eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Pediatric Otolaryngology

Cervicofacial Lymphangiomas: Treatment

Author: Ted L Tewfik, MD, FRCS(C), Professor, Department of Otolaryngology, Director of Continuing Medical Education of Otolaryngology, McGill University Medical School; Director, Director of Professional Affairs of Otolaryngology, Department of Otolaryngology, Montreal Children's Hospital; Senior Staff, Montreal General Hospital and Royal Victoria Hospital
Coauthor(s): Richard L Fabian, MD, Former Director, Former Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Harvard Medical School
Contributor Information and Disclosures

Updated: Oct 17, 2008

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.

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 where surgical treatment is associated with the possibility of serious functional or cosmetic side effects.4

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.

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.

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.

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.

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.

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.

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.5

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.

More on Cervicofacial Lymphangiomas

Overview: Cervicofacial Lymphangiomas
Workup: Cervicofacial Lymphangiomas
Treatment: Cervicofacial Lymphangiomas
Follow-up: Cervicofacial Lymphangiomas
References
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Further Reading

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Keywords

lymphangioma, lymphangiomas, cystic hygroma, cervicofacial lymphangiomas, lymphangioma circumscriptum, lymphangioma capillary type, lymphangioma cavernosa, lymphangioma cystica, lymphangioma complex

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Contributor Information and Disclosures

Author

Ted L Tewfik, MD, FRCS(C), Professor, Department of Otolaryngology, Director of Continuing Medical Education of Otolaryngology, McGill University Medical School; Director, Director of Professional Affairs of Otolaryngology, Department of Otolaryngology, Montreal Children's Hospital; Senior Staff, Montreal General Hospital and Royal Victoria Hospital
Ted L Tewfik, MD, FRCS(C) is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Society of Pediatric Otolaryngology, Canadian Medical Association, Canadian Society of Otolaryngology-Head & Neck Surgery, Quebec Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Richard L Fabian, MD, Former Director, Former Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Harvard Medical School
Richard L Fabian, MD is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Ophthalmology, American Academy of Otolaryngology-Head and Neck Surgery, American Association for the Advancement of Science, American College of Surgeons, American Laryngological Rhinological and Otological Society, American Medical Association, American Society for Head and Neck Surgery, Association of Military Surgeons of the US, and Undersea and Hyperbaric Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Daniel J Kelley, MD, Consulting Staff, Eastern Shore ENT and Allergy Associates and Peninsula Regional Medical Center
Daniel J Kelley, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Head and Neck Society, American Laryngological Rhinological and Otological Society, and Pennsylvania Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Karen Hall Calhoun, MD, William E Davis Professor and Chair, Department of Otolaryngology-Head and Neck Surgery, University of Missouri
Karen Hall Calhoun, MD is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Head and Neck Society, American Medical Association, American Rhinologic Society, Association for Research in Otolaryngology, Society of University Otolaryngologists-Head and Neck Surgeons, Southern Medical Association, Texas Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown

 
 
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