Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Wednesday, April 12, 2006

Lymphatic malformations of the head and neck: Introduction of a disease score for children, Cologne Disease Score (CDS)

Wittekindt C, Michel O, Streppel M, Roth B, Quante G, Beutner D, Guntinas-Lichius O.

Clinic of Otorhinolaryngology, Head and Neck Surgery, University of Cologne, Cologne, Germany.


A clinical staging system for children with lymphatic malformations that would allow for a standardized comparison of disease and treatment outcomes.


We developed an examination sheet for the determination of a disease score ranging from 0 (worst) points to 10 (best) points, Cologne Disease Score (CDS). Disfigurement, dysphagia, dysphonia, dyspnea and an observer statement towards progression were contributing to CDS. Each parameter yielded two, one or zero points. Two points were given when no limitation was seen in the patient concerning the respective item. One point was given at mild limitation and zero points were given when considerable limitation in the respective item could be observed. We evaluated 26 patients with lymphatic malformations of the head and neck retrospectively by completing one examination sheet for each patient-visit.


Four patients had an initial CDS value of three or less points (severe disease-group). After therapy the mean value of their score increased only slightly to 3.9+/-2.6 points. Six patients had an initial CDS value of four, five or six points (advanced disease-group). After treatment their mean score value increased dramatically to 9.0+/-1.5 points (P<0.001).>


The evaluation of the CDS was easy and expeditious. The score itself was a good predictor in view of the outcome. This new disease score for paediatric patients might be appropriate to evaluate therapeutic trials in paediatric patients.

PMID: 16460815

[PubMed - as supplied by publisher]


Management of lymphatic malformations in oral and maxillofacial regions: the rationale according to the new classification

Zheng JW, Qin ZP, Zhang ZY.

According to the classification of ISSVA, Waner and Suen, the traditionally called lymphangiomas are now referred to as lymphatic malformations, including both macrocystic and microcystic lesions. They are commonly seen vascular anomalies, and most frequently diagnosed at birth and most often occur in the head and neck area. The etiology of lymphatic malformations is still unknown, resulting in a variety of treatment modalities.

This paper reviewed the recent literatures with respect to the indications, contraindications, advantages and disadvantages of surgical excision, sclerotherapy and laser ablation of lymphatic malformations of the head and neck, with detailed discussion of the advances in molecular biology and clinical treatment prospects. It is concluded that although lymphatic malformations are benign lesions, they seldom involute spontaneously. Of all vascular malformations, lymphatic malformations are the most difficult to be eradicated. Their infiltrating nature coupled with the difficulty in distinguishing involved important structures of the head and neck from adjacent normal tissues makes complete surgical extirpation even more difficult. The likelihood of postsurgical recurrence and complications is thus higher than other vascular lesions.

Although many treatment protocols are available in clinic, indications, contraindications, advantages and disadvantages exist in each modality. The selection of treatment modalities should depend on the patients' status and techniques available. The treatment protocol should be individualized and comprehensive as well as sequential, in order to obtain the best treatment outcomes. In general, treatment will vary according to the depth and the extent of the lesions. Superficial mucosal microcystic lesions and cervicofacial macrocystic lesions are amenable to ablation with sclerotherapy using Bleomycin and OK-432 with the advantages of the absence of a surgical scar.

The sclerosing agent OK-432 is effective for macrocystic lymphatic malformations but showed less promise for microcystic lesions, mixed lesions, and lesions outside the head and neck region. In addition, superficial mucosal microcystic lesions are also amenable to CO2 laser therapy. Deeper microcystic lesions are still challenging head and neck surgeons, which are usually extensive and sometimes need to be resected in stages, and even may be impossible to be completely removed.

Somnoplasty shows promise for reduction of tongue lymphatic malformations. Surgical excision, staged when necessary, continues to be integral to management in many cases, but should not be overused without consideration of the histologic types and extent. Localized mucosal microcystic lesions and major cervicofacial macrocystic lesions are amenable to primary excision.

Care should be exercised in identifying and preserving important cervical and facial structures because anatomical planes are often distorted. Combined sequential approach is recommended for mixed lesions as well as extensive lesions involving both the mucosa and soft tissues.

Publication Types:

PMID: 16400476 [PubMed - in process]