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Disseminated Hemangiomatosis


Presentation

  • With a central motif of the biologic dichotomy of vascular tumors and vascular malformations, this book is organized into chapters which address clinical presentation, diagnostic imaging, molecular genetics, pathogenesis, histopathology, and management[books.google.de]
  • It present clinically as slowly growing, solid, firmly attached gingival mass sometimes arising between teeth and sometimes displacing teeth.[slideshare.net]
  • Topic: Digital Learning Presentation Type: Rapid-Fire Presentation Submission Type: Single Presentation Building: Mermaid Room: Room 3 - Upper River Room Date: 2013-09-23 04:00 PM – 06:00 PM Last modified: 2013-09-25 If you are the presenter of this[medicine20congress.com]
  • When present, symptoms may be due to hemorrhage from spontaneous or traumatic rupture of a hemangioma. A few patients present with splenomegaly. Splenic hemangiomas may be single or multiple and usually present as a hyperechoic mass in the spleen.[sonoworld.com]
Easy Bruising
  • Kasabach-Merritt Syndrome A patient developing sudden enlargement and firmness in a preexisting large hemangioma, with easy bruising and petechiae, should be suspected of having Kasabach-Merritt syndrome, a coagulopathy characterized by thrombocytopenia[aafp.org]
Splenectomy
  • Hone and expand your surgical skills by watching videos of minimally invasive procedures for recto urethral fistula, biliary atresia, laparoscopic splenectomy, uterine horn, and more.[books.google.de]
  • In such a scenario, splenectomy is often necessary. Ultrasound is often the initial modality used to diagnose splenic lesions, and when in doubt, a contrast enhanced CT or MRI scan is performed to characterize the lesions further.[sonoworld.com]
  • […] diet, chemotherapy, sclerotherapy, transplant; Abdominal [ edit ] interferon alpha 2b, sclerotherapy, resection, percutaneous drainage, Denver shunt, Total Parenteral Nutrition (TPN), medium chain triglyceride (MCT) and high protein diet, transplant, splenectomy[en.wikipedia.org]
  • […] septicemia 敗血症 serosa漿膜 sexual transmitted disease (STD)性病 short bowel syndrome短腸症候群 signet ring cell carcinoma印環細胞癌 sinusoid類洞 small intestine小腸 spasm of the esophagus食道痙攣 sphincter of Oddi (SO)オッジ括約筋(オッジ筋) splanchnic nerve内臓神経 splanchnic plexus内臓神経叢 splenectomy[tokyo-med.ac.jp]
Pathologist
  • Vascular Anomalies: Hemangiomas and Malformations is a comprehensive and interdisciplinary textbook ideal for dermatologists, interventional radiologists, surgical specialists, ophthalmologists, pathologists, geneticists, pediatricians, hematologic-oncologists[books.google.de]
  • “Arthur Purdy Stout (1885-1967), a pioneer of surgical pathology: a survey of his Notes on the Education of an “Oncological” Surgical Pathologist..” Annals of Diagnostic Pathology 2.4 (1998): 271-9. PubMed.gov. Web. 2 Sep 2011. 7. WRIGHT, I.S.[lgdalliance.org]
  • […] cartilage, fat, muscles or blood vessels ** Sarcomas show the cytological malignant features of cellular and nuclear pleomorphism, mitotic figures and Hyperchromatism  Other lesions of fibrous tissue:  Fibrous histiocytoma  There is disagreement amongst pathologists[slideshare.net]
Surgical Procedure
  • procedure is planned Fresh frozen plasma if PT or APTT 1.5 times the normal value Cryoprecipitate if fibrinogen 150 mg/dL Packed RBCs if: If acute traumatic coagulopathy transfuse packed RBC, FFP, and platelet concentrate in a 1:1:1 ratio Synthetic protease[amboss.com]
  • Cartilaginous destruction can also occur at the nasal alae and the pinna It is important to realize that the bony distortion will magnify the disfigurement and further more it may require multiple complicated surgical procedures to be corrected.[hemangioma.gr]
Chills
  • Acute side effects, which are reversible, include fever, chills, arthralgias and retinal vasculopathy. Fatigue, leukopenia, anemia, thrombocytopenia, nausea, vomiting, elevated liver transaminase levels and neurotoxicity may occur with chronic use.[aafp.org]
