A rare case of persistent lateral marginal vein of Servelle in Klippel Trenaunay Syndrome: A successful surgical management

Dahal, Sujata; Karmacharya, Robin Man; Vaidya, Satish; Gautam, K; Bhatt, Swechha; Bhandari, Niroj

doi:10.1016/j.ijscr.2022.107052

Cited by 5 publications

(4 citation statements)

References 11 publications

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“…According to the KTS Boston Children's Hospital guideline, there is suspicion in the gene PI3K and mTOR, but the gene is still being investigated further 11 . It is said that the cause is due to mesodermal developmental abnormalities that result in increased size and number of veins, leading to increased blood flow in the affected extremities.…”

Section: Discussionmentioning

confidence: 99%

“…Thus, it hypothesizes that soft tissue and bone hypertrophy are not the direct causes of the static vein in patients KTS. 6,7, 11 Klippel-trenaunay syndrome lacks a definite treatment consensus due to an unknown etiology that is still being investigated. It involves diverse structures and various problems patients face, from abnormal walking posture to psychological disorders and age group differences to the disease.…”

Section: Discussionmentioning

confidence: 99%

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Endovascular laser ablation management of pediatric Klippel trenaunay syndrome: a case report

Annisa,

Joalsen MT,

Michael Caesario

2023

JINAVascAccess

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Introduction: Klippel trenaunay syndrome (KTS) is a rare and complex congenital vascular anomaly as a combination of vascular capillary malformations (port wine stain), venous malformations (VM) and excessive extremity growth with or without lymphatic malformations. The incident KTS is estimated to be 2 to 5 per 100,000 and found in the male gender without distinguishing race. This syndrome is rare, with a low incidence. Klippel trenaunay syndrome uses conservative management with the use of compression stockings. Other KTS management, such as minimally invasive intervention with endovascular laser ablation (EVLA) therapy, is rarely used due to the rare number of diseases. Thus, this study aims to perform the management of KTS in pediatrics. Case description: We report 11-year-old girls complaining of pain and swelling on the right leg when standing long and doing activities. Physical examination appears to show a purplish-red birthmark that arises on the surface of the skin. Ultrasound and CT scan angiography with contrast in the lower region extremities showed hypertrophy accompanied by peripheral branches in the cutaneous layer. Patients are performed endovascular laser ablation using general anesthesia. After endovascular laser ablation, the patient was treated for 2 days without any complaints. Conclusion: KTS is a rare, complex and varied syndrome that has not been found yet. KTS only performs symptomatic treatment depending on clinical and severity. Endovascular laser ablation is a minimally invasive intervention management that must be considered and has a fairly low risk and complication, especially in pediatrics.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Endovascular laser ablation management of pediatric Klippel trenaunay syndrome: a case report

Annisa,

Joalsen MT,

Michael Caesario

2023

JINAVascAccess

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“…The presence of lateral marginal vein was associated with a statistically significant odds ratio (OR) of 2.71, insufficient vena perforans of upper leg OR of 4.08, and convoluted intramuscular veins OR of 2.67 for increased risk of VTE. These aberrant veins can be closed or removed to prevent VTE [ 20 , [24] , [25] , [26] , [27] , [28] ]. The risk of pulmonary embolism is highest in patients who have significant venous ectasia or persistent embryonic veins, which allow for a connection from the superficial vascular malformation to the deep veins [ 23 ].…”

Section: Vtementioning

confidence: 99%

Anticoagulation and vascular anomalies

Crary,

Mack

2024

Research and Practice in Thrombosis and Haemostasis

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“…Minor adverse effects include neutropenia, lymphopenia, infection, and ulcer formation. Patients with KTS have reported immediate improvements with good cosmetic results, including the resolution of lateral veins [52 ▪▪ ,54].…”

Section: Treatmentsmentioning

confidence: 99%

Overgrowth syndromes, diagnosis and management

Klein,

Nisbet,

Kalish

2023

Current Opinion in Pediatrics

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Purpose of review This review will focus on the current knowledge of the diagnosis and management of overgrowth syndromes with specific focus on mosaic conditions and treatment strategies. Recent findings With the implementation of massively parallel sequencing, the genetic etiology many classically described overgrowth syndromes has been identified. More recently, the role of mosaic genetic changes has been well described in numerous syndromes. Furthermore, the role of imprinting and methylation, especially of the 11p15 region, has been shown to be instrumental for growth. Perhaps most importantly, many overgrowth syndromes carry an increased risk of neoplasm formation especially in the first 10 years of life and possibly beyond. The systematic approach to the child with overgrowth will aide in timely diagnosis and efficient alignment them with appropriate screening strategies. In some cases, precision medical interventions are available to target the perturbed growth signaling pathways. Summary The systematic approach to the child with overgrowth aids in the standardization of the diagnostic pathway for these young patients, thereby expediting the diagnostic timeline, enabling rigorous monitoring, and delivering tailored therapeutic interventions.

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A rare case of persistent lateral marginal vein of Servelle in Klippel Trenaunay Syndrome: A successful surgical management

Cited by 5 publications

References 11 publications

Endovascular laser ablation management of pediatric Klippel trenaunay syndrome: a case report

Endovascular laser ablation management of pediatric Klippel trenaunay syndrome: a case report

Anticoagulation and vascular anomalies

Overgrowth syndromes, diagnosis and management

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