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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 –

Vascular Anomaly Treatment Centers

When looking for a physician to manage and treat, hemangioma, vascular malformation, PWS, KT, PHACES, and other vascular anomalies, there are several key things to consider. Whether you choose a large medical center, university based center or a private practice physician the center should offer a comprehensive multidisciplinary approach to treatment. The center should have a physician staff with experience, dedication and interest in the diagnosis and current treatment modalities available. There should be coordination with a variety of support services and scientific research labs. Establish financial aspects of the treatment including insurance and payment plans. Patients need to determine the philosophy in the treatment of vascular anomalies used at the clinic and evaluate if the specific center is the best match for their specific diagnosis.

Ideally a Vascular Anomaly Treatment Center would offer a comprehensive and multidisciplinary group from the following medical disciplines.

* Dermatology * Plastic surgery * ENT (otolaryngology) * Surgery * Hematology/Oncology * Dental/Periodontal * Pediatrics * Psychology/Social Services * Radiology ** Neurology/neurosurgery ** Ophthalmology ** Orthopedics

** denotes a medical discipline that is available for specific cases requiring the area of expertise

While large medical centers and university based hospital centers would have direct access to all of these disciplines there are many smaller clinics and private practice physicians that offer excellent medical care that refers patients and consults with the larger centers for complex issues.

More important then the size of the clinic; is the program should have a dedication, interest and physicians experienced in the diagnosis and treatment of vascular anomalies and the syndromes associated with these lesions. The center should be apprised of current terminology and new approaches to the management of vascular anomalies. Physicians should be board certified and have completed a Fellowship following their residency that included training in the management of vascular anomalies. Not all residency programs include training in hemanigoma and vascular malformation. In addition physicians can receive continuing education in vascular anomalies through programs offered by the International Society for Vascular Anomaly Physicians. (IVSAP)

In addition to the physicians the VAC should offer access to a variety of support services for patients and families. These services include patient advocacy, social services, patient education materials and non-profit organizations. Many VAC are coordinated by a Nurse manager assigned to the program. The nurse manager is a vital part of the clinic. The nurse manager assists the patient and family with education about diagnosis and treatment, answers questions about procedures and post-operative care and serves as a liaison between the center and the other support services.

There are several philosophies to the treatment of vascular anomalies. Some centers are conservative and prefer a careful observation of diagnosed infants with vascular anomalies and others offer early and aggressive management with a variety of treatment modalities. Today treatments available for vascular anomalies and the related syndromes include medical management, laser treatment, surgery, scelerotherapy and physical therapy procedures. A VAC should include physicians experienced in these treatment options and specifically how they are used to manage vascular anomalies. Since hemangioma and vascular malformations vary from patient to patient the treatment philosophy employed by the center should offer individualized treatment plans based on the size, location, complexities and potential for complications for the patient.

Many centers are affiliated with or collaborated on scientific research in order to understand the biology of vascular anomalies and to modify and improve treatments currently used. The National Institute of Health and other private groups offer grant funding for research. Some clinics collaborate with the university based research labs providing lab specimens and data for the research. Collaborative research allows the physicians in private practice, small clinics or in the large centers to all be involved in the large grant funded research projects. There are many excellent clinics that are not directly involved with research, it is important that these facilities are apprised of the new approaches and methods used in the treatment of vascular anomalies that come from the research. These studies are published in the peer review medical journals.

Once a VAC is identified, the experience and specialty of the physician is considered by the patient or family for managing their individual hemangioma or vascular malformation and the availability of support services are incorporated into the treatment plan the patient and family should consider several questions before making their final decision.

• Does the Center offer a variety of treatment modalities?
• Does the Center provide post surgical support and direction?
• What is the complete treatment plan and expected time line for treatment?
• What type of surgical or laser techniques will be used to manage this patient?
• Will prosthetics be used and are they directly available from the center?
• Can the patient be monitored by the local primary care doctor for lab work, blood pressure and other routine testing in between visits to the VAC?
• Are there other parents/patients of the VAC available to talk to prior to treatment?
• What form of payment does the VAC accept?
• Which Insurance Companies do they participate with?
• Does the VAC offer a payment plan for cash patients?
• Will the VAC assist with insurance appeals?

Choosing a physician or team of physicians to manage and treat a vascular anomaly is important for the overall success and satisfaction of the patient. Considering the medical disciplines needed and provided for care, treatment methods used, support services offered, philosophies employed in treatment and overall individualized treatment offered will increase patient satisfaction.

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 2-25-2010 klh

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

Supporting NOVA

Dear Friends of NOVA:

2010 has brought some challenges to NOVA and most other charities.  The economy in the US and around the world has led to a decline in donations across the board.  At NOVA we have “tightened” our belts and have made our organizaiton work more cost effectively then ever.  Many of you already know that NOVA works on on a streamlined budget.  Most of our services are offered by the support of volunteers.  Our physicians, scientists, support staff and board of directors are all volunteers.  We continue to provide information and assistance to everyone one that contacts us.  Most months we are contacted by 100 different individuals!

Eventhough NOVA is an all volunteer organization we still have expenses.  We have been so forutnate that we have never not had enough to cover our needs. 

This year we want to host another conference for families.  In order to make this happen we are begining an active fundrasing season.  We are asking every family that we have assisted over the years to help us in this effort. 

If every family we assisted in 2009 send us a $10 donation we would rasie $12,000 in March! 

In the fall of 2009 one NOVA family hosted a raffle.  $6000. was donated to NOVA from this single event. Other families sent out a letter to friends and family asking for support of the organization that helped their child.  Each of these families raised over $1000 with their letter campaign.

Currently there is a February event being hosted by Wee Rock Coulture-

http://www.weerockcouture.com/

NOVA needs your help this year! 

Send your tax deductible donation to NOVA: PO box 38216 Greensboro NC  27438-8216.  Donations can be designated as Memorial or Honorary Donations for a specific individual.

Help NOVA host the next Family Conference!

NOVA Board of Directors

Karla, John, Lillian, Bob, Kaye, Roy & Denise

Facebook Update

NOVA is moving our link on facebook from a fan page to a group page.  The new link will be-  http://www.facebook.com/group.php?gid=306674032112&ref=mf   you will need to rejoin at this new site.  We will keep the old site up for a while but please transfer as soon as you can.

Karla

Register for Human Race

*  Registration is now open for the 2010 Human Race, March 20, in Greensboro, NC visit the race web page for NOVA at http://www.active.com/donate/THEHUMANRACE2010/NOVAsupport

At this web page you can register to participate in the 5k walk/run or sign up to donate to the race.  Donations can be mailed to NOVA directly as well.

Please visit the human race web site and Join the 2010 Human Race.

Son Pham

Son Pham is in Boston today having his final surgery today:

Olwyn writes: