Introduction: Lymphatic malformations are rare congenital tumors that consist of abnormal lymphatic channels. Lymphatic malformations include a wide spectrum of disorders that can occur on any part of the body, most often the head and neck. These nonmalignant fluid filled spaces are thought to be due to aberrant development of the lymphatic system. Here we will explore a rare case of congenital lymphatic malformation that demonstrates possible connection with deeper lymphatic structures in an 11 month old female.

Presentation: An 11 month old female presents to the clinic for evaluation of a skin lesion on her left upper arm. According to the parents, they state that the mass waxes and wanes in size, notably becoming smaller in the past few months. The parents state that the lesion is asymptomatic and that the child isn’t in any obvious discomfort.

Physical examination reveals a spongy patch on the distal portion of her right upper volar arm midway between the elbow and shoulder that measures approximately 6.5 cm * 3 cm. The skin overlying the lesion has become pink and thinned. There is also a smaller, more proximal pink macule measuring 1 cm * 1 cm. Punch biopsy is subsequently performed on the patch. Histopathology results show large dilated spaces lined by plump endothelial cells, recognizable as lymphatic vessels, that are seen in the dermis. Expansion of the dermis and some slight papillomatosis is also noted above the numerous ectatic lymphatic vessels.

Diagnosis: A diagnosis of congenital lymphatic malformation is made based on clinical presentation and histopathology results. Of particular interest, the changes noted are distinct from a typical lymphangioma and more likely represents a true malformation that could possibly connect with deeper lymphatic structures. Typical lymphangiomas generally show no evidence of communication with the regular lymphatic channels.

Management: The patient’s family was instructed on the typical clinical course associated with lymphatic malformations including the potential for lymphangioma circumscriptum. Potential treatment options include conservative management with watchful waiting, compression therapy, sclerotherapy, and surgery which would entail an excisional biopsy/debulking. The family agrees to pursue the recommended course of conservative treatment with watchful waiting.

References and Resources:

1. Dimitrios Patoulias, Ioannis Patoulias, Christos Kaselas, Maria Kalogirou, Chatzopoulos Kyriakos, Farmakis Konstantinos, Thomas Feidantsis, Papacrivou Eleni, “Cystic Lymphangioma of the Chest Wall in a 5-Year-Old Male Patient: A Rare and Atypical Localization—A Case Report and Comprehensive Review of the Literature”, Case Reports in Pediatrics, vol. 2017, Article ID 2083204, 6 pages, 2017. https://doi.org/10.1155/2017/2083204.
2. Prashant K. Minocha, Lakhan Roop, Rambachan Persad, “Cases of Atypical Lymphangiomas in Children”, Case Reports in Pediatrics, vol. 2014, Article ID 626198, 6 pages, 2014. https://doi.org/10.1155/2014/626198.