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Table 1 Clinical characteristics associated with CLOVES syndrome, K–T syndrome, and M-CM

From: Mapping the PIK3CA-related overgrowth spectrum (PROS) patient and caregiver journey using a patient-centered approach

PROS condition Clinical characteristics
CLOVES [3, 9] Overgrowth (lipomatous, spinal/paraspinal, bony, limb and digit); cutaneous and vascular malformations (lymphatic, venous, capillary ± arteriovenous malformations); varicose embryonic veins and blood clots; musculoskeletal abnormalities; visceral abnormalities; neurologic abnormalities
K–T [3, 10, 11] Vascular malformations (capillary, venous, ± lymphatic malformations) or varicose embryonic veins and blood clots; hypertrophy of bone and soft tissue (features are typically isolated to the pelvis and a lower extremity, but upper extremities and other organs can be involved)
M-CM [3, 9] Segmental overgrowth of brain tissue (megalencephaly or hemimegalencephaly); cutaneous capillary malformations with focal or generalized somatic overgrowth; digital anomalies; tone abnormalities; mild to severe intellectual disability may be present
  1. CLOVES, congenital lipomatous overgrowth, vascular malformations, epidermal nevi, scoliosis/skeletal and spinal anomalies; K–T, Klippel–Trénaunay; M-CM, megalencephaly-capillary malformation