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Congenital hemangiomas are vascular tumors that are fully formed at birth, typically without postnatal growth. Noninvoluting congenital hemangiomas (NICH) have a distinctive clinical, radiologic, and histopathological profile and lack of expansion or involution over time. Herein, we describe two cases of NICH with atypical postnatal growth., (© 2019 Wiley Periodicals, Inc.)

Background: The proliferative phase of infantile hemangiomas (IHs) is usually complete by 9 months of life. Late growth beyond age 3 years is rarely reported., Objective: To describe the demographic and clinic characteristics of a cohort of patients with late growth of IH, defined as growth in a patient >3 years of age., Methods: A multicenter, retrospective cohort study., Results: In total, 59 patients, 85% of which were female, met the inclusion criteria. The mean first episode of late growth was 4.3 (range 3-8.5) years. Head and neck location (55/59; 93%) and presence of deep hemangioma (52/59; 88%) were common characteristics. Posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities (PHACE) syndrome was noted in 20 of 38 (53%) children with segmental facial IH. Systemic therapy (corticosteroid or β-blocker) was given during infancy in 58 of 59 (98%) and 24 of 59 (41%) received systemic therapy (β-blockers) for late IH growth., Limitations: The retrospective nature and ascertainment by investigator recall are limitations of the study., Conclusion: Late IH growth can occur in children after 3 years of age. Risk factors include head and neck location, segmental morphology, and involvement of deep dermal/subcutaneous tissues., (Crown Copyright © 2018. Published by Elsevier Inc. All rights reserved.)

Capillary hemangioma is the most common benign vascular eyelid tumor in childhood. The periocular lesion appears within the first few weeks after birth and usually has superficial or deep components. Primary conjunctival capillary hemangiomas are rarely reported. We present the case of a 2-day-old child with a pedunculated capillary hemangioma arising from superior palpebral conjunctiva. A complete surgical excision was performed under general anesthesia, and the child was asymptomatic at follow-up of two months.

Pulmonary capillary hemangiomatosis is a rare entity characterized by the proliferation of capillaries into alveolar walls, interlobular septa, pleura and pulmonary interstitium, without malignant characteristics, with almost constant association with pulmonary hypertension. Until now two cases of congenital presentation have been reported in the literature. This is the third case in a newborn; he has not followed the usual pattern associated with pulmonary hypertension as occurs in most patients with this pathology; the highest incidence is among 20-40 years old. We report a preterm newborn patient of 36 weeks of gestation with progressive respiratory distress requiring mechanical ventilation by constant desaturation during his clinical evolution without clinical, radiological or ultrasonographic signs of pulmonary hypertension., (Sociedad Argentina de Pediatría.)

Objective: To investigate the clinical efficiency of electrocoagulation for the treatment of noninvoluting congenital hemangioma., Methods: Sixteen infants with noninvoluting congenital hemangioma who were admitted to our hospital from January 2011 to June 2013 were included in this study. Color Doppler ultrasound was used to determine the hemangioma location, as well as its size and depth. High frequency electrocoagulation was adopted for the treatment. The output power was set at 10-20 W. The probes were inserted around the tumor or at the surface of the tumor. After switching on for 1-2 seconds, the direction and position of the probe was modulated until covering the whole tumor. After the treatment, the absorption of tumor was about 3-6 months. The efficiency was evaluated during the follow-up., Results: Tumor atrophy was obvious after treatment in all patients. The temperature around the tumor mass was decreased, and the aberrant blood signals were decreased under the ultrasonic examination. Complete or partial atrophy were observed. The efficiency was graded as level I, II, III, IV in 0, 2, 9 and 5 patients, respectively. One patient showed local infection due to improper nursing, which was completely relieved after corresponding treatment. No severe adverse events were observed., Conclusions: High-frequency electrocoagulation is effective for treating noninvoluting congenital hemangioma through coagulating the aberrant blood vessels in the tumor, interrupting the vascular endothelial cell, blocking the aberrant blood flow, as well as leading to atrophy and absorption of tumor mass. Besides, no obvious scar is observed after the surgery.

