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Year : 2013  |  Volume : 14  |  Issue : 1  |  Page : 36-38

Cutis marmorata congenita telangiectatica, Klippel-Trenaunay-Weber syndrome or both combined

Department of Neonatology, Princess Anne Hospital, Southampton, United Kingdom

Correspondence Address:
Alok Sharma
Department of Neonatology, Princess Anne Hospital, Coxford Road, Southampton
United Kingdom
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.116860

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Cutis marmorata telangiectatica congenita (CMTC) is a congenital cutaneous vascular anomaly evident as persistent cutis marmorata, telangiectasia and phlebectesia. Klippel-Trenaunay-Weber syndrome (KTW) consists of port wine stains, varicose veins and limb hypertrophy. A term baby was delivered shocked with an extensive cutaneous malformation resembling a port wine stain with hypertrophied forearm and fingers. Deep substance hemangiomas were seen in the liver raising the possibility of KTW syndrome. With resuscitation over the next few days, the malformation evolved into a more marbled appearance with axillary phlebectesia. This along with the presence of syndactyly raised the potential for CMTC but did not explain the liver hemangioma or bony hypertrophy. In this case, there some features of CMTC and some of KTW syndrome raising the possibility of their coexistence in the same case. Sturge-Weber syndrome, KTW syndrome and CMTC may be included in a spectrum of vascular diseases that are associated with other developmental defects of the mesoderm during embryonic life. The variability in presentation with overlap, which is seen in these conditions, may have a common pathophysiology explained by the Happle gene hypothesis.

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