Pediatric submental cavernous lymphangioma.

A 6-year-old girl presented with a 3-week history of a painless, rapidly enlarging right submental swelling that had become noticeable following an upper respiratory infection. The patient denied any constitutional symptoms. Physical examination revealed that she had a right nonpulsatile, compressible mass in the submandibular triangle that extended into the right floor of the mouth. The lesion was easily transilluminated. Radiologic studies included magnetic resonance imaging (MRI) of the neck with contrast, which demonstrated findings consistent with a congenital cystic lesion (figure 1). The patient underwent complete excision of the mass without injury to adjacent neurovascular structures (figure 2). Findings on histologic analysis of the surgical specimen were consistent with a cavernous lymphangioma. The patient fully recovered without postoperative complication, and she exhibited no evidence of recurrence at 1 year of follow-up.

Lymphangiomas are subclassified into three morphologic types: capillary lymphangioma (lymphangioma simplex), which is predominantly made up of capillary-sized, thinwalled lymphatics; cavernous lymphangioma, which is made up of dilated lymphatics; and cystic hygroma, which is made up of lymphatic cysts of various sizes. The various forms of lymphangioma are all the result of developmental anomalies in the fetal jugular lymphatic system. At some stage during development, the lymphatic and venous systems fail to mature. As a result, the communications necessary for normal drainage and recirculation of lymph do not form. These isolated systems gradually expand and develop into lymphatic malformations.

Lymphangiomas may occur anywhere in the head and neck, but they have a predilection for the tongue musculature and the deep cervical neck planes. Some 60% are noticeable at birth, and 80 to 90% manifest by 2 years of age. (1) Clinically, capillary lymphangiomas are the least significant; they generally appear as asymptomatic superficial skin lesions. Of more clinical concern are the cavernous and cystic forms, which may become so large that they cause dysphagia or obstruct the airway. Diagnosis is based on the clinical presentation and findings on radiographic imaging, with MRI being the study of choice.

Management includes an investigation for concomitant infection and possible abscess. When no infection is present, lymphangiomas are traditionally treated by surgical excision. When an incomplete excision is performed in order to preserve vital structures, recurrence is common. An alternative to excision is administration of a sclerosing agent such as OK-432, a streptococcal derivative. These agents elicit an inflammatory response that leads to shrinkage of the lymphatic malformation. (2)

References

(1.) de Serres LM, Sie KC, Richardson MA. Lymphatic malformations of the head and neck. A proposal for staging. Arch Otolaryngol Head Neck Surg 1995;121:577-82.

(2.) Ogita S, Tsuto T, Tokiwa K, Takahashi T. Intracystic injection of OK-432: A new sclerosing therapy for cystic hygroma in children. Br J Surg 1987;74:690-1.

Lorraine Smith MD, MPH, FACS; Jason S. Hamilton, MD

From the Head and Neck Cancer Center, Cedars-Sinai Medical Center (Dr. Smith), and the Osborne Head and Neck Institute (Dr. Hamilton), Los Angeles.