Cervicofacial Lymphangiomas Treatment & Management

Updated: Apr 13, 2016
  • Author: Ted L Tewfik, MD; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Treatment

Medical Therapy

Alternate therapy has been proposed as the primary treatment for lymphangiomas, particularly for sensitive areas (eg, the orbit) and, more commonly, for recurrent disease after surgical therapy.

Radiation therapy has been effective but abandoned because of later malignant transformation or retardation of growth sites.

Carbon dioxide laser therapy has been effective in managing upper airway lesions and superficial mucosal microcystic lesions.

Angiero et al (2009) used a 980-nm diode laser to treat 250 children with head and neck hemangiomas and venous malformations. They observed good resolution and aesthetic results. [8]

Intralesional sclerotherapy with group A Streptococcus pyogenes of human origin (OK-432) has had some success controlling lymphangiomas. The mechanism suggested is the stimulation of increased permeability of the endothelium, accelerating lymphatic fluid drainage and size reduction of the lymphangioma. Peral Cagical et al consider OK-432 the treatment of choice, especially in cases in which surgical treatment is associated with the possibility of serious functional or cosmetic side effects. [9]

Ozeki et al (2013) have studied the possibility of using propranolol as an alternative treatment for pediatric lesions. [10] Propanolol is now well recognized in the treatment of infantile hemangioma. They concluded that some plasmatic growth factors were significantly lower after the treatment and that further trials must be carried out to prove the clinical effectiveness.

Somnoplasty shows promise for reduction of tongue lymphatic malformations.

Occasional reports have described the use of triamcinolone, cyclophosphamide, bleomycin, fibrin glue, and alcohol (Ethibloc). Results have been inconsistent, and success is limited.

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Surgical Therapy

The treatment of choice for lymphangiomas is surgery. The primary intention is to accomplish total resections. However, because of lesion size, lesion location, and a myriad of previously mentioned variables, total resection is not always possible. Nowhere in surgery is careful planning and attention to detail more important than when dealing with these elusive lesions. If significant cosmetic or functional deficits are probable, consider partial staged reduction or alternative therapy.

Combined sequential approach is recommended for mixed lesions as well as extensive lesions that involve both the mucosa and soft tissues.

The particular surgical procedure relates to the location of the lymphangioma and the structures involved.

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Preoperative Details

The surgeon must prepare the patient with lymphangioma and the parents of a child who has lymphangioma for the potential problems associated with resection of a lymphangioma. Airway distress necessitates a tracheostomy, which, in a child, may be required for a considerable period. A careful preoperative consent and a discussion with the patient and family need to include the possible functional and cosmetic deficits that may occur as a result of the surgery. These potential deficits are determined by the age of the patient at onset and the complexity, size, and location of the lymphangioma. Acknowledging the probability of recurrence is important to avoid unpleasant confrontations at a later time. Consider second opinions to solidify the bond between the operating surgeon, the patient, and the family.

Preoperative testing should confirm with a high probability that the diagnosis is correct. In borderline cases, open biopsy may be performed before definite surgery; however, this is rarely necessary. The responsible surgeon should be an experienced head and neck surgeon with a surgical background in the resection of lymphangioma and all its ramifications.

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Intraoperative Details

Slow deliberate dissection with meticulous attention to anatomic detail, excellent hemostasis, and wide field exposure is essential for a favorable outcome of surgery. Complex disease of the head and neck may require innovative exposures, such as midline mandibulotomy, craniofacial exposure, facial translocations, or degloving incisions. Because of the high incidence of nerve injury, a nerve stimulator is essential to identify at least the motor nerves. Immediately repair transection of a motor nerve (eg, facial) by a nerve graft. Attempts to identify the serpiginous pathways taken by lymphangiomas and hygromas by dye injection or casting with dental materials provide no assurance that disease does not remain. When penetration occurs, decompression flattens the saccular distentions, eliminating the identification and resection of additional disease. If this should occur, repair of the breach may allow for redistention. If not, terminate additional dissection.

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Postoperative Details

Physical and psychological support is required, particularly when adverse cosmetic and functional problems occur as a result of surgery or the disease process itself. Tissue diagnosis should reassure the family and/or the patient that the process is benign. Standard postoperative care includes infection prevention, drain removal, airway maintenance, and proper nutrition.

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Follow-up

Following discharge from the hospital, the frequency and duration of follow-up visits should be related to the nature of the surgery, its complexity, and problems generated by the surgery or disease process. After initial healing has occurred, a baseline MRI of the operative bed represents a snapshot to which perceived future problems can be compared. In view of the high recurrence rate, providing clinical care for a child with lymphangioma into early adulthood is recommended. For rare adult disease, 5 years is a reasonable time for follow-up evaluation and care. Beyond this period, individual considerations determine patterns of follow-up care.

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Complications

The most common complication is incomplete resection and recurrence. A recurrence rate as high as 50% has been reported. Cranial nerve injury exceeds the rate of 20%; the facial nerve is the most common neural deficit reported. Ozen et al (2005) reviewed the medical records of 17 patients who were operated for cervicofacial cystic hygroma between 1985 and 2004 and documented the following 4 postoperative complications: 1 recurrence (6%), 2 facial paralyses (12%) and 1 collection of fluid (6%) at the resection site. [11]

Additional complications include ocular motility problems, difficulty swallowing, aspiration, lingual and hypoglossal nerve injury, spinal accessory nerve loss, brachial plexus, and phrenic nerve injury. Secondary infection, cosmetic deficits, thrombocytopenia, and secondary airway obstruction have also been recorded.

Liu et al (2009) reviewed their experience with 53 cases treated surgically. The age of the patients ranged from 6.5 months to 41 years. The rate of residual or recurrent lesions was significantly higher in the suprahyoid region than that in the infrahyoid region. The perioperative complications were paralyses of the mandibular branch of the facial nerve, Horner syndrome, secondary hemorrhage, fluid collection at the resection site, local infection, and parotid fistula. [12]

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Outcome and Prognosis

Outcome and prognosis relate to age of onset, the number of sites affected, the size of the lesion, the location of the lesion, and cosmetic implications. Negative prognostic factors include an age of onset younger than 1 year, multiple lesion sites, large complex lesions, severe widespread cosmetic changes with extension into subcutaneous tissues, and incomplete excision. Lesions of the lip, tongue, floor of the mouth, and larynx/hypopharynx have a high rate of recurrence. The mortality rate is less than 6%.

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Future and Controversies

Interferon alfa-2A has been shown to be effective in the treatment of hemangiomas. No studies have shown consistent success in the treatment of lymphangiomas. Genetic therapy for treatment and prevention, improved sclerosing therapy, and advances in molecular biology may eventually provide more acceptable and effective treatments. At present, surgical therapy continues to offer the best chance at a cure for cervicofacial lymphangioma. Unfortunately, this cure may come at a price.

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