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)
References
1.Vincenzo Suma, MD, Alberto Marini, MD, Piergiorgio Gamba, MD, Claudia Luzzato, MD1992-03-13-21 Cystic hygroma, axillary, cervico-mediastinal © Suma www.thefetus.net/
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.