Background: Propranolol has become the first-line treatment for complicated infantile hemangiomas (CIHs) worldwide. Recommendations for monitoring infants undergoing propranolol therapy vary. Data on long-term blood pressure (BP) monitoring have not been reported before., Objective: The objective of the current study was to monitor BP in full-term infants during the induction and maintenance phase of propranolol therapy., Methods: BP was monitored prospectively in 109 infants (mean age 2.8 mos, range 1-5 mos) with CIHs during the induction (3-4 days in the hospital during up-dosing from 0.5 to 2.0 mg/kg/day) and maintenance (6 mos) phases of oral propranolol therapy., Results: Four children were excluded from the study because of sinus bradycardia (n = 2 [1.8%]) or lethargy (n = 2 [1.8%]). Mean systolic BP (SBP) decreased by 5 mmHg with the increase in propranolol dosage. Low (<5th percentile) SBP or diastolic BP (DBP) was observed in 2 of 105 children (1.9%) each. During the maintenance phase, 2 of 105 children (1.9%) had occasional SBP readings of less than 70 mmHg. No hypotension was observed after the third month of therapy. Low DBP (<36 mmHg) was recorded in 16 (15.2%) children after the first month, in 8.6% after the second, and in 2.9% during the third and fourth months of therapy. No patients exhibited clinical hypotension, bradycardia, or other known side effects of propranolol. Clinical response to therapy was excellent., Limitations: Reference BP values were derived from published tables, not from an untreated control group., Conclusions: In healthy full-term infants, propranolol (2 mg/kg/day divided in three doses) is well tolerated. No clinically significant hypotension was observed. We conclude that for otherwise healthy infants, BP monitoring during long-term propranolol therapy for CIHs is not necessary., (© 2015 Wiley Periodicals, Inc.)

Objectives: Hepatic hemangiomas are often found in association with multiple cutaneous infantile hemangiomas. Screening abdominal ultrasonography has been recommended for patients with five or more cutaneous lesions. We sought to determine whether hemangiomas found through screening had improved clinical outcomes., Methods: Patients entered into our hepatic hemangioma registry between 1995 and 2012 were reviewed., Results: Seventy-two patients with multiple cutaneous and hepatic hemangiomas were identified; 43 (60%) were detected through screening. The median age at diagnosis was 41 days for screened patients and 53 days for those not screened. Screening detected 40 (93%) multifocal and 3 (7%) diffuse hemangiomas, compared to 18 (62%) and 11 (38%), respectively, in the nonscreened group. Patients identified by screening had lower incidences of congestive heart failure and hypothyroidism and were less likely to receive treatment for their hemangiomas. The mortality rate in the children not screened was 28% (n = 8). None of the patients found by screening died (p < 0.001). Multivariate analysis of treated patients demonstrated that screening was a significant predictor of reduced mortality (p = 0.04)., Conclusion: Hepatic hemangiomas found through screening ultrasonography are less likely to develop serious clinical sequelae. Although the reasons for this may include detection of hemangiomas that are less likely to progress to symptomatic disease, it appears that it also allows for earlier intervention for more concerning (e.g. diffuse) subtypes. Screening may allow for closer surveillance and earlier treatment before life-threatening progression in a subset of infants with liver hemangiomas, preventing complications and reducing mortality., (© 2015 Wiley Periodicals, Inc.)

Background/objective: Oral propranolol has been shown to be safe and effective in infants with infantile hemangioma (IH). Side effects such as sleep disturbances have been associated with propranolol. The aim of this study was to evaluate the efficacy and safety of oral nadolol in a small series of patients whose propranolol therapy was discontinued due to sleep disturbances., Methods: A retrospective study of patients with IHs who were treated with oral nadolol due to propranolol-related sleep disturbances at a pediatric tertiary care center between July 2008 and March 2013. Clinical response to oral nadolol and disappearance of propranolol-related side effects were analyzed., Results: A total of 97 patients presenting IH received oral propranolol. Nine patients (9.3%) developed sleep disturbances. Oral propranolol was discontinued in seven patients and switched to oral nadolol, with resolution of these side effects in 5 (71%) of the cases. One patient developed sleep disturbances again after four months of oral nadolol., Limitations: The sample size was too small to draw generalizable conclusions and to draw any statistical inference as to the incidence of sleep disturbances with nadolol therapy., Conclusions: The use of oral nadolol in the treatment of IH in our series of 7 patients, resolved the propranolol-related sleep disturbances in 5 (71%), while in one patient the symptoms recurred after 4 months of oral nadolol at a dose of 2 mg/kg/day. In most cases, switching beta-blockers did not compromise efficacy, and is recommended when sleep disturbance necessitates discontinuation of beta-blocker therapy of IH., (© 2015 Wiley Periodicals, Inc.)

