Cystic lymphangioma with enlarged tongue: A case report

Abstract

T he cystic lymphangioma., “cystic hygroma,” is a congenital abnormality of the lymphatic system. Thought to be due to sequestration of the primitive lymph sacs, these benign tumors are most commonly posterior cervical masses that are present at birth. When laterally placed and well loculated, complete surgical excision is the treatment of choice along with marsupialization of unresectable cysts. Unfortunately, many lymphangiomas send fingerlike processes into surrounding tissues, so that extension into the axilla and mediastinum is not infrequent. When this lesion is sublingual and/or submental in position, bilateral, and especially with invasion of tongue and suprahyroid structures, complete resection is virtually impossible. Over the past 4 years we have had under our care a boy, now 8 years old, with such a remarkably extensive lesion. Because of the massive tongue involvement at birth, the child had undergone an immediate tracheotomy and subsequently had undergone two partial neck resections of the lesion. Surgical trauma to the facial nerve at the second resection had caused a complete peripheral facial paralysis on the left side. When first seen, the boy had massive tongue enlargement, constant drooling, and protrusion with a very obtuse maxillary-mandibular angle. He was unable to breathe with his tracheotomy tube obstructed. On full evaluation, it was noted that the grapelike lymphangioma masses were studded throughout the floor of the mouth and over the soft palate as well as being deeply infiltrated into the tongue. Furthermore, cystic masses were also noted throughout the entire supraglottic larynx. Remarkably, the child could swallow well and there was minimal aspiration. Prior to orthodontic consultation, osteotomy to reduce the mandible was recommended. After examination of the patient with diagnostic aids, it appeared desirable to attempt orthodontic treatment without surgical intervention. A head