Lymphatic Vascular Malformations Treatment & Management

Local pressure

Elastic support stockings may help to decrease the swelling and the functional handicap associated with lymphatic VMs of the extremities (see image below).

Antibiotics

Viral or bacterial infection can cause acute infection of a lymphatic VM. Infection may be associated with acute enlargement, pain, local warmth, redness, and elevated systemic fever. Intravenous (IV) antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs) are indicated during such episodes.

Sclerotherapy

Transcutaneous injection of a sclerosant such as alcohol or sodium tetradecyl sulphate (STS) may help to decrease a lymphatic VM. ^[4] It is mostly useful for macrocystic malformations and for combined venous-lymphatic lesions. The procedure is painful; therefore, it is performed by an invasive radiologist under general anesthesia.

Initially, 5 mL of contrast fluid is injected through a venous catheter to delineate the anatomy of the lesion and to detect escape of contrast to the systemic circulation (see the upper left portion of the image below). The upper middle portion of the image below shows injection of contrast into a facial venous-lymphatic malformation. The catheter was relocated when escape of contrast to the facial vein was detected (upper right). When a lumen filled with lymphatic fluid is detected, the lymph is aspirated. Alcohol (100%) mixed with a small amount of contrast fluid (alcohol-to-contrast ratio of 20:5) is then injected through the same venous catheter.

The total amount of alcohol injected into a lesion of a full-sized male is approximately 50 mL. Exceeding a dose of 1 mL/kg is not advised. The catheter is left in place following alcohol injection in case some of the alcohol needs to be withdrawn. Injection is discontinued when skin changes such as peau d'orange, erythema, and bruising are observed. The upper left portion of the image above shows bruising and peau d'orange changes following injection with alcohol of a chest venous-lymphatic VM.

Additionally, doxycycline percutaneous image-guided sclerotherapy of macrocystic intra-abdominal lymphatic malformations has been shown to be safe and effective. ^[15]

To decrease the swelling, 4 mg of dexamethasone (PO/IV) is administered 3 times every day for 3 days after the procedure. NSAIDs are administered for pain control. The patient usually stays in the hospital overnight for pain control and for monitoring of possible vascular or neurologic limb compromise. Systemic leakage of alcohol may cause myocardial depression. Injection of alcohol close to the skin or mucosa may cause skin slough or skin necrosis. The lower right and left portions of the image above) shows necrosis of skin and mucosa following injection of alcohol to the forearm and tongue, respectively. It was found that more complications were seen with the use of alcohol, prompting a change of practice to favour STS as the primary agent, especially for head and neck lesions. ^[16]

Sclerotherapy with intralesional bleomycin and OK-432 have been reported to have dramatic results. ^{[14, 17]} For example, a retrospective study by Mohan et al indicated that sclerotherapy with serial intralesional bleomycin injections can effectively treat slow-flow VMs, including the lymphatic kind. The study involved 32 children, including 27 with mixed venous-capillary malformations and five with lymphatic malformations, with 29 patients (91%) responding to the treatment and 28% achieving complete resolution of the malformations (mean follow-up period, 38 mo). ^[18]

Another study, by Paramasivam et al, found image-guided bleomycin sclerotherapy to be effective in treating orbital lymphatic VMs. The study involved 14 patients, all of whom experienced improvement in exophthalmos and either stable or improved vision. ^[19]

Surgery is the only way to "cure" a lymphatic malformation. It should be considered in the following situations:

• When intraoperative and postoperative bleeding can be controlled

• When surgery does not put another organ at risk (eg, injury to the eye or facial nerve)

The surgery must be well planned. Most lesions cannot be resected completely; therefore, the extent of the resection needs to be defined before the procedure. In certain situations, such as eyelid surgery, performing the surgery under local anesthesia is better. This facilitates intraoperative navigation. The image below shows an upper right eyelid lesion before and after resection under local anesthesia.

Lymphangioma circumscriptum is a superficial lymphatic malformation of the skin. This often can be resected completely and the defect reconstructed with a skin graft (see image below). Administration of hemostatic agents such as recombinant factor VIIa (rVIIa) may decrease bleeding and improve surgical efficiency. ^[20]

A study by Bonilla-Velez et al indicated that surgery can serve as a safe and effective first-line treatment for selected macrocystic lymphatic malformations of the head and neck. Most of the patients in the report had unilateral infrahyoid, unilateral suprahyoid, or unilateral infrahyoid and suprahyoid lesions, affecting the neck. Patients experienced such surgical complications as seroma/hematoma (9.5%), transient nerve weakness (facial nerve, sympathetic chain, or phrenic nerve; 6.3%), and infection (1.6%). At median 12-month follow-up, 90.5% of patients had experienced a complete response with a single surgery. In addition, over an observation period of up to 15 years, 86% of patients achieved recurrence-free survival. ^[21]

Vision

Obstruction of vision during the first 6 months of life by a periorbital, cheek, forehead, or nasal VM may cause long-term visual damage. Early treatment with transcutaneous sclerosis and/or surgery is indicated in such patients.

Breathing

Lymphatic VM, which invades the neck, may compress the soft tracheal rings of infants and present as stridor. Direct excision of the lesions is impossible in most patients because of the close proximity of the lesion to vital structures. Consider tracheostomy in patients in whom pharmacologic treatment has failed. ^[12]

Psychosocial complications

The presence of a VM psychosocially affects both the patient and his or her parents. Parents may be subjected to comments, questions, and unsolicited advice from friends, family, and complete strangers. Early psychosocial support by primary caregivers with the help of a dedicated vascular birthmark clinic team is mandatory. ^[22] Early surgery may be indicated in patients with visible facial VMs.

Lymphatic lesions gradually enlarge and worsen with time. ^[5] Surgery is the only complete cure for this problem. However, this is not always possible, and the goal of treatment in many patients is improvement rather than cure.

A significant contribution to the understanding of vascular lesions is the introduction of a classification method by Mulliken and associates. ^[1] This made diagnosis and treatment more accurate and predictable. However, confusing and occasionally misleading terms used by different subspecialties are still found.

Improvement in patient monitoring and anesthesia during and after surgery made early excisions and transcutaneous sclerosis safer and more acceptable.

A major controversy is the timing of operative procedures. No clear-cut answer exists to this question. The authors believe that decisions should be made according to the individual patient. The psychosocial consequences of growing up with a facial deformity always should be taken into consideration.

Future research of specific genes and their angiogenic growth factor products will contribute to the understanding of the mechanism underlying the formation of lymphatic VMs and may provide new modalities of treatment at the gene level. ^[7]