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The two most prevalent childhood vascular abnormalities are infantile hemangioma (IH) and port-wine stain (PWS). They become apparent shortly after birth but have distinct pathophysiology and clinical manifestations. The goal of this study was to determine if mother's history of angioma or PWS is associated with these vascular abnormalities. We evaluated an UK anonymized electronic medical records database with medical records that were linked between children and their mothers. Cox proportional hazards models were used to evaluate the association between maternal factors and the time of onset of either IH or PWS in children. Between 2004 and 2021, 639,085 children were linked to their mom's medical data with a total of 4,270,773 person-years of follow up. Children born to mothers with an angioma as compared to a mother without an angioma were more than 60% more likely to have an IH (HR: 1.64 [1.07, 2.52]). Children born to mothers with a PWS as compared to children born to mothers without a PWS were nearly 20 times more likely to have a PWS (18.95 [4.71,76.26]). Mothers with angiomas were not more likely to have children with PWS and mothers with PWS were not more likely to have children with IH. The effect estimates were minimally changed after adjustment. We demonstrated that children born to mothers with angiomas or PWS were at increased risk of IH or PWS, respectively., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Heightened intraocular pressure resulting in glaucoma and impaired vision is treatable if detected early. It is therefore necessary to identify populations at risk for glaucoma for regular screening visits., Objective: To investigate the prevalence of glaucoma in patients with facial port-wine stains (PWSs), nevus of Ota, and phakomatosis pigmentovascularis (PPV) and to establish the association between facial vascular birthmarks and ocular complications., Methods: This study is a retrospective chart review of 166 patients with facial PWS, PPV, and nevus of Ota over a 10-year period., Results: Of the 166 cases, 76 patients were diagnosed with PWS, 83 with nevus of Ota, and 7 with PPV. The mean age of patients was 12.8 years, ranging from newborn to 63 years old. Fifteen patients were diagnosed with glaucoma. Of 15 patients, 11 presented with PWS, and 4 presented with both PWS and PPV. Of 83 patients with nevus of Ota, only 2 (2.4%) presented with increased ocular pressure., Limitations: The relatively short follow-up period is a limiting factor in this study., Conclusions: Early and periodic ophthalmic examinations in patients with PWS, PPV, and nevus of Ota are essential to minimizing the risk of developing glaucoma in these groups of patients., (Copyright © 2020 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is a rare syndrome with characteristic skin lesions that are associated with fast-flow vascular malformations (FFVMs) in one-third of patients. Few case series have been described, and none in Spain., Aim: To identify the prevalence of dermatological parameters, FFVMs and associated features in a large series of patients with CM-AVM., Methods: We conducted an observational study of patients with CM-AVM syndrome diagnosed in 15 Spanish hospitals over 3 years. The main clinical, radiological, genetic findings and associated diseases were analysed., Results: In total, 64 patients were assessed. In 26.5% of cases, the diagnosis was incidental. In 75% of patients, there was one significantly larger macule, which we termed the 'herald patch'. FFVMs were detected in 34% of the patients, with 30% located on the skin, 7.8% in the brain and in 1.5% in the spine. There was a positive family history in 65% of the 64 patients. Genetic analysis was performed for RASA1 mutations in 57 patients, of whom 42 (73%) had a positive result. All 4 patients tested for EPHB4 mutations had a positive result. No tumour lesions were detected in the series, except for five infantile haemangiomas., Conclusions: Our data on clinical lesions, associated FFVM, family history and genetics are similar to those previously published in the literature. An extensive data analysis failed to demonstrate any statistically significant association between the presence of an FFVM and any clinical, familial or genetic parameter that could predict its onset, although a link between the presence of a herald patch on the midline face and the presence of a brain FFVM was observed. We did not detect any genotype-phenotype correlation., (© 2020 British Association of Dermatologists.)

