Physiologic changes in vascular birthmarks during early infancy: Mechanisms and clinical implications

doi:10.1016/j.jaad.2008.11.020

Journal of the American Academy of Dermatology

Volume 60, Issue 4, April 2009, Pages 669-675

https://doi.org/10.1016/j.jaad.2008.11.020 Get rights and content

Vascular birthmarks are the most common birthmarks encountered in newborns. Many dermatologists are unfamiliar with the normal physiologic changes that affect and alter their appearance during early infancy. In this article we discuss normal newborn hemodynamic/neurovascular physiology and the associated clinical findings in neonatal skin. The physiologic anemia of infancy and its resultant effects on skin color and the appearance of vascular birthmarks are detailed. Finally, the pitfalls and obstacles to early diagnosis of vascular birthmarks and the subtle differences between them are reviewed, as are the challenges in assessing response to early pulsed dye laser treatment in the context of the changing vascular physiology.

Introduction

Vascular birthmarks are the most common birthmarks encountered in newborns. Their classification has been the subject of some controversy. The International Society for the Study of Vascular Anomalies has adopted a classification scheme that has stood the test of time and is widely used.1, 2 Several factors unique to the newborn period can make the evaluation of vascular birthmarks challenging. In this commentary, we elaborate on both physiologic and disease-related vascular skin findings in newborns and young infants that create diagnostic difficulty, and we discuss their implications for management.

Section snippets

Physiologic changes in the newborn skin

Neonatal vasomotor instability results in predictable and unique clinical features that can make it difficult to delineate a vascular stain from normal physiologic changes. (Fig 1). Transient generalized rubor due to vasodilatation and hyperemia, which can be particularly striking in the cephalic region, is accompanied by intermittent, bilaterally symmetric temperature-dependent acrocyanosis.3, 4 Prominent vascular mottling of the extremities and occasionally the trunk is observed in

Physiologic changes in hemoglobin concentration in the newborn

A phenomenon not previously emphasized that can impact the appearance of vascular birthmarks is the so-called physiologic anemia of infancy. The transition from a relatively hypoxic intrauterine environment to a well-oxygenated external environment initiates a complex series of physiologic adaptations. Neonates are relatively polycythemic at birth, with hemoglobin values of 16 to 18 g/dL, due to the low intrauterine partial pressure of oxygen (the placenta is in the venous circulation).5, 6

Morphologic changes in birthmarks

The morphologic similarity of several common vascular birthmarks in the neonatal period can also create diagnostic difficulty. Salmon patches (nevus simplex) affect nearly 50% of newborns, without gender predilection, and are the most common vascular stains present at birth. They represent a subset of true capillary malformations that occupy a midline location, such as the nape of the neck and occipital region (“stork-bite”), glabellar region (“angel's kiss”), nose, upper cutaneous lip, and

Discussion

The unique clinical characteristics of normal neonatal skin reflect the underlying immature and transitioning neurovascular, hematologic, and cutaneous physiology. Even experienced clinicians may have difficulty determining the extent of, and accurately diagnosing, vascular birthmarks in the presence of generalized rubor, soft tissue swelling, mottling, and acrocyanosis. However, timely distinction between PWS, salmon patches, and IH in young infants is important, as the natural history and

