Coagulation Abnormalities in Pediatric and Adult Patients After  Sclerotherapy or Embolization of Vascular Anomalies

Mason, Keira P.; Neufeld, Ellis J.; Karian, Victoria E.; Zurakowski, David; Koka, Babu V.; Burrows, Patricia E.

doi:10.2214/ajr.177.6.1771359

Cited by 80 publications

(52 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…[2][3][4][5][6][7][8] Hematologic evidence of low-grade consumptive coagulopathy has been documented repeatedly for patients with large venous malformations. [4][5][6] Mason et al, 4 Mazoyer et al, 5 and Enjolras et al 6 studied coagulation parameters among patients with large venous malformations and recognized that many patients had hematologic evidence of coagulopathy, defined by elevated D-dimer and soluble fibrin complex levels, decreased fibrinogen levels, and elevated prothrombin times, with normal to moderately low platelet counts. The magnitude of the coagulopathy correlated with the severity of the malformation.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Thromboembolism Associated With Klippel-Trenaunay Syndrome

Huiras¹,

Barnes

Eichenfield

et al. 2005

Pediatrics

View full text Add to dashboard Cite

ABSTRACT. Klippel-Trenaunay syndrome (KTS) is a rare congenital anomaly characterized by unilateral limb overgrowth, venous varicosities, and capillary malformations (port wine stains) of the affected limb or limbs. Large venous malformations such as those observed in KTS are rare, and many physicians are unfamiliar with the potential complications, which include hypercoagulability, thrombosis, and pulmonary embolism (PE). As a result, patients may suffer from delayed diagnosis of a potentially life-threatening thromboembolic event. We present 2 cases of children with KTS complicated by PE, and we review the English-language literature regarding pathophysiologic features, interventions, and outcomes of PE in the setting of KTS among both pediatric and adult patients, with emphasis on issues relevant to pediatricians. I n 1900, French physicians Klippel and Trenaunay described a patient with massive hemihypertrophy of the lower extremity associated with a large, confluent, red-blue plaque and venous varicosities. 1 The term Klippel-Trenaunay syndrome (KTS) has since been used to describe rare, congenital, vascular malformations identified by the classic clinical triad of venous varicosities, ipsilateral cutaneous capillary malformations, and bony/soft-tissue overgrowth of the affected limb or limbs. As our understanding of vascular lesions evolves, it is clear that the constellation of findings reported by Klippel and Trenaunay represents complex, mixed, vascular malformations, often with venous, capillary, and lymphatic components, with various clinical consequences, including hypercoagulability and the complications that ensue. We describe 2 children with KTS of the lower extremity who suffered from pulmonary embolism (PE). CASE REPORTS Patient 1A 14-year-old boy with a combined capillary-venous-lymphatic malformation of the right lower extremity who had been diagnosed as having KTS at birth presented to an outside institution after 2 episodes of syncope. During these episodes, he was unresponsive for several minutes and was observed to have circumoral cyanosis and tonic posturing. Two weeks earlier, the child had been treated for cellulitis, with fever, pain, and warmth of his affected extremity; blood cultures were positive for Staphylococcus aureus, and the patient was treated with a course of orally administered cephalexin. After the second episode of syncope, the patient was transferred to Children's Hospital of Wisconsin (Milwaukee, WI) for evaluation.In the physical examination, the child was pale and lethargic, with a heart rate of 119 beats per minute, a respiratory rate of 28 breaths per minute, and oxygen saturation ranging from 93% to 97% with room air. A cardiac examination revealed a new grade II/VI systolic murmur. The lungs were clear. The right lower extremity was enlarged, with 2ϩ edema at the ankle to mid-shin level, which was its usual size, according to the boy's family. There was an extensive red-purple patch extending from the right buttock to the lateral ankle (Fig 1). There were hundred...

show abstract

Section: Discussionmentioning

confidence: 99%

Pulmonary Thromboembolism Associated With Klippel-Trenaunay Syndrome

Huiras¹,

Barnes

Eichenfield

et al. 2005

Pediatrics

View full text Add to dashboard Cite

show abstract

“…Sclerotherapy is the primary treatment, alone or in combination with surgical resection (3,4). Sclerotherapy can cause swelling, skin necrosis, peripheral nerve deficits, and rarely, cardiac arrest, depending on the sclerosing agent used (9)(10)(11)(12). Extensive VMs commonly persist after treatment (4).…”

Section: Introductionmentioning

confidence: 99%

Rapamycin improves TIE2-mutated venous malformation in murine model and human subjects

Boscolo¹,

Limaye²,

Huang³

et al. 2015

Journal of Clinical Investigation

181

235

View full text Add to dashboard Cite

R e s e a R c h a R t i c l e3 4 9 2 jci.org Volume 125 Number 9 September 2015Other factors are likely involved, since over 80 genes were differentially expressed (at least 2-fold) in the TIE2-L914F endothelial cells compared with WT (30). We hypothesized that mutant TIE2 in endothelial cells is sufficient to cause VM. Here, we show that HUVECs engineered to Previous studies on mutant TIE2 showed that expression of TIE2-L914F or TIE2-R849W in HUVECs increased activation of AKT and of . Elevated AKT signaling in these cells has been linked to increased survival (29, 30) and to reduced production of PDGF-B (30), a major player in mural cell recruitment.

show abstract

“…34,35 Most likely, the increased D-dimer levels mainly reflect local fibrinolytic effects in and around the vascular malformations. 23 The high prevalence of a history of VTE in our cohort suggests that this localized intravascular coagulation can eventually evolve to the formation of deep vein thrombi and consequently (recurrent) PE. 21 Because there is overwhelming localized fibrinolytic activity in the vascular malformations, fibrinolytic activity elsewhere in the body will be obscured.…”

Section: Discussionmentioning

confidence: 75%

“…Although up to 30 KTS case reports with PE have been published, 4,10,[12][13][14][15][16][17][18][19][20][21] the exact mechanism underlying the hypercoagulability in vascular malformations remains unclear; however, coagulation activation may be attributed to the stagnation of blood within the distorted, enlarged venous blood vessels. [22][23][24] This could lead to a continuous formation of thrombi, resulting in recurrent PE, as reported in several cases. 14,16,18 Moreover, unresolved recurrent PE, originating in the vascular malformations, may lead to chronic thromboembolism (CTE) or even CTE pulmonary hypertension (CTEPH) in patients with KTS.…”

mentioning

confidence: 99%