Objective: The aim of the study is to determine the prevalence of RASopathies in a polyhydramnios cohort selected by postnatal medical genetics evaluation., Methods: In this retrospective study, we reviewed 622 pregnancies with polyhydramnios seen at Lucile Packard Children's Hospital between 2008 and 2017. The findings from 131 cases evaluated by Medical Genetics were included in our final analysis. Genetic testing information was extracted to determine the rate of chromosomal or single gene conditions focusing on the RASopathies. Additional variables collected were: maternal characteristics, ultrasound findings, and the severity and timing of diagnosis of polyhydramnios., Results: Postnatal genetic testing or clinical examination identified a genetic disorder in 63 (48.1%) cases, more than half (n = 33) of which had a single gene condition. Postnatal testing revealed an underlying RASopathy in 15 (11.5%) cases. An underlying RASopathy was significantly associated with the severity and timing of polyhydramnios (p < 0.05)., Conclusion: Focusing on a selected cohort postnatally evaluated by Medical Genetics, our study identified a chromosomal or genetic disorder in almost half of pregnancies complicated by polyhydramnios. Specifically, an underlying RASopathy was found in 11.5% of cases with 13/15 of these cases having additional ultrasound findings., (© 2020 John Wiley & Sons Ltd.)

Background/objectives: To determine the role of sex in port-wine stain (PWS) distribution and describe the epidemiologic and anatomic differences between syndrome-associated and non-syndrome-associated PWS using modern criteria., Methods: A retrospective review of PWS patients aged 18 years and younger from 1995 to 2018 seen in the Department of Dermatology at an academic tertiary referral center. Cases were reviewed for sex, anatomic location, and presence of associated syndrome. 4,527 records were reviewed on the basis of ICD billing codes for congenital vascular malformations, with 516 meeting inclusion criteria., Results: 516 patients were included in the analysis: 234 (45.4%) men and 282 (54.6%) women. A female preponderance of Sturge-Weber syndrome (18 of 23, 78%, P = .03) and a trend toward more female-isolated PWS (149 of 269, 55%, P = .72) were found. No lateral predominance observed for isolated PWS was found: 112(41.6%) limited left-side lesions and 113(42%) limited right-side lesions (P = .41). A trend toward Klippel-Trenaunay syndrome (KTS)-associated PWS occurring more commonly isolated to the left side (76 (45.5%) vs 59 (35.12%) P = .29) was found. Nine percent of SWS patients had a PWS on the body. Five percent of KTS patients had a facial PWS. The lower limb was the most common location overall of body PWS with 33.8% of isolated PWS and 81.5% of KTS patients having a lower limb lesion., Conclusions: Female children were more likely to be diagnosed with SWS, and a trend toward more isolated PWS in women was found. No lateral predominance of isolated PWS was found, but KTS-associated PWS was more common on the left. A considerable proportion of lesions do not appear in anatomic locations traditionally considered typical in the setting of associated syndromes, which underscores the importance of conducting a complete physical examination and adhering to diagnostic criteria for those syndromes., (© 2020 Wiley Periodicals LLC.)

Background: Sturge-Weber syndrome (SWS) is caused by a somatic mutation in GNAQ leading to capillary venous malformations in the brain presenting with various neurological, ophthalmic, and cognitive symptoms of variable severity. This clinical variability makes accurate prognosis difficult. We hypothesized that the greater extent of physical factors (extent of skin, eye, and brain involvement), presence of possible genetic factors (gender and family history), and age of seizure onset may be associated with greater symptom severity and need for surgery in patients with SWS., Methods: The questionnaire was collected from 277 participants (age: two months to 66 years) with SWS brain involvement at seven US sites., Results: Bilateral brain involvement was associated with both learning disorder and intellectual disability, whereas port-wine birthmark extent was associated with epilepsy and an increased likelihood of glaucoma surgery. Subjects with family history of vascular birthmarks were also more likely to report symptomatic strokes, and family history of seizures was associated with earlier seizure onset. Learning disorder, intellectual disability, strokelike episodes, symptomatic stroke, hemiparesis, visual field deficit, and brain surgery were all significantly associated with earlier onset of seizures., Conclusion: The extent of brain and skin involvement in SWS, as well as the age of seizure onset, affect prognosis. Other genetic factors, particularly variants involved in vascular development and epilepsy, may also contribute to neurological prognosis, and further study is needed., (Copyright © 2018. Published by Elsevier Inc.)

