Transarterial Embolization in Neonatal Kasabach-Merritt Syndrome.

Background: Kasabach-Merritt syndrome (KMS) is characterized by large hemangiomas and persistent thrombocytopenia, which may result in visceral hemorrhage and disseminated intravascular coagulation. This study aimed to evaluate the value of transarterial embolization (TAE) in neonatal KMS patients. Patients and Methods: The clinical course of 11 neonates with KMS who underwent TAE in the Department of Neonatology, Anhui Provincal Children's Hospital, Anhui Medical University, China, were reviewed retrospectively. Results: Eleven neonates with KMS (nine male and two female) were admitted to our hospital between the age of 1 h and 6 days. All were born with progressively enlarged hemangiomas and persistent thrombocytopenia. The largest lesion had its maximum size reached at 15 × 8 × 8 cm. Eight patients had cutaneous hemangiomas (1 right face, one oropharynx, one left upper arm, two back, one left lumbar, one right lower leg, and one right thigh), and three patients had liver hemangiomas. All 11 patients underwent TAE. Nine patients underwent two TAEs, and two patients underwent only one embolization procedure. They all obtained >80% devascularization of their lesions without a major complication. The platelet count increased at 2-5 days after treatment and reached normal count and coagulation profile at 18-28 days after the TAE. Conclusions: TAE is a safe and effective alternative therapy for neonatal KMS patients.

Hemangioma hepático congénito gigante: síndrome de Kasabach-Merritt / Giant Congenital Liver Hemangioma: Kasabach Merritt Syndrome

Resumen Los hemangiomas son las proliferaciones más frecuentes en la edad pediátrica, y el hemangioma hepático es de los más comunes entre los viscerales. El diagnóstico precoz es importante para orientar el tratamiento. Se presenta el caso de un neonato con compromiso ventilatorio secundario al efecto de una masa gigante, que ecográficamente correspondió a un hemangioma hepático. Se realizó resección quirúrgica y, ulteriormente, fue enviada a patología, que confirmó el diagnóstico.

Abstract Hemangiomas are the most frequent proliferations in children, and liver hemangiomas are the most common among those in the viscera. Early diagnosis to focus treatment is important. We present the case of a neonate with compromised breathing secondary to the effect of a giant mass that echographically appeared to be a hepatic hemangioma. Surgical resection and subsequent pathology confirmed the diagnosis.

Successful Management of Kaposiform Hemangioendothelioma with Long-Term Sirolimus Treatment: a Case Report and Review of the Literature.

BACKGROUND: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour of the infancy and the first decade of life. It is locally aggressive and potentially life threatening when associated with consumptive coagulopathy, known as Kasabach-Merritt syndrome (KMS). No consensus or guideline for the therapy has been reached because of the lack of prospective trials, and the different standard care suggestions are based on retrospective case series. CASE REPORT: We report the case of a 9-month-old male with KHE and KMS in which the initial response, obtained with prednisone and vincristine, was subsequently consolidated and strengthened by long-term treatment with sirolimus, a mTOR inhibitor. A summary of the published data is presented as well. CONCLUSIONS: The inhibition of mTOR pathway represents the most important therapeutic innovation introduced in the last few years for KHE. Our case shows the effectiveness and good tolerance of long-term therapy with sirolimus.

Angioma en penacho / Tufted angioma

El Angioma en Penacho es un raro tumor vascular de la infancia, que comparte características con el hemangioendotelioma kaposiforme, teniendo ambos posibilidad de desarrollar Síndrome de Kasabach-Merrit. Presentamos una paciente portadora de un angioma en penacho desde el nacimiento, que hemos seguido por 13 años: clínicamente, con estudios de imágenes y biopsias. El caso presenta similitudes con la literatura, con algunas interesantes peculiaridades en su evolución. Consideramos que por el comportamiento clínico e histológico, el Angioma en Penacho es parte de la misma enfermedad que el Hemangioendotelioma Kaposiforme.

Tufted angioma is a rare vascular tumor of childhood that shares features with the Kaposiform hemangioendothelioma, having both tumors the possibility of developing Kasabach-Merrit phenomenon. We report the case of a patient with a tufted angioma since birth, which we have followed for 13 years: clinically, with imaging studies and biopsies. The case presents similarities with literature, with some interesting peculiarities in its evolution. We consider that because of the clinical and histological behavior, Tufted angioma is a part of the same disease that Kaposiform hemangioendothelioma.

Intrathoracic hemangioendothelioma presenting as refractory unilateral pleural effusion and thrombocytopenia

A variety of diseases are associated with the development of unilateral pleural effusion. Although unilateral pleural effusion is common, refractory unilateral pleural effusion is rare. It is important to make an accurate diagnosis using proper diagnostic tools. Thrombocytopenia is one of the rare conditions occurring from various diseases such as severe infection or autoimmune diseases. It can be life-threatening if accurate diagnosis and treatment are delayed and be a clue to accurate diagnosis in differential diagnosis from refractory pleural effusion. Kasabach-Merrit syndrome (KMS) is often accompanied by extensive vascular tumors and characterized by consumptive coagulopathy with profound thrombocytopenia. It is also important to have a high index of suspicion for the diagnosis. We report a case of KMS in a 2-month-old female infant with a vascular tumor on her left intrathoracic cage, who had presented refractory unilateral pleural effusion and thrombocytopenia. Initially, the patient was diagnosed as having complications of severe infection, and a chest tube was inserted for aggressive treatment. However, her unilateral pleural effusion persisted, and thrombocytopenia and hypofibrinogenemia were refractory. Chest imaging revealed an infiltrating large vascular tumor involving the cardiac border, diaphragm, and chest wall. The patient’s unilateral pleural effusion was misidentified as an infectious condition at the initial stage. As a result of the ultrasonography-guided biopsy, it was revealed to be Kaposiform hemangioendothelioma. The patient was cured after treatment for KMS.

A rare case of kaposiform hemangioendothelioma presenting as intussusception in a 4-month-old child without Kasabach-Merrit syndrome: A case report.

We report the identification of Kaposiform hemangioendothelioma of the cecum presenting as colocolic intussusception in a 4-month-old boy without Kasabach-Merrit syndrome. To our knowledge this is the first reported case in the literature of such a presentation.