.
Thorner and A. Daneman, “Congenital Thymic Cysts in
Children—Mostly Misdiagnosed,” Journal of Pediatric
Surgery, Vol. 33, No. 6, 1998, pp. 821-825.
[13] J. L. Mic hel, Y. Revillon, P. Montupet, F. Sauvat, S.
Sarnacki, N. Sayegh and C. Nihoul-Fekete, “Thoraco-
scopic Treatment of Mediastinal Cysts in Children,”
Journal of Pediatric Surgery, Vol. 33, No. 12, 1998, pp.
Copyright © 2010 SciRes. SS
Copyright © 2010 SciRes. SS
[14] N. Borecky, F. Gudinchet, R. Laurini, B. Duvoisin, J.
Hohlfeld and P. Schnyder, “Imaging of Cervico-Thoracic
Lymphangiomas in Children,” Pediatric Radiology, Vol.
25, No. 2, 1995, pp. 127-130.
[15] L. Ghedira, S. Haddad, K. Lajmi, et al., “Isolated Medi-
astinal Cystic Lymphangioma in Children: About Two
Cases.” Respiratory Medicine CME, Vol. 1, No. 4, 2008,
pp. 270-273.
[16] A. M. Kavunkal, J. Ramkumar, S. Gangahanumaiah, K.
N. Parimelazhagan and V. K. Cherian, (2007) “Isolated
Mediastinal Cystic Lymphangioma in a Child,” The
Journal of Thoracic and Cardiovascular Surgery, Vol.
134, No. 6, pp. pp. 1596-1597.
[17] D. K. Robie, M. H. Gursoy and W. J. Pokorny, “Medi-
astinal Tumors—Airway Obstruction and Management,”
Seminars in Pediatric Surgery, Vol. 3, No. 4 , 1994, pp.
[18] A. Adil and M. Ksiyer, “Unusual Mediastinal Cystic
Lymphangioma. Report of a Case and Review of the Lit-
erature,” Interventional Radiology, Vol. 39 , 1996, pp.
[19] L. E. Swischuk, J. C . Hoeffel and S. D. John, “Primary
Intrathoracic Lymphangioma Masquerading as Tera-
toma,” Pediatric Radiology, Vol. 26, No. 11, 1996, pp.
[20] S. S. Rothenberg, “Thoracoscopy in Infants and Children:
The State of the Art,” Journal of Pediatric Surgery, Vol.
40, No. 2, 2005, pp. 303-306.
[21] G. Lemoine and P. Montupet, “Mediastinal Tumors in
Infancy and Childhood. In Current Topics in General
Thoracic Surgery,” Elsevier Science Publishing, 1991, pp.
[22] H. Takamatsu, H. Noguchi, H. Tahara, H. Kajiya and H.
Akiyama, “Giant Mediastinal Lymphangioma in a Neo-
nate: Report of a Case,” Surgery Today, Vol. 26, No. 7,
1996, pp. 527-531.