• Cause of Port Wine Stain/SWS Identified in Recent Study
  
  
  
  
• Dealing with Birthmarks - a Psychotherapeutical Approach by Leslie Graff
  
  
  
• Investigation into optimal treatment intervals of facial port-wine stains using the pulsed dye laser
  
  
  
• Attention-Deficit/Hyperactivity Disorder After Early Exposure to Procedures Requiring General Anesthesia
  
  
  
  
• Port Wine Stain and Gum Involvement
  
  
  
  
• Initial Experiences with Propranolol Treatment of Infantile Hemangiomas: Report of Three Cases
  
  
  
  
• Medical News: Hemangiomas Erased With Propranolol
  
  
  
  
• Propranolol Treatment for Hemangioma of Infancy: Risks and Recommendations
  Leslie P. Lawley, M.D.,* Elaine Siegfried, M.D., and Jane L. Todd, M.D.à
  
  
  
• Oral Propranolol for Hemangiomas of Infancy
  Marcia L. Buck, Pharm.D., FCCP, FPPAG
  
  
  
• Propranolol for Infantile Hemangiomas: Early Experience at a Tertiary Vascular Anomalies Center
  Lisa M. Buckmiller, MD; Patrick D. Munson, MD; Umesh Dyamenahalli, MD; Yuemeng Dai, MD, PhD; Gresham T. Richter, MD
  
  
  
• Propranolol for infantile haemangiomas: insights into the molecular mechanisms of action
  C.H. Storch and P.H. Hoegerà
  
  
  
• Early Surgical Intervention for Proliferating Hemangiomas of the Scalp: Indications and Outcomes
  Jason A. Spector, M.D.; Francine Blei, M.D.; Barry M. Zide, D.M.D., M.D.
  New York, NY
  
  
  
• Review of Literature of the Treatment of Lymphatic Malformations of the Tongue
  M. Guntau, H. Kubiena, J. Roka, K. Hirschl, J. Lammer, E. Frey and M. Frey
  VASCULAR ANOMALIES CENTER
  Div.of Plastic and Reconstructive Surgery, Dep.of Surgery, Medical University Vienna, AUSTRIA
  
  
  
• Blood flow dynamics after laser therapy of port wine stain birthmarks.
  Huang YC, Tran N, Shumaker PR, Kelly K, Ross EV, Nelson JS, Choi B.
  Lasers Surg Med. 2009 Sep 3;41(8):563-571.
  FYI---laser speckle imaging is system for viewing blood flow. This study uses LSI to view the blood flow in port wine stains and how it changes or reacts after laser treatment.
  
  
• Trigeminal Dermatome Distribution in Patients with Glaucoma and Facial Port Wine Stain.
  Mehta M, Hernandez Salas A, Fay A.
  Dermatology. 2009 Aug 11.
  FYI—Trigeminal distribution refers to the location of the PWS on the face, which is broken into 3 sections. V1 is the forehead area, V2 extends from the upper eyelids to mouth area, V3 is the chin/beard area.
  
  
• Port wine stain progression: a potential consequence of delayed and inadequate treatment?
  Minkis K, Geronemus RG, Hale EK.
  Lasers Surg Med. 2009 Aug;41(6):423-6.
  
  
• Paper on Lymphatic Malformation Treatment (pdf)
  
  
• VB Article in Pediatrics for Parents (pdf)
  
  
• Pilot Study Examining the Combined Use of Pulsed Dye Laser and Topical Imiquimod Versus Laser Alone for Treatment of Port Wine Stain Birthmarks (pdf)
  
  
• Ulcer Care and Treatment Guide (pdf)
  
  
• Case study: A minimally invasive approach to the treatment of Klippel-Trenaunay syndrome (pdf)
  
  
• Simple things you can do to manage KTS
  By Linda Rozell-Shannon and Kamiab Delfanian, M.D.
  
  
• Port Wine Stains: Clearance, Cure, and Recurrence
  To Treat or Not to Treat
  
  
• Multifocal Lymphangioendotheliomatosis With Thrombocytopenia
  A Newly Recognized Clinicopathological Entity
  Article Located at Archives of Dermatology
  
  
• Multifocal Lymphangioendotheliomatosis With Thrombocytopenia:
  A Rare Cause of Gastrointestinal Bleeding in the Newborn Period
  Article Located at Pediatrics
  
  
• Available offline only: VINAY PRASAD, STEVEN J. FISHMAN, JOHN B. MULLIKEN, VICTOR L. FOX, MARILYN G. LIANG, GIANNOULA KLEMENT, MARK W. KIERAN, PATRICIA E. BURROWS, DAVID A. WALTZ, JULIE POWELL, JOS!E DUBOIS, MOISE L. LEVY, ANTONIO R. PEREZ-ATAYDE, AND HARRY P. W. KOZAKEWICH: Cutaneovisceral Angiomatosis With Thrombocytopenia, Pediatric and Developmental Pathology 8, 407–419, 2005.
  
