Thursday, December 10, 2009

Microcystic Lymphatic Malformations of the Tongue – an Article Review

I stumbled across this article while previewing JAMA & Archives CME articles (full reference below). The article gives an overview of lymphatic malformations, noting that both sexes are equally affected, and there is no predilection for any race.

Lymphatic malformations are vascular malformations with an unknown cause. They are estimated to make up 6% of all benign soft-tissue tumors in children. While they may be rare, 50% of all lymphatic malformations are already obvious at the time of birth. Most (90%) are diagnosed by the end of the second year of life owing to clinical symptoms.

About 60% of all lymphatic malformations are found in the head and neck region. Regarding the mouth, the tongue is most commonly affected.

When the malformations occur in the tongue, the symptoms may include hemorrhage, excessive salivation, speech disturbances, difficulties chewing and swallowing, airway obstruction, and orthodontic abnormalities such as mandibular prognathism and malocclusion. Functional impairment and cosmetic deformity significantly affect the quality of life of patients with lymphatic malformations of the tongue.

Along with the overview, the authors present the review of their patients between January 1, 1998, through December 31, 2008, with respect to age and sex distribution, symptoms, clinical presentation, management, treatment outcome, and follow-up.

Twenty patients (13 male and 7 female) with microcystic lymphatic malformations of the tongue were included in the evaluation. Their ages at initial presentation ranged from newborn to 20 years (mean age, 7.4 years). Thirteen of them had been treated at another hospital before the initial presentation at our department. The treatment methods included surgical reduction, laser therapy, corticosteroid therapy, and OK-432 (Picibanil; Chugai Pharmaceutical Co, Ltd, Tokyo, Japan) injections.

The authors present the classification of lymphatic malformations (photo credit)

  • Isolated superficial microcystic lymphatic malformations of the tongue (stage I)
  • Isolated lymphatic malformations of the tongue with muscle involvement (stage II; stage IIA, involving a part of the tongue; stage IIB, involving the entire tongue)
  • Microcystic lymphatic malformations of the tongue and the floor of mouth (stage III)
  • Extensive microcystic lymphatic malformations involving the tongue, floor of mouth, and further cervical structures (stage IV)


The article discusses treatment options:

In the present series of patients with microcystic lymphatic malformations of the tongue, it was possible to perform complete excision with a CO2 laser in all patients with stage I disease and in 3 patients with stage IIA disease. …….. The advantages of the CO2 laser compared with conventional surgery include less postoperative edema, tissue trauma, and blood loss……... For stages I and IIA microcystic lymphatic malformations of the tongue, CO2 laser surgery seems to be an excellent curative treatment option. In stages IIB, III, and IV disease, CO2 laser surgery seems to be useful as a part of a combined or staged approach.

Other treatment modalities discussed include radiofrequency ablation, sclerotherapy (specifically OK-432 injections), and other surgical options, including wedge resection, bilateral marginal resection, U-shaped resection, and Jian or Dingman glossectomy.

Treatment of infected cysts before surgery:

The combination of antibiotics and short-duration systemic corticosteroids usually leads to a reduction of symptoms and a decrease of swelling and inflammation as described in patient 2.

I think the article is well written and well worth reading.

REFERENCE

Microcystic Lymphatic Malformations of the Tongue: Diagnosis, Classification, and Treatment; Arch Otolaryngol Head Neck Surg. 2009;135(10):976-983; Susanne Wiegand, MD; Behfar Eivazi, MD; Annette P. Zimmermann, MD; Andreas Neff, MD, PhD; Peter J. Barth, MD, PhD; Andreas M. Sesterhenn, MD, PhD; Robert Mandic, MD, PhD; Jochen A. Werner, MD, PhD

My post: Vascular Birthmarks (July 15, 2007)

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