Background: Facial port-wine stains (PWS) are considered by some an aesthetic skin problem, yet impact on quality of life (QoL) has not been objectively documented., Objective: We sought to (1) characterize the effect of PWS on QoL in adults, (2) to identify the clinical and demographic factors that affect QoL, and (3) to compare our results with QoL studies in other skin conditions., Methods: In total, 244 adults with facial PWS completed an online QoL survey, which included the Skindex-29 instrument., Results: QoL in adults with facial PWS was diminished, especially from an emotional perspective. Variables associated with reduced QoL in all Skindex-29 subdomains included comorbid depression, limited facial mobility, and presence of other skin conditions. Persons with hypertrophy had more emotional and symptomatic impairment. The composite dermatologic-specific QoL scores were similar to those of cutaneous T-cell lymphoma, rosacea, alopecia, and vitiligo., Limitations: Selection bias was a potential limitation, as participants were primarily recruited from patient support groups., Conclusion: Our analysis demonstrates that the presence of a facial PWS has a significant negative impact on QoL. Dermatologists caring for patients with PWS should inquire about QoL, provide appropriate support and resources, and consider QoL when discussing treatment options and obtaining authorization for these procedures., (Published by Elsevier Inc.)

Background: Sturge-Weber syndrome is characterized by a facial port-wine stain associated with either or both a retinal angioma and a cerebral pial angioma. Because a pial angioma may not be evident on the initial imaging studies, individuals at risk for epilepsy are often not identified before their first seizure. The aim of this study is to identify predictive factors predisposing Sturge-Weber patients to epilepsy., Methods: The medical archives and photography database of our institution were reviewed to identify Sturge-Weber Syndrome patients followed up between 1990 and 2015. Patients without epilepsy were compared with patients with epilepsy based on the location of the port-wine stain, its extent and cerebral imaging., Results: Twenty-four patients were included in the study. Thirteen did not develop epilepsy. Patients with bilateral port-wine stain were at higher risk of epilepsy (P = 0.03). Unilateral port-wine stain did not increase the risk of epilepsy (P = 0.29) regardless of its extent. The presence of developmental venous anomalies on brain imaging was also associated with a higher risk of epilepsy (P = 0.03)., Conclusions: Bilateral facial port-wine stain and cerebral developmental venous anomalies increase the risk of epilepsy in Sturge-Weber syndrome patients. Because they can be detected at birth, they might guide preventive management and follow-up., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Port-wine stains (PWS) may thicken and darken with age. Little is known about the pathogenesis and epidemiology of PWS hypertrophy because of the lack of large studies., Objective: We sought to assess the prevalence and characteristics of patients with hypertrophic PWS., Methods: Medical records and clinical photographs of all patients with PWS visiting our clinic between 2005 and 2009 were examined to identify hypertrophy. Patients were sent questionnaires regarding their hypertrophic PWS., Results: In all, 335 patients (age 0-81 years; 69% female) with PWS were included. Hypertrophy was found in 68 patients (20%; 32 male, 36 female) and classified as thickened (5%), nodular (8%), or both (7%). Color of hypertrophic PWS was mainly red (50%) or purple (44%). Patients with hypertrophy in their PWS were mostly (68%) older than 40 years, and rarely (7%) younger than 20 years. When older than 50 years, 71% of all patients had hypertrophy in their PWS. Median age of onset of PWS hypertrophy was 31 years (12 years for thickened, 39 years for nodular)., Limitations: This was a retrospective study in a selected population., Conclusion: Hypertrophy is an important feature in the development of PWS and affects a majority of patients older than 50 years. Depth of color of the PWS is associated with hypertrophy, whereas location and size appear not to be related. More attention should be drawn to therapy and prevention of hypertrophic PWS. Diffuse thickening and nodules should be distinguished, as a different age of onset may indicate different pathomechanisms., (Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Background: Patients with facial port-wine stains (PWS) often demonstrate oral manifestations of their disorder; however, the spectrum and prevalence of such findings among a cohort of patients with PWS has not been established. As a result, dermatologists and oral health specialists may be uncertain how to counsel their patients with PWS regarding oral hypervascularity, bony oral changes, and oral hygiene., Objectives: We sought to identify physical findings and complications involving the teeth, oral cavity, and perioral structures in individuals with facial PWS., Methods: This was a cross-sectional study of 30 patients with facial PWS. Descriptive data were collected through anonymous paired surveys completed by patients and their dentists, and analyzed (Fisher exact test) for trends based on physical findings and stage of the PWS., Results: The most common orodental manifestations according to patients were enlargement of the lip (53.3%), stained gums (46.7%), abnormal bite (30%), and spontaneous bleeding of the gums (26.7%). Staining of the gingiva correlated significantly with gingival hyperplasia (P = .006), maxillary hyperplasia (P = .014), and widened interdental spaces (P = .002), and in all cases gingival staining predated these findings. Lip hyperplasia was reported more frequently by patients than by their dentists (50% vs 18.2%, P = .008). Orodental manifestations were more common among patients with darker and thicker PWS. Hemorrhage after dental procedures was rare (4.5%)., Limitations: Modest sample size and difficulty recruiting control subjects are limitations., Conclusions: Facial PWS commonly affect the orodental structures, and intraoral staining may predict future complications., (Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Facial port-wine stain (PWS) may be associated with cerebrovascular abnormalities such as Sturge-Weber syndrome (SWS). In a large series, we aimed to assess which topography of facial PWS can predict SWS. This was a cross-sectional study of consecutive patients with facial PWS seen in pediatric dermatologic or angiodysplasia consultations from 1993 to 2005 at the University Hospital Center of Tours. A standardized form was used to collect data on clinical and imaging findings. Patients with and without SWS were compared in terms of topography of the cutaneous angioma and related ophthalmologic and neurologic features. Two hundred fifty-nine patients were included, 15 with a diagnosis of SWS. All patients with SWS showed involvement of the V1 trigeminal cutaneous area. SWS was significantly associated with bilateral topography of the PWS, its extension to another territory, and involvement of the upper eyelid. Knowledge of the topography of facial PWS with risk of associated neurological or ocular anomalies allows for early diagnosis of SWS and avoids unnecessary and costly radiography for patients with uncomplicated facial PWS., (© 2011 Wiley Periodicals, Inc.)

