Kasabach-Merritt syndrome

el-Dessouky, M; Azmy, Amir; Raine, P.A.M.; Young, D.G.

doi:10.1016/s0022-3468(88)80135-0

Cited by 111 publications

(59 citation statements)

References 30 publications

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“…Platelet trapping in the haemangioma is thought to result in activation of platelets and the clotting cascade, resulting in a consumptive coagulopathy [22]. The mortality rate of Kasabach-Merritt syndrome approaches 30% [23]. …”

Section: Cavernous Haemangioma: Presentation and Natural Historymentioning

confidence: 99%

Giant Haemangioma of the Liver: Observation or Resection?

Duxbury¹,

Garden²

2010

Dig Surg

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Background: Haemangiomata are the most frequent benign solid liver lesion. The management of giant (≧5 cm) haemangiomata of the liver remains controversial. Methods: A search of relevant peer-reviewed literature was conducted using PubMed and original articles were reviewed. Results and Conclusions: The vast majority of giant haemangiomata remain asymptomatic and have a benign and uncomplicated natural history. Decisions regarding the optimal management of giant haemangiomata depend on a high level of confidence in diagnostic imaging. Diagnostic biopsy to differentiate giant haemangiomata from malignant lesions should be discouraged. Despite limitations and alternative modalities, surgery remains the only consistently effective curative treatment for giant haemangiomata. Surgery is not generally justified to prevent complications in asymptomatic patients. Principal indications for the surgical management of giant haemangiomata include established complications, incapacitating symptoms and uncertainty of diagnosis. Patients should only be selected for surgery based on a careful assessment of risks and benefits of intervention.

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Section: Cavernous Haemangioma: Presentation and Natural Historymentioning

confidence: 99%

Giant Haemangioma of the Liver: Observation or Resection?

Duxbury¹,

Garden²

2010

Dig Surg

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“…Since carcinomas are by far morecommon than hemangiomatosis, this disease has occasionally been diagnosed as metastatic cancer (16). It was also difficult in the present case to differentiate the splenic low density nodules from metastases of the germ cell tumor, because; 1) they developed in size and number after treatment of the primary tumor, 2) teratoma, which could be a component of the germ cell tumor in this case, mayform cystic lesions without elevation of tumor markers, and 3) hemangiomas usually grow in infancy but regress slowly or stabilize thereafter (15 heparin ( 17). In the current case, no clear explanation was given for the prolonged thrombocytopenia, because it mayhave been partially attributable to all of the following: 1) visceral hemangiomatosis, 2) DIC, 3) the cytotoxic therapy, and 4) production of anti-platelet antibody induced by massive platelet transfusion.…”

Section: Case Reportmentioning

confidence: 74%

Mediastinal Germ Cell Tumor Complicated by Visceral Hemangiomatosis.

et al. 1998

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This report describes an extremely rare combination of mediastinal germcell tumor and visceral hemangiomatosis in a 17-year-old boy who initially presented with chest pain and dyspnea. He was treated with chemotherapy consisting of cisplatin, cyclophosphamide, bleomycin, vinblastine, and dactinomycin followed by surgery. Multiple low-density nodules developed in the spleen three weeks later, suggesting metastases from the primary tumor, but the resected specimen showed cavernous hemangiomaswithin the splenic parenchyma. The patient died of recurrence of germ cell tumor 19 months after the initial treatment. Postmortem examination disclosed multiple hemangiomasin the lung and liver similar to those in the spleen.

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“…The authors chose to perform a spinal, and not an epidural, anesthetic to minimize potential injury to small, occult hemangiomas within the soft tissue or spinal canal that had not been previously identified in the MRI study. Kasabach-Merritt coagulopathy occurs in fewer than 0.3% of neonates with hemangiomatous disease, but the disorder may be associated with a mortality rate approaching 40% from uncontrollable bleeding [21]. Notably, the development of Kasabach-Merritt coagulopathy cannot be reliably predicted based on the relative size and location of neonatal hemangiomas during the neonatal period [21,22].…”

Section: Discussionmentioning

confidence: 99%

Successful use of spinal anesthesia in a patient with severe Klippel–Trénaunay syndrome associated with upper airway abnormalities and chronic Kasabach–Merritt coagulopathy

Holak

Pagel

2010

J Anesth

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Klippel-Trénaunay syndrome is a rare disorder characterized by the triad of capillary or cavernous hemangiomas, venous varicosities or malformations, and soft tissue or bone hypertrophy. Neuraxial anesthesia in patients with Klippel-Trénaunay syndrome has been infrequently described and has not been previously reported when accompanied by consumptive coagulopathy with thrombocytopenia (Kasabach-Merritt syndrome). The authors describe their clinical management of a 23 year-old woman with Klippel-Trénaunay syndrome who presented for elective total knee arthroplasty. Her past medical history was notable for chronic Kasabach-Merritt syndrome, hypersplenism with pancytopenia, and intermittent lower gastrointestinal bleeding resulting from colonic hemangiomas. The physical examination revealed several large cavernous hemangiomas located on her right face, neck, chest, arm, and leg. No hemangiomas were noted within the dermatomal levels innervated by the upper lumbar spine. The neck hemangioma was very large and filled with blood when the patient assumed a supine position, making it almost impossible for her to breathe. The oropharynx revealed markedly hypertrophied soft tissue, pharyngeal, and hypopharyngeal hemangiomas, and a Mallampati class IV airway. Spinal and epidural hemangiomas were excluded based on a magnetic resonance imaging study before surgery. Kasabach-Merritt coagulopathy was corrected preoperatively by administration of cryoprecipitate. These interventions allowed the authors to safely perform a spinal anesthetic for the operation. The current case illustrates that major conduction anesthesia may be safely performed in patients with Klippel-Trénaunay disease provided that preoperative imaging studies exclude neurovascular involvement and coexisting coagulopathy is appropriately corrected.

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Kasabach-Merritt syndrome

Cited by 111 publications

References 30 publications

Giant Haemangioma of the Liver: Observation or Resection?

Giant Haemangioma of the Liver: Observation or Resection?

Mediastinal Germ Cell Tumor Complicated by Visceral Hemangiomatosis.

Successful use of spinal anesthesia in a patient with severe Klippel–Trénaunay syndrome associated with upper airway abnormalities and chronic Kasabach–Merritt coagulopathy

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