Biomedical subjects
Publications and source records attributed to Alexander Roosen.
The Klippel-Trenaunay Syndrome (KTS) is defined as a triad of cutaneous capillary malformations, venous varicosities, bone and soft tissue hemi-hypertrophy. The urinary tract is involved in up to 10%. We report the clinical presentation and surgical management of a 9-year-old boy with extensive lympho-venous malformations of the bladder which led to massive recurrent gross hematuria.
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Impaired bone metabolism following urinary diversion through intestinal segments has always been a controversial subject of unclear clinical relevance. Whereas the perpetuated pathophysiological considerations seem conclusive in theory, the role of acidosis and malabsorption is less clear in animal experimentation and, even more so, in the clinical reality of modern continent diversion. In hardly any of the available contemporary case series was overt derangement of the acid-base balance, rickets or osteomalacia encountered. No consistent changes in osteotropic serum parameters could be found with normal calcium and phosphate in all patients. The assumption that colonic reservoirs have a higher risk of developing metabolic bone disease could not be confirmed by clinical data. As early correction of base excess is easy and probably a common policy in patients with intestinal urinary reservoirs, it will be virtually impossible to further study the natural history of bone metabolism after urinary diversion. While there is no need for a bone specific follow-up in asymptomatic adults with a normal acid-base balance, particular attention should be paid to children and to all patients with impaired renal function.