Purpose: To identify the sensitivity and specificity of risk factors for the development of glaucoma in patients with port wine stain (PWS)., Design: A retrospective case-control study involving a large cohort of patients with PWS., Participants: A total of 216 patients (total of 252 eyes) with unilateral or bilateral PWS seen in the eye department in Great Ormond Street Hospital, London, United Kingdom., Methods: We studied the anatomic distribution of PWS and the incidence of choroidal hemangioma, episcleral hemangioma, iris heterochromia, and Sturge-Weber syndrome (SWS). We analyzed the sensitivity and specificity of these features as risk factors for glaucoma., Main Outcome Measures: Development of glaucoma., Results: Mean age at presentation was 2.9 years (3 weeks to 18.8 years). Mean follow-up was 3.2 years (0-15 years). A total of 180 patients (83.3%) had unilateral lesion, and 36 patients (16.7%) had bilateral lesion. Thirty-one patients (14.3%) had isolated V1 lesion, 35 patients had V2 lesion only (16.2%), and 93 patients (43%) had both V1 and V2 involved. On the last visit, 46 eyes (18.3%) in 39 patients had glaucoma; their mean age was 3.25 years. Glaucoma was more common if PWS was bilateral (P=0.0001), both upper and lower lids were involved (P < 0.0001), and episcleral hemangioma (P < 0.0001), iris heterochromia (P=0.004), or choroidal hemangioma (P < 0.0001) was present. Twenty-four patients had SWS; this was significantly associated with upper lid PWS (P=0.001) and bilateral PWS (P=0.0003). Glaucoma was more common in patients with SWS compared with those without (66.7% vs. 18%, P=0.01). Combined upper and lower lid PWS, episcleral hemangioma, SWS, and iris heterochromia are sensitive prognosticators for the development of glaucoma., Conclusions: Iris heterochromia is associated with the development of early glaucoma in patients with PWS. Patients at high risk of glaucoma should be seen more often in clinic. Patients who do not have combined lid involvement or episcleral hemangioma have a lower risk and can therefore be seen less often in clinic., Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article., (Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limbs will discuss the dermatologist's role in the diagnosis of these lesions. At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. This article highlights the differences in clinical appearance and discusses the differential diagnosis of extensive vascular malformations in an attempt to ensure earlier diagnosis and better outcomes for these patients., (Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. Extensive vascular malformations are often more complex than they appear and require a multidisciplinary therapeutic approach. Vascular malformations may be associated with underlying disease or systemic anomalies. Part II of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limb highlights the systemic repercussions [corrected] (bone, articular, visceral, and hematologic involvement), diagnosis, and treatment of these lesions., (Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Background: Tuberous sclerosis complex is a multisystem inherited disorder characterized by the development of tumour-like growths in brain, skin and other organs. Although cutaneous vascular anomalies are not considered a common manifestation, we have encountered co-occurrence of port wine stains and tuberous sclerosis., Objective: To assess the prevalence of port wine stain in patients with previously diagnosed tuberous sclerosis., Methods: All cases diagnosed with tuberous sclerosis at two tertiary care centres from 2000 to 2009 were reviewed. Cases with clinically documented port wine stains were included for evaluation., Results: Of 24 patients diagnosed with tuberous sclerosis, three (12.5%) had clinically evident port wine stains. The prevalence of port wine stains in this series of tuberous sclerosis patients was significantly higher than the 0.3% prevalence of port wine stain in the general population., Conclusion: Port wine stain rate in this population was significantly greater than the expected rate. Further studies are needed to assess the frequency of port wine stains in tuberous sclerosis and to clarify whether the finding should be added to the list of cutaneous features of tuberous sclerosis., (© 2010 The Authors. Journal of the European Academy of Dermatology and Venereology © 2010 European Academy of Dermatology and Venereology.)