Gagging
  • A moderately increased urinary excretion of glycosaminoglycans (GAG) was found. The main GAG fraction excreted was tentatively identified as a heparan sulphate.[ncbi.nlm.nih.gov]
  • -Most common location of the stalk is fossa ovalis -stellate character of cells, cytologically regular cells, swimming in: -Collagen and gelatinous, big amounts of ECM (GAGs) Connective tissue tumors -tumors of mucoid tissue -myxosarcoma - malignant variant[quizlet.com]
Vascular Disease
  • OBJECTIVE: We hypothesized that many cases reported as diffuse neonatal hemangiomatosis did not have infantile hemangiomas (IH), but represented more recently described neonatal vascular diseases.[ncbi.nlm.nih.gov]
Angiomatosis
  • Cystic angiomatosis is a nonaggressive form of skeletal angiomatosis with multifocal involvement, predominantly affecting the trunk bones. The imaging modalities gave the diagnosis of cystic angiomatosis of humerus showing multicystic lytic areas.[ijpmonline.org]
  • Angiomatosis: a vascular malformation of infancy and childhood. Pathology 1987 ; 19 : 377 – 382. 56. Rao, VK, Weiss, SW. Angiomatosis of soft tissue: an analysis of the histological features and clinical outcome in 51 cases.[cambridge.org]
  • Angiomatosis of bone and soft tissue: a spectrum of disease from diffuse lymphangiomatosis to vanishing bone disease in young patients. Clin Radiol. 2001 Mar;56(3):184-90.[lgdalliance.org]
  • Differential Diagnosis- Infection (Bacillary angiomatosis); Tumors (Glomus tumor; Tufted angioma; Kaposiform hemangio-endothelioma; Angioendothelioma; Sarcoma); Congenital (Verrucous hemangioma); Miscellaneous (Pyogenic granuloma; Angiokeratomas).[carouselprotocols.com]
  • See also bovine cutaneous angiomatosis, hemangiomatosis, telangiectasia. disseminated cavernous hemangioma multiple, small hemangiomatous tumors found in skin and internal organs; in calves and pigs rarely. verrucous hemangioma hemangioma in superficial[medical-dictionary.thefreedictionary.com]
Hyperpigmentation
  • Glomeruloid Hemangioma Polyneuropathy Organomegaly (hepato, lymphadeno) Endocrinopathy (impotence, gynecomastia, amenorrhea, hypothyroid) M protein (a/w castlesmans disease) Skin changes (hemangiomas hyperpigmentation skin thickening)[cram.com]
  • After 1 to 2 weeks, the eruption usually desquamates leaving a temporary hyperpigmentation.[bobbybukamd.com]
Skin Thickening
  • Glomeruloid Hemangioma Polyneuropathy Organomegaly (hepato, lymphadeno) Endocrinopathy (impotence, gynecomastia, amenorrhea, hypothyroid) M protein (a/w castlesmans disease) Skin changes (hemangiomas hyperpigmentation skin thickening)[cram.com]
Myopathy
  • Side effects include nausea, vomiting, diarrhea and, in the case of aminocaproic acid, rare instances of renal insufficiency and myopathy. The Authors show all author info FERN A.[aafp.org]
  • […] fingers, photosensitive dermatitis) and two or more of the following: progressive symmetrical proximal muscle weakness, inflammatory and atrophic muscle histopathology, elevated muscle-derived enzymes, and EMG changes or MRI appearance of inflammatory myopathy[bobbybukamd.com]
Amenorrhea
  • Glomeruloid Hemangioma Polyneuropathy Organomegaly (hepato, lymphadeno) Endocrinopathy (impotence, gynecomastia, amenorrhea, hypothyroid) M protein (a/w castlesmans disease) Skin changes (hemangiomas hyperpigmentation skin thickening)[cram.com]
Renal Insufficiency
  • Side effects include nausea, vomiting, diarrhea and, in the case of aminocaproic acid, rare instances of renal insufficiency and myopathy. The Authors show all author info FERN A.[aafp.org]
Polyneuropathy
  • Glomeruloid Hemangioma Polyneuropathy Organomegaly (hepato, lymphadeno) Endocrinopathy (impotence, gynecomastia, amenorrhea, hypothyroid) M protein (a/w castlesmans disease) Skin changes (hemangiomas hyperpigmentation skin thickening)[cram.com]

