Cases reported "Hemangioma"

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21/147. Diffuse neonatal hemangiomatosis associated with Simpson-Golabi-Behmel syndrome: a case report.

    Diffuse neonatal hemangiomatosis (DNH) is a rare disorder characterized by multiple cutaneous and visceral hemangiomas that usually presents in newborns and has a high mortality rate.While previously reported cases of DNH describe multiple cutaneous hemangiomas, we present a patient with a single hemangioma of the ear, who also had DNH of the central nervous system and visceral organs. Furthermore, in this report we present a new constellation of findings, namely, a Simpson-Golabi-Behmel syndrome (SGBS). The practical implication on the basis of the experience with our patient is the need to ensure adequate diagnostics for patients with large hemangiomas as well as for patients with multiple cutaneous hemangiomas, because DNH also can occur in single hemangiomas in rare cases.
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keywords = angiomatosis
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22/147. prenatal diagnosis and management of fetuses with liver hemangiomata.

    OBJECTIVES: To study the relationship between prenatal appearance and perinatal outcome of fetuses with hepatic hemangiomata with special emphasis on criteria that may help to improve perinatal management. methods: In a tertiary referral center six fetuses with hepatic hemangiomata were evaluated by gray-scale, color, and pulsed wave Doppler ultrasound between 1994 and 2000. fetal blood sampling was performed in four cases. All data (computerized files and video tapes) were analyzed retrospectively. RESULTS: Two fetuses showed very similar sonographic findings. They had an isolated large ('giant') round hepatic hemangioma (diameter 43 and 68 mm, respectively) supplied by one hepatic artery and drained by one hepatic vein, both of them showing high velocity and low pulsatility blood flow. fetal blood count and coagulation parameters were normal in one case, whereas the other fetus showed a Kasabach-Merritt sequence with severe thrombocytopenia (10 platelets/nL) and mild disseminated intravascular coagulation. Intrauterine platelet transfusion was performed immediately prior to planned Cesarean delivery. Rapid platelet consumption continued postnatally, requiring several thrombocyte transfusions. Platelet counts stabilized only after tumor resection on the second day of life. One fetus with diffuse neonatal hemangiomatosis developed high-output cardiac failure with hydrops in addition to Kasabach-Merritt sequence (15 platelets/nL), and died following premature delivery. Three fetuses, however, showing an isolated small hyperechogenic hepatic hemangioma (5, 5, and 6 mm in diameter, respectively) did not develop any perinatal complications. CONCLUSION: Large fetal liver hemangiomata and diffuse hemangiomatosis may cause severe perinatal complications, particularly high-output cardiac failure and/or Kasabach-Merritt sequence with severe consumption of platelets and clotting factors and hemolytic anemia. fetal blood sampling enables the prenatal detection of these potential complications, allowing critical modification of perinatal management such as intrauterine platelet transfusion, especially directly before delivery. In contrast, isolated small hyperreflexic hepatic hemangiomata do not appear to be associated with any of these fetal and postnatal sequelae.
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ranking = 0.4
keywords = angiomatosis
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23/147. A newborn infant with sternal malformation/vascular dysplasia association.

    Sternal malformation/vascular dysplasia complex was described by Hersch et al. in 1985. The principle findings include cleft of the sternum covered by an atrophic skin, a midline abdominal raphe and hemangiomatosis. The inheritance pattern seems to be sporadic. We report a newborn baby with sternal defect, cleft lip and palate, supraumbilical raphe and hemangiomas.
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ranking = 0.2
keywords = angiomatosis
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24/147. Benign neonatal hemangiomatosis with conjunctival involvement. Report of a case and review of the literature.

    Hemangiomas are the most common tumours of infancy. When limited to the skin, multiple lesions have a benign course and excellent prognosis but in cases of visceral involvement, the morbidity and mortality rates are high. We report a rare case of a female infant with benign neonatal hemangiomatosis who had dramatic conjunctival involvement. The spectrum of neonatal hemangiomatosis is reviewed, highlighting the importance of differentiation of the two extremes of this disorder.
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ranking = 1.2
keywords = angiomatosis
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25/147. Diffuse neonatal haemangiomatosis: a rare cause of haemorrhagic shock and refractory coagulopathy in the newborn.

