Venous Malformation and Localized Intravascular Coagulopathy in Children

Abstract

Localized intravascular coagulopathy (LIC) has been described in adults with venous malformation (VM) but rarely reported in children. This study aims to determine the prevalence of LIC in children with VM and associated risk factors. Patients younger than 18 years with VM from 2010 to 2014 were reviewed. Diagnosis was confirmed by Doppler ultrasound and/or magnetic resonance imaging. Demographics data and VM characteristics including volume, site, extension, painful symptoms, and palpable phleboliths were studied. Plasma D-dimer level of greater than 500 ng/mL was considered as abnormal. Total 24 children were included, of whom 8 were boys. Median age of presentation was 9 months (range: 0-12 years). Head-and-neck VM occurred in 17 (70.8%) patients and 3 (12.5%) had multifocal lesions. Seven (29.2%) patients had VM volume greater than 10 mL. Five (20.8%) patients had painful symptoms. Palpable phleboliths were found in two patients. Plasma D-dimer was raised in eight cases (33.3%). One patient with Klippel-Trenaunay syndrome (KTS) had D-dimer level of 5,000 ng/mL. Raised D-dimer was found in 23.5% of small VM (volume < 10 mL) and 57.1% of large VM ( = 0.167). D-dimer was significantly raised in multifocal VM ( = 0.028) and showed increasing trend in lesions with palpable phleboliths ( = 0.101). All patients had sclerotherapy performed with indications (cosmesis 41.7%, enlarging lesion 29.2%, pain 20.8%, bleeding 8.3%). Perioperatively, bolus intravenous fluid and mannitol were given to selected patients. All patients had VM volume reduction after sclerotherapy. There were no major thromboembolic complications. LIC with raised D-dimer level occurred in one-third of pediatric VM. It was more common in large, multifocal VM and in those with palpable phleboliths or KTS.

Combined neodymium-doped yttrium aluminum garnet laser and sclerotherapy in Gorham-Stout syndrome

“…About one-third of pediatric VMF patients are affected by localized intravascular coagulation and have D-dimer levels greater than 500 ng/mL. 9 Lesion size, presence of phleboliths, truncal location, and spongiform morphology on MRI were correlated with localized intravascular coagulation. 10 As shown in Fig 2 , laser plus sclerotherapy was accompanied in our patient by normalization of localized intravascular coagulation.…”

Clinical efficacy of absolute ethanol combined with n‑butyl cyanoacrylate sclerotherapy in the treatment of Puig's classified advanced venous malformation in children

“…It has also been indicated that notable sclerosing agent reflux causes serious complications. The effective rate of absolute ethanol treatment for low draining venous malformations has been reported as 75–95% (14,15,20); however, the efficacy of interventional therapy for Puig's classified advanced venous malformations has been unsatisfactory, which was reported to be at only 41% (21). To the best of our knowledge, this is the first study to report the use of absolute ethanol combined with n-butyl cyanoacrylate sclerotherapy in patients for the treatment of Puig's classified advanced venous malformation.…”

Sclerotherapy with Adjunctive Stasis of Efflux (STASE) in Venous Malformations: Techniques and Strategies

“…Depending on the series, 33%-88% of patients have some degree of d-dimer elevation and approximately 5% with decreased fibrinogen levels. [14][15][16][17] The detection of LIC and coagulopathy is important for 3 reasons. First, painful flareups may be associated with aggravation of those hematologic parameters associated with LIC and therefore conservative (eg, compression garments) or medical treatment directed to this portion of the coagulation cascade, that is, low molecular-weight heparin (LMWH) or direct anti-Xa agents may control and improve patient symptoms.…”

“…12,14,[18][19][20][21] Second, patients with coagulopathy are more likely to develop painful phleboliths, thrombophlebitis, deep venous thrombosis, and pulmonary embolism and therefore must be identified to allow appropriate thromboprophylaxis. 15,17 Third, and most importantly to the interventional radiologist, minor sclerotherapy among other factors can cause LIC to rapidly progress onto disseminated intravascular coagulation (DIC) either intra-or postprocedurally with life-threatening consequences. 13 The interventional radiologist must ensure patients are screened for risk of LIC and sent for appropriate lab studies, and where appropriate, be treated medically both periprocedurally and/or chronically.…”