Hormonal receptors in cutaneous vascular malformations: 51 cases.

Vascular malformations (VMs) are rare congenital anomalies that develop during embryogenesis in different types of vessels. Several triggering factors of cutaneous VMs include trauma, infections, or hormonal changes. We investigated the expression of hormonal receptors (androgen, estrogen, progesterone) in tissue samples of well-characterized VMs. A secondary objective was to identify self-reported triggering factors for these VMs, including hormonal changes, in the cohort of patients. We included patients with VM samples obtained in the tertiary center for vascular anomalies of the University Hospital Center of Tours, France, from January 1, 2007, to August 1, 2018. Immunohistochemistry was used to detect the expression of hormonal receptors (estrogen, progesterone, androgens). We obtained 51 samples from 51 patients: 13 cystic lymphatic malformations (CLMs), 16 venous malformations (VeMs), 11 arteriovenous malformations (AVMs), 4 combined VMs, 4 PIK3CA-related overgrowth spectrum, 1 Parkes-Weber syndrome, 1 Gorham syndrome, and 1 multiple lymphangioendotheliomatosis with thrombopenia. In total, 38 (74.5%) samples were positive for androgen receptor: 11 (84.6%) CLMs, 12 (75.0%) VeMs, 8 (72.2%) AVMs, and 7/11 (63.5%) other samples. All samples were negative for estrogen and progesterone receptors. Triggering factors were self-reported in 7 cases and were most frequently hormonal changes (n = 6, 18.2%). Hormonal triggers were frequent in AVMs (n = 4). Among patients with identified hormonal triggers, VM samples were positive for androgen receptor in 3 and negative in 3. Three-quarters of our VM samples expressed androgen receptor, and most CLM, VeM, and AVM samples were positive. Hormonal triggers were identified in 6/33 patients, mostly with AVMs.

Aneurysmal bone cyst: A 19-case series managed by percutaneous sclerotherapy.

INTRODUCTION: Sclerotherapy offers an alternative to surgery for the treatment of aneurysmal bone cyst (ABC). The main objective of the present study was to assess the radiological efficacy of sclerotherapy in terms of ossification on MRI. Secondary objectives were to assess clinical efficacy on pain evaluation and to analyze recurrence and complications according to type of sclerosing agent and intraoperative imaging technique. MATERIALS AND METHODS: Between 2006 and 2014, 19 patients (7 females, 12 males, aged 3 to 17 years) with ABC treated by sclerotherapy were included. Six received Ethibloc(®), 9 Aetoxisclerol(®), 2 liquid absolute alcohol, and 2 absolute alcohol gel. Assessment used fluoroscopy in 17 cases and CT in 2. Ossification was assessed on MRI and pain on a visual analog scale and HEDEN score. RESULTS: Ossification was complete in 11 cases (84.6%) and partial in 2 (15.4%). Eighteen patients (94.7%) were pain-free at 3 months. There was no recurrence, at a minimum 2 years' follow-up. One case of skin necrosis was observed, associated with use of liquid absolute alcohol; there was 1 case of arterial reflux of Ethibloc(®) under CT control. DISCUSSION: Sclerotherapy enables minimally invasive treatment of lesions that are deep, difficult of access to surgery and potentially damaging. Use of absolute alcohol gel and fluoroscopic control seems to improve the risk/benefit ratio, limiting complications by vascular extravasation of the sclerosing agent, thanks to real-time visualization of diffusion. Its clinical and radiological efficacy makes sclerotherapy and alternative primary treatment choice in ABC. LEVEL OF EVIDENCE: IV, retrospective study.

Early symptoms and long-term clinical outcomes of distal limb's cutaneous arterio-venous malformations: a retrospective multicentre study of 19 adult patients.

INTRODUCTION: Cutaneous arterio-venous malformations (AVM) are high-flow vascular malformations made up of a direct link between arteries and veins without intermediary capillary space. 'Distal limb's AVM', which mean involving hands or feet, are rare and their functional prognosis is often poor. Little is known about their early clinical symptoms. The objectives of this study were to identify early clinical symptoms of distal limb's cutaneous AVMs and to determine their long-term clinical outcome. METHODS: A retrospective study was carried out including adult patients who had distal limb's AVM, who were followed up between January 2000 and November 2013 in two regional tertiary care centres. The information was collected from patients' clinical records and completed by a structured telephone questionnaire. RESULTS: Nineteen patients were included in the study: four (21%) with foot AVM and 15 (79%) with hand AVM. The first clinical symptoms were as follows: swelling (47%), pain (47%), one or several venous dilatations (37%) and rarely abnormal skin colour, hyperthermia and pulsating sensation. The median diagnosis delay was 9 years after the onset of first manifestations. Amongst the 17 patients who underwent a treatment, 53% had embolotherapy session(s), 12% surgery and 35% had both. After an average follow-up of 57.6 months, 31% of the 13 patients contacted who were receiving treatment were in complete remission; 31% had partial remission; 15% had relapse after initial improvement and 23% had treatment failure. Overall, 74% of patients had a serious development of the AVM: 37% had digital or hand amputation, and 42% remained symptomatic and/or unstable. CONCLUSION: This study suggests that initial manifestations of distal limb's AVMs are discreet and non-specific, leading to a diagnosis delay of about 10 years, with poor prognosis. Doctors should evoke the diagnosis earlier, when these symptoms are shown: pain and/or swelling, sometimes with a large vein.

