Extensive Venous Malformation Research Articles

Intraoperative Anaphylaxis Following Injection of a Bleomycin-Gelatin Solution for Sclerotherapy

During the perioperative period, the most commonly identified agents that are responsible for acute allergic reactions include antibiotics, neuromuscular blocking agents, opioids, chlorhexidine, and iodinated dyes for radiologic imaging. However, whenever an allergic reaction is suspected, all of the agents to which the patient has been exposed must be considered. Although bleomycin is utilized as the primary agent for sclerotherapy in the treatment of vascular malformations, other substances such as Surgiflo® may be added to the bleomycin solution to increase its efficacy and promote thrombosis of the smaller vessels. These products are derived from animal collagen and contain gelatin which may lead to an allergic reaction. We present an 11-year-old girl undergoing sclerotherapy treatment of an extensive left lower extremity venous malformation who subsequently developed perioperative hemodynamic instability requiring intervention after receiving an injection of a bleomycin and Surgiflo® solution. Further investigation identified gelatin in the Surgiflo® as the causative agent of the allergic event. Previous reports of such reactions are reviewed and the perioperative care and treatment of patients who experience anaphylaxis is addressed.

Total Hip Arthroplasty in Extensive Venous Malformations: An Experience of Four Cases

Background: Extensive venous malformation (VM) (Klippel-Trenaunay syndrome, KTS) and associated degenerative joint disease of the hip is a rare condition. The difficulty of total hip replacement (THR) depends on tissue involvement around the joint, with the risk of severe blood loss. To evaluate the feasibility and outcome of hip replacement in this disease. Methods: Between 2010 and 2020, 4 patients with extensive VM of the limb and degenerative joint disease had a total hip replacement. Investigations included X-ray exams, T2 MRI with fat saturation, and hematologic tests. The main outcome end- points of the study were success of hip replacement, postoperative complications and quality of life. Results: Four patients underwent THR. Le sex ratio was (3 males, 1 female) with a median age of 37 years (range 27-55). All were symptomatic, with worsening hip pain and use of a mechanical aid. MRI identified VM in the muscles and cellular space of the buttock. The choice of surgical approach was anterior in three patients, and lateral in one. Mean blood loss was 1500mL. Surgery was completed by peroperative sclerotherapy to reduce blood loss (3 patients). The median follow-up time was 82 months (range 12- 120). There were no postoperative complications. At 6 months, three patients were walking with normal gait, and one was using an orthopedic walker after knee arthrodesis. There was no reoperation during the follow-up. All patients returned to their professional activities. Conclusion: The difficulty of total hip replacement depends on the extent of involved soft tissue around the hip area. The choice of surgical approach, surgical skill in vascular and orthopedic procedures and per-operative sclerotherapy permit to reduce surgical risk.

Sirolimus for the treatment of vascular anomalies in children

Vascular anomalies (VA) comprise a heterogeneous group of diseases associated with congenital angiogenesis disorder. There are no currently developed unified protocols and treatment regimens for systemic forms of VA. Numerous advantages show sirolimus, an mTOR inhibitor, as a well tolerated and effective antiproliferative and antiangiogenic therapy in patients with VA. The article presents the results of treatment of 211 patients with VA (6 patients with vascular tumors and 205 patients with vascular malformations) aged 2 months to 17 years (median – 9 years), who received sirolimus therapy for 1–86 months (median – 24 months). Sirolimus was administered at a starting dose of 0.8 mg/m2/day orally in two doses with an interval of 12 hours. The concentration of the blood preparation was maintained in the therapeutic range of 6–15 ng/ml. Since 2015, concomitant therapy with co-trimoxazole for the prevention of Pneumocystis pneumonia has been prescribed only to tracheostomy carriers. When infectious episodes occurred in patients, sirolimus therapy continued without changes in the dose of the drug and did not affect the disease, provided that the therapeutic concentration was maintained. A positive response to therapy was observed in 89.1 % of patients with VA in the form of the size of the vascular mass according to the data of visual examination and instrumental control. All patients showeda clinical response to therapy in the form of relief of painsyndrome, reduction/relief of lymphorrhea, reduction/improvement of hemostasis parameters, and an increase in functional activity and quality. For the entire observation period 2012–2020, when taking sirolimus, not a single severe adverse event occurring in post-transplant patients has been reported that would require discontinuation of the drug. The article presents two clinical cases of sirolimus use in the treatment of patients with kaposiform hemangioendothelioma and extensive venous malformation. Parents are encouraged to use the information in scientific research and publications.

