ISSVA-2010

The 18^th meeting of the general assembly of the International Society for the Study of Vascular Anomalies (ISSVA) was held in Brussels April 20-24, 2010.  The meeting was nearly canceled due to the eruption of a volcano that literally shut down  air travel throughout Europe/Asia for nearly a week.  Fortunately the dedicated and hardworking members of the ISSVA conference team utilized modern day technology to pull together the first Trans-Atlantic Web-Conference for  Vascular Anomalies.  Live-stream web broadcasting was coordinated with Montreal and Brussels with internet viewing available for those that could not travel to either location.  Dr. Mikka Vikkula, the entire Brussels team and  Dr. Josie DeBois, of Montreal must be commended for the success of this important scientific meeting that at least appeared to be planned for web broadcasting!

 

Nearly 100 presentations from the spectrum of vascular anomalies diseases were made by centers across the globe.  These presentations outline the dedication by clinicians and scientists to better understand and manage the complex spectrum of vascular anomalies. 

 

What started as an idea to advance vascular anomalies, by Dr. John Mulliken nearly 20 years ago has grown into ISSVA today!  Everyone affected by vascular anomalies owes these dedicated researchers our gratitude.

 

Karla Hall

Executive Director NOVA

Migraines, Malformations and More

Migraines, malformations and more….

 

 

There is a lot of new information coming out about Migraine headaches.  Much of this information has to do with the mechanism of migraine and the occurrence of migraine.  There is still not clear answers to these questions but the research is ongoing.  We are hoping that some of the information about migraines will help us better understand the migraines that children with PHACE often experience. 

 

 

Summary of information:

 

Migraines

 

• Migraine headaches have a female predominance with a peak occurring in the 30’s and 40’s.  Migraines can be divided into two major groups: migraine with aura and migraine without aura.  Migraine without aura is the most common type of migraine headache.  In both groups the headacahes are unilateral, moderate in intensisty, often pulsate, aggravate with routine physical activity, associated with nausea, photophobia and or phono-phobia.  Each migraine lasts from 4-72 hours.  Migraine with aura occurs in 37% of the women with reported migraine.  This type of migraine is associated by the presence of a neurological symptom that develops over 5-20 minutes and lasts for less then 60 minutes.  The aura precedes the headache in most cases.

 

• The most widely accepted mechanism for the development of migraine headache with aura is excitability of the neuronoal cells membranes of the occipital cortex of the brain.  The excitation spreads through the cortex leading to activation of the trigeminal nerve pathway causing pain. 

 

• In the absence of aura the mechanism of migraine pain is debated.  The main area of dispute is whether the migraine pain originates from the activation of the trigeminal nerve pathway due to a release in vasoactive chemicals from blood vessels or does this originate in the center of the trigeminal pathway in the brainstem.  Regardless of the origination pathway of the migraine the underlying cause appeases to be due to dilation of the large cranial vessels beneath the surface of the brain, (dura mater).  These vessels are interwoven in the trigeminal pathway.  Research has identified several vasoactive peptides in the trigeminal nerve cells including calcitonin gene related peptide, (CGRP).  This peptide seems to be the most important identified peptide in the development of a migraine headache.

 

 

• Pregnancy has a variable effect on both the intensity and the frequency of migraine without aura headaches.  A literature search shows that most women seem to have either some improvement or no change in migraine during pregnancy.  No data can be found establishing and objective set of criteria that will predict which patients will see improved symptoms of migraine and which patients will see no improvement.  Women with migraine with aura headaches are less likely to see improvement of symptoms during pregnancy. 

 

• Ovulation also seems to have an effect on migraine occurrence.  In one study reported in the literature women with history of migraine, undergoing induced ovulation had a higher incidence of headaches then those without history of migraine.  During ovulation levels of estrogen fluctuate.  Similarly in the perimenopausla state estrogen levels fluctuate.  The observation that migraine attacks increase during the 30’s – 40’s supports the suggestion that fluctuations and abrupt changes in estrogen levels triggers migraine headaches.

