Search

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.] [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

16 Mentzel T, Reisshauer S, Rütten A, Hantschke M, Soares de Almeida LM, Kutzner H. Cutaneous clear cell myomelanocytic tumour: a new member of the growing family of perivascular epithelioid cell tumours (PEComas). Keywords: clear cell; mesenchymal; neoplasm; soft tissue EN clear cell mesenchymal neoplasm soft tissue 835 838 4 09/21/22 20221001 NES 221001 INTRODUCTION Distinctive dermal clear cell mesenchymal neoplasm (DDCCMN) is a very rare entity. The clinical presentation of a solitary, exophytic nodular tumor and histopathologic findings of large cells with abundant clear cytoplasm and characteristic staining pattern were compatible with distinctive dermal clear cell mesenchymal neoplasm. Primary clear cell sarcoma of the skin is rare, most often affecting tendons and aponeurosis of the limbs.7-11 Sharing similar pathologic properties, clear cell sarcoma is considered the soft tissue counterpart of malignant melanoma. [Extracted from the article]

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. 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. 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. 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. IV Retrospective cohort study. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]