Novel Approach to Management of Localised Intravascular Coagulopathy in Extensive Low Flow Venous Malformations Using Rivaroxaban

Introduction: Extensive low flow venous malformations, such as Klippel Trenaunay Syndrome (KTS), are rare conditions. Abnormal blood flow in these structures leads to activation of coagulation, sometimes referred to as localised intravascular coagulopathy (LIC). This results in spontaneous, sometimes painful, thrombus formation and fibrinolysis as indicated by raised D-dimer levels.In some cases the LIC can be so severe that it leads to a consumptive coagulopathy with bleeding complications. It is important to assess for this, particularly as many of these patients undergo corrective surgeries. It has been noted that low fibrinogen and, more recently, low FXIII levels correlate with severity of LIC and bleeding risk in these cases while other coagulation factors and platelet counts seem to be unaffected.There is very little published evidence to help with antithrombotic and haemostatic management of these rare and complex patients. Anticoagulation with low-molecular-weight heparin (LMWH) has been used to reduce LIC and consumptive coagulopathy on a case-by-case basis to either manage thrombotic events or reduce bleeding risk. The main issues with long term use of LMWH are that it can result in reduction of bone density and impact on quality of life due to requirement for daily administration of subcutaneous injections. An oral agent would be preferable but vitamin K antagonists, such as warfarin, have been found to be not as effective. Direct oral anticoagulants (DOACs) are being increasingly used as an alternative anticoagulant in more common conditions, such as atrial fibrillation and venous thromboembolism. These could be a potential alternative in this rare group of patients.Aims: We present the use of direct oral factor Xa inhibitor rivaroxaban (off-label) as a novel treatment option for LIC and consumptive coagulopathy in three patients with extensive low flow venous malformations. Serial measurements of D-dimer, fibrinogen and factor XIII levels were used as surrogate markers for the severity of LIC.Methods: Patients managed with rivaroxaban at our tertiary centre were identified retrospectively by the lead consultant. All had a diagnosis of extensive venous malformation with laboratory features of LIC, defined by at least one of the following parameters: high D-dimer levels, low fibrinogen (Clauss), low FXIII levels with the latter only being monitored for the last two years since its clinical significance was realised. Case notes and laboratory results were reviewed.Results: Three patients fulfilled the inclusion criteria (Table 1). Mean age was 29 years. Two cases had KTS, the third had an extensive low flow venous malformation on the back. All cases received prior LMWH. Rivaroxaban (10-20mg once daily) was administered for between 6 weeks and 23 months.In all patients there was an improvement in laboratory parameters (Table 1). D-dimer levels were reduced by 66, 97 and 69% (Cases 1 - 3 respectively). A low fibrinogen increased by 1.1, 0.5 and 0.6 g/L on rivaroxaban (Cases 1 - 3 respectively), with all patients maintaining a level above 1 g/L. Results for FXIII were only available for Case 3: the level more than doubled on rivaroxaban treatment.All patients had a normal platelet count.In addition good clinical response was observed in the two patients with painful phleboliths and venous thromboembolism.No adverse effects were seen in this small cohort.Conclusions: This is the first case series reporting the successful use of rivaroxaban in extensive low flow venous malformations for the management of LIC and consumptive coagulopathy. Our findings suggest that this novel approach can provide good control of laboratory parameters and clinical symptoms and is more convenient for patients. The optimal dose and duration of therapy remains to be defined. In light of these findings other DOACs may also have a role in the treatment of these conditions. [Display omitted] DisclosuresMathias:Bayer: Speakers Bureau. Drebes:Bayer: Consultancy; Bayer: Other: Educational Grant.

Surgical Treatment of Intra-articular Knee Venous Malformations: When and How?