 

 

• Migraines headaches do not seem to directly affect the outcome of pregnancy.  There are no outcome studies that report an increase in the preterm labor, low birth weights or congenital anomalies.  There is a noted indirect effect.  Patients with migraine headaches have a higher risk of developing pre-eclampsia then those without migraine.  Migraines that develop during pregnancy or delivery must be evaluated and considered for other complications associated with headache such as pre-ecalmapsia and post-epidural headache.

 

 

• At the 14^th International Headache Congress it was reported that abdominal migraine represents %15 of gastrointestinal pain in the pediatric population.  If you track children with gastrointestinal migraine a significant number of these children will go on to have migraine headaches later in life.  Abdominal migraine is characterized by episodes of moderate to severe midline, periumbilical, or poorly localized pain lasting for 1 to 72 hours with nausea, anorexia, pallor, and occasionally headache. “But headache is not one of the cardinal features. It’s episodes of belly pain,” reports Dr. Donald Lewis Professor of Pediatrics Eastern Virginia Medical School.  In a retrospective study it was found that there is a large subset of children who have had a vague diagnosis of GI pain who most likely have abdominal migraine.  Making the correct diagnosis says Dr. Lewis, “ means the spectrum of migraine medications will become available to children.” 

 

Rare Vascular Tumors

 

• Verrucous hemangioma is an uncommon, localized vascular malformation associated with reactive epidermal acanthosis, papillomatosis and hyperkeratosis on histological examination.^[1] The lesions are bluish-red, well demarcated, soft, and compressible. In time, verrucous hemangiomas gradually enlarge and satellite nodules may arise. 

 

• It as a congenital vascular malformation comprising a capillary or cavernous hemangioma in the dermis and subcutaneous tissue associated with reactive epidermal acanthosis, papillomatosis, and hyperkeratosis, distinguishing it from angiokeratoma.^[4]

 

• Most verrucous hemangiomas are located on the lower extremities and involvement is generally unilateral. Lesions have usually been present since birth or have occurred in early childhood, although they may appear later or even in adult life.

 

• Verrucous hemangiomas do not resolve spontaneously and have a tendency to relapse. Early diagnosis is important to get a better cosmetic result. Verrucous hemangioma requires a large, deep excision. Incomplete excision leads to recurrence because of deeper components. Cryosurgery, electrocautery, and laser therapy can be used for smaller lesions.  It must be confirmed by histopathological examination.

 

 

Treatment:

 

• A recent published report in Pediatrics supports the opinion that Propranolol is a safe and effective treatment for severe infantile hemangioma.  Use of propranolol can markedly shorten the disease course according to the small study published in the September 2009 issue of Pediatrics. Authors of the study note that within a few hours of treatment with the beta-blocker Propranolol the color of the hemangioma lightens and the growth is reduced.  The authors conclude that if the results are confirmed by a large comparative study, propranolol could be come the first line treatment for infantile hemangioma.

NOVA provides this information as a resource.  It is not intended to engage in the practice of medicine or to replace the physician.  NOVA does not claim to have medical knowlege.  NOVA does not endorse any particular physician, treating facility or treatment protocol.  In all cases NOVA, the NOVA Board of Directors and associates recommends that you seek the opinion of a physician experienced in the diagnosis and treatment of hemangioma and vascular malformations.

 

 

 

 

 

photo page

We are adding photos of vascular anomalies to the web site.  They can be found on the drop down menu of the Information Page or http://www.novanews.org/information/photos-of-vascular-anomalies.

National Geographic Show

If you missed the National Geographic Channel’s special on Rare Anatomy: Blood,  Featuring  Friends of NOVA Son Pham and Michale Politzer you can view it now online at:  http://channel.nationalgeographic.com/series/extraordinary-humans/4214/Overview#tab-Videos/07899_00

Additional Broadcast dates have not been announced.  We are hoping for updates soon.

ISSVA- 2010 Brussels

18th International Worskhop on Vascular Anomalies

21-24 April 2010, Brussels, BELGIUM

Visit the ISSVA website for more information: www.issva.org

Using the NOVA Blog

The NOVA blog is a way for NOVA to quickly communicate with our members. As a user you are able to leave comments, or read other comments.
To read a comment: scroll over the area at the top of the post which says: File Under: “xyz”. This will take you to the topic category. This will take you to the blog category where in the top right corner is a marker which says, “# comment” click on this and scroll down to the bottom of the page to read the comments. Once here you cal also leave a new comment.

