Kasabach-Merritt syndrome: clinical vs. surgical treatment

Kassabach-Merritt syndrome is a combination of capillary hemangioma and thrombocytopenia that predisposes to bleeding with petechiae, ecchymosis and spontaneous bruising. Treatment is generally started with corticosteroids, interferon alpha or chemotherapy. We present the case of a child (aged 1 year and 9 months) with a giant hemangioma, from the root of the thigh to the knee, and thrombocytopenia. Treatment was started with corticosteroids, without improvement, and then intra-tumor and cutaneous bleeding appeared spontaneously. The patient’s clinical condition precluded prescription of vincristine and interferon and emergency tumor resection was conducted because of extreme thrombocytopenia and bleeding. The child then began to develop sepsis with hypotension and ischemia of remnant tissues. This case presented a therapeutic challenge, which is the subject of this article.

Surgical interventions for treating pectus excavatum

Pectus excavatum is characterized by a depression of the anterior chest wall (sternum and lower costal cartilages) and is the most frequently occurring chest wall deformity. The prevalence ranges from 6.28 to 12 cases per 1000 around the world. Generally pectus excavatum is present at birth or is identified after a few weeks or months; however, sometimes it becomes evident only at puberty. The consequence of the condition on a individual’s life is variable, some live a normal life and others have physical and psychological symptoms such as: precordial pain after exercises; impairments of pulmonary and cardiac function; shyness and social isolation. For many years, sub-perichondrial resection of the costal cartilages, with or without transverse cuneiform osteotomy of the sternum and placement of a substernal support, called conventional surgery, was the most accepted option for surgical repair of these patients. From 1997 a new surgical repair called, minimally invasive surgery, became available. This less invasive surgical option consists of the retrosternal placement of a curved metal bar, without resections of the costal cartilages or sternum osteotomy, and is performed by videothoracoscopy. However, many aspects that relate to the benefits and harms of both techniques have not been defined. Objectives To evaluate the effectiveness and safety of the conventional surgery compared with minimally invasive surgery for treating people with pectus excavatum. Search methods With the aim of increasing the sensitivity of the search strategy we used only terms related to the individual’s condition (pectus excavatum); terms related to the interventions, outcomes and types of studies were not included. We searched the Cochrane Central Register of Controlled Trials (CENTRAL), PubMed, Embase, LILACS, and ICTPR. Additionally we searched yet reference lists of articles and conference proceedings. All searches were done without language restriction.