To assess the efficacy of surgical treatment of intra-articular knee venous malformations (VM). Between 1998 and 2010, 8 children (mean age: 12.3 y) underwent surgical resection of their vascular malformation (7 venous and 1 capillary venous) involving the knee joint. The lesion was diffuse in 6 cases and well-demarcated in 2 cases. All children were suffering from knee pain and had recurrent hemarthroses. Color-Doppler ultrasonography, magnetic resonance imaging, computed tomography scan, and blood test were performed preoperatively. Preoperative and postoperative physical examination, clinical symptoms, and orthopaedic evaluation were retrospectively reviewed. Surgery consisted in arthrotomy with total excision of the vascular malformation for the 2 well-demarcated lesions and synovectomy with squeezing of the surrounding vascular malformation for 5 diffuse lesions. One patient with an extensive venous malformation associated with severe localized intravascular coagulopathy and mild hemophilia A had undergone synovectomy by knee arthroscopy. Immediate postoperative follow-up was uneventful in 6 patients, whereas 2 patients with diffuse vascular malformation and coagulopathy suffered from postoperative hemarthroses, delaying their rehabilitation. After a mean follow-up of 5.1 years, persistence of the VM within the joint was observed in the 6 initially diffuse lesions. The 2 well-demarcated lesions showed no evidence of disease at latest follow-up. Four patients with preoperative chondropathy and functional impairment were not substantially improved regarding their range of knee motion at latest follow-up, whereas the 4 others were free of symptoms. Only 1 patient presented a recurrent hemarthroses after a 5-year-symptom-free period and had to be reoperated. Patients without preoperative chondropathy recovered normal knee function mobility. This retrospective study highlights the importance of early surgery in patients with intra-articular venous malformation, even if asymptomatic, to prevent joint impairment. For well-demarcated lesions, total resection by arthrotomy can provide definitive healing without resuming of symptoms. Although diffuse lesions treated by synovectomy still persist in the joint, treatment avoids recurrence of hemarthroses and, therefore, protects the cartilage from further erosion. Level IV-cases series.

Nd:YAG LASER treatment of a complex airway venous malformation in a child

Abstract Nd:YAG LASER therapy is traditionally used for the treatment of superficial cutaneous and mucosal venous malformations. Venous malformations of the endolaryngeal airway may complex and difficult to manage given the sensitivity and access to this anatomic area. Nd:YAG LASER, may be a good modality to be included in management of these rare lesions with its versatility in delivery systems and hemostatic properties. We reviewed the presentation and response to treatment with Nd:YAG LASER in a 10-year-old child with extensive venous malformation involving the larynx.

Extensive ossification of an intramuscular venous malformation in the submental region

We describe a 62-year-old male patient with an extensively ossified venous malformation within the mylohyoid muscle. Phleboliths and calcifications are characteristic of venous malformations, while massive ossification is rare. To our knowledge, no extensive ossified venous malformation within the mylohyoid muscle has been reported. Intramuscular vascular lesions occur with an incidence of 0.8 in 10, occurring in the head and neck region in 15% of cases. Most are venous malformations. The diagnosis is rarely made before surgery and requires a definitive histological analysis, as there are no pathognomonic clinical or radiographic findings, especially with extensive ossification. Spontaneous regression does not occur, and treatment must be based on aesthetic and functional disturbances. We reviewed the classification of vascular anomalies, focusing on differentiating between venous malformations and haemangiomas, which are frequently confused in the literature.

Correction: Extensive Intramuscular Venous Malformation in the Lower Extremity

This corrects the article "Extensive Intramuscular Venous Malformation in the Lower Extremity" on page 893.

Extensive Cerebellar Venous Malformation Associated with a Varix and Cavernous Malformations: a Case Report

Venous malformations are the most common cerebral vascular malformation, frequently associated with cavernous malformation, and rarely accompanied by a varix. We report the case of a 67-year-old woman with tinnitus, whose cranial magnetic resonance images and selective catheter angiograms showed extensive cerebellar venous malformation with cavernous malformations and varix. Injury of vessel walls by increased blood flow in the draining vein of the malformation may have led to the development of cavernous malformations and a varix. To our knowledge, such association with an extensive cerebellar venous malformation has not been reported.

Extensive Intramuscular Venous Malformation in the Lower Extremity

Typical venous malformations are easily diagnosed by skin color changes, focal edema or pain. Venous malformation in the skeletal muscles, however, has the potential to be missed because their involved sites are invisible and the disease is rare. In addition, the symptoms of intramuscular venous malformation overlaps with myofascial pain syndrome or muscle strain. Most venous malformation cases have reported a focal lesion involved in one or adjacent muscles. In contrast, we have experienced a case of intramuscular venous malformation that involved a large number of muscles in a lower extremity extensively.