To leave a comments on an item simply click on the marker which says, “leave a comment” then write your thoughts.

All comments will need approval from the web master- we set the blog in this format to prevent spam and junk mail.

Hope this makes using the blog easier.
Karla

Literature Search and update 03/2010

Recent Publications

 

A review of the medical literature leads to thousands of hits for vascular anomalies.  NOVA attempts to provide a listing of the recent literature that is relevant to the diagnosis and treatment of vascular anomalies.  Some publications provide summaries (abstracts) and others do not.  Complete articles are available on line from a variety of sources for a fee. 

Buckmiller, LM (2009) “Propranolol treatment for infantile hemangioma” Current Opinion Otolaryngol of Head and Neck Surgery 17(6): 458-459

Codisco, Maria (2009) “Update on lasers in children.” Curr Opin Pediatrics 21(4): 499-504

Dayicioglu, dE (2009) “vascular Anomalies of the upper extremity in children” J Craniofacial Surgery 20(4) 1025-1029

Fay A, Nguyen J, Jakobiec FA, Meyer-Junghaenel L, Waner M. (2010), “Propranolol for isolated orbital infantile hemangioma.” Arch Ophthalmol. Feb;128(2):256-8.

Frieden IJ, (2009) “conditions masguerading as infantile hemagnioma” Australas J Dermatology 50(3): 153-168

Horii KA, Drolet BA, et al, (2010) “Risk of hepatic hemangiomas in infants with large hemagnioma” Arch Dermatol 2010 Feb:146(2):201-3

Lawley, LP, Siegfried,  (2009) “Propranolol treatment for hemangioma of infancy: fisks and recommendations” Pediatri Dermatolol 26(5):610-614

Marsciani, AR (2009) “Massive response of severe infantile hepatic hemangioma to propranolol: Pediatric Blood Cancer 54(1)”176

Metry, D., et al (2009) “PHaCE syndrome: current knowledge, future dirctions.” Pediatric Dermatology 26(4):  381-398

Redondo PG (2009) “Efficacy and safety of microfoam sclerotherapy in a patient with KT” Arch Dermatol 145(10):1147-1151

Sharan, SB, Swamy et. al. (2009) “Port Wine vascular malformation and glaucoma risk in Sturge-Weber syndrome” JAAPOS 13(4):374-378

Yoon, HS., J.H> Lee, et al (2009) “Successful Treatment of Retropperiitoneal Infantile hemangiomaendothelioma with Kasabach-Merrit Syndrome using steroid, interferon and vincristin.” J Pediatric Hematolo Oncol. 31 (12):952-4

 

 

Available Abstracts

 

Arneja jS, Mulliken JB, (2010), “resection of amblyogenic periocular hemangioms: indication and outcomes”, Plast Reconstru Surg, 2010 Jan:125(1):274-81

BACKGROUND: Periocular hemangiomas can induce irreversible amblyopia by multiple mechanisms: visual deprivation, refractive error (astigmatism and/or anisometropia), or strabismus. There is a subset of complicated periocular hemangiomas most effectively managed by resection. METHODS: The authors reviewed all patients from 1999 to 2008 with a periocular hemangioma that was either completely resected or debulked; whenever necessary, the levator apparatus was reinserted. Infants were included in the study if they had complete preoperative and postoperative ophthalmic assessments and there was more than a 6-month follow-up interval. RESULTS: Thirty-three children were treated with a mean operative age of 6.2 months and a mean follow-up interval of 48.2 months. The majority of hemangiomas were well-localized and caused corneal deformation with astigmatism or blepharoptosis. Intralesional or oral corticosteroid administration was attempted in almost one-half of patients. Postoperatively, the degree of astigmatism was statistically improved: from 3.0 diopters to 1.11 diopters (p < 0.001). When resection was performed in infants younger than 3 months (19 patients), astigmatism was less severe preoperatively and the correction was slightly greater postoperatively (from 2.76 diopters to 0.80 diopters). Resection performed after 3 months (14 patients) of age also resulted in improvement of astigmatism (from 3.39 diopters to 1.38 diopters). Reinsertion of the levator expansion was required in 34 percent of patients. CONCLUSIONS: The authors advocate early resection of a well-localized periocular hemangioma to prevent potentially irreversible amblyopia caused by either corneal deformation or blepharoptosis. The longer a complicated periocular hemangioma is observed, the greater the astigmatism and the less amenable it will be to correction following tumor removal.

