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PIK3CA‐related overgrowth spectrum (PROS) encompasses different clinical entities caused by somatic activating mutations in PIK3CA. Among PROS, CLOVES syndrome represents a severe phenotype with poor survival rate. We present the case of a 4‐month‐old girl with CLOVES syndrome successfully treated with alpelisib, a PIKC3A inhibitor. [ABSTRACT FROM AUTHOR]

The burden of treatment (BOT) related to propranolol treatment for infantile hemangiomas (IH) has never previously been explored. A modified validated questionnaire, the Treatment Burden Questionnaire, and one‐on‐one semi‐structured interviews were used to assess the BOT for propranolol for IH. Out of 80 caregivers, the overall burden score was very low at 1.2 out of 10; thematic analysis of interviews grouped themes into administration, monitoring, financial, and associated anomalies. The BOT of propranolol for IH is very low but could be reduced further by offering age‐based risk stratification related to feeding frequency and risk of hypoglycemia, pragmatic advice around timing of doses before sleep, and reducing frequency of vital sign monitoring. [ABSTRACT FROM AUTHOR]

PHACE (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac anomalies, eye anomalies) association has many recognized clinical features. A link between PHACE and non‐vascular intracranial lesions has not been well‐described. We report three pediatric patients with PHACE and non‐vascular intracranial lesions. [ABSTRACT FROM AUTHOR]

High‐flow vascular malformations have been associated with multiple syndromes including capillary malformation–arteriovenous malformation (CM–AVM) syndrome, hereditary hemorrhagic telangiectasia syndrome, and less commonly, phosphatase and tensin homolog hamartoma tumor syndrome (PHTS). We present a series of three patients with clinically challenging complex AVMs who were found to have underlying PHTS. In all patients, diagnosis was delayed, and the presence of the AVM prompted sampling and genetic testing for PHTS in the absence of other clinical features of the condition. This series highlights the importance of screening for PHTS in the setting of high‐flow vascular malformations. [ABSTRACT FROM AUTHOR]

Background/Objectives: Ulceration is a common complication of infantile hemangioma (IH). Severe, persistent ulceration occurs in a minority of patients. This study aims to characterize the clinical features of IH with aggressive ulceration (AU). Methods: Multicenter retrospective study of clinical features of IH with AU. Results: Thirty‐five patients with AU were identified and included in the study. The majority of AU occurred in segmental IH (23/35, 65%). Segmental IH with AU were large (≥10 cm2; 16/23, 69%, p <.001) with a thin (<3 mm) superficial component (16/23, 69%, p <.001). Localized IH with AU had a thick (>3 mm) superficial component (11/12, 92%, p <.001). All diaper area IH with AU (9/35) were segmental with thin superficial component (100%, p =.02). IH with AU in the head/neck (10/35) were more commonly localized (67%) and mixed (62.5%), while segmental, thick superficial morphology was more common on trunk (9/35) and upper extremities (7/35). Conclusions: IH resulting in AU differ in clinical features by anatomic site. Those in the diaper area are nearly always segmental with thin superficial component, whereas other sites tend to be localized, mixed, with thick superficial component. These distinct phenotypes may prove useful in the clinical setting for physicians to identify patterns of IH ulceration with increased risk of aggressive, persistent ulceration. [ABSTRACT FROM AUTHOR]

Metopic ridge (MeR) is a midline osseous forehead prominence resulting from physiologic closure of the underlying metopic suture. This mass‐like ridge can be mistaken for serious conditions such as a craniosynostosis or vascular anomaly, prompting concern and workup. We reviewed patients presenting for a forehead mass to Vascular Anomalies and Dermatology clinics and diagnosed with MeR to increase familiarity with this finding and to encourage MeR in the differential diagnosis of pediatric midline forehead masses. [ABSTRACT FROM AUTHOR]

Congenital hemangiomas (CHs) are unusual and diverse tumors distinguished from infantile hemangiomas by being largely developed at birth and glucose transporter (GLUT1)‐negative. We describe three infants who presented in utero or at birth with segmentally distributed vascular tumors that were GLUT1‐negative, had histology compatible with congenital hemangioma, and exhibited spontaneous clinical involution. One of the three patients had high‐output cardiac failure and was found to have a mutation in GNAQ (c.626A>c, p.Gln209Pro); another had high‐output cardiac failure, heterotaxy, and transient hematologic abnormalities and was found to have a mutation in GNA11 (c.626_627delinsCC, p.Gln209Pro). In addition to describing a novel segmental pattern of congenital hemangioma variant with genetic correlations, these cases illustrate the utility of targeted genetic testing to elucidate the exact mutation and thus classification of vascular tumors. [ABSTRACT FROM AUTHOR]

Objective: To quantify complications and rates of adverse events associated with surgical excision of hemangiomas in children using a national surgical database. Data source: Pediatric American College of Surgeons (ACS) National Surgical Quality Improvement Program® (ACS NSQIP®‐pediatric), years 2012‐2016. Methods: Subjects included children under 18 years with a postoperative diagnosis of International Classification of Diseases (ICD), 9th revision code: 228.00 and 228.01, or ICD 10 codes D18.00 and D18.01—hemangioma, any site and hemangioma, of skin and subcutaneous tissue. Results: A total of 1180 patients were included. The median age was 3.2 years (interquartile range [IQR] 1.2 to 6.4 years old). No occurrences of deep incisional surgical site infection, nerve injury, mortality, sepsis, septic shock, or blood stream infections were noted. In multivariate analysis, age ≤ 3 years and advanced American Society of Anesthesiologists class were significant predictors of adverse events (P = 0.035 and 0.001, respectively). Conclusions: For children with infantile hemangioma who fail to respond or have an incomplete response to medical therapy, surgical resection may be a relatively safe treatment alternative. Children older than 3 years of age with hemangiomas had less surgical adverse events than younger children. Overall, however, the surgical complications rates were low and deemed relatively minor. [ABSTRACT FROM AUTHOR]

Congenital hemangiomas are benign vascular tumors, categorized by their postnatal behavior as rapidly involuting, non‐involuting, or partially involuting. They are typically solitary, with a predilection for the head or limbs near a joint. We present two infants with small, multifocal congenital nonprogressive hemangiomas of the skin, one associated with hepatic and intracranial lesions, and another with an in utero intracranial hemorrhage and hydrocephalus. These cases further extend the differential diagnosis of congenital multifocal vascular lesions or "hemangiomatosis." [ABSTRACT FROM AUTHOR]