Objective: To investigate the clinicopathologic features and differential diagnoses of non-involuting congenital hemangioma (NICH) in children., Methods: The clinical, morphologic and immunophenotypic characteristics of 22 cases of NICH were retrospectively analyzed., Results: The mean patients' age at diagnosis was 4.2 years, with a male to female ratio of 1.75:1. The tumors were located in the head and face (5 cases), neck (3 cases), body (6 cases), upper limbs (5 cases), and lower limbs (3 cases). Histologically, the tumor was dominated by rather large lobules of small vessels that were mostly rounded, curved, small and thin-walled, and were lined by endothelial cells surrounded by one or more layers of pericytes. The center of the lobules was occupied by one or more thin or thick walled vessels, which were surrounded by fibrous and fatty tissue, which contained abnormal arterial and venous structures. At the edge of the lobules there were lymphatic vessels. Immunohsitochemical study showed that tumor cells in NICH were positive for CD34 (22/22), CD31 (22/22), SMA (22/22), vimentin (22/22) and Glut1 (0/22). D2-40 expression was located at the edge of the capillary lobules., Conclusions: NICH is a benign lesion. Clinically and pathologically, it needs to be differentiated from rapidly involuting congenital hemangioma, infantile hemangiomas, tufted angioma, vascular malformation, and others.

We describe a 6-month-old girl with medically intractable seizures, multiple congenital hemangiomas, and developmental delay. The patient underwent two surgical resections. Pathological findings at both the first and second resections were consistent with focal cortical dysplasia. The literature was reviewed on focal cortical dysplasia associated with cutaneous hemangiomas., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

In this report we detail the case of an infant presenting with a giant intracranial congenital hemangioma and describe the clinical features and surgical management. Congenital hemangiomas are benign vascular tumors that typically present as skin lesions in neonates and infants. On rare occasions they present as intracranial tumors. The possibility that these tumors may undergo spontaneous regression poses a treatment dilemma.

Background: Infantile hemangiomas have a dramatic response to propranolol, a nonselective beta-blocker. However, this treatment is not risk-free and many patients are excluded because of respiratory comorbidities. Atenolol is a cardioselective beta-blocker that may have fewer adverse events., Objective: We sought to evaluate the effectiveness of atenolol against propranolol in a noninferiority trial., Methods: In all, 23 patients met the inclusion criteria and were randomized to receive either atenolol or propranolol. Thirteen patients were treated with atenolol and 10 with propranolol. Follow-up was made at baseline, 2 weeks, 4 weeks, and then monthly for 6 months., Results: Patients treated with atenolol had a complete response of 53.8% and 60% with propranolol, respectively. These results were nonsignificant (P = .68). Relevant adverse events were not reported., Limitations: The reduced number of patients could have influenced our results., Conclusion: Atenolol appears to be as effective as propranolol. We did not find significant differences between these results or any adverse events., (Copyright © 2014 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Objectives: Infantile hemangiomas (IHs) in the airway may be potentially life-threatening during the proliferative phase. Available treatments like oral corticosteroids (OCS) and chemotherapeutic agents usually showed variable responses and serious side effects. Propranolol is a new and promising treatment option., Methods: A case series of five IH patients with airway involvement is presented, supplemented with a review of literature. Propranolol treatment (2.0-3.0mg/kg/day) was initiated between 3 weeks and 6 months of age. Three cases were treated with propranolol monotherapy, 2 cases with OCS primarily and propranolol secondarily, in which treatment with OCS could be reduced rapidly., Results: In our case series a dramatic, fast response was observed in all cases, with a permanent effect after discontinuation in four cases. In one patient a relapse of airway problems occurred two months after discontinuation of propranolol at 16 months of age; this resolved after re-start of propranolol. Review of literature together with these five cases showed 81 patients with airway IHs treated with propranolol. Propranolol was effective in 90% of the cases and seven patients were classified as non-responders. Eight IHs relapsed while weaning of propranolol or after discontinuation; dose adjustment or restart was effective in most cases but one patient appeared resistant to therapy., Conclusions: Propranolol seems to be a rapidly effective and safe treatment strategy for most IHs obstructing the airway. Because of the fast and important effects of propranolol, randomized controlled trials are hardly justifiable for this specific, relatively rare but, acute treatment indication. Despite the efficacy of propranolol, close monitoring of the patients with an airway IH is required, considering the risk of relapse of symptoms during or after treatment and the reported resistance to propranolol in at least 9% of the published cases. The dose and duration of treatment should be high and long enough to prevent relapse. Further research should focus on the optimal treatment protocol; the actual percentage of non-responders and also the mechanism of resistance to propranolol is unknown and needs to be illuminated., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Background: Oral propranolol produces dramatic results in severe infantile haemangioma. We report a case of rapid and significant regression of an infantile haemangioma in infant treated topically with 2% propranolol ointment., Patients and Methods: A female infant aged 11 weeks was seen as an outpatient for two infantile haemangiomas on the front of the left knee and the vulva. After parental consent was given, the child was treated with a topical preparation of 2% propranolol ointment prepared by the pharmacy. Regression was rapid and on the 45th day, lesion size had been reduced by an estimated 75%. No adverse effects were observed., Discussion: Topical propranolol appears to be effective and safe for the treatment of superficial haemangiomas. Clinical trials are required to determine the optimal dosage and pharmaceutical form, method of use and treatment duration., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: Infantile hemangioma is the most common vascular tumor in newborns, with an incidence from 12 to 23% among preterm infants with low weight at birth and a female to male ratio of 3:1. The head and neck is the most frequently affected area (60%), and the scalp is a typical site for such large lesions., Objective: We describe some clinical and medical aspects in comparison with the surgical approach to giant infantile hemangioma of the scalp., Methods: The indications to treatment are discussed. An outcome basis evaluation, by reviewing some clinical cases, is provided to help readers better understand when and how to undergo surgery safely., Conclusion: Early excision of huge infantile hemangioma of the scalp is the treatment of choice if feasible within 5 months of age.

Infantile hemangiomas (IHs) are increasingly being treated with propranolol or other beta-blockers, but before this therapeutic option was available, oral glucocorticosteroids (GCSs) were the criterion standard treatment and are still the alternative modality in problematic cases. Nevertheless, there is no standard treatment protocol for the dose and duration of GCSs. Long-term treatment with GCSs is associated with unwanted side effects such as growth suppression, behavioral changes, and reflux. Twenty-one children with troublesome IHs were treated according to an algorithm with 3 mg/kg/day of oral prednisolone divided three times per day with varying duration and number of GCS courses. Two blinded investigators independently interpreted therapy results using the Hemangioma Activity Score (HAS). Side effects were determined according to reports in patient charts and parental questionnaires. The median duration of a short course of GCSs was 2 weeks (range 1-6 weeks). The number of courses was 2 (range 1-5). The median cumulative dose was 91 mg/kg. Growth stabilized in all patients, with a good response (>50% reduction in HAS) in 62% and a favorable response (30-50% reduction is HAS) in 23%. Twelve of the 21 children (57%) had minor side effects. Persistent side effects did not occur. Intermittent short course, systemic, high-dose GCS therapy is an effective and safe treatment modality for IH, with a substantially lower cumulative dose of GCSs compared to prolonged therapy and no major side effects. This treatment is an alternative in cases in which propranolol fails or is contraindicated., (© 2012 Wiley Periodicals, Inc.)

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.)