Bourdelat D, 2010 ” Congenital prenatal hemangioma: diagnosis and treatment” (article in French), Arch Pediatric  (ahead of print)

 

An echogenic tumor (22 × 22 mm) isolated from the anterior part of the left leg at 27 WA of pregnancy suggested an angiomatous structure. This mass appeared to be well vascularized on Doppler examination. Three-dimensional imaging showed connections with adjacent tissues. The limbs were mobile. Delivery of a 3990-g-boy occurred normally. On the anterior part of the left leg, there was a plate-shaped mass with a pink hole at the superior part. Excision under general anesthesia was performed at the first month because of the risk of rupture and bleeding. The dissection was easy with the deep plane. Healing was complete 10 days after the surgical procedure and the aesthetic aspect was good 1 year later. Histological findings of the suspect mass emphasize the vascular aspect of the tumor. A hemangioma capillary with venous structures in the dermal hypodermic area was found.

Benoit MM, North PE,McKenna MJ, Mihm MC, Johnson MM, Cunningham MJ, (2010), “Facial Nerve Hemangiomas:vascular tumors or malforamtions?  Otolaryngol Head Neck surg 2010 Jan 142(1) 108-14

OBJECTIVE: To reclassify facial nerve hemangiomas in the context of presently accepted vascular lesion nomenclature by examining histology and immunohistochemical markers. STUDY DESIGN: Cohort analysis of patients diagnosed with a facial nerve hemangioma between 1990 and 2008. SETTING: Collaborative analysis at a specialty hospital and a major academic hospital. SUBJECTS AND METHODS: Seven subjects were identified on composite review of office charts, a pathology database spanning both institutions, and an encrypted patient registry. Clinical data were compiled, and hematoxylin-eosin-stained specimens were reviewed. For six patients, archived pathological tissue was available for immunohistochemical evaluation of markers specific for infantile hemangioma (glucose transporter protein isoform 1 [GLUT1] and Lewis Y antigen) and for lymphatic endothelial cells (podoplanin). RESULTS: All patients clinically presented with slowly progressive facial weakness at a mean age of 45 years without prior symptomatology. Hemotoxylin-eosin-stained histopathological slides showed irregularly shaped, dilated lesional vessels with flattened endothelial cells, scant smooth muscle, and no internal elastic lamina. Both podoplanin staining for lymphatic endothelial cells and GLUT1 and LewisY antigen staining for infantile hemangioma endothelial cells were negative in lesional vessels in all specimens for which immunohistochemical analysis was performed. CONCLUSION: Lesions of the geniculate ganglion historically referred to as “hemangiomas” do not demonstrate clinical, histopathological, or immunohistochemical features consistent with a benign vascular tumor, but instead are consistent with venous malformation. We propose that these lesions be classified as “venous vascular malformations of the facial nerve.” This nomenclature should more accurately predict clinical behavior and guide therapeutic interventions

Levitin GM, Thompson SH, Berenstein A, Waner M. (2010), “Surgical treatment of buccofacial region vascular anomalies using intraoral buccumucosal flap procedure, Arch Otolaryngol Head Neck Surg Feb:136(2):134-7

OBJECTIVE: To report our experience in and our surgical technique of treating vascular anomalies of the buccofacial region using an intraoral buccomucosal flap approach. DESIGN: Retrospective medical record review and illustration of a specific surgical procedure. SETTING: Academic tertiary care center. PATIENTS: Thirty-two patients with vascular anomalies of the buccofacial region who have been treated using the intraoral buccomucosal flap approach. INTERVENTION: Surgical therapy using an intraoral buccomucosal flap approach. MAIN OUTCOME MEASURES: Surgical outcomes and complications. RESULTS: Thirty-two patients were treated using the intraoral buccomucosal flap approach. The vascular anomalies treated at this site were venous malformations (17 [53.1%]), lymphatic malformations (13 [40.6%]), and hemangiomas (2 [6.3%]). Surgical removal was accomplished without occurrence of facial nerve dysfunction or other morbidity. The most frequent postoperative problem encountered was scarring with lymphatic malformation treatment. CONCLUSION: The intraoral buccomucosal flap procedure is an effective surgical technique for treating vascular anomalies of the buccofacial region.