  
• Sturge–Weber syndrome and paroxysmal hemiparesis: epilepsy or ischaemia? (pdf)
  
  
• Liver Hemangiomas (pdf)
  
  
• "Everyone Deserves to Look Normal":
  The Communication of Uncertainty, Support, and Information in an Online Community for Families Affected by a Vascular Birthmark
  Liesbeth Wiering
  M.A. Candidate
  San Diego State University
  
  
• New Paper on Port Wine Stain Treatment
  Read more here (pdf)
  
  
• Comparing The Effects of Surgical Intervention, Chemical Intervention, and Compression Therapy in Patients with Klippel-Trenaunay Syndrome.
  From:
  Matthew S. Nole, Baldwin-Wallace College.
  
  
• "Science, Math and Medicine - Working Together To Understand the Diagnosis, Classification and Treatment of Port-Wine Stains" by Martin Mihm, Jr. MD; Linda Rozell-Shannon, M.S.
  This paper was presented in Mt. Tremblant, Quebec, Canada in August at the Controversies and Conversations in Cutaneous Laser Surgery - An Advanced Symposium. It was met with rave reviews and presenters encouraged all physicians to read this paper to better understand the evolution of the diagnosis, management and treatment of port wine stains. Permission to reprint or use this paper should be directed to Linda Shannon at hvbf@aol.com
  
  
• Treatment of an Ulcerated Hemangioma With Recombinant Platelet-Derived Growth Factor
  http://www.dermatology.medschool.ucsf.edu/
  faculty/frieden.aspx
  
  

• Practical Considerations in the Treatment of Capillary Vascular Malformations, or Port-Wine Stains
  Samuel M. Lam, M.D* and Edwin F. Williams, III, M.D.**
  
  
• A Psychological Profile of Children and Families Afflicted with Hemangiomas
  Edwin F. Williams III, M.D., Marcelo Hochman, M.D., Bret J. Rodgers, M.D., David Brockbank, B.A., Linda Shannon, M.S., Samuel M. Lam, M.D.
  
  
• Management of an Arteriovenous Malformation
  Samuel M. Lam, M.D., Ravi Dahiya, M.D., Edwin F. Williams, III, M.D.
  
  
• Vascular Anomalies:  Review & Current Therapy
  Samuel M. Lam, M.D., and Edwin F. Williams III, M.D.
  
  
• Thrombosis and Haemostasis (pdf)
  This manuscript is essentially a theoretical study that combines the limited knowledge we have about the biological, biochemical, and physiological effects of laser therapy with information from related, ancillary fields such as haematology, immunology, physiology, and vascular biology in an attempt to explain what occurs inside a vessel following a laser pulse and how the laser-treated section of the skin gets remodeled after laser therapy. We expand on our previous hypothesis that thrombosis is a consequential effect of laser irradiation and, on top of the currently accepted view that the birthmark loses its redness by the replacement of abnormally large vessels by smaller capillaries, propose an additional model for this phenomenon. This model is based on the involvement of so-called endothelial progenitor cells (these cells are one step further down from stem cells in that they also possess largely undifferentiated, or “generic,” cellular features). In the latter section of the manuscript we outline the development of a drug delivery system that can be used to increase the rate of vascular occlusion through thrombotic events. Since we have recently proven (to be published) that thrombosis consistently results from laser irradiation, it is not unreasonable to believe that the administration of prothrombotic (thrombus enhancing) and/or anti-fibrinolytic (thrombus breakdown deterring) pharmaceuticals prior to laser therapy may improve treatment outcome by facilitating the complete occlusion of target vessels. In order to protect birthmark-carrying patients from the undesired effects of these drugs, the drugs must be encapsulated in a liposomal or nanocapsule system and modified to include targeting to the site of irradiation. As with the Darkfield Orthogonal Polarized Spectral Imaging device, developing such a treatment modality will be a long-term project. Nevertheless, it offers a novel alternative for treating resistant birthmarks.
  