Retrospective Analysis of Delayed Intraparenchymal Hemorrhage after Flow-Diverter Treatment: Presentation of a Retrospective Multicenter Trial.

BACKGROUND AND PURPOSE: Intracranial aneurysm treatment with flow diverters has shown satisfying results in terms of aneurysm occlusion, and while some cases of delayed intraparenchymal hemorrhage have been described, no systematic analysis of the risk factors affecting its occurrence has been conducted in a large series of patients. This retrospective analysis of delayed intraparenchymal hemorrhage after flow-diverter treatment is a multicenter, retrospective study using a large series of treated patients to analyze factors affecting the occurrence of delayed intraparenchymal hemorrhage. MATERIALS AND METHODS: Patients treated with flow diverters and presenting with delayed intraparenchymal hemorrhage were included from December 2007 to December 2014 in 7 participating centers in France. Patient and aneurysm characteristics were recorded as were characteristics of bleeding (size, lateralization, and time to bleed), treatment, and clinical outcome after 1, 3, and 6 months. RESULTS: Delayed intraparenchymal hemorrhage occurred in 11 patients between 1 and 21 days after the procedure. In 10 of these patients, hemorrhages were ipsilateral to the treated aneurysms. Five of the 11 underwent surgery, and 9 of the 11 had good clinical outcomes at 6 months (mRS ≤2). CONCLUSIONS: The pathogenesis of delayed intraparenchymal hemorrhage occurring after flow-diverter treatment remains unclear. The multidisciplinary management of delayed intraparenchymal hemorrhage yields a relatively low morbidity-mortality rate compared with the initial clinical presentation.

Visceral and mucosal involvement in neonatal haemangiomatosis.

BACKGROUND: Two types of neonatal haemangiomatosis (NH) are distinguished: diffuse which is associated with a high rate of mortality linked to mucosal/visceral involvement, and benign. OBJECTIVES: First, this study aimed to examine the frequency of mucosal and visceral (especially hepatic) involvement in NH, according to skin extension, and second, it aimed to examine clinical, pathological (with glucose transporter 1 (GLUT-1) immunostaining), and imaging features of NH, including follow-up data. METHODS: This was a descriptive retrospective study carried out in the University Hospital Center of Tours, France. RESULTS: The study included 19 patients with cutaneous NH (number of skin haemangiomas ranging from 5 to >100). Mucosal involvement was observed in 32% of all cases (100% and 19% in diffuse and other cutaneous cases respectively) and hepatic involvement in 42% (67% and 38% respectively). The number of hepatic haemangiomas ranged from 1 to >10. Half of the hepatic haemangiomas cases exhibited increased hepatic arterial blood flow. CONCLUSIONS: Mucosal and hepatic involvement was frequent in cases with a high number of cutaneous haemangiomas (>100), but only frequency of mucosal involvement was statistically significant (P = 0.021).

[French first results using magnetic resonance-guided focused ultrasound for myoma treatment]. / Résultats préliminaires de la destruction des fibromes utérins par ultrasons focalisés contrôlée par résonance magnétique.

