The biker-glove pattern of congenital melanocytic nevi.

Congenital melanocytic nevi (CMN) are common birthmarks with 20% occurring on the limbs. We describe 4 patients with acral CMN with a "biker-glove" distribution with sparing of the distal digits, as has previously been described in acral infantile hemangiomas (IH). The existence of the biker-glove pattern suggests that CMN arise from early mutations in melanocyte precursors and supports the recently described Kinsler-Larue hypothesis of mesenchymal distribution of melanocyte migration occurring in a circular field from a central point. Developmental errors in mesenchymal precursors with similar migration patterns may explain this shared pattern among CMN and IH

Rudimentary meningocele: remnant of a neural tube defect?

Background: Rudimentary meningocele, a malformation in which meningothelial elements are present in the skin and subcutaneous tissue, has been described in the past, under a variety of different terms and has also been referred to as cutaneous meningioma. There has been debate as to whether rudimentary meningocele is an atretic form of meningocele or results from growth of meningeal cells displaced along cutaneous nerve

Neurodevelopmental abnormalities in children with PHACE syndrome.

Prior case reports have identified neurodevelopmental abnormalities in children with PHACE syndrome, a neurocutaneous disorder first characterized in 1996. In this multicenter, retrospective study of a previously identified cohort of 93 children diagnosed with PHACE syndrome from 1999 to 2010, 29 children had neurologic evaluations at ≥ 1 year of age (median age: 4 years, 2 months). In all, 44% had language delay, 36% gross motor delay, and 8% fine motor delay; 52% had an abnormal neurological exam, with speech abnormalities as the most common finding. Overall, 20 of 29 (69%) had neurodevelopmental abnormalities. Cerebral, but not posterior fossa, structural abnormalities were identified more often in children with abnormal versus normal neurodevelopmental outcomes (35% vs. 0%, P = .04). Neurodevelopmental abnormalities in young children with PHACE syndrome referred to neurologists include language and gross motor delay, while fine motor delay is less frequent. Prospective studies are needed to understand long-term neurodevelopmental outcomes

Stroke in children with posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities (PHACE) syndrome: a systematic review of the literature.

Background and purposePHACE is an acronym for posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Several case reports of arterial ischemic stroke (AIS) in individuals with PHACE have been published, but risk factors for AIS in PHACE have not been clearly defined. The objective of this article is to review all cases of stroke in PHACE in children and describe clinical characteristics that may be associated with an increased risk of AIS.MethodsA literature and registry search was conducted to identify patients with PHACE who had experienced AIS. Data were analyzed to determine age of onset, presenting signs and symptoms, and clinical features among this cohort compared with PHACE without AIS.ResultsTwenty-two individuals with PHACE and AIS were identified. Imaging of the arteries of the head and neck was reported in 20 of 22. Narrowing or nonvisualization of at least 1 great cerebral vessel was present in 19 of 20 and of those, 15 had ≥ 2 vessels involved. Aortic arch anomalies were reported in 13 of 22 individuals.ConclusionsAplasia, hypoplasia, or occlusion of a major cerebral artery appears to be a significant risk factor for AIS in children with PHACE, especially when >1 vessel is involved or if there is coarctation of the aorta

Management of infantile hemangiomas during the COVID pandemic

This article is made available for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.The COVID‐19 pandemic has caused significant shifts in patient care including a steep decline in ambulatory visits and a marked increase in the use of telemedicine. Infantile hemangiomas (IH) can require urgent evaluation and risk stratification to determine which infants need treatment and which can be managed with continued observation. For those requiring treatment, prompt initiation decreases morbidity and improves long‐term outcomes. The Hemangioma Investigator Group has created consensus recommendations for management of IH via telemedicine. FDA/EMA‐approved monitoring guidelines, clinical practice guidelines, and relevant, up‐to‐date publications regarding initiation and monitoring of beta‐blocker therapy were used to inform the recommendations. Clinical decision‐making guidelines about when telehealth is an appropriate alternative to in‐office visits, including medication initiation, dosage changes, and ongoing evaluation, are included. The importance of communication with caregivers in the context of telemedicine is discussed, and online resources for both hemangioma education and propranolol therapy are provided

The biker-glove pattern of congenital melanocytic nevi.

Congenital melanocytic nevi (CMN) are common birthmarks with 20% occurring on the limbs. We describe 4 patients with acral CMN with a "biker-glove" distribution with sparing of the distal digits, as has previously been described in acral infantile hemangiomas (IH). The existence of the biker-glove pattern suggests that CMN arise from early mutations in melanocyte precursors and supports the recently described Kinsler-Larue hypothesis of mesenchymal distribution of melanocyte migration occurring in a circular field from a central point. Developmental errors in mesenchymal precursors with similar migration patterns may explain this shared pattern among CMN and IH

Randomized Controlled Trial of Spaced Education for Pediatric Residency Education

BackgroundSpaced education (SE) has shown promise as an instructional tool that uses repeated exposure to the same questions, but information on its utility in graduate medical education is limited, particularly in assessing knowledge gain with outcome measures that are different from repeat exposure to the intervention questions.ObjectiveWe examined whether SE is an effective instructional tool for pediatrics residents learning dermatology using an outcome measure that included both unique and isomorphic questions.MethodsWe randomized 81 pediatrics residents into 2 groups. Group A completed an SE course on atopic dermatitis and warts and molluscum. Group B completed an SE course on acne and melanocytic nevi. Each course consisted of 24 validated SE items (question, answer, and explanation) delivered 2 at a time in 2 e-mails per week. Both groups completed a pretest and posttest on all 4 topics. Each group served as the comparison for the other group.ResultsFifty residents (60%) completed the study. The course did not have a statistically significant effect on the posttest scores for either group. Overall, test scores were low. Eighty-eight percent of residents indicated that they would like to participate in future SE courses.ConclusionsUsing primarily novel posttest questions, this study did not demonstrate the significant knowledge gains that other investigators have found with SE