Capillary haemangioma or infantile haemangioma (IH) is the most common congenital vascular tumour in the periocular region. Several treatment modalities have been documented, with variable degree of success. Propranolol has recently been reported to be an effective and safe alternative. The aim of this systematic review is to examine the evidence base for the use of propranolol administered orally in the management of periocular capillary haemangioma, and use this information to guide future research. A systematic review of literature was carried out by two independent reviewers using the search strategies highlighted below. A total of 100 cases of oral propranolol use in periorbital or orbital capillary haemangiomas have been documented in the literature. Of the 85 cases that had details of previous treatment, it was used as first-line treatment in 50 (58.8%). The commonest dose used was 2 mg/kg/day. Adverse events were documented in one-third of cases; in most cases these were minor. Improvement or complete resolution of the lesions occurred in 96% of cases. Recurrence was noted in one-fifth of cases. Propranolol has shown a lot of promise in the therapy of IH and further research in the form of properly designed randomized trials is certainly warranted. Treatment guidelines based on literature available to date is included in this review.

Aims: To investigate the expression of the placental cell-specific associated proteins in infantile haemangioma (IH)., Methods: Immunohistochemical staining was used to investigate the expression of human chorionic gonadotrophin (hCG), human placental lactogen (hPL), human leucocyte antigen-G (HLA-G), cytokeratin 7 (CK7) and smooth muscle actin in paraffin-embedded sections of proliferating and involuted IHs., Results: The proteins hCG and hPL were expressed by the endothelium but not the pericyte layer of proliferating IH, but these proteins were not detected in involuted lesions. There was no expression of CK7 and HLA-G in IH., Conclusions: The expression of hCG and hPL, but not CK7 or HLA-G, by the endothelium of proliferating IH supports a placental chorionic villous mesenchymal core cellular origin for IH rather than a trophoblast origin.

Cardiac hemangiomas can occur at any age, but they are extremely rare when they occur early after birth. We describe the case of a 5-month-old infant who had a giant right atrial capillary hemangioma associated with massive pericardial effusion. The tumor was incidentally diagnosed during routine clinical follow-up. The hemangioma was removed successfully under cardiopulmonary bypass, and the patient's postoperative course was uneventful. The occurrence of giant capillary hemangioma in infancy represents an unusual event in the relevant literature. Herein, we discuss the clinical features and surgical management of this rare primary tumor of infancy., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: The purpose of this report was to describe 2 cases of periocular infantile hemangiomas (IHs) that were successfully treated with low-dose oral propranolol alone and in combination with oral prednisolone., Methods: Two infants aged 3 months and 6 weeks, respectively, were referred for management of vision-threatening periocular IHs causing ocular displacement and obscuration of the visual axis. The first infant had a superficial left upper eyelid capillary hemangioma with extraconal extension and the second infant had a deep preseptal capillary hemangioma in the right lower eyelid with intraconal extension. Both cases were started on oral propranolol 0.5 mg/kg/day in divided doses and titrated up to 1.5 mg/kg/day as first-line therapy. The first infant was also given oral prednisolone 2 mg/kg/day during the initial first month of treatment., Results: Rapid regression in sizes of the hemangiomas was seen within the first 3 days of treatment. By 2 months of therapy, both infants had achieved normal ocular alignment. The second infant experienced a transient period of hypotension after the first dose of propranolol was started but recovered spontaneously. Both infants did not experience any adverse effects of propranolol throughout the treatment period., Conclusions: Low-dose oral propranolol is an effective first-line therapy for the management of vision-threatening IH. Dose escalation in combination with oral prednisolone after pediatric assessment might be useful in avoiding adverse effects of propranolol in young infants.

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.)

Airway hemangiomas are most often seen in association with cutaneous hemangiomas involving the "beard area." We report two unusual cases of extensive airway hemangiomas developing in patients with facial hemangiomas predominantly involving the upper face, emphasizing the need to consider the possibility of airway hemangiomas even in the absence of "beard area" hemangiomas., (© 2011 Wiley Periodicals, Inc.)

We describe the case of a 3-month-old infant suffering from rapidly progressive orbital capillary hemangioma which we treated with systemic propranolol over a period of 3.5 months. A few weeks after the onset of treatment, the exophthalmus had regressed and after 3 months of treatment the tumor completely vanished. This result is consistent with recent publications and supports the role of propranolol in the treatment of problematic hemangioma.

