婴儿血管瘤并发卡-曼综合征的血管栓塞术治疗

目的探讨婴儿血管瘤合并消耗性凝血病(卡-曼综合征)血管栓塞术的临床应用。方法以选择性栓塞方法,对4例血管瘤合并卡-曼综合征的患儿实施血管瘤的病理血管床和供血动脉的栓塞。结果3例患儿接受栓塞治疗后1～2周内血管瘤明显缩小,局部软组织充血、肿胀迅速缓解并局限,血小板快速回升并稳定在正常水平,卡-曼综合征获得有效控制。1例于栓塞术后2周血小板再次快速回落,经补充栓塞治疗后病情得到缓解。结论血管栓塞术对血管瘤合并消耗性凝血病有着较为理想的疗效,对于外科手术治疗困难合并有卡-曼综合征的血管瘤患儿,倡导给予及时的血管栓塞术治疗。     

Successful management with interferon alpha-2a after prednisone therapy failure in an infant with a giant cavernous hemangioma

A giant cavernous hemangioma of the left arm with severe thrombocytopenia and consumptive coagulopathy was observed in a neonate. Initial treatment with prednisone, platelet transfusions, and clotting replacement failed to control the bleedings. The child was then treated with daily subcutaneous infusions of interferon alpha-2a. Coagulopathy rapidly improved and transfusions were drastically reduced. The hemangioma regressed progressively and disappeared after 4 months of treatment. Med. Pediatr. Oncol. 28:213–215 © 1997 Wiley-Liss, Inc.     

Diffuse cavernous hemangioma of the spleen with Kasabach-Merritt syndrome misdiagnosed as idiopathic thrombocytopenia in a child

Background  Most cavernous hemangiomas in the spleen are small lesions that are found incidentally and patients usually present with no symptoms. Imaging is able to detect the lesions that are considered as diagnostic evidence. But some patients with diffuse cavernous hemangioma may present with anemia, thrombocytopenia, coagulopathy and bleeding, which might be misdiagnosed as idiopathic thrombocytopenia with disseminated intravascular coagulation (DIC). Splenectomy is the most effective therapy for diffuse cavernous hemangiomas with symptoms. Methods  The history, imaging results, pathologic findings, diagnosis and treatment of a 34-month-old boy with severe petechiae were reviewed. Results  The boy was diagnosed as having refractory idiopathic thrombocytopenia (ITP) because of low platelet count and bleeding at a local hospital. He had no response to a full-dose of corticosteroid and a high-dose of immunoglobulin (2 g/kg). Huge splenomegaly and DIC were found after 7 months. Diffuse cavernous hemangioma of the spleen was highly suspected, but it was not confirmed by B ultrasound, enhanced CT or MRI. DIC and bleeding were solved by low molecular weight heparin, supplement of fibrinogen and prothrombin complex. A diffuse cavernous hemangioma involving the whole spleen was confirmed pathologically following a successful splenectomy. The boy recovered completely without any complication after the operation. Conclusions  Diffuse cavernous hemangioma of the spleen should be differentiated from ITP associated with splenomegaly. Radiological and overall physical examination should be emphasized for refractory ITP cases.     

Consumptive Coagulopathy in Utero Associated with Multiple Vascular Malformations

We report a postmature male infant who died at age 2 h of asphyxiating pulmonary hemorrhage. Autopsy found edema, serous ascites, and vascular malformations within myocardium, lung, mediastinal soft tissue, thoracic-wall skeletal muscle, spleen, thyroid and adrenal glands, and pancreas. The pulmonary malformations had arteriovenous features, while those elsewhere were predominantly capillary; many of the latter contained fibrinous thrombi. Circulating nucleated erythrocytes, siderosis of proximal renal tubular epithelium, and intrahepatocytic and intracanalicular cholestasis were present. We believe that these findings represent an unusual instance of hemangioma-associated consumptive coagulopathy and intravascular hemolysis (Kasabach-Merritt syndrome) of antenatal onset, associated with features suggestive of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu 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.     

婴儿血管瘤合并PHACE综合征的发病机制与诊治进展

婴儿血管瘤(IH)是儿童时期最常见的良性肿瘤,以出生后迅速增长和后期缓慢自然消退为其典型的临床特征。部分面部节段型IH患者可合并PHACE综合征。PHACE综合征为累及全身多个系统的血管神经、血管皮肤病变,常常造成机体结构以及功能损害。近期的研究表明,PHACE综合征发病的可能机制主要包括:缺氧假说、中胚层血管内皮细胞异常假说、基因异常假说、胎盘绒毛膜绒毛间质干细胞假说。目前,IH合并PHACE综合征的治疗药物主要包括β受体阻滞剂、糖皮质激素、m TOR受体抑制剂。本文主要针对IH合并PHACE综合征的发病机制、诊断以及治疗进展进行综述,以期为该病的临床诊治提供帮助。     

Sudden spinal hemorrhage in a pediatric case with total body irradiation‐induced cavernous hemangioma

Compared to cerebral radiation‐induced cavernous hemangiomas (RICHs), little is known about intraspinal RICHs. A 13‐year‐old male suddenly developed symptomatic spinal hemorrhage eight years after hematopoietic stem cell transplantation using a total body irradiation (TBI) based myeloablative regimen. A solitary small hemangioma was detected on follow‐up T2 star weighted magnetic resonance imaging of the spine. His neurological symptoms gradually improved with supportive treatment and rehabilitation, although he experienced rebleeding 2 years later. Intraspinal RICH is very rare but should be recognized as a possible late adverse effect in pediatric patients who received TBI.     

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.