Oral Lymphangiomas Clinical Presentation

Updated: May 09, 2018
  • Author: Kruti Parikh; Chief Editor: William D James, MD  more...
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Presentation

History

Although most lesions appear in the individual's first 2 years of life, lesions occasionally appear in early adulthood. Typical histories include annoying and repetitive surface bleeding, feeding difficulties in infancy, paroxysms of lesion expansion (usually in association with upper respiratory tract infections), intralesional hemorrhage, macroglossia, or repeated infections. [13]

Oral lymphangiomas can be seen in association with Turner syndrome, Noonan syndrome, various trisomies, fetal hydrops, and fetal alcohol syndrome. A thorough medical and family history can help rule out these associated syndromes. [14]

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Physical Examination

The clinical appearance of the lymphatic malformations generally depends on the depth of extension. Superficial lymphatic malformations have a pebbly surface and are usually pink or yellow, with the appearance of grouped translucent vesicles. Some may also be bluish, which may be caused by either a venous component or an intralesional hemorrhage.

See the images below.

Superficial lymphatic malformation. Superficial lymphatic malformation.
Superficial lymphatic malformation. Superficial lymphatic malformation.

Deeper lesions may have a superficial component. When a superficial component is absent, deeper lesions usually appear as soft, ill-defined masses that markedly distort the local anatomy.

See the images below.

Marked lingual enlargement caused by lymphatic mal Marked lingual enlargement caused by lymphatic malformation. Note the pebbly surface in areas not covered by materia alba. Also note the ecchymotic lesions protruding from the buccal mucosa in the mandibular vestibules.
Note the significant left buccal and submandibular Note the significant left buccal and submandibular swelling.
Profile view of a young adult with oral lymphangio Profile view of a young adult with oral lymphangioma (same patient as in Media File 2).

Macrocystic lesions may be fluctuant and readily transilluminating.

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Complications

The severity of the sequelae varies with the extent of the lesion.

Airway compromise is a possible complication. Oral and hypopharyngeal lesions often result in a compromised airway. Approximately 50% of children with oral lymphangiomas require tracheotomy. Close cooperation between the surgeon and an anesthesiologist skilled in fiberoptic intubation is imperative.

The surgeon must be cognizant that these lesions may later expand and compromise the airway as a result of trauma, hemorrhage, infection, or upper respiratory tract infection. Surgeons should have a low threshold for performing a tracheostomy in patients in whom the potential for airway compromise exists. [15]

When a tracheostomy is not performed after a lesion on the tongue or floor of mouth has been debulked, the author prefers to manage the airway expectantly. The patient is left intubated overnight and reevaluated for extubation in the subsequent days.

Dental caries are proportionately prevalent in these patients with oral lymphangiomas and are most likely a result of patient's and dentist's difficulties in maintaining adequate oral hygiene. The finding of dental caries is important because the spread of odontogenic infection to a lymphatic malformation is potentially life threatening. Additionally, the premature loss of dentition can further hamper proper nutrition in an already compromised patient. Therefore, aggressive dental care and meticulous hygiene are warranted. Pediatric dentists should be involved early in the care of children with oral lymphangioma.

Dysmorphogenesis of the maxillofacial skeleton is frequently observed in association with oral and cervicofacial malformations.

The proposed etiologies for these changes include local pressure effects, increased blood flow, and direct bony involvement. Bony changes appear to progress until growth is complete, regardless of whether the soft-tissue lesions are treated. In other words, soft-tissue debulking does not appear to affect the progression of this deformity; this finding appears to support the direct bony involvement hypothesis because debulking eliminates local pressure and blood flow effects. In addition, the bony overgrowth appears to behave as a malformation, mirroring somatic growth trends.

Although no histologic evidence suggests the presence of lymphatics in the long bones, lymphatics appear to be present in the alveolar bone of the mandible and maxilla. Histologic examination of resected mandibular specimens reveals abnormal dilated channels in marrow spaces lined by a flat, adult-type endothelium; this observation further supports the direct bony involvement hypothesis.

Speech pathologies are common. These may result from abnormal morphology and mobility of the tongue, poor oral compliance, lip incompetence, and palatal hypomobility. Palatal hypomobility may contribute to eustachian tube dysfunction with resultant ear infections and conductive hearing deficits.

Feeding difficulties are common in neonates. Alternate enteral feeding routes, including a gastrostomy, are often necessary.

Repeated paroxysms of bleeding may occur secondary to trauma. These repeated paroxysms can be nuisances with superficial lesions, they can be life threatening with the expansion of deep lesions. Because the lesion is not lethal as long as airway compromise is avoided, the reported surgical mortality rates are 2.5-11.4%.

Complications can result from surgical treatment.

Complication rates of surgical debulking are typically 20-30%. Complications include airway obstruction, seromas and hematomas, infections, and cranial nerve palsies. Reported surgical mortality rates are 2.5-11.4%.

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