Combined Neurovascular Gracilis Muscle and Jejunal Free-Flap Reconstruction for Extensive Venous Malformation of the Face

The defect left after the resection of an extensive venous malformation in the cheek was reconstructed with a combined neurovascular gracilis muscle and patched jejunal free flap. At 25-year follow-up, the patient demonstrated good oral competence, had an adequate oral aperture allowing dental hygiene, and had symmetrical corners of the mouth.

Phacomatosis pigmentovascularis and extensive venous malformation of brain vessels. A unknown association or a new vascular neurocutaneous syndrome?

Phacomatosis pigmentovascularis and extensive venous malformation of brain vessels. A unknown association or a new vascular neurocutaneous syndrome?

In reply: extensive venous malformation: an alternative diagnosis to Klippel–Trénaunay syndrome

In reply: We thank Dr. Alomari for his interest in our recent report [1]. The alternative diagnosis of extensive venous malformation that he proposes for our patient is certainly quite plausible. Our patient’s original diagnosis of Klippel– Trenaunay syndrome was established during infancy based on the classic triad of capillary, lymphatic, and venous malformations in an enlarged lower extremity [2]. The patient received much of her continuing medical care at the Mayo Clinic and our affiliated children’s hospital, where her consultants concurred with the original diagnosis. The patient subsequently developed additional vascular malformations as described over the intervening two decades after her original diagnosis was made. Her hematology team established the presumptive diagnosis of Kasabach–Merritt syndrome after the patient sustained an intracerebral hemorrhage during infancy. The patient also suffered from chronic intermittent lower gastrointestinal hemorrhage as a consequence of the presence of colonic vascular malformations and this chronic coagulopathy. As Dr. Alomari correctly notes, Kasabach–Merritt syndrome may be associated with Kaposiform hemangioendothelioma and tufted angioma [3], but Kasabach–Merritt syndrome may affect nearly one-half of patients with Klippel–Trenaunay syndrome independently of these specific pathologic abnormalities [4]. Thus, we feel confident that her original diagnosis of Klippel–Trenaunay syndrome complicated by chronic Kasabach–Merritt coagulopathy was correct. We specifically asked our patient for permission to photograph her peripheral and upper airway vascular malformations, but she adamantly refused because other physicians and health care providers had repeatedly photographed her in the past and she was very self-conscious about her appearance. Indeed, concerns about body image are very common in patients with Klippel–Trenaunay syndrome [5], and we accordingly respected her wishes. Regardless of the original diagnosis, we chose to present this difficult case because of its specific challenges, including the presence of vascular malformations in the upper airway, the potential for neuraxial vascular malformations within the dermatomal distribution of cutaneous lesions, and the presence of a chronic coagulopathy affecting both anesthetic and surgical management.

Extensive muscle necrosis and infection following treatment of a lower extremity vascular malformation with Sotradecol and absolute ethanol

Venous malformations are a subset of low-flow vascular malformations. These are usually present at birth and grow commensurate with the child. The treatment of low-flow vascular malformations has been studied extensively. Many interventions have been devised to benefit this patient population in regard to the pain, ulcerations, infections, cosmetic concerns, and overall bulk associated with these malformations. Treatment can begin with compression garments. Another treatment is sclerotherapy. This can be done as a stand-alone treatment or as an adjunct to surgical excision. Percutaneous sclerosis of venous malformations has an efficacy of between 74 and 90% in relieving symptoms. We present a case of percutaneous sclerotherapy with Sotradecol and ethanol into an extensive lower extremity venous malformation in the setting of orthopedic megaprosthesis. We feel that this led to extensive soft tissue necrosis and infection of the limb and created a precipitous situation.

Spontaneous Thrombosis of Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation) Simulating Inguinal Hernia Incarceration

Tender lumps in the inguinal region are often explored emergently to treat suspected hernial strangulation. We discuss the case of an adult male who presented acutely with a tender inguinal swelling and raised inflammatory markers and was therefore deemed as requiring surgical exploration. However preoperative abdominal computerized tomography (CT) revealed an extensive thrombosing congenital venous malformation of portosystemic origin with extension into the symptomatic inguinal canal. A potentially lethal exsanguination from surgery was thus avoided.

Spontaneous splenic rupture due to extensive venous malformation with consumptive coagulopathy: treatment with splenic artery embolization

Extensive venous malformation (VM) is often associated with chronic consumptive coagulopathy, which may result in bleeding complications. We report herein the case of a 25-year-old man with extensive VM involving the left lower extremity who presented with hypovolemic shock and abdominal distension without abdominal trauma. Abdomen computed tomography revealed splenic rupture with massive hemoperitoneum. He was managed successfully by repeated splenic artery embolization.