Background and Objectives: Almost all newborn children have some sort of birthmark or transient benign skin lesion. Few studies, however, have analyzed their frequency, particularly in Spain. The aims of this study were to determine their prevalence in 1000 newborn children in the health care area of Ferrol in northwest Spain and to compare the results with those of 9 other studies with similar characteristics., Patients and Methods: We undertook a descriptive study of 1000 newborn infants seen in the first 3 days of life at the neonatal clinic in the Department of Pediatrics, Hospital Arquitecto Marcide, Ferrol, Spain. Each infant was examined for the presence of 19 different transient benign skin lesions and 11 birthmarks., Results: Birthmarks or benign skin lesions were present in 994 neonates (99.4%). Transient skin lesions were present in 99.2% and birthmarks in 72%. The 5 most prevalent lesions were sebaceous hyperplasia (75%), salmon patch (64.2%), hypertrichosis (59%), sucking calluses (54%), and palatine cysts (53.7%)., Conclusions: The results of this study show that most neonates have benign skin lesions. The findings of studies to assess their frequency are influenced not only by geographic location (affecting variables such as climate, social and health care conditions, and ethnic group) but also by the timing of examination, the inclusion criteria applied, and the terminology used., (Copyright © 2010 Elsevier España, S.L. y AEDV. All rights reserved.)

Background and Objectives: salmon patch is a congenital venous malformation that usually affects the midline. Although it is very common, few studies have analyzed its prevalence or predisposing factors. The aim of this study was to determine the prevalence and clinical characteristics of salmon patch in a group of newborn infants from a health care area in northwest Spain and to assess its association with neonatal and maternal variables., Patients and Methods: a descriptive study was undertaken of live newborn children seen in the neonatal unit of the Department of Pediatrics at Hospital Arquitecto Marcide, Ferrol, Spain between May 1, 2008 and January 31, 2009. The study protocol included collection of data on neonatal variables (including gestational age, sex, ethnic origin, weight, and presence and anatomical site of salmon patch) and maternal variables (including age and number of previous pregnancies)., Results: of the 600 newborn infants included in the study, 59% had salmon patches. The most commonly affected sites were the nape of the neck (226 infants, 37.6%) and eyelids (211 infants, 35.1%). In a number of cases, more than one part of the body was affected. There was a higher prevalence of salmon patch in full-term or post-term births, in girls, white children, heavier children, and infants born to mothers aged between 30 and 34 years or who had not been pregnant previously., Conclusions: salmon patch occurred most frequently on the nape of the neck, the eyelids, and the glabella. Its prevalence was associated with certain neonatal and maternal factors., (Copyright © 2010 Elsevier España, S.L. y AEDV. All rights reserved.)

Purpose: A representative compilation of data on the incidence and prevalence of benign cutaneous vascular neoplasms and malformations in childhood has been made., Patients, Materials and Methods: A review of the literature has been made and basic data from an own epidemiological survey in the province of Tyrol (Austria) in 6-year-old children are evaluated., Results: For capillary malformations with spontaneous regression (salmon patches), the reported numbers usually vary between 20 and 30% of the newborns, while true, persisting capillary malformations (port-wine stains) can be found in about 1%. Strawberry angiomas are found in about 3% of mature newborns but in up to 12.5% of preterm children. Complex vascular malformations or severe cases of vascular tumours are very rare., Conclusion: All in all, vascular lesions are very common findings in childhood, but in most cases harmless and transient in nature. The number of lesions that may require adequate but usually uncomplicated treatment amounts to about one percent of children (strawberry angiomas and port-wine stains). Complex and/or life-threatening severe vascular tumours and malformations that represent a considerable therapeutic challenge are extremely rare events.

