title: Renal lymphangiomatosis, interrupted IVC with persistent primitive hepatic venous plexus and multiple anomalous venous channels: parts of an overlap syndrome?
abstract: A 5-year-old girl with cutis marmorata telangiectasia congenita (CMTC) and congenital glaucoma, who had previously presented with seizures, transient hemiplegia, upper gastrointestinal bleeding and hemihypertrophy, developed a large pleural effusion. Subsequent imaging revealed renal lymphangiomatosis, multiple anomalous intra-abdominal venous channels, an interrupted inferior vena cava with a persistent primitive hepatic venous plexus (PPHVP) and meningeal angiomas. To the best of our knowledge, the CT findings of PPHVP and the combination of the demonstrated abnormalities have not been previously reported. They may represent an overlap syndrome of CMTC, Sturge-Weber syndrome and Klippel-Trenaunay syndrome. The complexity and degree of overlap highlights the importance of an accurate clinical and anatomical description and good communication among clinicians.
authors.affiliation: Clarendon Wing Radiology Department, Leeds General Infirmary, Leeds, LS2 9NS, UK.
authors.forename: Tom
authors.initials: T
authors.lastname: Watson
authors.forename: Ester
authors.initials: E
authors.lastname: Martinez
authors.forename: David
authors.initials: D
authors.lastname: Crabbe
authors.forename: Phillip
authors.initials: P
authors.lastname: Chetcuti
authors.forename: Jeannette K
authors.initials: JK
authors.lastname: Kraft
mesh_terms: D000015:Abnormalities, Multiple; D002675:Child, Preschool; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D005260:Female; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000175:diagnosis*; D054068:Livedo Reticularis; D008099:Liver / Q000098:blood supply; D017445:Skin Diseases, Vascular / Q000175:diagnosis*; D013341:Sturge-Weber Syndrome / Q000175:diagnosis*; D013684:Telangiectasis / Q000151:congenital* / Q000175:diagnosis; D014680:Veins / Q000002:abnormalities*; D014682:Vena Cava, Inferior / Q000002:abnormalities*