Pr08 lymphatic malformations and the molecular basis of lymphangiogenesis.

[lymphede...@gmail.com]

Pr08 lymphatic malformations and the molecular basis of
lymphangiogenesis.

ANZ J Surg. 2007 May

Ch'ng S, Tan ST.
Wellington Regional Plastic Unit, Hutt Hospital, Wellington, New
Zealand.

This paper reviews the clinical features of lymphatic malformations
and the molecular basis of embryonic lymphangiogenesis.

Lymphatic malformations are classified as microcystic, macrocystic, or
combined. Most commonly found in the axilla/chest and cervicofacial
region, they can be localised or diffuse. The commonest complications
are intralesional bleeding and infection. Other significant
complications are due mainly to their mass effect on nearby anatomic
structures including the airway and eyeball, and soft tissue and
skeletal overgrowth including macrocheilia, macroglossia, macrotia,
macromala and mandibular prognathism, resulting in functional problems
in feeding, speech, occlusion, oral hygiene, and disfigurement.

The characteristic radiological finding of a LM on gadolinium-enhanced
T1-weighted MRI is a low-density lesion with septation or rim
enhancement. Histologically, LMs are cystic lesions that contain
eosinophilic proteinaceous fluid whose walls are composed of smooth
and skeletal muscle fibres, collagen and lymphocytes. Management
options range from observation, comfort cares, empirical antibiotic
treatment for LM cellulitis to sclerotherapy, surgical excision and
Nd:YAG laser for selected cases.

Lymphangiogenesis is believed to occur in four sequential but
overlapping stages: lymphatic endothelial cell competence, bias and
specification, and finally lymphatic vessel terminal differentiation
and maturation. Multiple genes are involved in this process including
Lyve1, Nrp2, podoplanin, Prox1, VEGFR3, VEGFC and Ang2. Developmental
defects during embryonic lymphangiogenesis result in lymphatic
malformations.

PMID: 17490238 [PubMed - in process]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17490238&itool=iconabstr&query_hl=5&itool=pubmed_docsum

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Pat O'Connor
Lymphedema People
http://www.lymphedemapeople.com