History
The presenting signs and symptoms of a lymphatic malformation (LM) vary, depending on the lesion's location.
The microcystic form tends to predominate over macrocystic LM in the oral cavity and oropharynx. Microcystic LMs commonly appear as clusters of clear, black, or red vesicles on the buccal mucosa or tongue. Macrocystic LMs tend to predominate below the mylohyoid muscle and can involve both the anterior and posterior triangles of the neck. The cysts are typically large and thick-walled and have little involvement of surrounding tissue. The overlying skin can take on a bluish hue or may appear normal.
LMs often present after a sudden increase in size secondary to infection or intralesional bleeding. Spontaneous decompression or shrinkage is uncommon.
Rarely, children with LMs display symptoms of newly onset obstructive sleep apnea syndrome (OSAS). This situation may involve children with LM or other space-occupying lesions of the supraglottic or paraglottic region. Suprahyoid lymphangiomas tend to cause more breathing difficulties than infrahyoid lesions.
Potentially life-threatening airway compromise that manifests as noisy breathing (stridor) and cyanosis is a possible symptom of lymphangiomas.
Feeding difficulties, as well as failure to thrive, may alert the clinician to a potential lymphangioma. This is especially true when the lesion affects structures of the upper aerodigestive tract.
Rare locations, such as the middle ear, have been reported. [6]
Physical Examination
On physical examination, the typical LM is a soft, painless, compressible (doughy) mass that usually transilluminates.
In children who present with LM of the neck, closely evaluate for tracheal deviation or other evidence of impending airway obstruction. Closely inspect the tongue, oral cavity, hypopharynx, and larynx; any involvement of these structures may lead to airway obstruction.
Referral to an airway expert (otolaryngologist) to potentially perform a fiberoptic airway evaluation is justified in patients with a lymphatic malformation.
Complications
Complications of LMs include the following:
-
Airway obstruction
-
Hemorrhage
-
Infection
-
Deformation of surrounding bony structures or teeth (if an LM is left untreated)
-
Reconstruction of CT in sagittal dimension. This patient was intubated because of respiratory distress caused by the large lymphatic malformation.
