Management of Combined Vascular Malformations
Section snippets
Capillary-lymphatico-venous malformation
The first reports of patients with a slow-flow capillary-lymphatico-venous malformation (CLVM) were published in the nineteenth century by Hilaire, Trélat, and Monod.2, 3 It was not until 1900 that this constellation of findings was considered more than mere coincidence. French physicians, Maurice Klippel and Paul Trénaunay,4 were the first to recognize Klippel-Trénaunay syndrome as a distinct entity.5 They proposed the main characteristics of the syndrome were a localized vascular nevus,
Capillary-arteriovenous malformation and capillary-arteriovenous fistulas
Capillary-arteriovenous malformation (CAVM) and capillary-arteriovenous fistulas (CAVFs) correspond to the old eponym Parkes Weber syndrome. This syndrome is characterized by the presence of a confluent or patchy CM with underlying multiple microarteriovenous fistulas in association with soft tissue and skeletal hypertrophy of the affected limb (Fig. 10).7, 34 There is often an associated lymphatic component. Like other vascular malformations, the diagnosis may be suspected antenatally but
CLOVES syndrome
Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies (CLOVES) syndrome is a newly recognized syndrome.37, 38 Its main features are truncal lipomatous masses, vascular malformations, and acral/musculoskeletal anomalies (Fig. 11). Not all features must be present to make the diagnosis of CLOVES syndrome. Until recently, many patients with this syndrome were misdiagnosed as either having CLVM or Proteus syndrome. Like CLVM and Parkes Weber syndrome,
Summary
Proper diagnosis of patients affected by complex combined vascular malformations is essential. These patients benefit from an interdisciplinary approach involving many medical and surgical specialists. Interventions must be tailored to the specific needs and symptoms of the patient. Outcomes are optimized with careful preoperative planning, identification of comorbidities, and realistic expectations on behalf of both the patient and surgeon.
References (38)
- et al.
Vascular anomalies
Curr Probl Surg
(2000) - et al.
Novel roles of GATA1 in regulation of angiogenic factor AGGF1 and endothelial cell function
J Biol Chem
(2009) - et al.
Klippel-Trenaunay syndrome: spectrum and management
Mayo Clin Proc
(1998) - et al.
Endorectal pull-through abates gastrointestinal hemorrhage from colorectal venous malformations
J Pediatr Surg
(2000) - et al.
Portomesenteric venous thrombosis associated with rectal venous malformation
J Pediatr Surg
(2010) - et al.
Persistent sciatic vein: diagnosis and treatment of a rare condition
J Vasc Surg
(1996) - et al.
Extensive pure venous malformations in the upper or lower limb: a review of 27 cases
J Am Acad Dermatol
(1997) - et al.
Endovenous laser and echo-guided foam ablation in great saphenous vein reflux: one-year follow-up results
J Vasc Surg
(2008) Histoire generale et particuliere des anomalies de l’organisation chez l’homme et les animaux
(1832)- et al.
De l’hypertrophie unilaterale partielle ou totale du corps
Arch Gen Med
(1869)
Memoires originaux: du noevus variquex osteo-hypertrophique
Arch Gen Med
Klippel-Trenaunay syndrome: diagnostic criteria and hypothesis on etiology
Ann Plast Surg
Angioma formation in connection with hypertrophy of limbs and hemi-hypertrophy
Br J Dermatol
Hemangiectatic hypertrophy of limbs: congenital phlebarteriectasias and so-called congenital varicose veins
Br J Child Dis
Identification of association of common AGGF1 variants with susceptibility for Klippel-Trenaunay syndrome using the structure association program
Ann Hum Genet
Wilms tumor screening is unnecessary in Klippel-Trenaunay syndrome
Pediatrics
Klippel-Trenaunay syndrome: the risks and benefits of vascular interventions
Surgery
The Klippel-Trenaunay syndrome: clinical, radiological and haemodynamic features and management
Br J Surg
Surgical implications of Klippel-Trenaunay syndrome
Ann Surg
Cited by (21)
Surgical Resection of Vulvar Labial Venous Malformations: A Single Center Experience
2023, Journal of Pediatric SurgeryOvergrowth syndromes and new therapies
2020, Seminars in Pediatric SurgeryCitation Excerpt :Hematuria can also result. Recurrent infections are also a feature of this disease, likely due to bacterial proliferation in abnormal lymphatic spaces.21 Some patients with massive and disabling overgrowth undergo surgical debulking to reduce the size of the affected extremity.
Transcatheter embolization of persistent embryonic veins in venous malformation syndromes
2017, Journal of Vascular Surgery: Venous and Lymphatic DisordersVascular Anomalies in Pediatrics
2015, Advances in PediatricsCitation Excerpt :The marginal veins should be obliterated with excision or embolization or photocoagulated with endovenous laser in childhood prior to the veins becoming large and ectatic. Staged, contour resection of the extrafascial soft tissue overgrowth of the calf, thigh, and buttock can improve function and decrease infection [117]. Orthopedic follow-up is necessary for limb length discrepancy.
Overgrowth syndromes with complex vascular anomalies
2014, Seminars in Pediatric SurgeryCitation Excerpt :Pulse dye laser can be used for treatment of capillary malformation. In patients with disabling, massive overgrowth or large complex lymphatic malformation, surgical debulking of the extrafascial overgrowth can considerably reduce the excessive size of the extremities and limb girdle and considerably improve the quality of life (Figure 2).13 For patients with severe chronic rectal bleeding and blood transfusion-dependence, endorectal pull-through operation may be considered.14
Arteriovenous malformations: A diagnostic and therapeutic challenge
2014, Actas Dermo-Sifiliograficas