Sclerotherapy of voluminous venous malformation in head and neck with absolute ethanol under digital subtraction angiography guidance

Venous malformation (VM) is the most common symptomatic low-flow vascular malformation, which predominantly occurs in the head and neck region. The aim of this paper was to evaluate the results of endovascular sclerotherapy of voluminous VM, when the lesion is either >or=15 cm in maximum diameter or the lesion invades more than one anatomical space, in the head and neck region using absolute ethanol under digital subtraction angiography (DSA) guidance. A total of 23 patients with head and neck VMs between October 2005 and December 2008 were retrospectively reviewed. All patients received direct puncture ethanol sclerotherapy under DSA guidance. Follow-up assessments were performed at 3-25 months after therapies were completed, and complications were reported in some cases. All patients were satisfied with the results of therapy. Seventeen patients (73.9%) achieved excellent responses and six patients (26.1%) achieved good responses in magnetic resonance imaging assessments. Minor complications developed during the procedures, all of which were successfully managed with full recovery during follow-ups. Serious complications such as acute pulmonary hypertension, cardiovascular collapse and pulmonary embolism were not encountered. It is concluded that sclerotherapy with absolute ethanol under DSA guidance is an important alternative therapy for voluminous and extensive VM, as the procedure is reasonably safe and offers good therapeutic results.

Extensive venous malformation associated with atrophy, osteoporosis, coagulopathy, and visceral involvement

Extensive venous malformation associated with atrophy, osteoporosis, coagulopathy, and visceral involvement

Oral 11, Potential life-threatening complications of vascular malformations

We present two patients with vascular anomalies which illustrate important learning points. Patient 1 was a 7‐year‐old girl who was being followed up for a vascular malformation which had been present at birth and had slowly enlarged over the years. It was located midline, overlying her thoracic spine. Magnetic resonance (MR) imaging revealed the lesion to be an arteriovenous malformation. An MR angiogram, performed to delineate further the extent of involvement around the spine, surprisingly revealed multiple pulmonary arteriovenous malformations. The patient admitted to having regular nosebleeds but was otherwise asymptomatic and had no telangiectasia on her face or in her mouth. There was no family history of bleeding disorders. Genetic testing has confirmed hereditary haemorrhagic telangiectasia. We present this girl to highlight the significance of midline vascular lesions, which should be treated with suspicion and physicians should have a low threshold for investigation. Patient 2 was an 18‐year‐old man attending the multidisciplinary vascular anomalies clinic with an extensive lymphatic venous malformation present since birth. Imaging showed this involved the bladder and rectum, and extended the whole way down the left leg to include the foot. Over the years he was troubled with haematuria, rectal bleeding, and recurrent painful episodes. D‐dimers were elevated. His condition was complicated by bilateral extensive pulmonary emboli and he was started on low‐molecular‐weight heparin. Despite this he went on to suffer a fatal pulmonary embolus 2 months later. These cases highlight the associated risks of vascular malformations.

Spontaneous Thrombosis of a Venous Malformation Leading to Intracerebral Hemorrhage -Case Report-

A 33-year-old man presented with consciousness disturbance (Glasgow Coma Scale score 7) and right hemiplegia after suffering headache persisting for 10 days. Head computed tomography revealed an irregular intracerebral hematoma in the left temporoparietal region, associated with a tubular high density area compatible with a thrombosed transcerebral vein in the left temporal lobe. The patient was free of coagulopathy. Craniectomy was performed to remove the intracerebral hematoma and venous thrombosis was confirmed. Postoperative cerebral angiography demonstrated extensive venous malformation in the left parietal and occipital lobes. Multiple transcerebral draining veins converged in the vein of Galen associated with a varix. Segmental narrowing of the straight sinus was suggestive of congestion in the venous anomaly. The patient showed progressive recovery following surgery, and was discharged with moderate aphasia, mild right hemiparesis, and right homonymous hemianopsia 1 month later. Venous malformations are usually silent, but occasionally become symptomatic due to thrombosis of the draining vein. The presence of stenosis in the draining route may lead to venous congestion, thrombus formation, and catastrophic hemorrhagic venous infarct.