Hepatic infantile hemangiomas may be associated with morbidity and mortality, and traditional therapies may be associated with significant side effects. Since propranolol has been recently used successfully to treat cutaneous infantile hemangiomas, we decided to use it in three patients who presented with hepatic and skin hemangiomatosis. Three patients with skin and hepatic infantile hemangiomas, two of whom had evidence of cardiovascular compromise and one of whom had extensive liver involvement and hypothyroidism, were treated with oral propranolol. Regression of both skin and hepatic hemangiomas was noted in all patients, as was resolution of the cardiac symptoms and decreased thyroid requirement in two patients each. Propranolol was well tolerated without any adverse effects. Propranolol should be considered as a potential first-line therapy in patients with symptomatic hepatic hemangiomatosis., (© 2011 Wiley Periodicals, Inc.)

Congenital dacryocystocele is a uncommon type of nasolacrimal duct obstruction. Differential diagnosis for masses in the medial canthal region of a newborn include encephalocele, hemangioma, nasal glioma, and dermoid cyst. Because of the risk of becoming infected (acute dacryocystitis) and potentially lethal due to septicemia, aggressive management, including admission for intravenous antibiotics and surgical removal, is now advocated by many pediatric ophthalmologists if the cyst cannot be decompressed. Because of the commonly associated nasal cyst, infants with nasolacrimal dacryocyctocele may also experience respiratory distress especially when breast-feeding. The following case of dacryocystocele, which had progressed to dacryocystitis, was misdiagnosed as an infantile hemangioma. It is important to diagnose this entity quickly and refer for appropriate antibiotic and surgical management to avoid more serious sequelae of sepsis and possible death., (© 2011 Wiley Periodicals, Inc.)

Infantile hemangioma precursors or those without a proliferative phase may mimic a port-wine stain. We describe a case of infantile hemangioma precursor, which proved to be Glut-1 positive in biopsy, with the dermoscopic features of red round globular vessels, red comma-like vessels, and red linear vessels. These dermoscopic features should help to distinguish infantile hemangioma precursors or those with an absent or minimal growth phase from a port-wine stain., (© 2011 Wiley Periodicals, Inc.)

Background: infantile hemangioma (IH) is a most common tumor of infancy. Using infantile hemangioma-derived stem cells (HemSCs), we recently demonstrated that corticosteroids suppress the expression of VEGF-A, monocyte chemoattractant protein-1 (MCP-1), urokinase plasminogen activator receptor (uPAR), and interleukin-6 (IL-6); each of these are known targets of the transcription factor nuclear factor κ-light-chain-enhancer of activated B cells (NF-κB). In the present study, we examined the expression of these NF-κB target genes in IH tissue specimens and the effect of NF-κB regulation on the expression of pro-angiogenic cytokines, and in particular VEGF-A, in HemSCs., Materials and Methods: RNA extracted from IH tissue and hemangioma-derived stem cells (HemSCs) was used to analyze NF-κB target gene expression by reverse transcription-quantitative PCR (RT-qPCR). The effects of NF-κB blockade were examined in HemSCs. Immunostaining, immunoblotting and ELISA were used to assess protein expression., Results: MCP-1, uPAR, and IL-6 were found to be differentially expressed in proliferating versus involuting IH. Corticosteroids suppressed NF-κB activity of HemSCs. Velcade (Bortezomib), a proteosome inhibitor that can indirectly inhibit NF-κB, impaired HemSCs viability and expression of pro-angiogenic factors. Furthermore, specific inhibition of NF-κB resulted in suppression of VEGF-A., Conclusions: we demonstrate expression of NF-κB target genes in proliferating IH. In addition, we show that the expression of several pro-angiogenic factors in HemSCs, and in particular VEGF-A, is regulated by NF-B activity.