  
• Optics Express (pdf)
  The Optics Express article details a novel microscopic imaging technique which was used to study the response of rat intestinal blood vessels to laser light. The ultimate goal of this project is twofold: First, we want to develop a non-invasive microscopic device coupled to a laser system so that we can image the response of a patient’s port wine stain as laser treatment is being carried out. This will enable the clinician to adjust laser parameters in conformity with the birthmark-specific vasculature, which has been a significant problem in relation to attaining complete lesional clearance. Although this proof-of-principle study with a semi-analogous vessel model demonstrates that this technique (“Darkfield Orthogonal Polarized Spectral Imaging, DFOPS”) can prove very useful for these purposes, several features must still be optimized before it can be used on patients. Despite this long-term prospectus, we attained some interesting data at the level of laser-tissue interactions. Since the laser procedure that we employed, referred to as selective photothermolysis, is the same procedure as is used in the clinic (the underlying mechanisms is cooking blood just as you would cook an egg – by addition of heat), we aimed to acquire insight into the events that take place inside and at the periphery of the irradiated vessels immediately after a laser pulse. These included the formation of (undefined) aggregates, reversal in direction of blood flow, vessel disappearance (due to absence of blood), and possibly thrombosis inside the vessel. Around the vessel we observed heat-induced collagen damage. By understanding these mechanisms, novel therapeutic modalities can be developed to enhance the treatment options for people with mildly and non-responding birthmarks. For example, thrombosis could be used as a means to increase blood vessel occlusion, which is required for lesional disappearance. This is discussed in the Thrombosis and Haemostasis article.
  

 

A bibliography of papers for offline research

• North P, Waner M, Mizeracki A, Mrak R, Nicholas R, Kincannon J, Suen J, Mihm M: A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol 137:559-570, May 2001
  
• VINAY PRASAD, STEVEN J. FISHMAN, JOHN B. MULLIKEN, VICTOR L. FOX,
  MARILYN G. LIANG, GIANNOULA KLEMENT, MARK W. KIERAN,
  PATRICIA E. BURROWS, DAVID A. WALTZ, JULIE POWELL, JOS!E DUBOIS,
  MOISE L. LEVY, ANTONIO R. PEREZ-ATAYDE, AND HARRY P. W. KOZAKEWICH: Cutaneovisceral Angiomatosis With Thrombocytopenia, Pediatric and Developmental Pathology 8, 407–419, 2005.
  
• North PE, Waner M, Brodsky MC. Are infantile hemangiomas of placental origin? Ophthalmology, April 2002: 109 (4) 633-6.
  
• Waner M, North PE, Scherer KA, Freiden IJ, Waner A, Mihm MC. The nonrandom distribution of facial hemangiomas. Arch. Dermatol. 139 (9); 869 – 875, June 2003
  
• Waner M: Recent development in lasers and the treatment of birthmarks. Arch Dis Child. May 2003: 88 (5) 372-4.
  
• North P, Waner M, James C, Mizaraki A, Frieden I, Mihm M: Congenital nonprogressive hemangioma, A distinct clinicopathologic entity unlike infantile hemangioma. Arch Dermatol, 137:1607-1620, Dec 2001.
  
• Walter JW, North PE, Waner M, Mizeracki A, Blei F, Walker JW, Reinisch JF, Marchuk DA: Somatic mutation of vascular endothelial growth factor receptors in juvenile hemangiomas. Genes Chromosomes & Cancer 2002: 33 (3) 295-303.
  
• Shafirstein G, Baumler W, Lapidot M, Ferguson S, North P E, Waner M: A mathematical approach to the diffusion approximation theory for selective photothermolysis modeling and it’s implication in laser treatment of portwine stains. Laser Med. Surg. 34:335-347, 2004.
  
• North PE, Antany DC, Young TL, Waner M, Brown HH, Brodsky MC: Retinal neovascular markings in retinopathy of prematurity: aetiological implications. Br J Ophthalmology, March 2003: 87 (3) 275-8.
  
• .Madathada V M, Mehta P, Waner M, Fink L M: Recombinant factor VIIa in the treatment of bleeding. Am. J. Clin. Path. 120: 1 – 14, Dec. 2003.
  
• Waner M Novel Hemostatic alternatives in reconstructive surgery. Sem. In Hematol. 41: 1, suppl. 163 – 167. Jan. 2004.
  
• Zharov V P, Ferguson S, Eidt J F, Howard P C, FinK L M, Waner M : Infrared imaging of subcutaneous veins. Lasers Surg. Med. 34: 56 – 61. Jan. 2004.

Abstracts for further research

Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-evaluated improvement.

Alomari AI, Karian VE, Lord DJ, Padua HM, Burrows PE.

Division of Vascular and Interventional Radiology, Department of Radiology, Children's Hospital Boston and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.

