Lymphatic Malformation (Cystic Hygroma) Clinical Presentation

Updated: Dec 14, 2020
  • Author: Vijay A Patel, MD; Chief Editor: Ravindhra G Elluru, MD, PhD  more...
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Presentation

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. [9]

Rare locations, such as the middle ear, have been reported. [10]

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Physical Examination

On physical examination, a typical LM is a soft, painless, compressible (doughy) mass that usually transilluminates.

A comprehensive head and neck examination should be performed to evaluate for any compressive cranial neuropathies related to LM.

In children who present with neck LM, attention must be given to assess for tracheal deviation or other evidence of impending airway obstruction.

Flexible laryngoscopy performed by an otolaryngologist can be a useful adjunct to evaluate vocal fold mobility as well as airway patency.

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Complications

Complications of LMs include the following:

  • Airway obstruction
  • Hemorrhage
  • Infection
  • Deformation of surrounding bony craniofacial structures or dentition (if an LM is left untreated)
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