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Treating pediatric port-wine stains in aesthetics
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Citation Excerpt :
PWSs are comprised of ectatic blood vessels of varied morphology, diameter, and depth.1 Facial PWS can be unilateral or bilateral, commonly occurring in dermatomal or segmental patterns.2 PWS has an incidence of 0.3% to 0.5%2-5 without predilection for sex.
A port-wine stain (PWS) is a vascular birthmark present in 0.3% to 0.5% of newborns. If untreated, this erythematous patch will grow proportionally with the child to thicken and darken with age. PWSs have implications for the child’s quality of life for many years, with cosmetic, medical, and psychosocial disability. Controversy exists in many aspects surrounding laser treatment of these birthmarks in the pediatric population. We have reviewed the clinical features as well as the historic and current laser treatment of PWS. We have also examined the current hot topics of debate surrounding the treatment of PWS in the pediatric population. These controversies include the patient age of treatment initiation, the long-term psychologic impact, the use of general anesthesia, the application of eye shields, and alternative treatments for recalcitrant PWS. We have concluded with a discussion on the future directions of management and treatment.
A clinical study of HMME-PDT therapy in Chinese pediatric patients with port-wine stain
2018, Photodiagnosis and Photodynamic Therapy
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The treatment of PWS is still challenging for pediatric patients worldwide [8,9]. Pulsed dye laser (PDL) is currently the golden standard treatment of PWS [10,11]. Although newer PDL systems (e.g. 595 nm, longer pulse durations and built-indynamic cooling system) are more effective, vessels smaller than 50 um appear resistant to laser treatment [8,12].
To report our observations from a trial of the effectiveness and safety of hematoporphyrin monomethyl ether photodynamic therapy (HMME-PDT) in treating Chinese Pediatric patients port-wine stains (PWS).
From October 2017 to December 2017, the study recruited 82 patients of PWS with negative HMME skin test results. The patients received an intravenous injection of 5 mg/kg HMME and the affected areas were exposed to 532 nm LED green light, with the irradiation power density between 80–85 mW/cm². Each light spot was irradiated for 20–25 min. After two treatments, the clinical efficacy and posttreatment reactions at each treated area were observed.
The patient age between 1 and 14 years old. 24 of the 82 cases were cured (29.27%) after two HMME-PDT treatment, 34 cases indicates a good efficacy (41.46%), 16 cases showed alleviation (19.51%), while 8 cases displayed no efficacy (9.76%). Pain was observed during treatment, and post-treatment edema was observed at the treated areas. No other obvious systematic adverse reaction were reported.
HMME-PDT is an effective and safe treatment for pediatric patients with PWS.
Capillary Malformations (Portwine Stains) of the Head and Neck: Natural History, Investigations, Laser, and Surgical Management
2018, Otolaryngologic Clinics of North America
Clinical study on clinical operation and post-treatment reactions of HMME-PDT in treatment of PWS
2017, Photodiagnosis and Photodynamic Therapy
Citation Excerpt :
In the past, PWS was treated by surgery, which however features a long treatment cycle and invasiveness; Worse still, other therapies, like cryotherapy, dermabrasion, isotope therapy and copper vapor laser, had a poor efficacy, and were easy to produce scars thus not widely applied [4]. Later, pulsed dye laser (PDL) came to be widely applied in treating PWS and recognized as the golden standard for treating PWS at present [8,9], but still with a low response rate and cure rate [10]. In 1990, Orenstein et al. [11] verified the feasibility of PDT in treating PWS through a cockscomb model.
To analyze the clinical efficacy of hematoporphyrin monomethyl ether photodynamic therapy (HMME-PDT) in treating port-wine stains (PWS), and its clinical operation skills and operation essentials.
A total of 16 cases of PWS with negative HMME skin test results were included in this study. Their treated areas were exposed and injected with 5 mg/kg HMME, and then irradiated under 532 nm LED green light (produced by Wuhan YaGe LED1 modified machines), with the irradiation power density between 80 and 100 mW/cm². Each light spot was irradiated for 20–25 min. After one treatment, the clinical efficacy and post-treatment reactions at each treated area were observed.
Under standard operation, two of the 16 cases were cured (12.5%) after one HMME-PDT treatment, eight cases indicates a good efficacy (50.0%), four cases showed alleviation (25.0%), while two cases displayed no efficacy (12.5%), indicating an one-time response rate of 87.5%. Pain was reported during treatment, and significant post-treatment edema was observed at the treated areas; And no allergy or other adverse reaction were reported.
In adherence to operation standards and operation essentials during treatment, HMME-PDT shows a satisfactory clinical efficacy in treating PWS.
Greater Detection of Small Caliber Blood Vessels in Port Wine Stain with X Mode Than With Conventional Doppler: A Pilot Study
2017, Actas Dermo-Sifiliograficas
Prognosis and response to laser treatment of early-onset hypertrophic port-wine stains (PWS)
2016, Journal of the American Academy of Dermatology
There is limited information regarding early development of soft-tissue and/or bone hypertrophy with facial port-wine stains (PWS).
We sought to characterize patients with hypertrophic PWS presenting during childhood.
Patients with a facial PWS and underlying hypertrophy that developed before the age of 18 years were included in a multicenter retrospective study. Age at onset of the hypertrophy, its location, association with odontologic problems, presence of other associated complications, and response to laser treatment were recorded.
A total of 98 patients were included. The mean age at onset of hypertrophy, retrieved for 77 of 98 patients, was 5.6 years. The hypertrophy was congenital in 26%. Odontologic problems were noted in 39.8% of cases. Other complications, including cataract, asymmetric development of the maxillary bone, and speech delay/disorders, were reported in 18.4%. In all, 67 patients received laser treatment. Only 3% achieved complete or nearly complete clearance of the PWS.
As only cases of PWS with early-onset hypertrophy were included, we were unable to calculate the prevalence of this manifestation.
PWS with early-onset hypertrophy are associated with a high rate of complications and a poor response to laser treatment. Periodic monitoring is recommended for early detection and treatment of complications.

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ReviewPhysiologic changes in vascular birthmarks during early infancy: Mechanisms and clinical implications

Introduction

Section snippets

Physiologic changes in the newborn skin

Physiologic changes in hemoglobin concentration in the newborn

Morphologic changes in birthmarks

Discussion

J Pediatr

Clin Haematol

J Pediatr

J Pediatr

J Am Acad Dermatol

J Invest Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

Br J Plast Surg

Vascular tumors and vascular malformations (new issues)

Adv Dermatol

Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics

Plast Reconstr Surg

Heat production and temperature regulation

Periodic variations in skin perfusion in full-term and preterm neonates using laser Doppler technique

Acta Paediatr Scand

Hematologic problems in the newborn. Third edition

Major Probl Clin Pediatr

The physiology of the newborn infant

Review
Physiologic changes in vascular birthmarks during early infancy: Mechanisms and clinical implications