Infantile Hemangiomas and Vascular Anomalies.

Vascular anomalies represent a diverse group of disorders of abnormal vascular development or proliferation. Vascular anomalies are classified as vascular tumors and vascular malformations. Significant advances have been made in the understanding of the pathogenesis, natural history, and genetics of vascular anomalies, allowing for improvements in management including targeted molecular therapies. Infantile hemangiomas are the most common vascular tumor of childhood and follow a distinct natural history of proliferation and involution. Although benign, infantile hemangiomas can be associated with important complications. The use of beta-blockers has revolutionized the management of infantile hemangiomas. Other vascular tumors include pyogenic granulomas, congenital hemangiomas, and kaposiform hemangioendotheliomas, among others. Vascular malformations are categorized based on the type of involved vessel, including capillary malformations, venous malformations, lymphatic malformations, arteriovenous malformations, and mixed vascular malformations. Expert multidisciplinary management of vascular anomalies is critical to optimize outcomes in these patients. [Pediatr Ann. 2024;53(4):e129-e137.].

Delayed ulceration following combination pulse dye laser and topical sirolimus treatment for port wine birthmarks: A case series.

Port wine birthmarks (PWB) are capillary vascular malformations within the papillary and reticular dermis, most commonly occurring on the head and neck and may darken and thicken with age. Pulsed dye laser (PDL) is the gold standard of treatment for PWB as it selectively targets involved vessels. Sirolimus is a macrolide antibiotic that selectively inhibits mammalian target of rapamycin, thereby suppressing the angiogenesis pathways that can be activated by PDL. Sirolimus and PDL may be used together to treat PWB. We present a case series describing three cases of delayed ulceration and systemic sirolimus absorption following combination therapy, highlighting a potential complication and patient safety concern.

Longitudinal evaluation of two different COVID-19 vaccination strategies in individuals with and without previous SARS-CoV-2 infection.

Background: We tested the antibody response to SARS-CoV-2 vaccination in individuals with and without previous infection that received different vaccination strategies. Methods: We recruited 203 volunteers. Individuals who have had SARS-CoV-2 infection during the six months preceding vaccination received one dose (group 1), the others received two (group 2). After 3 months, 98 subjects received a booster dose. Anti-SARS-CoV-2 Spike RBD IgG were tested in all subjects before vaccination (T0), and at 15 (T15), 90 (T90), 180 (T180) and 360 (T360) days after second or single dose; additionally, in group 2, IgG were tested 10 days after the vaccination (T10). Results: The difference of IgG concentration between the groups was statistically significant (p<0.05) at T0, T15 and T90, but not at T180 (p=0.713) and T360 (p=0.069). At T0 and T90 the antibody titre was higher in group 1, but it dropped in all volunteers 90 days after vaccination. Most of infections after vaccination occurred between T90 and T180. Conclusions: Antibody titre is significantly associated with a previous SARS-CoV-2 infection. Probability of contracting the infection increases after three months from primary vaccination, even among who had a previous infection, confirming the efficacy of vaccination as a preventive measure against SARS-CoV-2 infections and the need of booster administrations.

Rosacea and the Microbiome: A Systematic Review.

Rosacea, a chronic inflammatory skin disease characterized by recurrent episodes of facial flushing, erythema, pustules, and telangiectasia, largely affects fair-skinned women over 30 years of age. Although a long-recognized entity, the exact pathophysiology of this disease is still debated. Current theories highlight the role of the cutaneous microbiome and its associated inflammatory effects in rosacea's pathogenesis. However, microbiological reverberations are not limited to the skin, as recent studies have described the potential cutaneous effects of alterations in the gastrointestinal (GI) microbiome. Associations with additional GI pathologies, including small intestinal bacterial overgrowth (SIBO), irritable bowel syndrome (IBS), and inflammatory bowel disease (IBD), have been investigated, as well as Helicobacter pylori infection. In an attempt to better understand and characterize these relationships, as well as current treatment options, we conducted a systematic review of the literature in PubMed, Cochrane, and Embase from their inception to August 6, 2020. We have synthesized the literature findings within three sections of this manuscript: the cutaneous microbiome, the gut microbiome, and therapeutic strategies. Future studies should focus on specific mechanisms linking GI pathology with rosacea manifestations and the role of enteral drugs in mitigating cutaneous symptoms.

Optimizing Management of Otophyma: A Case Series Highlighting the Role of Surgical and Retinoid Therapy.

