Infected cystic hygroma resulting in septic shock and respiratory failure: A case report

• Lymphatic malformations (LM) can become infected but rarely lead to septic shock.
• Without infection, treatment can be with sclerotherapy, surgery, or novel therapies.
• Once infected, small loculated cysts may be inaccessible by incision and drainage.
• Urgent surgery may be needed for source control in those with septic shock from LM.

Macrocystic lymphatic malformations (cystic hygromas) are a common cause of cystic neck lesions. These lesions are often diagnosed prenatally in children. In cases without airway compromise, these children are discharged from the hospital for elective treatment. Surgical excision is one treatment modality while sclerotherapy has recently shown adequate results as well. While infection is a relatively common problem for lymphatic malformations, the majority can be treated with antibiotics alone. We present a case in which septic shock and respiratory failure resulted from primary infection of a macrocystic lymphatic malformation in a term infant discharged with a lymphatic malformation of the neck. Urgent surgical drainage failed, and complete excision was ultimately required for source control due to numerous small multiloculated small cysts inaccessible via incision and drainage.