Discussion
Lymphangiomas are non-infiltrating cystic tumors containing non-communicating lymphatic sequestrations that have developed outside the lymphatic system. 75% of the cases are localized in cervical, 15% in axillar, and 10% in cervico-mediastinal and retroperitoneal splenic or colonic regions. In the mediastinum, the cystic lymphangiomas tend to develop between the normal structures, and their mass effect on the airways (as it was in our case) is rare and leads to death in 3.4% of cases [2].
Detection of lymphangiomas is based on prenatal two-dimensional ultrasound where they appear as anechoic, thin-walled multilocular masses of variable size. The cystic content may be heterogeneous in some cases. Color Doppler may demonstrate a small vascular flow within the septa.
Prenatal ultrasound specifies locations of cystic lymphangiomas and allows differentiating them from cystic hygroma. Fetal MRI may be helpful in assessment of extension of the masses and their relation to surrounding organs of the mediastinum. The cystic lymphangiomas may be associated with all forms of haemolymphatic malformations [3].
Four main rare syndromes have been described in association with these tumors: Klippel-Trenaunay syndrome, Parkes-Weber syndrome, Maffucci syndrome and Proteus syndrome [4].
The Proteus syndrome is characterized by disproportional overgrowth of various organs - from macrodactyly to hemi-hypertrophy of the body, bony, subcutaneous, or skin tumors (nevi), and vascular malformations [5]. Most of the time, the capillary and venous malformations are present, while lymphatic tumors are less common. The lymphangiomas may cause dystocia during delivery. Other complications like hemorrhage, inflammation, infection, and mass effect of on adjacent organs may also occur.
References
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