Document Detail


Klippel-Trenaunay and Sturge-Weber syndromes with renal hemangioma and double inferior vena cava.
MedLine Citation:
PMID:  3016342     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe a 3 1/2-year-old boy with the Klippel-Trenaunay and Sturge-Weber syndromes. The child had congenital superficial capillary hemangiomas, congenital glaucoma and mild hydrocephalus. During the first year of life he experienced intermittent hematuria. When he was 3 years old he presented with seizures and left hemihypertrophy first was noted. Several months later radiological examination of a large abdominal mass demonstrated its origin to be in the right kidney. Radical nephrectomy documented the presence of renal hemangioma with complicating perirenal hematoma. A double inferior vena cava was another unexpected surgical finding that complicated the course of this patient. All of these unusual features in these rare syndromes with their clinical, pathogenetic and therapeutic implications are discussed. The differential diagnosis of renal masses in these syndromes also is presented.
Authors:
D Schofield; G S Zaatari; B B Gay
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Journal of urology     Volume:  136     ISSN:  0022-5347     ISO Abbreviation:  J. Urol.     Publication Date:  1986 Aug 
Date Detail:
Created Date:  1986-09-17     Completed Date:  1986-09-17     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0376374     Medline TA:  J Urol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  442-5     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Angiomatosis / complications*
Child, Preschool
Glaucoma / congenital
Hemangioma / complications*,  pathology
Humans
Hydrocephalus / complications
Kidney / pathology
Kidney Neoplasms / complications*,  pathology
Klippel-Trenaunay-Weber Syndrome / complications*
Male
Skin Neoplasms / complications
Sturge-Weber Syndrome / complications*
Vena Cava, Inferior / abnormalities*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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