Purpose: The objective of this study was to investigate the prevalence of intraoral findings and other minor developmental malformations in newborns from San Luis Potosi, Mexico., Methods: Study subjects were neonates born in San Luis Potosi Morones Prieto Hospital between September 1989 and February 1990. All subjects are examined at this hospital within 20 hours of birth. Premature babies or those requiring intensive care were excluded. Examinations are performed by a team consisting of a geneticist, an oral pathologist, 2 dentists, and an oral surgeon using mirrors, tongue blades, and a flashlight., Results: The team examined 2,182 neonates and found a frequency of 99% for congenital oral cysts, 2% for natal teeth, 11% for ankyloglossia, 8% for commissural lip pits, and 54% for congenital vascular malformations. The male/female ratios for ankyloglossia and natal teeth were 1.5:1 and 1:2.3, respectively., Conclusions: Babies born at the same hospital demonstrated a high rate of oral cysts, natal teeth, ankyloglossia, and commissural lip pits.

Background: Laser therapy is the optimal approach for treating port-wine stains, but whether it is effective for patients with facial dermatomal port-wine stains and Sturge-Weber syndrome is undetermined. This project aimed to verify the incidence of Sturge-Weber syndrome and glaucoma within the population, compare the response in syndromic and nonsyndromic patients with facial dermatomal port-wine stains to laser, and determine the treatment response in relation to dermatome and color., Methods: The authors retrospectively reviewed 874 patients with facial port-wine stains (203 displayed a dermatomal pattern). Pretreatment and posttreatment photographs were assessed clinically and recorded on a statistically reliable percentage gradient., Results: Sturge-Weber syndrome was diagnosed in 30 patients. Twelve patients had glaucoma, with the port-wine stains involving the ophthalmic (V1) division of the trigeminal nerve. The highest proportion of patients with glaucoma came from those with involvement of the maxillary (V2) and/or mandibular (V3) division of the trigeminal nerve. No patients had Sturge-Weber syndrome with V3 port-wine stains alone. After laser therapy, only 45 percent of syndromal patients and 55 percent of nonsyndromic patients had a satisfactory outcome (>50 percent) in color and size reduction of dermatomal port-wine stains., Conclusions: The incidence of Sturge-Weber syndrome was 3 percent in patients with a facial port-wine stain. There was an increased risk of Sturge-Weber syndrome with involvement of V1 port-wine stains and no risk with involvement of V3 port-wine stains alone. Laser treatment produced unsatisfactory outcomes in patients with facial dermatomal port-wine stains. V3 port-wine stains responded best and V2 worst to laser.

Vascular skin lesions in children are common, but their management is complicated by the ongoing use of inappropriate and often misleading terms. Inaccurate explanations of the diagnosis and progression of these lesions may cause unnecessary distress. This study describes an audit of vascular lesions in children from one center in which the classification system described by Mulliken and Glowacki was used to establish diagnosis. Seventy patients with vascular lesions attending vascular lesion clinics, miscellaneous plastic surgery clinics and laser lists in the Royal Victoria Infirmary, Newcastle-upon-Tyne, were studied. A proforma was completed using information from the patients and their medical records. The initial diagnosis given to parents was inaccurate in 69% of patients. Furthermore, 53% of parents were given an incorrect account of what to expect with regard to progression of the lesion and the prospect of future treatment. Altogether, in 71% of instances some inappropriate terminology was applied during the course of the child's management. Plastic surgeons were the most accurate diagnosticians. This study highlights the fact that a selection of misleading and erroneous terms is still regularly used in medical practice. The recommended classifications are rarely adhered to and in consequence efficient communications between professionals and families is often lacking.