OBJECTIVE: Many women with myomas desire uterine conservation. Magnetic resonance-guided focused ultrasound surgery (MRgFUS) is a new non-invasive therapy. We describe our early results regarding efficacy and safety of MRgFUS for the treatment of uterine leiomyomas. PATIENTS AND METHODS: Fifty-two French women, over 18 years of age, who were candidates for surgical myomectomy, hysterectomy or uterine artery embolization due to symptomatic myomas were treated by MRgFUS (ExAblate 2000(®), InSightec) and followed up for at least 6 months. Intramural or subserous myomas with a size between 4-12 cm and a T2 low intensity MRI image were selected in women with no abdominal scar and no bowel interposition. The modified symptom severity score (SSS) was examined before and after the treatment at 6 months. Second treatment rate during the first follow-up year was reported. RESULTS: No serious complications were recorded during the treatments or follow-up period. Seven women had initial failure mostly because of bowel interposition, six of them had uterine artery embolisation. The mean modified SSS value for patients before MRgFUS was 41/100 and the values diminished significantly to a mean value of 22/100. A total of 65% of women had a reduction of at least 10 points (n=22) (initial score of 41 [29; 62] and 22 [16; 46] at 6 month follow-up). Good correlation was observed between myomas destruction and the symptoms score. During the follow-up period, ten patients (19%) required invasive interventions (six hysterectomies, two embolisations and two myomectomies). The average reduction in myoma volume determined by MR imaging at 6 months after treatment was 14%. This volume reduction is poorly correlated with the myoma's treated volume of 36.4% (6-74%). CONCLUSION: MRgFUS can safely be used for symptomatic treatment and avoid the need for surgical intervention in most patients. Additional follow-up is needed to determine the long-term durability of this promising non-invasive approach and to obtain reimbursement.

[Cortical malformations and epilepsy: Role of MR imaging]. / Malformations corticales et épilepsie: apport de l'IRM.

Malformations of cortical development are increasingly recognized as important causes of epilepsy, developmental delay and other neurological disorders. Our purpose is to present the relevance of the MRI in these pathologies with the clinical, genetic and therapeutic aspects. This classification is based on the three fundamental events of cortical formation: proliferation of neurons and glie in the periventricular zone, migration of postmitotic neurons to the periphery, subsequent cortical organization. MR analysis evaluates particularly the cortical thickness, sulcal and cortical morphology, gray-white matter junction, and looks for gray matter in abnormal location. These data coupled with the familial history, the seizure characteristics and genetic findings should allow an appropriate classification of the lesions. MR imaging allows the detection and classification of cortical malformations. MR imaging findings are primordial to consider surgery when the epilepsy becomes refractory to the anti-epileptic drugs. An adequate classification of these malformations should help to provide to the family an appropriate counseling both in terms of genetics and outcome.

Endovascular treatment of brain arteriovenous malformations using onyx: preliminary results of a prospective multicenter study.

SUMMARY: The main final goal of the treatment of brain AVMs is to prevent bleeding and to obtain a complete occlusion of the nidus. The strategy of treatment of brain arteriovenous malformations (AVM) has to be defined by a multidisciplinary team and will usually combine several modalities (surgery, radiosurgery, embolization). Embolization is generally the first step of treatment. Occasionally embolization is able to completely occlude a small AVM. Otherwise embolization is the first step before surgery or radiosurgery. Several embolic agents have been proposed for embolization of brain AVMs like particles or cyanoacrylates. The main disadvantage of particles is the high frequency of recanalization. The cyanoacrylates are probably more appropriate, providing a permanent occlusion. However, due to the polymerizing characteristics of the glue, the time of injection is relatively short (few seconds or minutes) and a complete or substantial occlusion of the nidus is difficult to obtain, especially in the case of medium or largesized AVMs. Onyx is a nonadhesive liquid polymer made of a mixture of ethylene-vinyl-alcohol copolymer and dimethylsulfoxide. The theoretical advantage of a nonadhesive liquid is to eliminate the risk of gluing the microcatheter and subsequently to perform a more durable injection with a larger amounts of agent delivered in a single injection. Several French centers have undertaken a prospective, multicentric study to evaluate the clinical value of Onyx in embolization of brain AVMs. As required, 50 patients were included. Preliminary results are presented regarding the first 48 patients, the last 2 patients being included after writing of this paper. The treatment is now completed in 15 patients. As expected, it was possible with Onyx to perform long duration injections (5 to 70 minutes with a mean of 34 minutes). Volumes injected per session were also important (0.25 to 6 ml with a mean of 1.6 ml). According to the clinical experience of the centers, duration and volume injected were most important with Onyx than with cyanoacrylates. Out of the 15 patients for whom embolization is now completed, 14 had a percentage of occlusion of the nidus greater than 60% (with 2 complete occlusion). With regard to procedural complications, four acute postembolization hemorrhages (APEH) were observed (8% per patient, 4% per procedure). This rate of APEH is in the same range as with cyanoacrylates.

[Percutaneous sclerotherapy for the treatment of soft-tissue venous malformations: a retrospective study of 68 patients]. / Sclérose percutanée des malformations veineuses superficielles: étude rétrospective de 68 cas.