An echogenic tumor (22 x 22 mm) isolated from the anterior part of the left leg at 27 WA of pregnancy suggested an angiomatous structure. This mass appeared to be well vascularized on Doppler examination. Three-dimensional imaging showed connections with adjacent tissues. The limbs were mobile. Delivery of a 3990-g-boy occurred normally. On the anterior part of the left leg, there was a plate-shaped mass with a pink hole at the superior part. Excision under general anesthesia was performed at the first month because of the risk of rupture and bleeding. The dissection was easy with the deep plane. Healing was complete 10 days after the surgical procedure and the aesthetic aspect was good 1 year later. Histological findings of the suspect mass emphasize the vascular aspect of the tumor. A hemangioma capillary with venous structures in the dermal hypodermic area was found., (Copyright 2010. Published by Elsevier SAS.)

Unlabelled: Periocular haemangioma of childhood can severely impact visual development., Objective(s): To review our experience with 20 periocular haemangioma patients; to review infant ocular development in the context of periocular capillary haemangioma; to identify early clinical warning signs that may precede devastating visual outcomes in the absence of timely management and to review our experience with surgical debulking for the treatment of selected periocular haemangioma., Design: Retrospective case series., Interventions: Twenty children with congenital periocular haemangiomas received care by a multidisciplinary team consisting of doctors from the specialties ophthalmology, plastic surgery, paediatrics and dermatology. The patients were separated by age at presentation to our centre (1 year). Based on consensus amongst the team, certain patients were considered to be at high risk for development of amblyopia, permanent cortical visual change or blindness. These patients were scheduled for urgent surgical excision or debulking. The effect of treatment on visual development over time was evaluated., Results: Patients presenting to our centre after 1 year of age were more likely to have amblyopia (75% vs. 0% if presenting at

While infantile haemangiomas are the most common tumours of childhood, rare congenital haemangiomas grow to maximum size at birth and then spontaneously and rapidly regress (rapidly involuting congenital haemangiomas). However, certain congenital haemangiomas, described recently as 'non-involuting congenital haemangiomas', evolve differently and do not regress. There are several options regarding the treatment of infantile haemangiomas, but few reports have addressed the treatments of non-involuting congenital haemangiomas, though a small number have recommended surgical excision. However, the treatments of non-involuting congenital haemangiomas with multiple epidermal cysts have not been investigated. Epidermal cysts can cause recurrent infection, and we suspect that recurrent ulceration might cause the implantation of epidermal cells into the dermis, and result in the development of multiple epidermal cysts. Currently, epidermal cysts are treated by surgical excision, which should reduce complication rates. Herein, we report our experience of treating repeatedly infected non-involuting congenital haemangioma with multiple epidermal cysts., (Copyright (c) 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Infantile haemangioma is the most common tumour of infancy, yet the pathogensis of this lesion remains unknown and the predictable life cycle is poorly understood. Though much new information on infantile haemangioma has emerged over the past decade, researchers continue to debate the fundamental features; including cells of origin, nonrandom distribution, and mechanisms regulating the sometimes explosive growth and slow involution. The development of biomarkers has shed light on the pathogenesis and management of infantile haemangioma. Several useful biomarkers and their suggestions as to the aetiology of infantile haemangioma are reviewed. In addition, the application in clinical diagnosis and choice of treatment methods of infantile haemangioma is summarised.

Hemangiomas are benign tumors of the capillary endothelium involved in varied clinical and pathologic entities. Congenital hemangioma is quite rare compared to infantile hemangioma. Hemangiomas rarely occur in bone. Intraosseous hemangiomas are most commonly found in the vertebral column and skull. The mandible is the most frequently affected skull bone. Intraosseous hemangiomas commonly present in the second decade of life. Here, we report a neonate with noninvoluting congenital hemangioma of the mandible. To our knowledge, she is the youngest patient reported in the literature.

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.