PURPOSE: To evaluate the midterm outcomes of percutaneous sclerotherapy of lymphatic malformations (LMs) as judged by patients.
MATERIALS AND METHODS: A 13-item survey questionnaire was sent to 74 patients who had undergone at least one sclerotherapy procedure in our hospital from January 1997 through January 2003. Information regarding the anatomic location, specific symptoms reported, history, treatment satisfaction, postprocedural complications, and number of treatment sessions was elicited. Four sclerosing agents (as single agents or in combination with other agents) were used: ethanol, sodium tetradecyl sulfate 3% (STS), OK-432, and doxycycline.
RESULTS: Fifty-five patients or their caregivers completed the survey. The patients' ages ranged from 6 months to 48 years at the time of the first procedure (mean, 12 y; median, 4 y). A majority of LMs were located in the cervicofacial region. The size and location of the lesion, recurrent infection, and pain were the most frequent indications for treatment. Fifty-one percent of these patients received sclerotherapy alone or in conjunction with surgery as primary treatment. Ethanol was the most common sclerosing agent used, followed by doxycycline, STS, and OK-432. Response varied with the type of LM, with 100%, 86%, and 43% of the patients reporting good to complete response for macrocystic, microcystic, and combined-type LMs, respectively. Skin blistering and ulcers were the most common complications. Permanent complications were uncommon and were largely related to ethanol use.
CONCLUSIONS: Percutaneous sclerotherapy provides effective midterm primary treatment for LMs. Treatment outcomes appear to vary according to the morphology of the malformation.

The full text of this article may be accessed for a fee at:
http://www.jvir.org/cgi/content/full/17/10/1639
Journal of Vascular and Interventional Radiology Online

Lymphatic malformation of the lingual base and oral floor.

Edwards PD, Rahbar R, Ferraro NF, Burrows PE, Mulliken JB.

Craniofacial Center, Division of Plastic and Oral Surgery, Department of Radiology, Children's Hospital, Harvard Medical School, Boston, Mass, USA.

BACKGROUND: Lymphatic malformation of the tongue and floor of the mouth is associated with chronic airway problems, recurrent infection, and functional issues related to speech, oral hygiene, and malocclusion. There are no accepted anatomic guidelines or treatment protocols.
METHODS: This retrospective review focused on anatomic extent, treatment, complications, and airway management in 31 patients with lymphatic malformation of the lingual base and oral floor.
RESULTS: Involved adjacent structures included the neck (77 percent), mandible (41 percent), face (42 percent), lips (10 percent), pharynx (45 percent), and larynx (26 percent). Fifty-eight percent of patients required tracheostomy during infancy; decannulation was possible in two-thirds of these patients. Management included resection alone (42 percent), resection and sclerotherapy (26 percent), resection and laser coagulation (16 percent), sclerotherapy and laser coagulation (16 percent), and resection and radiofrequency ablation (3 percent). Resection involved the neck (58 percent), floor of the mouth (52 percent), and tongue (42 percent); there were often multiple procedures. Aspiration was tried with little success in 10 percent of patients. Virtually all patients had residual abnormal lymphatic tissue. Complications and posttherapeutic problems included infection (81 percent), neural damage (27 percent), difficulty in speech (23 percent), feeding problems (10 percent), and seroma or hematoma (6 percent). Associated dental/orthognathic conditions, particularly prognathism and anterior open bite, were documented in one-third of patients.
CONCLUSIONS: The initial step in the protocol is control of the neonatal airway. Staged cervical resection is undertaken in late infancy to early childhood; resection should also include abnormal tissue in the oral floor. Sclerotherapy is primarily for macrocystic disease or secondarily for recurrent cysts following partial extirpation. Vesicles of the mucous membranes and dorsal tongue are treated either by sclerotherapy, laser (carbon dioxide, yttrium-aluminum-garnet, or potassium-titanyl-phosphate), or radiofrequency ablation. Reduction for macroglossia is indicated for persistent protrusion or to allow correction of malocclusion. Embolization controls lingual bleeding. Orthognathic procedures are undertaken at the appropriate age, only after lingual size and position are acceptable.

The full text of this article may be accessed for a fee at:
http://www.plasreconsurg.com/pt/re/prs/abstract.00006534-200506000-00014.htm
Journal of the American Society of Plastic Surgeons

Periorbital lymphatic malformation: clinical course and management in 42 patients.

Greene AK, Burrows PE, Smith L, Mulliken JB.

Vascular Anomalies Center, Division of Plastic Surgery, and the Department of Radiology, Children's Hospital, Harvard Medical School, Boston, Mass 02115, USA.