Otophyma is a rare condition that can result in conductive hearing loss. Current otophyma literature does not examine validated treatment outcomes for patients. Utilizing a medical and surgical approach to maintain a patent canal can lead to significant objective improvements. The aim of this case series is to describe a combined successful approach in 3 cases from an academic, multidisciplinary center. The main outcomes analyzed were pre and post air-bone gap audiogram analysis and disimpaction frequency. The results showed that post-management, patient 1 had substantial improvement in hearing, recovering 49 dB in his right ear and 25 dB in his left ear, demonstrating near complete air-bone gap closure. Patient 2 showed a similar dermatologic and functional improvement, although objective audiometric assessment related to otophyma could not be performed due to coexisting chronic otitis media and cholesteatoma. Patient 3, in the 12 months prior to comanagement, had 8 bilateral disimpactions, and following comanagement had 2 disimpactions in 23 months. All 3 patients were pleased with the resultant functional and physical appearance following comanagement. By presenting this approach and objective measures of treatment, we hope to improve future clinical decision-making in a rare condition.

The influence of spatial location on same-different judgments of facial identity and expression.

The "spatial congruency bias" is a behavioral phenomenon where 2 objects presented sequentially are more likely to be judged as being the same object if they are presented in the same location (Golomb, Kupitz, & Thiemann, 2014), suggesting that irrelevant spatial location information may be bound to object representations. Here, we examine whether the spatial congruency bias extends to higher-level object judgments of facial identity and expression. On each trial, 2 real-world faces were sequentially presented in variable screen locations, and subjects were asked to make same-different judgments on the facial expression (Experiments 1-2) or facial identity (Experiment 3) of the stimuli. We observed a robust spatial congruency bias for judgments of facial identity, yet a more fragile one for judgments of facial expression. Subjects were more likely to judge 2 faces as displaying the same expression if they were presented in the same location (compared to in different locations), but only when the faces shared the same identity. On the other hand, a spatial congruency bias was found when subjects made judgments on facial identity, even across faces displaying different facial expressions. These findings suggest a possible difference between the binding of facial identity and facial expression to spatial location. (PsycInfo Database Record (c) 2020 APA, all rights reserved).

Does Hirschsprung-Associated Enterocolitis Differ in Children With and Without Down Syndrome?

BACKGROUND: Children with Hirschsprung disease (HD) and Down syndrome (DS) are reported to experience a higher incidence of Hirschsprung-associated enterocolitis (HAEC) than those without DS, although whether their individual episodes are more severe is unknown. METHODS: A single-institution retrospective cohort study was performed on all patients with HD admitted with HAEC between 2009 and 2017. Patients were divided into two groups based on an associated diagnosis of DS, and demographics, clinical characteristics at presentation, and clinical outcomes were compared. RESULTS: Of 86 admissions for HAEC over the study period, 12 (14%) were for patients with DS. Patients admitted with and without DS were similar in terms of age (3.0 versus 2.6 y, P = 0.72), male gender (58% versus 77%, P = 0.17), and proportion with disease proximal to the left colon (9% versus 12%, P = 0.70). Patients admitted with DS were more likely to be tachycardic (75% versus 19%, P ≤ 0.001) and hypotensive (33% versus 7%, P = 0.01) at presentation and presented for treatment after a longer duration of symptoms than those without DS (84 versus 24 h, P = 0.001). Patients admitted with DS were more likely to require intensive care unit admission (58% versus 12%, P = 0.006) and received a longer course of antibiotics (13 d versus 7 d, P = 0.02). The length of stay was similar (6 versus 4 d, P = 0.34). CONCLUSIONS: The results of our study suggest that patients with DS experience more severe episodes of HAEC. Ongoing study is needed to identify preventive strategies to decrease the morbidity of HAEC in children with HD and DS.

Anal sphincter botulinum toxin injection in children with functional anorectal and colonic disorders: A large institutional study and review of the literature focusing on complications.

BACKGROUND AND AIM: Botulinum toxin (botox) is a commonly used treatment for functional anorectal and colonic disorders. Although generally regarded as safe, complications associated with botox injection into the anal sphincters in children with severe defecation disorders are not well described. We aimed to review our institutional experience and the existing literature to better understand the safety of this practice. METHODS: We performed a retrospective review of pediatric patients undergoing botox administration into the anal sphincter for treatment of a variety of defecation disorders between 2014 and 2018. Additionally, we performed a review of all published literature reporting complications from botox injection in this patient population. RESULTS: 881 patients ranging from 5 weeks to 19.7 years underwent a total of 1332 botox injections including our institution (332 patients/526 injections) and the reviewed series (549 patients/806 injections). Overall, complications were seen after 9 (0.7%) injections and included urinary incontinence (n = 5), pelvic muscle paresis (n = 2), perianal abscess (n = 1), pruritis ani (n = 1), and rectal prolapse (n = 1). Patient age, weight, and diagnosis were not associated with an increased rate of complication in our institutional experience. All complications were self-limited and did not require intervention. There were no episodes of systemic botulinum toxicity. CONCLUSION: Botox injection into the anal sphincters is accepted practice in children with Hirschsprung disease, severe functional constipation, and internal anal sphincter achalasia and appears to be safe from this review. The precise dosing and age at which complications are more likely to arise could not be ascertained and require further study. LEVEL OF EVIDENCE: IV TYPE OF STUDY: Retrospective cohort study.