From the Guidelines
Visceral hemangiomas can be a part of Klippel-Trenaunay syndrome (KTS), and their management depends on their location, size, and associated symptoms. The diagnosis of KTS is based on the presence of capillary malformations, venous malformations, and limb overgrowth, but visceral hemangiomas can occur less frequently as part of the syndrome 1.
Key Considerations for Diagnosis
- The classification of vascular anomalies, as adopted by the International Society for the Study of Vascular Anomalies, is crucial for accurate diagnosis and management 1.
- Visceral hemangiomas in KTS can affect various organs, including the gastrointestinal tract, liver, spleen, or genitourinary system, with varying clinical implications.
Management Approach
- Asymptomatic visceral hemangiomas are typically monitored with regular imaging (ultrasound, MRI, or CT scans) every 6-12 months.
- For symptomatic lesions, treatment options include pharmacological therapy with propranolol (starting at 0.5-1 mg/kg/day divided into three doses, gradually increasing to 2-3 mg/kg/day) or sirolimus (0.8 mg/m² twice daily, adjusted to maintain trough levels of 5-15 ng/mL) 1.
- Interventional procedures such as embolization may be necessary for bleeding hemangiomas, while surgical resection is reserved for cases with severe symptoms, complications, or failure of other treatments.
Multidisciplinary Care
- Multidisciplinary care involving vascular specialists, interventional radiologists, and surgeons is essential for optimal management of visceral hemangiomas in KTS.
- The approach to visceral hemangiomas in KTS requires individualization based on the specific organ involvement and associated symptoms 1.
From the Research
Diagnosis of Klippel-Trenaunay Syndrome
The diagnosis of Klippel-Trenaunay syndrome (KTS) is based on the clinical triad of:
- Capillary malformations (port wine stain)
- Soft tissue and bone hypertrophy or hypotrophy of usually one lower limb
- Atypical, mostly lateral varicosity 2, 3, 4 A thorough history and clinical examination is enough for the diagnosis of KTS in most cases, but noninvasive imaging techniques may be used to evaluate the disease in patients with certain complications 3.
Visceral Hemangiomas in Klippel-Trenaunay Syndrome
Visceral hemangiomas can be part of KTS, as the syndrome may affect internal organs 5. KTS is a mixed vascular malformation, with predominant capillary, venous, and lymphatic components, without significant arteriovenous shunting 2. The management of visceral hemangiomas in KTS is not specifically discussed in the available studies, but a multidisciplinary approach to management of KTS is warranted 2, 3.
Management of Klippel-Trenaunay Syndrome
The management of KTS is largely conservative and includes:
- Compression therapy to reduce edema due to chronic venous insufficiency
- Laser treatment for port wine stains
- Sclerotherapy, endovenous thermal ablation, or surgical stripping and phlebectomy for superficial veins and malformations 2, 6 A multidisciplinary approach to management of KTS is essential due to the diversity of presentation 3.