Table 7.

Recommended Surveillance for Individuals with TEK-Related Venous Malformations

System/ConcernEvaluationFrequency
VM Eval by vascular anomalies specialist
  • Annually or as needed (esp around puberty or other hormonal changes, as VM can become painful)
  • Lesions can ↑ in size over time & become painful or symptomatic.
GI lesions
  • CBC incl Hgb & hematocrit level
  • Iron levels (incl serum iron, serum ferritin, & transferrin levels)
  • Endoscopy
  • Annually or more frequently in case of symptoms (fatigue, bloody stools)
  • Consider endoscopy based on blood results.
Coagulopathy/Bleeding D-dimer & fibrinogen levelsShould be checked:
  • Every 5 yrs;
  • If lesions become painful;
  • Before any surgical &/or sclerotherapeutic procedure.
Treatment w/sirolimus See Table 8.See Table 8.

CBC = complete blood count; GI = gastrointestinal; Hgb = hemoglobin; VM = venous malformations

From: TEK-Related Venous Malformations

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