Clinical Characteristics and Complications in Patients with Complex Vascular Anomalies

BACKGROUND/OBJECTIVE: Vascular anomalies are rare complications of development, with some forms affecting less than 1% of the population. In addition to visible manifestations, they may cause pain, swelling, bleeding, thrombosis, and infection. These conditions often require more than one field of medical expertise, so incorporating multidisciplinary care is essential for optimizing management strategies. In an effort to better describe a cohort of these patients requiring complex interventions and understand the spectrum of care they need, we captured demographic, clinical, and quality-of-life data to serve as a launching point for future studies. METHODS: We designed a RedCap database and conducted a retrospective chart review of 100 patients who presented at the Vascular Lesions Clinic (VLC) at Riley Children’s Hospital from May 2020 to May 2022. Demographic, clinical, and quality-of-life data using the OVAMA scale was obtained from Cerner and captured on RedCap. Excel and RedCap software were used to characterize this patient population. RESULTS: The majority of patients had diagnoses of venous malformations and lymphatic malformations. These anomalies showed no male or female predominance and most lesions were segmental. Sclerotherapy was the most common intervention, with venous malformations receiving a higher median number of treatments than lymphatic malformations. Lymphatic malformations were associated with lower appearance satisfaction and a younger median age at the time of the VLC visit than venous malformations. Older age, larger lesion size, female sex, and lesion location on the lower extremities also correlated with worse quality-of-life outcomes. CONCLUSION AND POTENTIAL IMPACT: The characterization of this cohort will guide broader studies of treatments and quality-of-life trends among patients with complex vascular anomalies. Future directions could explore patient outcomes, complication rates, and influences on quality-of-life in a prospective study design

Peters anomaly in PHACE syndrome

PHACE syndrome is a rare neurocutaneous disorder, with a complex pathogenesis. It presents with a large facial hemangioma associated with anomalies of the posterior fossa of the brain, arterial anomalies, cardiac anomalies, coarctation of the aorta, and eye anomalies. Ocular abnormalities are rare. We report the first published case of an infant with PHACE syndrome and Peters anomaly

Neonatal lupus with left bundle branch block and cardiomyopathy: a case report

Background Cardiac manifestations of neonatal lupus include an array of structural and conduction abnormalities due to placental transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies. Late-onset neonatal lupus cardiomyopathies, occurring outside the neonatal period, is an infrequently reported manifestation with unknown pathophysiology and poorly defined treatment regimens. Due to the rarity of this condition, additional studies and case reports are required to better understand and manage late-onset neonatal lupus cardiomyopathies. Case presentation A 4-week-old female, born to a mother with known anti-SSA/Ro and anti-SSB/La autoantibodies, presents with classic cutaneous manifestations for neonatal lupus and is found to have left bundle branch block, severely dilated cardiomyopathy with an ejection fraction of 25%, and a thin echogenic dyskinetic ventricular septum. Weekly second trimester and 30-week fetal echocardiograms showed no signs of structural or conduction abnormalities. There were no histologic signs of inflammation on cardiac tissue biopsy. After a complicated hospital course, she was successfully treated with biventricular pacemaker, intravenous immunoglobulin, and plasmapheresis. Conclusions We present a case of late-onset neonatal lupus with severe dilated cardiomyopathy, a dyskinetic ventricular septum, and left bundle branch block. To our knowledge, the dyskinetic ventricular septum has never been reported and left bundle branch block is rarely reported in NL. This case further validates the need for long term cardiac follow up for patients born with NL, even if lacking cardiac manifestations in the peripartum period. We characterize a unique presentation of a rare clinical entity, highlighting the diagnostic challenges, and describe a successful treatment course

Seborrhea Herpeticum: Cutaneous Herpes Simplex Virus Infection Within Infantile Seborrheic Dermatitis

Eczema herpeticum has been well described in the setting of atopic dermatitis (AD) and other dermatoses. We present the case of a 2-month-old infant boy with cutaneous herpes simplex virus (HSV) infection within existing diffuse infantile seborrheic dermatitis. Providers should be aware that cutaneous HSV can be confined to a seborrheic distribution and may represent underlying epidermal dysfunction secondary to seborrheic dermatitis

Segmental infantile hemangioma and concomitant hypertension in three African American neonates

We present three African American infants with segmental, ulcerated infantile hemangiomas and concomitant, persistent hypertension. When treated with beta-blocker therapy, the hemangiomas decreased in size and the ulcerations resolved, but there was no impact on the elevated blood pressure in one of our patients. We failed to identify any associations between infantile hemangioma and hypertension in the literature

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

Congenital agminated segmental nevi of the chest

Grouped patterns of pigmented lesions are infrequent. Of the several reports of agminated nevi, most have been Spitz nevi or blue nevi. The distribution of these nevi is often segmental, following a dermatome or the lines of Blaschko. Most segmental nevi are not agminated and develop early in childhood [1]. We describe a rare case of congenital agminated segmental nevi on the chest

Congenital agminated segmental nevi of the chest

Grouped patterns of pigmented lesions are infrequent. Of the several reports of agminated nevi, most have been Spitz nevi or blue nevi. The distribution of these nevi is often segmental, following a dermatome or the lines of Blaschko. Most segmental nevi are not agminated and develop early in childhood [1]. We describe a rare case of congenital agminated segmental nevi on the chest

Rebound Growth of Infantile Hemangiomas Following Propranolol Therapy

BACKGROUND AND OBJECTIVES: Propranolol is first-line therapy for problematic infantile hemangiomas (IHs). Rebound growth after propranolol discontinuation is noted in 19% to 25% of patients. Predictive factors for rebound are not completely understood and may alter the management approach. The goal of the study was to describe a cohort of patients with IHs treated with propranolol and to identify predictors for rebound growth. METHODS: A multicenter retrospective cohort study was conducted in patients with IHs treated with propranolol. Patient demographic characteristics, IH characteristics, and specifics of propranolol therapy were obtained. Episodes of rebound growth were recorded. Patients' responses to propranolol were evaluated through a visual analog scale. RESULTS: A total of 997 patients were enrolled. The incidence of rebound growth was 231 of 912 patients (25.3%). Mean age at initial rebound was 17.1 months. The odds of rebound among those who discontinued therapy at <9 months was 2.4 (odds ratio [OR]: 2.4; 95% confidence interval [CI]: 1.3 to 4.5; P = .004) compared with those who discontinued therapy between 12 to 15 months of life. Female gender, location on head and neck, segmental pattern, and deep or mixed skin involvement were associated with rebound on univariate analysis. With multivariate analysis, only deep IHs (OR: 3.3; 95% CI: 1.9 to 6.0; P < .001) and female gender (OR: 1.7; 95% CI: 1.1 to 2.6; P = .03) were associated. Of those with rebound growth, 83% required therapeutic modification including 62% of patients with modifications in their propranolol therapy. CONCLUSIONS: Rebound growth occurred in 25% of patients, requiring modification of systemic therapy in 15%. Predictive factors for rebound growth included age of discontinuation, deep IH component, and female gender. Patients with these predictive factors may require a prolonged course of therapy