Lymphatic Malformation (Cystic Hygroma) Treatment & Management

Updated: Jan 31, 2023
  • Author: Vijay A Patel, MD; Chief Editor: Ravindhra G Elluru, MD, PhD  more...
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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. [17] ​ 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). [4]  Current studies are geared towards understanding the molecular pathology of LMs to allow for more targeted drugs. 

An infected LM should be treated with intravenous (IV) 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.


Medical Care


OK-432, though not currently approved by the US Food and Drug Administration (FDA), has been reported to be capable of successfully treating LM. [18] The mechanism of action is proposed to be an inflammatory response to the inactive bacteria, leading to fibrosis. 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. [19] 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, a cytotoxic antibiotic, 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. [20]

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 (including pulmonary fibrosis). [21]


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. [22]

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 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. [23]


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. [24]


Sirolimus (rapamycin) is a mammalian target of rapamycin (mTOR) inhibitor that may be helpful in treating LMs. It works through deregulation of the mTOR pathway, which is suspected of playing a role in the pathogenesis of vascular anomalies. Sirolimus causes a decrease in vascular endothelial growth factor (VEGF), which is a known regulator in lymphangiogenesis. [25]

Sirolimus has been studied in several small series, but the optimal dose and duration have not been identified. [26, 27, 28, 29] In a systematic review, treatment results from 105 patients with LM were reported, with more than 91% of patients showing at least a partial response to treatment. [25] Although complete response was seldom seen, many patients did benefit from the partial response and pain-relieving action. In complex cases where surgical therapy or sclerotherapy is difficult, sirolimus may be a useful option. [30]  A multicenter clinical trial is under way to assess the safety and efficacy of sirolimus in treating LMs; the hope is that sirolimus may become the new first-line therapy. [31]  


Surgical Care

The mainstay of treatment of LMs is surgical excision. [32] Although surgery is the standard treatment, 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; secondary excisions are complicated by fibrosis and distorted anatomic landmarks.

Microcystic lesions are much more difficult to remove, given their intimate association with nearby tissues. Laser therapy may be used for microcystic lesions as well.

The exceptions to excision at the time of diagnosis are few and include premature infants who are small 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 is around the facial nerve.

Signs of airway obstruction necessitate surgical evaluation at the time of diagnosis. In emergency situations, aspiration with an 18- 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. [33] 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 mo), with 81% of patients not requiring further treatment. [34]

Magnetic resonance–controlled laser-induced interstitial thermotherapy is a newer therapy that has been proposed for the 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, magnetic resonance imaging (MRI) should be performed to delineate the mass further. In these cases, a multispecialty team, including a high-risk obstetrician, a pediatric otolaryngologist, a pediatric surgeon, and a 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  also been reported in the literature.



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 neurovascular structures (including cranial nerves)
  • Chyle leak and chylothorax
  • Hemorrhage
  • Infection
  • Recurrence - Most recurrences develop within the first year, but some have arisen as long as 10 years following excision


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



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 as well as an interventional neurosurgeon/radiologist is appropriate.