Objective: Three previous retrospective studies noted a positive association between birthmarks and childhood cancer. The objective of this study was to determine whether the incidence of cancer is increased in children with birthmarks relative to those without birthmarks using data from the Collaborative Perinatal Project cohort, a large, prospective study., Methods: Our study population comprised 49,503 US children who were born between 1959 and 1968. Birthmarks were documented as definite or suspected during the first year through history or medical examinations and included hemangiomas (port-wine, strawberry, or cavernous), pigmented nevi, lymphangiomas, and café-au-lait spots. The association between birthmarks and childhood cancer was determined using Cox proportional hazards regression., Results: In the Collaborative Perinatal Project, 2505 individuals had a documented definite or suspected birthmark, including 7 of 47 children who developed cancer. Birthmarks were associated with a significant increase in the risk for cancer. There was a slight attenuation of the risk estimate when cases that were diagnosed in the first year of life were excluded. No specific childhood malignancies were notably affected by birthmarks., Conclusions: Although this study was based on a small number of cases, we found birthmarks to be in excess in children who received a diagnosis of cancer using prospective data. These findings provide additional support for the possibility of a shared etiology between birthmarks and childhood cancer that could offer insight into the pathogenesis of pediatric malignancy.

Objective: To establish whether the prognosis of bilateral facial capillary malformation (BFCM) is worse compared with that of unilateral facial port-wine stain., Design: Retrospective study., Setting: Paediatric Dermatology Department, Great Ormond Street Hospital for Children NHS Trust, a tertiary referral center for vascular anomalies., Patients: A cohort of 350 children who presented with facial CM was seen between January 1, 1994, and June 30, 2004. Twenty-seven children with BFCM were identified. A control group of 27 children with unilateral CM was randomly selected from the total cohort., Main Outcome Measures: Demographic, clinical, and radiographic characteristics were recorded and compared between the 2 groups: age at presentation, sex, distribution, extension, extrafacial lesions, glaucoma, ipsilateral leptomeningeal angiomatosis, and epilepsy. The recorded information was collected from the database of the Paediatric Dermatology Department, the hospital records, and the patients' photographs., Results: Compared with the 27 children with unilateral facial CM, the 27 with BFCM showed a higher frequency of association with extrafacial lesions (17 [63%] vs 6 [22%]), glaucoma (21 [78%] vs 2 [7%]), and ipsilateral leptomeningeal angiomatosis (14 [52%] vs 2 [7%]). All patients who had BFCM with bilateral and complete involvement of the ophthalmic area had ipsilateral leptomeningeal angiomatosis., Conclusion: Patients with BFCM must be considered as a group with a worse prognosis compared with patients with unilateral facial CM.

Background: Vascular nevi are cutaneous anomalies of angiogenesis and vasculogenesis resulting in various different clinical presentations., Aim: The purpose of our descriptive study was to observe the various types of vascular nevi in children and their features., Methods: A total of 4256 pediatric cases attending the dermatology OPD during the study period from August 2002 to August 2004 were screened for vascular nevi., Results: Out of these, 19 children (0.44%) had vascular nevi-17 hemangiomas of infancy (HOIs) and 2 port-wine stains. The mean age of the affected children was 1.3 years (ranging from 2.5 months to 8 years). There were 13 girls and 6 boys. Seventeen (89.5%) patients had progressing lesions and two (10.5%) had non-involuting ones. A solitary lesion was seen in ten (52.6%) cases and two to five lesions were present in five (26.3%) cases. The nevi were distributed over multiple sites in seven (36.8%) cases, the head and neck in six (31.6%) cases, the chest and lower limb in two (10.5%) cases each, and the upper limbs and genitalia in one (5.3%) case each. Among the hemangiomas of infancy, 15 (88.2%) cases of superficial type and 2 (11.8%) cases of deep type were seen. The cutaneous complications included ulceration in four cases and infection in one., Conclusions: HOIs were the most common vascular nevi of childhood.