Lymphatic malformation in the orbital cavity and surrounding region often causes disfigurement and visual problems. To better clarify the evolution and treatment of this condition, the authors studied a retrospective cohort of 42 consecutive patients seen between 1971 and 2003 and analyzed anatomic features, complications, and management. The ratio of female to male patients was 1:1. Most periorbital lymphatic malformations were noted at birth (59 percent), presenting as either unilateral swelling (60 percent) or a periorbital mass (24 percent). Sixty-two percent of lesions were on the left side. The ipsilateral cheek, temple, and forehead also were involved in 57 percent of patients. Twenty-two percent of lesions were intraconal, 30 percent were extraconal, and 48 percent were in both spaces. Forty-five percent of children had an associated cerebral developmental venous anomaly. Periorbital lymphatic malformation caused major morbidity; 52 percent of patients had intralesional bleeding and 26 percent of patients had a history of infection. Other common complications included intermittent swelling (76 percent), blepharoptosis (52 percent), proptosis (45 percent), pain (21 percent), amblyopia (33 percent), chemosis (19 percent), astigmatism (17 percent), and strabismus (7 percent). Ultimately, 40 percent of children had diminished vision and 7 percent became blind in the affected eye. Management of periorbital lymphatic malformation involved an interdisciplinary team that included an interventional radiologist, a craniofacial surgeon, and an ophthalmologist. The two therapeutic strategies were sclerotherapy (40 percent) and resection (57 percent); most patients required several interventions. A coronal approach was used for subtotal excision of fronto-temporal-orbital lymphatic malformation in 13 patients, whereas a tarsal incision was used for lesions isolated to the eyelid (n = 14). Ocular proptosis was temporarily managed by tarsorrhaphy (n = 9), but expansion of the bony orbit was needed to correct persistent proptosis (n = 8). Orbital exenteration was necessary in two patients.

The full text of this article may be accessed for a fee at:
http://www.plasreconsurg.com/pt/re/prs/abstract.00006534-200501000-00003.htm
Journal of the American Society of Plastic Surgeons

Percutaneous treatment of low flow vascular malformations.

Burrows PE, Mason KP.

Department of Radiology, Children's Hospital, 300 Longwood Avenue, Boston, Massachusetts 02115, USA. patricia.burrows@tch.harvard.edu

Low flow vascular malformations, especially venous and macrocystic lymphatic malformations, are effectively treated by percutaneous intralesional injection of sclerosant drugs, such as ethanol and detergent sclerosant drugs. Good to excellent results are possible in 75%-90% of patients who undergo serial sclerotherapy. Most adverse effects are manageable, but severe complications can result from the intravascular administration of ethanol. It is generally recommended that the treatment of vascular malformations be performed in a multidisciplinarysetting by practitioners with appropriate training and support.

The full text of this article may be accessed for a fee at:
http://www.jvir.org/cgi/content/full/15/5/431
Journal of Vascular and Interventional Radiology Online

Rapidly involuting congenital hemangioma: clinical and histopathologic features.

Berenguer B, Mulliken JB, Enjolras O, Boon LM, Wassef M, Josset P, Burrows PE, Perez-Atayde AR, Kozakewich HP.

Division of Plastic Surgery, Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA.

We define the histopathologic findings and review the clinical and radiologic characteristics of rapidly involuting congenital hemangioma (RICH). The features of RICH are compared to the equally uncommon noninvoluting congenital hemangioma (NICH) and common infantile hemangioma. RICH and NICH had many similarities, such as appearance, location, size, and sex distribution. The obvious differences in behavior served to differentiate RICH, NICH, and common infantile hemangioma. Magnetic resonance imaging (MRI) of the three tumors is quite similar, but some RICH also had areas of inhomogeneity and larger flow voids on MRI and arterial aneurysms on angiography. The histologic appearance of RICH differed from NICH and common infantile hemangioma, but some overlap was noted among the three lesions. RICH was composed of small-to-large lobules of capillaries with moderately plump endothelial cells and pericytes; the lobules were surrounded by abundant fibrous tissue. One-half of the specimens had a central involuting zone(s) characterized by lobular loss, fibrous tissue, and draining channels that were often large and abnormal. Ancillary features commonly found were hemosiderin, thrombosis, cyst formation, focal calcification, and extramedullary hematopoiesis. With one exception, endothelial cells in RICH (as in NICH) did not express glucose transporter-1 protein, as does common infantile hemangioma. One RICH exhibited 50% postnatal involution during the 1st year, stopped regressing, was resected at 18 months, and was histologically indistinguishable from NICH. In addition, several RICH, resected in early infancy, also had some histologic features suggestive of NICH. Furthermore, NICH removed early (2-4 years), showed some histologic findings of RICH or were indistinguishable from RICH. We conclude that RICH, NICH, and common infantile hemangioma have overlapping clinical and pathologic features. These observations support the hypothesis that these vascular tumors may be variations of a single entity ab initio. It is unknown whether the progenitor cell for these uncommon congenital vascular tumors is the same as for common infantile hemangioma.

Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations.

Eerola I, Boon LM, Mulliken JB, Burrows PE, Dompmartin A, Watanabe S, Vanwijck R, Vikkula M.

Laboratory of Human Molecular Genetics, Christian de Duve Institute of Cellular Pathology, Brussels, Belgium.