Infantile or capillary hemangioma is the most common vascular tumor of childhood. The tumors most frequently affect the head and neck area, but rare cases of intracranial lesions have been reported. Their natural history is marked by initial rapid growth velocity followed by a plateau and, in most cases, subsequent involution. Although the lesions are considered benign, 10% of affected children develop life-threatening complications (mortality rate 20-80% in this subgroup). When surgical intervention or other methods of local control are not possible, therapeutic options are limited. Corticosteroids have been the mainstay of therapy but therapeutic response is not predictable and the infectious risk is not negligible. Interferon alpha-2a may also be effective but has significant toxicities. Vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) have been implicated in the pathogenesis of hemangiomas, and antiangiogenesis agents are being evaluated in the treatment of these tumors. Thalidomide may be an ideal therapy for life-threatening hemangiomas because it inhibits new blood vessel formation by antagonizing both the bFGF and VEGF pathways and has a more acceptable toxicity profile than other agents. The authors present the case of an infant born with a life-threatening, unresectable intracranial hemangioma in which treatment with thalidomide resulted in a good clinical outcome.

The optic nerve glioma is a relatively rare pathology in children and only a few cases have been described in infants. This paper reports the symptoms of this exceptional case in a 4-month-old child and reviews the differential diagnoses at this age. Finally, we discuss the diagnosis and therapeutic possibilities.

Congenital pulmonary hemangiomas are extremely rare lesions. We present a unique case of a giant congenital pulmonary hemangioma in an otherwise healthy newborn who presented with respiratory distress at birth. This was managed with resection after an unsuccessful trial of nonoperative management. The clinical, radiographic, and pathologic features of this unique case are discussed in the context of a review of the literature.

Intracranial capillary hemangiomas are very rare, though several spinal capillary hemangiomas have recently been reported. We report here a case of intracranial capillary hemangioma with multiple cysts and review the current literature of similar cases. A 4-month-old girl was referred to our hospital for treatment of hydrocephalus and a cerebellar mass lesion. She presented with hemangiomas distributed widely over the body, as well as disseminated hemangiomas in the pleura, liver, spleen, pancreas, kidneys and vagina. Pathological examination of the specimen from the vagina confirmed the diagnosis of a capillary hemangioma made at another hospital. Radiological examination of the brain revealed a cystic mass lesion in the left cerebellar hemisphere with subsequent obstructive hydrocephalus. The cysts extended upward into the bilateral ventricle. Following neuroendoscopic fenestration of the cysts, resection of the left cerebellar mass was performed. Histological examination of the lesion demonstrated similarly sized capillaries, and the pathological diagnosis was confirmed as capillary hemangioma. There was no recurrence postoperatively. Our patient was treated successfully by surgical resection and neuroendoscopic procedures. Surgical intervention may therefore be indicated in intracranial capillary hemangiomas., (2008 S. Karger AG, Basel)

The authors report the case of a 3-week-old girl with two enhancing extraaxial masses in the posterior fossa, one in the left cerebellopontine angle (CPA) and the other to the right of the vein of Galen. Serial magnetic resonance images obtained in this patient at 3 months and then at 2.5 years of age documented regression of the enhancing mass in the left CPA and development of a cerebellar brain arteriovenous malformation (AVM) in the same CPA location. Also documented were regression of the pineal region mass and formation of the major draining vein of the AVM. The findings in this case support the theory that cerebral AVMs have early postnatal growth potential.

Background: A total of 620 healthy term neonates, randomly selected among babies born at the San Paolo Hospital in Milan (Italy), were examined to study the relationship between birthmarks and transient cutaneous lesions in newborns of different ethnic groups., Methods: Information on sociodemographic factors and on physiopathological variables of the pregnancy was collected. Multiple logistic analyses were performed to assess associations between diagnosed skin lesions and various factors. Odds ratios (OR) as a measure of association and the corresponding 95% confidence intervals were estimated., Results: A positive association was found between reduced hypoderm and pregnancy illness (OR = 2.78), hypertrophy genitalia and use of drugs (OR = 1.86) and illnesses in pregnancy (OR = 1.61). Hyperpigmentation in the genital area and Mongolian spot showed significant positive association with geographical area of origin, being systematically more frequent in non-European neonates, while for melanocytic congenital nevi a positive association was observed only for Asiatic newborns (OR = 4.67); salmon patch on the nape showed a significant OR of 1.81 among mothers aged > or = 35., Conclusions: Significant associations between some sociodemographic factors and cutaneous lesions of the newborn and anamnestic data related to the pregnancy were found. Dermatologic conditions are common in the newborns and may justify dermatologic examination., (2007 S. Karger AG, Basel)