An atypical case of Klippel-Trénaunay syndrome presenting with crossed-bilateral limb hypertrophy and postaxial polydactyly: a case report

Abstract

Background Klippel-Trénaunay syndrome (KTS) is a rare congenital condition characterized by the clinical triad of capillary malformations (port wine stains), varicose veins with or without venous malformations, and bony and/or soft tissue hypertrophy. Case presentation Here we report the first case of a one-day-old male with KTS presenting with crossed-bilateral limb hypertrophy and post-axial polydactyly. Conclusion This case serves to highlight the variable presentation and multiple problems faced by patients with KTS and why multidisciplinary management is mandatory.

Klippel-Trenaunay-Weber Syndrome – A Rare Case with Visceral Manifestation and Thrombosed Marginal Vein of Servelle

“…9 Treatment options include regular compression stockings and graded compression of the affected limb, stripping and sclerotherapy of varicosities and laser ablation for port wine stains. 10…”

A neonate with Klippel–Trénaunay syndrome: a case report

“…Surgical and orthopedic management can be applied too for the extremity asymmetry and spine deformities such scoliosis. Port wine stains are treated with pulsed dye laser therapy [ 16 ].…”

Periosteal new bone formation in Klippel-Trénaunay syndrome: a case report

“…Atypical clinical manifestations of KTS reportedly include hypersplenism, nephrotic syndrome, cerebral cavernous angioma, and puerperal hemorrhage [ 17 – 19 ]. Bone involvement in KTS is commonly noted and typically manifests as circumferential hypertrophy, longer extremities, ectrodactyly, polydactyly, syndactyly, camptodactyly, and clinodactyly, and in rare cases, intraosseous vascular malformation [ 1 , 2 , 20 , 21 ]. However, to the best of our knowledge, this report presents the first case of KTS with periosteal new bone formation.…”

“…Klippel-Trénaunay syndrome (KTS) in childhood is well-documented and commonly characterized by port-wine stains, varicose veins, and the overgrowth of long bones and soft tissues [ 1 , 2 ]. In this report, we describe an atypical pediatric case of KTS, in which a 17 cm-long periosteal new bone formation of the tibia developed rapidly within 7 weeks.…”