| < 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.
The full text of this article may be accessed for a fee at:
http://patient-research.elsevier.com/patientresearch/displayAbs?key=S0022346802075851
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.
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