Lymphatic Malformation (Cystic Hygroma) Treatment & Management

Updated: Jan 28, 2019
  • Author: Jason L Acevedo, MD; Chief Editor: Ravindhra G Elluru, MD, PhD  more...
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Treatment

Approach Considerations

Although some authors have reported watchful waiting for lymphatic malformations (LMs), this should be considered only in patients who are asymptomatic. Medical treatment of LMs consists of the administration of sclerosing agents, such as OK-432 (an inactive strain of group A Streptococcus pyogenes), bleomycin, pure ethanol, bleomycin, sodium tetradecyl sulfate, and doxycycline. [13] ​ Guidelines for the use of percutaneous sclerotherapy to treat LMs of the head and neck have been developed by the Society of NeuroInterventional Surgery (SNIS). [14]

An infected LM should be treated with intravenous antibiotics, and definitive surgery should be performed once the infection has resolved. Incision and drainage or aspiration results in only temporary shrinkage, and subsequent fibrosis can further complicate the resection. Radiotherapy has not been demonstrated to be effective. Some authors maintain that the preferred treatment of all LMs is surgical resection, positing that only resection can truly offer the potential for cure.

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Medical Care

OK-432

Although not currently approved by the US Food and Drug Administration (FDA), OK-432 has been reported to be capable of successfully treating LM. [15] The mechanism of action is proposed to be an inflammatory response to the inactive bacteria, leading to fibrosis of the hygroma. OK-432 may be a viable option for large unilocular cysts.

Currently, OK-432 is available in the United States only by protocol. It does not work well for small cysts. [16] Because the procedure for using OK-432 involves aspiration prior to injection of the sclerosant, some have hypothesized that the true effect is from the aspiration.

Bleomycin

Bleomycin has been considered a poor choice because of its toxicity (pulmonary fibrosis); LM is a benign disease, and other less toxic treatment options are available. Niramis et al studied 70 patients who underwent sclerotherapy with bleomycin; 83% obtained an excellent or good result, 43% had adverse reactions, and three patients died. [17]

Sainsbury et al studied 75 patients, 83% of whom had a complete or significant response. Five adverse reactions were reported, with no severe morbidity or mortality (to include pulmonary fibrosis). [18]

Alcohol

Absolute alcohol as a sclerosing agent has been used with some success in some patients; alcohol works well in vascular malformations. Imperizzilli et al studied computed tomography (CT)-guided ethanol injection and obtained complete resolution in seven of eight patients without complications. [19]

Interferon alfa-2a

This has been used in the treatment of hemangiomas, and its use has been proposed in lymphangiomas. However, its efficacy has never been documented, and it carries a serious side-effect profile.

Fibrin sealant

The use of a fibrin sealant after aspiration of LM has been reported in the literature.

Doxycycline

Doxycycline has been reported as a potential sclerotherapy agent, with both safety and efficacy. Like most other sclerosant agents, it has shown the highest efficacy in macrocystic lesions and the lowest efficacy in microcystic ones. [20]

Sildenafil

A limited case series has been reported using sildenafil for severe lymphatic malformations. A prospective trial has been performed, which did not demonstrate a significant effect on LM. [21]

Sirolimus

Sirolimus has been utilized in several small series, but the optimal dose and duration have not been identified. [22, 23, 24, 25, 26]

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Surgical Care

The mainstay of treatment of LMs is surgical excision. [27] Although surgery is the criterion standard for treatment, both the operating team and the family of the patient should go forward with the knowledge that LM is a benign lesion. If acute infection occurs prior to resection, surgery should be delayed at least 3 months.

The surgical team should attempt to remove the LM completely or, failing that, to remove as much as possible, sparing all vital neurovascular structures. Complete excision has been estimated to be possible in roughly 40% of cases.

LMs are ideally removed in a single procedure because secondary excisions are complicated by fibrosis and distorted anatomic landmarks.

Microcystic lesions are much more difficult to remove because of their intimate association with nearby tissues. Laser therapy is a recent advancement in the treatment of microcystic lesions.

The exceptions to excision at the time of diagnosis are few and include premature infants who are small in size and those with involvement of crucial neurovascular structures that are small and difficult to identify (eg, facial nerve). If no airway obstruction is present, surgery can be delayed until the child is aged 2 years or older, especially when the operation will be around the facial nerve in the parotid area.

Signs of airway obstruction necessitate surgical evaluation at the time of diagnosis. In emergency situations, aspiration with an 18-gauge or 20-gauge needle may obviate the need for an emergency tracheostomy.

Although traditional wisdom has dictated that LMs should not be aspirated, a study by Burezq et al documented success with serial aspiration of LM. [28] In this series, 14 patients were treated with aspiration alone (three needed multiple aspirations), with a mean follow-up of 5.75 years. No failures were reported. This technique may hold promise for the future management of LM. Other authors contend that aspiration has no role and believe that aspiration is often followed by recurrence, hemorrhage, or infection.

Radiofrequency ablation (RFA) has been advocated for use with intraoral LMs, especially microcystic lesions. Kim et al reported high long-term success rates with RFA (median follow-up, 47 months), with 81% of patients not requiring further treatment. [29]

Magnetic resonance–controlled laser-induced interstitial thermotherapy is a newer therapy that has been proposed for treatment of LM.

LM can present on routine antenatal ultrasonography (US) as a large obstructing airway mass, as can other pathologic conditions (eg, teratoma or rhabdomyosarcoma). If such a mass is visible on US, MRI should be performed to delineate the mass further. In these cases, a multispecialty team, including a high-risk obstetrician, pediatric otolaryngologist, pediatric surgeon, and neonatologist, should be present at the ex-utero intrapartum treatment (EXIT) procedure.

A planned cesarean delivery is performed, and intubation or tracheostomy is used to establish an airway. Extracorporal membrane oxygenation (ECMO) should also be available. Excision of the LM is delayed until the child is stable. Intrauterine cyst aspiration to facilitate vaginal delivery has been reported in the literature.

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Complications

Complications from surgical excision of an LM are myriad and are related to the location and structures adjacent to the mass; these include the following:

  • Damage to a neurovascular structure (including cranial nerves)
  • Chylous fistula
  • Chylothorax
  • Hemorrhage
  • Recurrence - Most recurrences develop within the first year, but some have arisen as long as 10 years after excision
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Activity

Patients with LM should be directed to avoid direct trauma to the area because intralesional bleeding or infection can be precipitated by trauma.

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Consultations

Depending on the anatomic location of the lesion, referral to a surgeon or surgical specialist is appropriate. In patients with LM of the head and neck, referral to an otolaryngologist is appropriate.

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