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.
References
Vissers W, Van Steensel M, Steijlen P et al (2003) Klippel-Trenaunay syndrome and Sturge-Weber syndrome: variations on a theme? Eur J Dermatol 13:238–241
Garzon MC, Huang JT, Enjolras O et al (2007) Vascular malformations. Part II: associated syndromes. J Am Acad Dermatol 56:541–564. doi:10.1016/j.jaad.2006.05.066
Telander RL, Kaufman BH, Gloviczki P et al (1984) Prognosis and management of lesions of the trunk in children with Klippel-Trenaunay Syndrome. J Pediatr Surg 19:417–422. doi:10.1016/S0022-3468(84)80266-3
Darwish K, Bleau BL (1998) Extensive small bowel varices as a cause of severe anemia in Klippel-Trenaunay-Weber Syndrome. Am J Gastroenterol 93:2274–2275
Verma A, Gupta RK, Kumar S et al (2006) Non-invasive imaging of persistent primitive hepatic venous plexus using doppler sonography and magnetic resonance imaging. Eur J Rad Extra 60:69–73. doi:10.1016/j.ejrex.2006.09.002
Gajinov Z, Misic-Pavkov G, Matic M et al (2008) Persistent hepatic venous plexus in a patient with Sturge-Weber Syndrome. Pediatr Dematol 25:452–454
Joshi M, Cole S, Knibbs D et al (1992) Pulmonary abnormalities in Klippel-Trenaunay syndrome. A histologic, ultrastructural, and immunocytochemical study. Chest 102:1274–1277. doi:10.1378/chest.102.4.1274
Levine C (1989) Primary disorders of the lymphatic vessels—a unified concept. J Pediatr Surg 24:233–240. doi:10.1016/S0022-3468(89)80001-6
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Watson, T., Martinez, E., Crabbe, D. et al. Renal lymphangiomatosis, interrupted IVC with persistent primitive hepatic venous plexus and multiple anomalous venous channels: parts of an overlap syndrome?. Pediatr Radiol 42, 253–256 (2012). https://doi.org/10.1007/s00247-011-2184-2
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-011-2184-2