Truong MT, Chang KW (2010)  “Propranolol for the treatment of a life threatening subglottic and mediastinal infantile hemagnioma”, J Pediatric 2010 Feb:156(2):335-8

An infant with a subglottic hemangioma remained in respiratory distress after multiple treatments failed and was found to have an enlarging mediastinal infantile hemangioma compressing the trachea. Treatment with oral propranolol resulted in resolution of symptoms within 2 days and a 50% reduction in lesion size within 1 week. Copyright 2010 Mosby, Inc. All rights reserved.

 

NOVA PROVIDES THIS INFORMATION TO FAMILIES AS A RESOURCE. IT IS NOT INTENDED TO ENGAGE IN THE PRACTICE OF MEDICINE OR TO REPLACE THE PHYSICIAN. NOVA DOES NOT CLAIM TO HAVE MEDICAL KNOWLEDGE. NOVA DOES NOT ENDORSE ANY PARTICULAR PHYSICIAN, TREATING FACILITY OR TREATMENT PROTOCAL. IN ALL CASES NOVA AND ITS BOARD OF DIRECTORS RECOMMENDS THAT YOU SEEK THE OPINION OF A PHYSICIAN EXPERIENCED IN THE MANAGEMENT OF HEMANGIOMAS AND VASCULAR MALFORMATIONS.

 

Revised 3/2/2010

Rare Diseases Top Ideas Contest

Rare Disease made the final round for the 2010 Change.org Top Ideas Contest.  This is really wonderful.
We beat out hundreds of ideas to make the finals and now must reach the top 10 out of 60 final ideas.http://www.change.org/ideas/view/25_million_it_is_time_to_care_about_rare_disease
 
You can vote every day through March 12 - ask family and friends to do the same!  Disease organization leaders, if you pass this to your members, we should win easily!  

Voting starts Monday, March 1 at 1 p.m. EST through Friday, March 12 at 5 p.m. EST.
 
This is truly an amazing chance to make rare disease a health priority in the USA (and world).
There are more than 25 million people in the USA alone with rare disease with millions of family members touched by rare disease as well.
 
Please vote at Change.org –

Introducing Anna

Welcome to Anna Rankin of Cincinnati.  Anna is a graduate student at the University of Cincinnati studding health communications.  She is specifically managing illness uncertainty through doctor/patient communications.  Anna was born with a venous malformation of right cheek, tongue, ear and neck.  She has been undergoing treatment since high school using laser, sclerotherapy and a debulking surgery.

 

Anna’s personal journey with VM sparked her interest in communication and health care, specifically how health communication can be used to help parents, children and families better manage the uncertainty that surrounds their disease.  She is currently doing research at the Cincinnati Children’s Hospital and the Hemangioma and Vascular Malformation Clinic with Dr. Denise Adams.  She is also representing NOVA to the clinic, sharing her experiences as a patient.  As a NOVA family advocate she will use her experience in health communication to improve communication for NOVA families and their doctors. 

 

Anna is a member of the NOVA facebook group and will be responding to posts there and on the NOVA blog.  If any of our families would like to communicate with Anna you can reach her through NOVA. 

Anna can be reached by email through NOVA at arankin@mail.novanews.org

 

Media Brief

 

NOVA Media Brief 2010

 

NOVA and the work we do.   In 2010 NOVA is asking each family we have helped to share their story with their local media.  By sharing your personal story with your local newspaper, radio station or television station you can reach tens of thousands of individuals with the message of NOVA.

 

Introduction:

“Since 1996, NOVA has been providing support to all individuals with hemangioma, vascular malformations and related medical conditions.  The dedication of  an all volunteer leadership team and medical & scientific professional advisors has been the foundation of  NOVA. 

