Treatment

Complications

Diagnosis

The differential diagnosis of LM includes hemangioma, VM, rhabdomyosarcoma, fibrosarcoma, and lymphoma. Most LM may be diagnosed by history and physical exam, with confirmation by MRI. Histologic confirmation is rarely necessary. Ultrasound is effective for diagnosis macrocystic LM. However, MRI with gadolinium is the most sensitive study to delineate the extent of lymphatic malformation and type of cysts. Because LM has a high water content, it is hyperintense on T2-weighted sequences. Macrocystic lesions often have fluid levels because of protein or blood within the space. Contrast thoracic duct lymphography may help delineate specific anatomic obstructions of thoracic duct anomalies.

Bleeding and infection are the two most common complications of LM. Intralesional bleeding occurs in 35% of cervicofacial LM, whereas infection has been cited to occur in 71% of cervicofacial LMs. Bleeding, either spontaneous or following trauma, may cause bluish discoloration and pain. Patients are treated conservatively with analgesic medication and rest.

LM will swell with systemic viral or bacterial infection. Cellulitis of the LM may lead to acute sepsis and functional problems, such as obstructed vision with a periorbital LM.

LMs may be treated with sclerotherapy, surgical resection, or both. Sclerotherapy works well for macrocystic LMs and in many cases may render lesions undetectable. Common sclerosant include pure ethanol, sodium tetradecyl sulfate, and doxycycline. Technically, sclerotherapy is usually straightforward. The cystic cavity is entered by direct puncture, and the fluid is aspirated. The needle is maintained in situ, and the sclerosant is injected. Multiple punctures, aspirations, and injections are often performed. Ultrasound guidance is useful. Staged sessions may be necessary for larger lesions. Sclerotherapy preceding resection can shrink LMs, allowing for less morbid surgical procedures.

LMs may re expand after treatment, requiring additional procedures. Microcystic LM is less responsive to sclerotherapy. Superficial lymphatic vesicles can be treated with local intravesicular injection to improve leakage. Complications of sclerotherapy to be avoided include injury to adjacent nerves, necrosis of overlying skin, and cardiotoxicity related to overdose.

Whereas sclerotherapy can achieve good long-term results, it is more common for LM to recur after such treatment. Consequently, most authors agree that resection offers the only potential for cure. However, complete removal is often difficult because the lesion permeates tissue planes, nerves, and muscles. Excision is indicated for patients with symptomatic microcystic lesion or problematic microcystic LM that fails sclerotherapy. Staged excision is usually necessary. The recurrence rate due to expansion of the lesion has been reported to be 17-40% after complete removal.