Capillary malformation (CM), or "port-wine stain," is a common cutaneous vascular anomaly that initially appears as a red macular stain that darkens over years. CM also occurs in several combined vascular anomalies that exhibit hypertrophy, such as Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and Parkes Weber syndrome. Occasional familial segregation of CM suggests that there is genetic susceptibility, underscored by the identification of a large locus, CMC1, on chromosome 5q. We used genetic fine mapping with polymorphic markers to reduce the size of the CMC1 locus. A positional candidate gene, RASA1, encoding p120-RasGAP, was screened for mutations in 17 families. Heterozygous inactivating RASA1 mutations were detected in six families manifesting atypical CMs that were multiple, small, round to oval in shape, and pinkish red in color. In addition to CM, either arteriovenous malformation, arteriovenous fistula, or Parkes Weber syndrome was documented in all the families with a mutation. We named this newly identified association caused by RASA1 mutations "CM-AVM," for capillary malformation-arteriovenous malformation. The phenotypic variability can be explained by the involvement of p120-RasGAP in signaling for various growth factor receptors that control proliferation, migration, and survival of several cell types, including vascular endothelial cells.

The full text of this article may be accessed for a fee at:
http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=14639529

Venous variations of the brain and cranial vault.

Burrows PE, Konez O, Bisdorff A.

Division of Interventional Radiology, Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA. patricia.burrows@tch.harvard.edu

Vascular anomalies involving both intra- and extra-cranial structures are more common than previously thought. It is important to evaluate the brain and its coverings carefully when imaging cervicofacial vascular malformations. Scientific knowledge regarding developmental mechanisms responsible for blood vessel formation is increasing rapidly and, hopefully, will contribute to better understanding of these clinical and imaging "patterns."

Angiographic features of rapidly involuting congenital hemangioma (RICH).

Konez O, Burrows PE, Mulliken JB, Fishman SJ, Kozakewich HP.

Vascular and Interventional Radiology, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA. konezo@ccf.org

Rapidly involuting congenital hemangioma (RICH) is a recently recognized entity in which the vascular tumor is fully developed at birth and undergoes rapid involution. Angiographic findings in two infants with congenital hemangioma are reported and compared with a more common postnatal infantile hemangioma and a congenital infantile fibrosarcoma. Congenital hemangiomas differed from infantile hemangiomas angiographically by inhomogeneous parenchymal staining, large and irregular feeding arteries in disorganized patterns, arterial aneurysms, direct arteriovenous shunts, and intravascular thrombi. Both infants had clinical evidence of a high-output cardiac failure and intralesional bleeding. This congenital high-flow vascular tumor is difficult to distinguish angiographically from arteriovenous malformation and congenital infantile fibrosarcoma.

The full text of this article may be accessed for a fee at:
http://www.springerlink.com/content/4cgv2h91dgtgdpuk/

Venous malformations of skeletal muscle.

Hein KD, Mulliken JB, Kozakewich HP, Upton J, Burrows PE.

Division of Plastic Surgery, Department of Radiology, Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.

Intramuscular venous malformations are often mistaken for tumors because of a similar presentation and improper nomenclature. This is a review of 176 patients with venous malformations localized to skeletal muscle compiled from the Vascular Anomalies Center at Children's Hospital from 1980 through 1999. The female-to-male ratio was 2:1. Two-thirds of skeletal muscle venous malformations were noted at birth; the remainder manifested in childhood and adolescence. Venous malformations occurred in every muscle group, most often in the head and neck and extremities. Pain and swelling were the usual presenting complaints. Skeletal problems, such as fracture, deformation, or growth abnormalities, were rare. Hormonal exacerbation and intralesional bleeding were infrequent. Magnetic resonance imaging showed the lesions to be isointense to surrounding muscle on T1-weighted sequences and hyperintense on T2-weighted images. Characteristic tubular or serpentine components were oriented along the muscular long axis. Thrombi were hyperintense on T1-weighted and hypointense on T2-weighted sequences; phleboliths were seen as signal voids on all sequences. Gross examination of resected specimens revealed multicolored tissue with dilated vascular channels, frequently containing phleboliths. Light microscopy showed aggregates of primarily medium-sized, thin-walled vascular channels with flat endothelium and variable smooth muscle, most closely resembling dysplastic veins. Three lesions had a different histologic appearance consisting predominantly of small vessels with capillary structure and proliferative activity admixed with large feeding and draining vessels, similar to a lesion called intramuscular capillary hemangioma in the literature. The endothelium in these three lesions was negative for glucose transporter-1 by immunostaining. Eight percent of the patients, who had minor or no symptoms, were not treated. Twenty-four percent of the patients were managed conservatively (with aspirin and compressive garments); for 17 of these patients (10 percent of 176), noninvasive therapy was not successful, and they proceeded to sclerotherapy, excision, or both. A total of 31 percent of the patients had sclerotherapy, 20 percent had excision, and 27 percent had combined sclerotherapy and excision. Sclerotherapy was used for diffuse lesions, except for those with multiple intralesional thromboses, neurologic impairment, or compressive signs and symptoms. Resection was preferred for venous malformations well localized to a single muscle or muscle group, particularly if the muscles are expendable. Therapeutic outcomes were recorded in the charts or obtained by telephone interview in 122 of the patients (69 percent). Of these, compression garment and aspirin, resection, sclerotherapy, or combined excision and sclerotherapy improved symptoms in 121 patients (92 percent); no change was noted in 10 patients (8 percent). Only one patient was worse (self-reported) after intervention.