PURPOSE: To evaluate the efficacy of percutaneous sclerotherapy for the treatment of venous malformations (VMs) with regards to cosmetic and functional outcome as a function of their size and to review the complications. MATERIALS AND METHODS: A retrospective study was performed between January 1997 and January 2002 on 68 patients (45 females and 23 males) ranging in age from 3 to 60 Years at the CHRU of Tours. RESULTS: Percutaneous sclerotherapy was a very effective treatment for small and medium-size VMs, for which the aim was to achieve cure. Aetoxisclerol and Ethibloc are the sclerosing agents used. They were associated with minimal side effects and no major complication. For larger lesions, the treatment was more complex and combined stronger and also more dangerous agents like absolute ethanol and Histoacryl. The aim was then a decrease of cosmetic and functional problems. CONCLUSION: Percutaneous sclerotherapy with Aetoxisclerol, Ethibloc, absolute ethanol or Histoacryl, either alone or before surgery, is a safe and effective method of managing soft-tIssue venous malformations.

[Management of cerebrospinal fluid fistulae: physiopathology, imaging and treatment]. / Stratégie d'exploration d'une brèche ostéoméningée.

Cerebrospinal fluid (CSF) fistulae can produce leakage through a defect in the bony skull and meninges into the contiguous air-filled cavities at the base of the skull. The major risk is central nervous system infection. When abundant clear rhinorrhea or otorrhea is present, the diagnosis is obvious and imaging is used to localize the fistula. Computed tomography (CT) with millimetric slices and magnetic resonance imaging (MRI) are the most effective diagnostic tools. CT cisternography, an invasive procedure, should only be used when the diagnosis remains uncertain following CT scan and MRI. When CSF leakage is sparse or intermittent, the diagnosis can be made by measuring beta-2 transferrine in the escaping fluid. CT scan followed by MRI are also useful for making the diagnosis and locating the fistula when exterior leakage is absent. CT scan alone is effective for assessing isolated otorrhea. If the diagnosis remains uncertain after all these studies have been used, the patient should be closely followed clinically and isotopic study or surgery should be considered.

Contrast-enhanced sonography during uterine artery embolization for the treatment of leiomyomas.

Uterine artery embolization (UAE) is a successful and safe treatment for symptomatic leiomyomas. However, rare complications such as premature menopause and uterine necrosis can arise because of embolization of non-target tissues. We studied the feasibility of using contrast-enhanced sonography with intravenous SonoVue just before, during and after complete occlusion of both uterine arteries. In a patient with multiple, large, symptomatic leiomyomas, contrast-enhanced imaging established that the UAE was technically successful and that myometrial vascularity was not reduced. Our case suggests that ultrasound contrast agents may have a role in monitoring UAE and thus may help prevent ischemic complications. Further studies are required to confirm this.

[Prospective clinical and sonographic assessment of uterine artery embolization as the treatment of symptomatic uterine leiomyomata]. / Evaluation prospective clinique et échographique de l'embolisation des artères utérines comme traitement des fibromyomes utérins symptomatiques.

OBJECTIVE: To evaluate the effectiveness of the uterine artery embolization as the treatment of symptomatic uterine leiomyomata. PATIENTS AND METHODS: Eighty-five women with symptoms caused by uterine leiomyomata underwent uterine artery embolization as an alternative to surgery from january 1997 to june 2000. The effectiveness of this method was evaluated by clinical and sonographic examination. RESULTS: The recession average was of 18.9 months. There were ten failures. We had immediate failures (n = 5) with a case of technical failure, one endometrium cancer, one adenomyosis, one larger subserosal leiomyomata and one parametrial leiomyomata. We had recurrences (n = 5) with the occurrence of new leiomyomatas (1 intramural and 3 submucosal) and an evolution of previous leiomyomata. The average volume reduction was 51% for the uterus and 65% for the main fibroid at one year follow-up. Minor complications occurred in 5%. Permanent amenorrhoea was observed for 3.75% of the women. Using cox model, no predictive factors of embolisation effectiveness were found. DISCUSSION AND CONCLUSION: In the treatment of symptomatic uterine leiomyoma, uterine artery embolization is an effective alternative to surgery. After one year and half, we had 12.5% of failures.

[Imaging of meningeal hemangiopericytomas]. / Imagerie des hémangiopéricytomes méningés.

PURPOSE: To study the imaging findings of meningeal hemangiopericytomas. MATERIAL: and method: Four cases of meningeal hemangiopericytomas are reported, two involving the spine and two intracranial in location. RESULTS: An extra-axial and heterogeneous pedunculated mass, without calcification should suggested a diagnosis of hemangiopericytoma especially if a bone erosion is noted; meningioma is the main differential diagnosis. CONCLUSION: CT scan as well as MR imaging are not specific but may provide findings consistent with a diagnosis of hemangiopericytoma. A long follow up of these patients is required because of frequent recurrences and delayed metastases.