Endovascular treatment of huge cervicofacial hemangioma complicated by Kasabach-Merritt syndrome

A 2-month-old girl with a huge cervicofacial hemangioma complicated by Kasabach-Merritt syndrome was presented. Two weeks corticosteroid treatment was ineffective, and deterioration of coagulation parameters and compromise of the airway and left vision due to huge hemangioma prompted use of endovascular treatment. Transfemoral arterial embolization using polyvinyl alcohol particles resulted in rapid clinical improvement. Endovascular treatment should be considered as an important therapeutic option for hemangioma complicated by Kasabach-Merritt syndrome.     

-Neonatal Kasabach-Merritt syndrome healed by complete surgical excision of the angioma-.

BACKGROUND: Kasabach-Merritt syndrome is an infrequent combination of a giant hemangioma and severe thrombocytopenia. This syndrome may be life-threatening. There are various but no definitive methods of treatment. We describe here a neonatal case cured by total excision of the tumor. CASE REPORT: A boy was admitted at birth with a giant hemangioma of the back with thrombocytopenia. Tumor compression, corticosteroids, acetylsalicylic acid and ticlopidine were ineffective. Total surgical excision was performed on day 51, resulting in complete recovery. CONCLUSION: Total surgical excision of a localized hemangioma in a non-functional area is probably the most appropriate and justified treatment for the Kasabach-Merritt syndrome.     

Successful Treatment of Kasabach-Merritt Syndrome with Prednisone and Epsilon-Aminocaproic Acid

The Kasabach-Merritt syndrome is characterized by thrombocytopenia and localized coagulopathy associated with a hemangioma. Most techniques applied to eradicate the tumor or accelerate its involution (surgery, radiation therapy, embolization) are invasive and require transfusion of large amounts of blood products. In some cases, medical treatment is the only alternative. Efficacy of steroids and antifibronolytic agents has already been described, but even this approach is associated with the administration of blood products. We report two cases of infants with Kasabach-Merritt syndrome associated with cardiac and hepatic hemangiomas. At admission, both had signs of cardiac failure. They were successfully treated with prednisone and epsilon-aminocaproic acid (EACA). Blood products were not required once the diagnosis was made. These observations have important implications for the management of patients with Kasabach-Merritt syndrome because they show that even in severe cases blood transfusions can be avoided by the use of prednisone and EACA.     

Development of Kasabach-Merritt syndrome following needle aspiration of a hemangioma.

A case of Kasabach-Merritt syndrome that developed following needle aspiration in an infant with hemangioma is presented. Enlargement of the tumor leading to severe respiratory distress accompanied by hemorrhages occurred within six to eight hours after this simple intervention. The symptoms continued despite repeated thrombocyte transfusions and prednisolone therapy. Radiotherapy and local intraarterial corticosteroid therapy were attempted and led to some improvement. It took approximately three months for the platelet count and one year for the clinical state to revert to normal. This case illustrates that minor trauma may lead to life-threatening symptoms in a young infant with congenital hemangioma.     

Radiotherapy in the management of Kasabach-Merritt syndrome: a case report

A 21-day-old infant with hemangioma was brought to the hospital with enlargement of the hemangioma of the left thigh and purple discoloration around umbilicus. This led to the diagnosis of Kasabach-Merritt syndrome. Initial treatment with corticosteroid failed. The patient under went radiotherapy that led success fully to improvement of both hemangioma and thrombocytopenia. After 2 years of follow-up, the patient has experienced long-term complications, including atrophy and growth retardation of the irradiated leg.     

RADIOTHERAPY IN THE MANAGEMENT OF KASABACH-MERRITT SYNDROME: A Case Report

A 21-day-old infant with hemangioma was brought to the hospital with enlargement of the hemangioma of the left thigh and purple discoloration around umbilicus. This led to the diagnosis of Kasabach-Merritt syndrome. Initial treatment with corticosteroid failed. The patient under went radiotherapy that led success fully to improvement of both hemangioma and thrombocytopenia. After 2 years of follow-up, the patient has experienced long-term complications, including atrophy and growth retardation of the irradiated leg.     

Kaposiform hemangioendothelioma associated with Kasabach-Merritt syndrome

OBJECTIVE: To describe a case of kaposiform hemangioendothelioma, the only malignant tumor of vascular origin specific of childhood.METHODS: We report a case of a 40-days-old girl who presented with a giant hemangioma of the face. Rapid enlargement of the tumor lead to laryngeal compression with severe respiratory distress. She had also a consumptive thrombocytopenic coagulopathy (Kasabach-Merritt syndrome).RESULTS: She was admitted to the pediatric intensive care unit of the Hospital das Clínicas da Universidade Estadual de Campinas and mechanical ventilation was introduced. A ten day course of dexamethasone was only partially effective. We then started alpha-2a interferon at a dose of 1.8 million units/m(2)/day by subcutaneous route, but she died four days after the onset of this therapy. The microscopic features showed a kaposiform hemangioendothelioma.CONCLUSION: We discuss this unusual fatal evolution of a rapidly growing hemangioma and its hematological complications.     

Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia

The authors report Kasabach-Merritt syndrome (KMS) in a patient with thrombocytopenia and splenic hemangioma. A 13-month-old boy with a history of anemia, thrombocytopenia, and abdominal mass was admitted to the hospital. The scintigraphic studies showed that a large mass contiguous to the spleen was responsible for the platelet uptake. After partial splenectomy, the platelet count returned to normal. This report of KMS in a child with splenic hemangioma suggests that the scintigraphic studies are mandatory to confirm diagnosis. Indium-111-labeled platelets are useful in identifying hemangiomatous sequestration of platelets in patients with thrombocytopenia.     

Kasabach-Merritt综合征5例临床分析

目的：分析5例婴儿Kasabach-Merritt综合征（KMS）临床数据及转归。方法1993年6月-2012年2月期间我科收治的5例KMS患儿（男4例,女1例）,回顾性收集上述病例的一般资料、血常规及出凝血检查、影像学和病理学检查结果并加以分析。结果5例患儿中3例为皮下型巨大血管瘤,2例为内脏型巨大血管瘤,除1例首诊时确诊为KMS外,其余4例首诊时均存在误诊。血液检查结果提示所有患儿均存在不同程度消耗性凝血功能障碍。所有患者均给予肾上腺糖皮质激素治疗及对症支持治疗,对激素敏感者3例,激素敏感率为60％,手术治疗1例。经治疗存活4例,死亡1例,死亡原因为严重DIC伴多脏器功能衰竭。结论应提高对KMS的认识以减少误诊发生率,对婴儿难治性血小板减少伴凝血功能异常者需注意除外KMS ,不同患儿对药物的治疗反应存在个体化差异,故应针对不同情况采用个体化综合治疗方法,以提高临床诊疗效果。     

A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging

Kaposiform hemangioendothelioma (KH) is an endothelial-derived spindle cell neoplasm often associated with Kasabach-Merritt syndrome. Most cases arise in infancy and childhood and are soft-tissue tumors. The tumor displays an appearance between capillary hemangioma and Kaposi's sarcoma. We report a case of KH in a 1-year-old girl involving a mass that showed abnormal enhancement of soft tissue superficial to the right temporal bone with partial destruction of the temporal bone, the temporomandibular joint, mandibular condyle, and occipital bone. The physical finding of a discolored mass led clinicians to consider a hemangiomatous lesion, whereas the radiological picture suggested a more aggressive diagnosis of rhabdomyosarcoma and aggressive fibromatosis. Received: 8 March 2001/Accepted: 6 August 2001     

婴儿突发皮肤紫癜

患儿,男,2个月,因发现皮肤紫癜、血小板减少2d就诊,体查发现背部紫红色肿块,软组织彩超提示组织内丰富血流信号,骨髓细胞学提示巨核细胞增多,经激素及丙种球蛋白治疗后血小板迅速上升并维持在正常;同时口服普萘洛尔,随访4个月,患儿背部瘤体明显减小。血管瘤和免疫性血小板减少症诊断明确。对于血管瘤伴血小板减少的患者,应增强对血管瘤血小板减少症 （KMS）的认识,拓展思路,理顺血小板减少与血管瘤的关系,警惕其它原因所致的血小板减少。     

Hydrops Fetalis Caused by Fetal Kasabach-Merritt Syndrome

There have been only 2 previous reports of nonimmunologic hydrops fetalis (NIHF) caused by fetal Kasabach-Merritt syndrome, both of which were pathological studies. This is the first clinical case report of NIHF due to fetal Kasabach-Merritt syndrome that was prenatally diagnosed by sonography, computerized tomography, and percutaneous umbilical blood sampling.     

Kasabach-Merritt syndrome in children]

Kasabach-Merritt syndrome is a combination of thrombocytopenia, intravascular coagulation, and a rapid increase in the size of an angioma. Anemia and disseminated intravascular coagulation may develop. This infrequent syndrome is severe and may be life-threatening. Pathophysiologic mechanisms underlying the condition are incompletely understood and, consequently, many different treatments are used, including systemic corticosteroids, compression, embolization, antifibrinolytic agents, platelet aggregation inhibitors, irradiation, and others. From findings in eight personal cases, the authors review clinical and biological features, pathophysiologic hypotheses and therapeutic strategies.     