Workup

  • The diagnosis is very difficult and usually requires an extensive workup. In a review of the Japanese literature, preoperative diagnosis was obtained in only 24.1% of patients [ 11 ].[em-consulte.com]
  • Great advances have taken place in the nomenclature, pathogenesis, immunohistochemistry, diagnostic workup and management of hemangiomas in the recent years.[ijdvl.com]
  • The diagnosis essentially is one of exclusion and must be based on combined clinical, radiological and histopathological findings. 9 X-rays, CT, MRI, ultrasound, and nuclear medicine (bone scans) are all important tools in the diagnostic workup and surgical[lgdalliance.org]
  • These patients require rapid diagnosis and treatment to prevent permanent complications (see Presentation, Workup, and Treatment.[emedicine.medscape.com]

Treatment

  • This era was followed by improved environmental factors which operate to produce methods of medical treatment, introduction congenital cardiac malformations.[books.google.de]
  • Treatment In general, the goals of hemangioma treatment are to prevent loss of life or function, and to prevent scarring, either before or as a result of therapy.[aafp.org]
  • What is the treatment for infantile proliferative haemangioma ? Propranolol is the treatment of choice for troublesome haemangiomas.[dermnetnz.org]
  • An improvement in the coagulopathy was observed after 10 days of treatment with interferon alfa-2a alone. After six months of treatment there was 95 percent regression of the lesion, and treatment was stopped.[nejm.org]

Prognosis

  •  Mixed cellularity  Nodular sclerosis  Lymphocyte depletion  Numerous Reed-Sternberg cells and extensive fibrosis  poor prognosis B.[slideshare.net]
  • Treatment and prognosis Various treatments are used, with varying degrees of success, in Kasabach-Merritt syndrome - systemic corticosteroids, radiation therapy, compression, embolization and/or pharmacotherapy (e.g. interferon, platelet aggregation inhibitors[radiopaedia.org]
  • Good prognosis- mostly cutaneous involvement U/S liver-- greater than 5 cutaneous lesions impart a 16% chance of liver involvement, leading to hypothyroidism check TSH-- MR and cardiac failure can ensue if hypothyroidism not detected ** Clinical syndromes[cram.com]
  • Accurate diagnosis of multifocal neonatal vascular lesions is imperative to facilitate appropriate evaluation, treatment, and prognosis. Copyright 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • The prognosis is often poor due to failure to thrive, fluid and electrolyte imbalances, and sepsis.[bobbybukamd.com]