The full text of this article may be accessed for a fee at:
http://www.plasreconsurg.com/pt/re/prs/abstract.00006534-200212000-00001.htm
Journal of the American Society of Plastic Surgeons

Magnetic resonance of vascular anomalies.

Konez O, Burrows PE.

Division of Cardiovascular and Interventional Radiology, Department of Radiology, Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.

More than half of the patients with vascular anomalies referred to the Vascular Anomalies Clinic at Children's Hospital, Boston, have been misdiagnosed. A major consequence of misdiagnosis is inappropriate treatment, including deferral of necessary treatment and inappropriate use of pharmacotherapy, radiation, surgery, and embolotherapy. Hemangiomas and vascular malformations are distinct categories with completely different biologic and clinical behavior, therapeutic requirements, and imaging features. This article reviews the biologic classification of vascular anomalies and corresponding MR imaging features, and presents a simplified guide to diagnosis.

Prenatal diagnosis of vascular anomalies.

Marler JJ, Fishman SJ, Upton J, Burrows PE, Paltiel HJ, Jennings RW, Mulliken JB.

Department of Surgery, the Vascular Anomalies Center, and the Advanced Fetal Care Center, Children's Hospital, and Harvard Medical School, Boston, MA, USA.

BACKGROUND/PURPOSE: Vascular anomalies are diagnosed prenatally with increasing frequency. The authors reviewed a group of children treated at their center who had an abnormal prenatal diagnosis to determine (1) fetal age at which the vascular anomaly was detected, (2) general diagnostic accuracy, and (3) impact on ante- and postnatal care. Their findings are compared with reported cases and series. The authors clarify appropriate terminology and underscore the need for interdisciplinary participation of specialists in the field of vascular anomalies.
METHODS: Patients referred during prenatal life and children with a history of abnormal antenatal findings seen at our vascular anomalies center during a 1-year period (September 1999 through August 2000) were included in this study. The fetal age at diagnosis, pre- and postnatal diagnoses, antenatal course, and neonatal outcome were obtained from the parents, through chart reviews, and through telephone interviews with the treating obstetricians.
RESULTS: Twenty-nine patients with vascular anomalies were identified: 17 had a correct prenatal diagnosis, and 12 had an incorrect diagnosis, an overall diagnostic accuracy of 59%. Capillary-lymphatic-venous malformations (CLVM) most often were correctly diagnosed (67%), followed by lymphatic malformation (LM, 62%) and hemangioma (59%). In the infants who received correct diagnoses in utero, there were no fetal deaths and there was no neonatal morbidity. Maternal steroids were administered for a fetus with an intrahepatic hemangioma and deteriorating cardiac function, with subsequent stabilization and successful delivery of a healthy neonate. Among infants with incorrect diagnoses, there was 1 postnatal death, 1 case of erroneous gender assignment, 1 case of unnecessary fetal surgical intervention, 1 unnecessary neonatal laparotomy, and 1 delay in diagnosis of a malignancy. Cesarean section was done for 65% of correctly diagnosed cases, (including 2 ex utero intrapartum [Exit] procedures) and for 33% of incorrectly diagnosed cases. Most diagnoses were made during the mid- to late second trimester and third trimester; only 4 cases (14%) were detected before 20 weeks.
CONCLUSIONS: In this series, accurate diagnosis optimized antenatal care by providing an opportunity for planning deliveries, for pharmacologic fetal intervention in 1 case, and for appropriate parental counselling. Inaccurate diagnosis was associated with significantly increased morbidity and mortality. Finally, the intrauterine diagnosis of LM should be distinguished from posterior nuchal translucency, an obstetric term applied to fetal lymphatic abnormalities detected in the first and second trimesters that do not manifest as postnatal LM. Copyright 2002 by W.B. Saunders Company.

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Pediatric hepatic vascular anomalies.

Burrows PE, Dubois J, Kassarjian A.

Department of Radiology, Children's Hospital, Boston, MA 02115, USA. patricia.burrows@tch.harvard.edu

The typical vascular anomalies (tumors and vascular malformations) that involve the liver in infants and children are summarized. Many of these lesions are complex and require multiple imaging modalities, often including angiography, for precise diagnosis.