Unlabelled: Lesions of port-wine stain (PWS) type are the most commonly occurring vascular malformations of the skin which concerns about 0.3% of the population. These important from aesthetic and psychological points of view capillaries malformations have always been difficult for treatment. Previously applied methods consisting in cryosurgery, dermabrasion, radiation therapy or surgery and skin grafting produced unsatisfactory results. It was only when highly selective lasers were introduced that PWS could be treated effectively and safely. A lot of available lasers and lack of experience of the health care providers result in not always proper qualification of the lesions to the given laser type. The aim of the study was to present a prospective analysis of the efficacy of treatment of PWS vascular malformations by means of KTP 532 nm laser. From January 2003 to May 2005, 155 patients (115 women, 40 men) aged from 2 to 62 years (mean age 18.23) were treated by means of laser at the Plastic Surgery Hospital in Polanica Zdrój, Poland. In the investigated population, the vascular malformation was a component of Sturge-Weber syndrome in 7 patients, Klippel-Trenaunay syndrome--in 2 patients, while in one case it accompanied Recklinghausen disease. The vascular lesions had not been treated before in majority of cases, while in 37 patients the laser therapy was the continuation of other previously attempted treatment, which included excision and skin grafting, argon laser therapy, radiation therapy and pulsed dye laser treatment. The procedures were performed with at least 4-week intervals without, or under local and in children general anaesthesia. Therapeutic efficacy was evaluated independently by 3 surgeons and a photographer on the basis of Subjective Scoring System (SSS) comparing simultaneously shown pictures of the patients taken prior to and after the last procedure. The outcomes of treatment were classified according to a 4-degree scale: excellent outcome--75-100% improvement, with 100% perceived as eradication of the lesion; good--50-74% improvement; fair--25-49% improvement and poor--less than 25% improvement, including no observable improvement. In 81% of the lesions (126 patients) treatment with KTP 532 nm laser produced significant improvement which was found satisfactory by the patient. excellent outcome of treatment was obtained in 49 patients (31%), good in 42 (27%), fair in 35 (23%). However, despite many laser treatments using various energy and pulse duration, there was almost not observable improvement in 19% of the study population (29 patients). The PWS which failed to treatment were most commonly located on the limbs. The results of treatment was poor in 74% of malformations which were located beyond the face and neck (all of them were on the limbs). There was no episodes of scarring or persistent pigmentary changes in any of the patients., Conclusions: 1. KTP (532 nm) laser is an effective and safe tool in the management of capillary malformations of PWS type. 2. The treatment is especially effective in adults and in lesions localized to the face and neck area. 3. Port-wine stain localized on the trunk and extremities often appears to be KTP laser resistant.

Videomicroscopy has been found to be useful in determining the depth of ectasia of vascular malformations. Different patterns that hypothetically could predict the response of a vascular malformation to pulsed dye laser have been described. Our purpose was to determine if the dermoscopy pattern was able to predict the response to pulsed dye laser therapy and if it was independent of other known clinical variables. Thirty-three consecutive children presenting for evaluation or treatment of vascular malformations underwent videomicroscopy previous to pulsed dye laser therapy. Sixty-nine representative areas were evaluated before and after laser therapy. Other clinical factors, including location of the malformation, the patient's age and sex, and previous therapy, were also included in the analysis. We found that the dermoscopy pattern was differently distributed depending on the anatomic area. A superficial pattern was not present in the centrofacial area. An undefined pattern was most often present when a previously treated area was imaged. A superficial pattern independently predicted a good response to laser. The location of the lesion was another independent factor influencing the outcome. A new pattern consisting of a pale circular area surrounding a central brownish dot is described as negatively influencing the response to laser therapy. We concluded that videomicroscopy is a good tool for assessing which vascular malformations can be adequately treated with laser therapy, although other anatomic factors can influence the response. Videomicroscopy is particularly helpful in deciding when to end the treatment because it objectively shows when no further response can be expected, and is helpful for demonstrating this to patients and their parents.

Background: Port wine stains can be treated successfully with the dye laser in most cases. The indication for treatment is made on the basis of the emotional stress arising from the port wine stain. Studies of the extent of psychosocial stress to date have led to contradictory results., Objective: To address the question how many patients with port wine stain suffer psychosocial stress and how this group can further be characterized. Moreover, the specific expectations of treatment were to be elucidated., Methods: During dye laser treatment, 76 patients with port wine stains were evaluated by questionnaires regarding (a) emotional well-being, (b) quality of life, (c) body perception and (d) disease-specific stress and expectations of therapy., Results: Patients with port wine stains showed significantly higher emotional stress than healthy controls. They were particularly impaired in quality of life with respect to their social life and felt less attractive than a healthy control group. Patients with high emotional stress placed high hopes on the laser therapy with regard to chances of employment and social contacts., Conclusion: The data point to the need for thorough patient consultation prior to laser therapy in order to avoid excessive expectations. Psychological consultation is recommended for emotionally labile PWS patients.