Etiology

  • Their etiology is not precisely century with the first clinical recognition of known, so we have included chapters that dis symptoms and patterns for diagnosis of car cuss many aspects of congenital cardiac mal diovascular diseases.[books.google.de]
  • Etiology Kasabach-Merritt syndrome is usually caused by kaposiform hemangioendothelioma or tufted angioma. Other vascular tumors may be responsible, such as infantile hemangioma. The radiographic features are specific to each vascular tumor.[radiopaedia.org]
  • Gorham’s (GOR-amz) disease is a very rare skeletal condition of uncertain etiology, characterized by the uncontrolled proliferation of distended, thin-walled vascular or lymphatic channels within bone, which leads to resorption and replacement of bone[lgdalliance.org]
  • Etiology Sepsis (more commonly with gram-negative organisms) Malignancies Surgery on thrombokinase-rich organs (e.g., lung, prostate, pancreas ) Acute traumatic coagulopathy Acute hemolytic transfusion reaction ( AHTR ) Traumatic brain injury Fat embolism[amboss.com]
  • […] scalp, dermoid cysts develop more frequently along the midline in the area covered by the anterior and posterior fontanelles, and epidermoids tend to occur laterally at suture sites between the pterion and the asterion along the squamosal suture. 3 The etiology[neupsykey.com]

Epidemiology

  • Related Journals: American Journal of Epidemiology, Journal of Clinical Epidemiology, Cancer Epidemiology Biomarkers and Prevention, International Journal of Epidemiology, Epidemiology, Journal of Immunology, Annual Review of Immunology, Journal of Allergy[clinical-pediatrics-dermatology.imedpub.com]
  • Incidence : 1% of all hospital admissions Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
  • Reporting guidelines exist for a variety of study designs including randomized controlled trials (Consolidated Standards of Reporting Trials, CONSORT) ( 10 ), observational studies (Strengthening the Reporting of Observational studies in Epidemiology,[aerzteblatt.de]
  • Optimizing Care for Infantile Hemangiomas: E-Learning Can Improve Parents’ Knowledge and (Risk) Evaluation Marlies de Graaf*, Department of Dermatology, University Medical Center Utrecht, Utrecht, Netherlands Mirjam Knol, Epidemiology and Surveillance[medicine20congress.com]
  • Because such disparate disciplines have been reporting the clinical series, epidemiologic studies have varied considerably in the incidence of the different types of lesions.[neupsykey.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Types of DIC Bleeding (hyperfibrinolytic) type Organ-failure ( thrombotic ) type Massive bleeding (consumptive) type Non-symptomatic DIC Pathophysiology Hyperfibrinolysis Cytokines Plasminogen activator inhibitor-I ( PAI-I ) and neutrophil[amboss.com]
  • Pathophysiology Metastatic spinal cord compression usually follows hematogenous dissemination of malignant cells to the vertebral bodies, with subsequent expansion into the epidural space.[emedicine.medscape.com]
  • Irrespective of the primary aetiology, electrophysiological studies suggest that the underlying pathophysiology relates to compression of the vasa nervosum of the nerve [ 6 Sunderland S.[benthamopen.com]
  • Pathophysiology of capillary haemangioma growth after birth. Med Hypotheses 2005;64:1093-6. [ PUBMED ] 37. Shuin T, Yamasaki I, Tamura K, Okuda H, Furihata M, Ashida S.[asianjns.org]
  • Endothelial cells in physiology and in the pathophysiology of vascular disorders. Blood 1998 ; 91 : 3527 – 3561. 2. Cotran, RS, Mayadas-Norton, T. Endothelial adhesion molecules in health and disease. Pathol Biol (Paris) 1998 ; 46 : 164 – 170. 3.[cambridge.org]

Prevention

  • Birth Defects This consumer information document provides general information on the various types of birth defects and includes causes, detection and diagnosis, treatment and prevention.[healthfinder.gov]
  • Early intervention is required to prevent complications ( 5 ) Therefore, The list of treatment includes surgical and non-surgical treatment.[ispub.com]
  • Treatment In general, the goals of hemangioma treatment are to prevent loss of life or function, and to prevent scarring, either before or as a result of therapy.[aafp.org]
  • Traditionally, the concept of cancer prevention as applied to childhood cancer has not been given much credence or attention.[clincancerres.aacrjournals.org]
  • Methods of prevention include avoidance of exposure to alcohol and tobacco products as well as vaccination of children and young adults against HPV.[clinical-pediatrics-dermatology.imedpub.com]

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