Table 7 - uploaded by Usama Lotfi
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Source publication
PurposeKTS represents a challenging problem in both diagnosis and management. This study presents an example of a radiological protocol which may prove extremely beneficial in the diagnostic and surgical planning strategy.Study designThis is a prospective study involving 20 patients that had been clinically diagnosed as having KT syndrome. The prop...
Context in source publication
Context 1
... skeletal anomalies associated with KT syndrome were found as toe macrodactyly and foot gigantism in 5, con- genital or acquired scoliotic deformity in 3 and lateral patellar subluxation in 2 patients (Table 7). Foot anomalies repre- sented an important focus of imaging as combined skeletal, vascular, and soft tissue anomalies were found (Table 8). ...
Citations
... Un complementario que ayudaría la confirmación del diagnóstico, el pronóstico y la conducta médica a seguir lo constituye la resonancia magnética tridimensional contrastada ya que muestra las diferencias en el tamaño de los miembros, los problemas vasculares venosos, el estado del hemangioma, su tipo y tamaño, y las comunicaciones arteriovenosas. 21 Existe el consenso de que el tratamiento más utilizado es el conservador o médico, lo que coincidió con el resultado obtenido, donde se trataron los enfermos con medias elásticas y la corrección ortopédica por la desigualdad del tamaño de los miembros. ...
Congenital limb length discrepancy disorders are frequently associated with a variety of vascular anomalies and have unique genetic and phenotypic features. Many of these syndromes have been linked to sporadic somatic mosaicism involving mutations of the phosphoinositide 3-kinase (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway, which has an important role in tissue growth and angiogenesis. Radiologists who are aware of congenital limb length discrepancies can make specific diagnoses based on imaging findings. Although genetic confirmation is necessary for a definitive diagnosis, the radiologist serves as a central figure in the identification and treatment of these disorders. The clinical presentations, diagnostic and imaging workups, and treatment options available for patients with Klippel-Trenaunay syndrome, CLOVES (congenital lipomatous overgrowth, vascular anomalies, epidermal nevi, and scoliosis/spinal deformities) syndrome, fibroadipose vascular anomaly, phosphatase and tensin homolog mutation spectrum, Parkes-Weber syndrome, and Proteus syndrome are reviewed. ©RSNA, 2019.
Klippel-Trenaunay syndrome (KTS) is a rare vascular malformation overgrowth syndrome that is characterized by the triad of (1) capillary malformation, (2) venous malformation or varicosities +/− lymphatic malformation, and (3) bony or soft tissue overgrowth. Recent advances have confirmed it to be caused by a somatic activating mutation in PIK3CA. While no cure exists, treatment is supportive and aimed at alleviating and preventing complications such as chronic venous insufficiency, thrombosis, and pain.
Case:
A 66-year-old Caucasian man with Klippel-Trenaunay-Weber syndrome (KTWS) presented with chronic changes related to the KTWS, along with worsening pain and motion associated with residual damage from an episode of spontaneous septic arthritis that occurred 1 year prior. He underwent total joint arthroplasty with a rotating hinged knee implant.
Conclusion:
Arthroplasty is a treatment option for patients with KTWS; however, there are risks that must be considered. This case report outlines the management of a patient with KTWS and a history of septic arthritis.
