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2012-10-13-09 Lymphangiomas ©Guzal Khodjaeva

Massive Lymphangiomas: head, neck, thorax

Guzal Khodjaeva, MD, Said-Azim Sultanov, MD, Alfiya Matchanova, MD, Ergash Tadjitdinov, MD.

Scientific Medical Center of Obstetrics and Gynecology, Republic of Uzbekistan
Synonyms: Lymphatic hamartomas, (cystic) lymphangiomas, hygroma colli cysticum and jugular lymphatic obstructive sequence [1]

Definition: Cystic lymphangiomas are congenital malformations of the lymphatic system that occur most commonly in the nuchal region. Nuchal lymphangiomas are often referred to as cystic hygromas. 

Case report: A 30-year-old woman (G2P1) was referred to our antenatal unit at 27-28 weeks for sonographic examination. Her family and medical histories were unremarkable. Our ultrasound examination found a single fetus in breech presentation with giant cystic axillar tumor. Prenatal sonography showed a multilocular, large cystic mass on the fetal left side, in the area of the head, neck, upper chest and upper abdomen. This cystic mass did not show flow on Doppler sonography.  

Oligohydroamnios was also present. Amniocentesis showed a normal 46, XX karyotype. Multiple fine-needle aspirations of the mass in the third trimesters obtained blood-stained chocolate-colored fluid containing numerous erythrocytes and lymphocytes but proved ineffective in lessening the progressive growth of the mass. 

These lymphangiomas enlarge progressively during fetal life and cause complications such as brain and limbs edema, ascites, cardiomegaly. Parents decided to terminate this pregnancy. 

Here are some of the images that we obtained:

Figure 1: Left side of head, nuchal edema and multilocular cystic mass.


Figure 2: Fetal chest and spine cystic lymphangiomas

Figure 3: Multilocular cystic mass surrounds the fetal upper chest.

Figure 4: Well-defined massive cystic mass on the level of upper abdomen, ascites

Figure 5: Upper limb edema.

Figure 6: Absence of flow on Doppler sonography.

Prevalence: It is not well defined. Reports range from 1.6:10,000 pregnancies to 0.8% of pregnancies at risk for having a structural anomaly [5]. 
Etiology: Variable, probably multigenic [3, 4].
Pathogenesis: Jugular lymphatic obstruction sequence, abnormal embryonic sequestration of lymphatic tissue, or abnormal budding of the lymphatics [3]
Recurrence risk: Not increased [2].
Obstetrical management:
When a prenatal diagnosis of cystic hygroma is made, the determination of the karyotype is recommended in all cases. Serial sonograms to assess the growth of the mass and monitoring for the development of hydrops should aid in the management of the pregnancy and are useful in the counseling of future pregnancies.
In the fetus with associated hydrops, the chance of survival is small; therefore, a non-aggressive approach is advisable. The option of pregnancy termination should be offered before viability [3].
If there is a small isolated cystic hygroma, no modification of standard obstetrical management is required. When large lesions are present, a cesarean section may be advisable [3, 4]. 
MESH Lymphangioma-diagnosis; Thoracic-Neoplasm,-congenital BDE 3284 MIM 257350 ICD9 2281 CDC 228.100 (non-neck location), 239.200 (neck location)

1.Vincenzo Suma, MD, Alberto Marini, MD, Piergiorgio Gamba, MD, Claudia Luzzato, MD1992-03-13-21 Cystic hygroma, axillary, cervico-mediastinal © Suma
2. Chervenak FA, Isaacson G, Blacemore KJ, et al: Fetal cystic hygroma. Causes and natural history. N Engl J Med 309:822-825,1983.
3. Garden AS, Benzie RJ, Misken M, et al: Fetal cystic hygroma colli: antenatal diagnosis, significance and management. Am J Obstet Gynecol 154:221-225,1986.
4. Romero R, Pilu G, Jeanty P, et al: The abdominal wall. Prenatal Diagnosis of Congenital Anomalies. Norwalk. Appleton and Lange. 1988.
5. Benacerraf BR, Frigoletto FD: Prenatal sonographic diagnosis of isolated congenital cystic hygroma unassociated with lymphedema or other morphologic abnormalities. J Ultrasound Med 6:63-66, 1987.

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