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 –