Embryological Basis of Cystic Hygroma: A Case Report

We intend to present a cystic hygroma (CH) case in a 2-year-old baby in the anterior cervical triangle, which is a rare site, as the most common site for the occurrence of CH is the supraclavicular fossa of the posterior cervical triangle.

Among developmental abnormalities in the lymphoid system, CH are usually seen in the posterior neck. Lymphatic malformations are generally exhibited either at birth or before the age of 2 years. Lymphatic channels are attenuated endothelium-lined spaces devoid of any cells and smooth muscle layer. Also, morphologically distinguishing normal lymphatic channels from venules or capillaries is a challenge.

A 2-year-old female patient reported having a chief complaint of swelling in the left submandibular region for 4 days. The patient underwent surgery for CH 18 days after birth. Swelling was rubbery in texture and firm in consistency.

A D2-40 immunoexpression was an identifying clue for normal lymphatics in comparison to morphology. Henceforth, this can be concluded that such tumors depict at least partial differentiation of endothelial cells lining lymphatic spaces.

The present article helps in illuminating the role of D2-40 in the diagnosis of lymphatic malformations, such as CH, and also highlights the embryological basis of the pathogenetic mechanism of this rare disease, which potentiates the role of various treatment modalities in pediatric cases for management considerations.

Yadav S, Gulati N, Shetty DC, et al. Embryological Basis of Cystic Hygroma: A Case Report. Int J Clin Pediatr Dent 2022;15(6):774-778.