    A term newborn infant developed hypovolaemic shock shortly after birth. She was pale with gross hepatomegaly. She required multiple boluses of intravenous fluids, blood products as well as inotropic support. Blood investigations showed persistent thrombocytopenia, anaemia and disseminated intravascular coagulopathy (DIC). She also developed heart failure. She finally succumbed on the eleventh day of life. autopsy revealed haemangiomatosis involving the liver, lungs, gastrointestinal tract, kidneys and adrenals.
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keywords = angiomatosis
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26/147. Diffuse congenital hemangiomatosis with infantile glaucoma.

    A white male infant was born with multiple hemangiomas of the skin and mucous membrane. At 7 months of age he exhibited the signs and symptoms of infantile glaucoma in each eye, but goniotomy was not possible because of vascularity in the angles. Cyclocryotherapy was used on multiple occasions to control intraocular pressure until a goniotomy could be done safely in one eye. The other eye has had good control of intraocular pressure without operation.
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ranking = 0.8
keywords = angiomatosis
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27/147. Recombinant interferon alfa 2a in hepatic hemangiomatosis with congestive heart failure: a case report.

    A 45-day-old patient was admitted with dyspnea, hepatomegaly, tachycardia, holosystolic murmur in the precordial region, and continuous murmur at the right hypochondrium. Four cutaneous angiomas were noted. Instrumental examinations revealed congestive heart failure and multiple focal lesions in the liver with typical features of hemangiomas. The therapy with subcutaneous interferon-alfa-2a (IFN-alpha) was administered for 12 months with progressive regression of cutaneous hemangiomas, liver lesions, and cardiological alterations. IFN-alpha therapy was effective without any significant adverse effects.
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ranking = 0.8
keywords = angiomatosis
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28/147. Massive chronic feto-maternal bleeding associated with placental chorioangiomas.

    After pregnancy complicated by polyhydramnios and the antenatal discovery of a very large placenta a newborn infant suffered from anemia, thrombocytopenia and hypoproteinemic edema, and was successfully treated by exchange transfusion. The placenta contained two chorioangiomas and there was diffuse placental hypertrophy with edema and patchy chorioangiomatosis. There was evidence of major chronic feto-maternal bleeding which could be the explanation for most of the hematological and biochemical problems which occurred. The child was developing normally at subsequent follow-up aged 15 weeks.
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ranking = 0.2
keywords = angiomatosis
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29/147. Littoral cell angiomatosis with poorly differentiated adenocarcinoma of the lung.

    We report on a 64-year-old male united states Navy Veteran of world war ii, one of two identical twins, diagnosed with littoral cell angiomatosis of the spleen, liver, and lymph nodes, later found to have a massive poorly differentiated adenocarcinoma involving the mediastinum, adjoining lung, and sternum with widespread metastases. Herein we include our findings at autopsy, pertinent immunohistochemical studies, and a review of the literature pertaining to littoral cell angiomatosis with comment on its association with visceral malignancies.
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ranking = 1.2
keywords = angiomatosis
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30/147. von hippel-lindau disease type 2A in a family with a duplicated 21-base-pair in-frame insertion mutation in the VHL gene.

    BACKGROUND: von hippel-lindau disease (VHL), also called angiomatosis retinae, is inherited as an autosomal dominant trait. It is frequently associated with other tumors in the central nervous system, kidneys, or adrenal glands. In order to investigate the relationship between genotype and corresponding phenotypes, we performed molecular genetic analysis in a Japanese patient with VHL type 2A. methods: After informed consent had been obtained, the three exons of the VHL gene were PCR-amplified and sequenced either directly or after subcloning. Clinical features were also examined. RESULTS: A novel in-frame duplication of the 21 base pairs at nucleotide 806 (the position of codon 198) of the VHL gene was found in our patient. The clinical phenotype of the patient included retinal hemangiomas associated with vitreous hemorrhage and traction retinal detachment, pheochromocytoma, and hemangioma-like lesions in the cerebellum which corresponded to those of VHL type 2A. Abnormal diffuse vascular leakage was observed in the apparently intact retina by fluorescein angiography. CONCLUSION: An insertion mutation of the VHL gene is a rare association with VHL type 2. This insertion mutation may interfere the binding between the VHL gene and elongins. Abnormal retinal vascular leakage suggests the possible effects of overexpressed vascular permeability factors such as vascular endothelial growth factor from hemangiomas associated with defective VHL gene.
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ranking = 0.2
keywords = angiomatosis
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