CLOVES syndrome is characterised by congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/scoliosis/skeletal deformities [1].

It results from a somatic mosaicism in the phosphatidylinositol 3-kinase catalytic subunit alpha (PIK3CA) gene and is a PIK3CA-related overgrowth spectrum (PROS) disorder that often presents at birth or childhood [2,3]. It is an extremely rare, nonhereditary disorder, with an estimated prevalence lower than 1:1,000,000 [4].

Skeletal abnormalities are in the upper and lower extremities and can include macrodactyly, sandal gap, leg-length discrepancy, hemihyperplasia, chondromalacia and furrowed soles [5]. Spinal abnormalities are common among affected individuals and can include tethered cord, scoliosis, and neural tube defects [6]. Other findings not included in the acronym include seizures, renal agenesis/hypoplasia, lymphatic malformations, splenic lesions, gastrointestinal involvement and an increased risk of developing Wilms tumours [6,7]. The vascular malformations typically present in childhood and include (venous, lymphatic and capillary) slow-flow and deeper (arteriovenous) high-flow vascular lesions [8]. Vascular and lymphatic lesions can lead to pain, haemarthrosis, bony abnormalities, bleeding, pulmonary embolism, thrombophlebitis, and even airway obstruction [9].

The prognosis of CLOVES syndrome depends on the severity of illness [5]. Mild disease tends to have a good prognosis. Treatment modalities for CLOVES syndrome includes supportive therapies, surgical interventions, and possible (mTOR) inhibitors [10].