 

NOVA has successfully influenced the medical community to revise an 85 year old attitude that vascular anomalies were simple birthmarks- better left untreated. By educating patients and their families to the classification, diagnosis and treatment of vascular anomalies individuals have been empowered to not accept the status quo and to work with their pediatricians and primary care doctors to find treatment centers to manage their medical care.

 

NOVA has joined with professional organizations like the International Society for the Study of Vascular Anomalies, the Coalition of Skin Disorders and the Genetic Alliance to raise awareness for these common yet misunderstood disorders.

 

NOVA has embraced every individual that has needed support, assistance and information in finding medical care that many had thought did not exist.”

 

 

 

 

National Organization of Vascular Anomalies

www.novanews.org

 

 

 

 

 

 

 

 

Personalization:

 

            1.  Share your story: example

 

We were one of those families that NOVA helped.  In 2004 our daughter was born with what we thought was a small red birthmark on her face.  We were told in the hospital that it would be gone by the time she was 5 and not to worry about it.  Within the first few weeks the birthmark had grown so large it was closing her eye and had several small wounds on it.  Despite the obvious pain our child was in and the bleeding that occurred from each wound on our daughter’s face our doctors told us not to worry and that it would get a bit worse but eventually go away.  After weeks of thinking our daughter had a simple birthmark they finally used a new word, “hemangioma”.  I did not know what a hemangioma was but as soon as I got home I sat at my computer and looked it up.  As the images of other children with hemangioma scrolled across the screen I was shocked.  My child did not have a mere birthmark that would go away at age 5, rather she had a tumor!  A tumor that would grow for at least a year and one that was associated with several complications.  On the website that provided me with all this information was an email address, I wrote to the email address and much to my surprise I received an answer the next morning. 

 

The website was for NOVA- the national organization of vascular anomalies, www.novanews.org.  The email that replied to my request for help offered a private telephone number to call.  Later that morning I was speaking with NOVA’s Karla Hall.  Karla and NOVA provided me with information on hemangioma  and an associated syndrome known as PHACE.  I was given assistance in finding a doctor that could help treat my daughter.  Within one week I was at Cincinnati Children’s Hospital with my child.  We had testing and started treatment right away.  Within a few days my daughter’s eye was opening, the wounds were healing and we were on our way to making sure our child was cared for.  My daughter does have PHACE and will need care for years as a result of it.  I can not even imagine what would have happened if I had not found NOVA.

 

Not only did NOVA provide me with information on doctors but they were able to connect me with other families through social networking sites like facebook, message boards and blog sites.  The families that connect on these sites share information that helps us simply get through the day.

 

 

 

National Organization of Vascular Anomalies

www.novanews.org

 

 

 

 

 

 

 

 

Last year NOVA hosted a conference in Houston Texas for PHACE families and we were able to attend. We were able to meet many of the families that we had been networking with.  We were able to speak with the leading doctors in the field.  We were able to enroll our children in some research that will eventually lead to better diagnosis and treatment for PHACE kids.

 

            2.  Include Photos of the child- a picture says a thousand words.  This is a visible disease let the photos speak for you.

 

 

Conclusion:  Bring it back to NOVA & tie it to your community.

 

Each year approximately 40,000 children will be diagnosed with a significant vascular anomaly.  Yet according to research from clinics treating vascular anomalies about 50% will be misdiagnosed and 35% of these children will receive improper treatment.  My child was one of the 35% that received improper or no treatment.  It could have led to life threatening complications. 

 

At the “list the name of your local hospital”,  _________ children are born each year.  Many of these children will be born with a vascular anomly like my child.  Early and accurate diagnosis and treatment is so important.  Please share my story with your readers and how NOVA can help families  find the medical care and support they need.

 

Provide Contact information for you.

 

 

Provide Contact information for NOVA

National Organization of Vascular Anomalies

www.novanews.org

PO Box 38216

Greensboro, NC  27438-8216

Karla Hall, Executive Director, khall@mail.novanews.org

John DuBiel, President, jdubiel@aol.com

Denise Adams, MD, Medical Director.

 

NOVA is a 501c3a organization.

 

 

National Organization of Vascular Anomalies

www.novanews.org

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