Successful treatment by coil embolization for infantile hemangioma with Kasabach–Meritt syndrome of newborn

Infantile hemangioma (IH) is the most common tumor of infancy, and it sometimes associated with Kasabach–Meritt syndrome (KMS) characterized by anemia, intraperitoneal hemorrhage secondary to rupture, coagulopathy, jaundice, and vascular malformations involving the brain, skin, gut, and other organs. Here, we report two newborn patients having IH with KMS at birth. The first patient had a giant hemangioma in the liver, which was successfully treated with i.v. corticosteroid and coil embolization. The second patient had a large hemangioma of the right axillary region, which was also successfully treated with i.v. corticosteroid, beta‐blocker, coil embolization and local irradiation. All symptoms were controlled without any side‐effects in both patients. According to these findings, combination therapy including coil embolization and corticosteroid is effective for IH patients with KMS. The indications for and timing of coil embolization should be determined further cases have been accumulated.     

Tranexamic acid in the treatment of Kasabach-Merritt syndrome in infants

Kasabach-Merritt syndrome (thrombocytopenia, consumption coagulopathy, and, occasionally, microangiopathic hemolysis) is an infrequent but often lethal complication of giant capillary hemangiomas in the infant. We report the clinical course of an infant who came to us with this syndrome shortly after birth. She was successfully treated with transfusions of blood products, high-dose oral prednisone, and a fibrinolytic inhibitor, tranexamic acid. No complications of the treatment occurred. The hematological abnormalities resolved completely by 2 years of age. Although prednisone may have been necessary for stabilization and involution of the hemangioma, the addition of tranexamic acid allowed earlier tapering of the steroid therapy and fewer transfusions. Tranexamic acid was extremely well tolerated in this newborn.     

Giant cavernous haemangioma with Kasabach-Merritt syndrome: a case report and review

We report the case of a 4-month-old boy presenting with a giant cutaneous haemangioma complicated by Kasabach-Merritt syndrome (KMS) with severe thrombocytopenia. After poor response to corticosteroid therapy and subsequent treatment with interferon alpha-2a, radiotherapy led to tumour regression and resolution of the disseminated intravascular coagulopathy over a 14-month period of follow up. Whereas the various available treatment options are reviewed and discussed in this article, the therapy of choice should be chosen individually. CONCLUSION: to date prospective randomised and controlled trials are required to investigate the optimal management of patients with Kasabach-Merritt syndrome.     

16例新生儿卡梅综合征临床分析

目的 分析总结新生儿卡梅综合征（Kasabach-Merritt syndrome,KMS）的临床特点、治疗及预后,为优化该病的诊断及治疗提供参考依据。方法 回顾性分析安徽医科大学附属省儿童医院2016年1月至2020年12月收治的16例KMS新生儿的住院及随访资料。结果 16例KMS患儿中,男13例（81%）,女3例（19%）,入院年龄为1 h至10 d。13例（81%）为皮肤血管瘤（头面部2例、躯干5例、四肢6例）,3例（19%）为肝脏血管瘤。10例（62%）临床表现以出血倾向、全身散在瘀点瘀斑为主。16例患儿均有不同程度的血小板减少及凝血功能异常。所有患儿入院后均采取糖皮质激素保守治疗,7例（44%）有效,其中复发4例。糖皮质激素治疗无效患儿中3例接受西罗莫司治疗,1例治疗4周后瘤体缩小58.8%,血小板计数及凝血功能恢复正常;2例瘤体无明显缩小,血小板计数无明显回升,联合博来霉素动脉栓塞治疗4周后瘤体缩小（43.7±0.4）%,血小板计数及凝血功能恢复正常。4例单独接受博来霉素动脉栓塞治疗4周后瘤体缩小（52.0±3.4）%,血小板计数及凝血功能恢复正常。2例接受外科钝性+锐性剥离切除术,术中切除全部瘤体,术后无感染及复发,血小板计数及凝血功能恢复正常,1例术后病理结果为卡波西样血管内皮瘤。结论 KMS具有特征性临床表现、组织病理特征及实验室检查结果。对激素反应不敏感的KMS通过动脉栓塞和西罗莫司治疗可取得较好的疗效。     

Embolization therapy in the management of infantile hemangioma with Kasabach Merritt syndrome

A case is presented in which a giant infantile hemangioma with thrombocytopenia is managed successfully by serial transcatheter embolization.     

Successful treatment with interferon alfa in infiltrating angiolipoma: a case presenting with Kasabach-Merritt syndrome.

We report a case of infiltrating angiolipoma who presented with a large mass on the shoulder and the signs of Kasabach-Merritt syndrome. She improved dramatically within one month and the mass completely disappeared after six months of interferon treatment.     

Successful treatment with interferon alfa in infiltrating angiolipoma: a case presenting with Kasabach-Merritt syndrome

We report a case of infiltrating angiolipoma who presented with a large mass on the shoulder and the signs of Kasabach-Merritt syndrome. She improved dramatically within one month and the mass completely disappeared after six months of interferon treatment.