Diffuse venous malformations of the upper limb: morphologic characterization by MRI and venography.

Claudon M, Upton J, Burrows PE.

Department of Radiology and Bouriez Research Foundation, University of Nancy 1, Hopital de Brabois, France.

OBJECTIVES: To define the morphologic abnormalities in patients presenting with diffuse pure venous malformations (VM) of the upper extremity.
SUBJECTS AND METHODS: A retrospective review of MRI and venography was performed on five patients, aged 6 months to 20 years, with extensive VM of the upper limbs. Abnormalities of major conducting veins were categorized as varicosities, stenoses, and asymmetrical pouches; anomalous venous spaces were classified into confluent lakes, interconnecting channels and spongelike plexiform networks. MRI and venographic data were reviewed separately and then simultaneously in order to establish correlation between types, location, and extent of lesions.
RESULTS: In all patients, the percentage of replacement of normal tissues by VM was shown by MRI to be significantly higher in the distal limb than in the proximal limb. Involvement of multiple tissue layers was seen in all cases, including, with a decreasing rate, muscles, tendons, interosseous membrane of the forearm, and bone. Venography showed superficial varicosities, frequently associated with stenoses and assymetric pouches in all patients. Interconnecting channels and venous lakes were noted in half of the segments, typically in muscle and other deep locations, and subcutaneous spongelike lesions were seen in two patients. MRI provided a more accurate evaluation of tissue extent. Venograms better demonstrated morphological details and provided more information about the venous drainage. Direct comparison of MR images with venograms helped to identify and characterize venous lesions on cross-sectional MR data.
CONCLUSION: Diffuse VM of the upper extremity are most extensive distally, and all tissues layers can be involved, each with a characteristic morphologic appearance. The morphology of different components of the VM is related to the nature of the surrounding tissue.

Life-threatening anomalies of the thoracic duct: anatomic delineation dictates management.

Fishman SJ, Burrows PE, Upton J, Hendren WH.

Department of Surgery, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.

Congenital anomalies of the thoracic duct are rare, poorly characterized, and difficult to manage. The spectrum of pathophysiologic perturbations, presenting symptoms, radiographic findings, and interventions performed in 4 patients are shown. Accurate anatomic delineation of the malformation was only possible by direct injection contrast lymphangiography. Therapies tailored to address the anatomic aberrations included intralesional sclerotherapy, surgical excision and ligation, lymphovenous anastomosis, and omental interposition to interrupt dysfunctional collateral lymphatics to the lung. Accurate anatomic diagnosis of central lymphatic channel anomalies by contrast lymphangiography facilitates an individualized multidisciplinary approach to repair. Copyright 2001 by W.B. Saunders Company.

Noninvoluting congenital hemangioma: a rare cutaneous vascular anomaly.

Enjolras O, Mulliken JB, Boon LM, Wassef M, Kozakewich HP, Burrows PE.

Consultation des Angiomes and Service d'Anatomie Pathologique, Hopital Lariboisiere, Paris, France.

The authors studied a rare, congenital, cutaneous vascular anomaly that grows proportionately with the child and does not regress. A total of 53 patients were compiled from three vascular anomaly centers. These patients' lesions were analyzed for presentation, physical findings, radiologic and histopathologic characteristics, natural history, and outcome after resection.The lesions occurred slightly more often in male patients, always appeared alone, and were located (in order of frequency) in the head/neck region, extremities, and trunk. They were round-to-ovoid in shape, were plaque-like or bossed, occurred in variable shades of pink to purple, and had an average diameter of 5 cm. The overlying skin was frequently punctuated by coarse telangiectasia, often with central or peripheral pallor. The lesions were warm on palpation; fast-flow was further documented by Doppler ultrasonography. Magnetic resonance imaging and angiographic findings were similar to those of common hemangioma of infancy. All lesions were easily excised without recurrence.Histologic examination revealed lobular collections of small, thin-walled vessels with a large, often stellate, central vessel. Interlobular areas contained predominantly dilated, often dysplastic veins; arteries were also increased in number. Small arteries were observed "shunting" directly into lobular vessels or into abnormal extralobular veins. "Hobnailed" endothelial cells lined the small intralobular vessels. Mast cells were increased. Tests for glucose transporter-1, a recently reported reliable marker for common hemangioma of infancy, were negative in all 26 specimens examined.In conclusion, the authors think these clinicopathologic and radiologic features define a rare vascular lesion for which the term "noninvoluting congenital hemangioma" is proposed. These lesions of intrauterine onset may be a variant of common hemangioma of infancy or another hemangiomatous entity with persistent fast-flow.

Vascular anomalies.

Mulliken JB, Fishman SJ, Burrows PE.

Harvard Medical School, Boston, Massachusetts, USA.