Kasabach-Merritt综合征研究进展

Kasabach-Merritt综合征是以巨大血管肿瘤伴发血小板减少和全身出血倾向为特征的一种综合征,其病理生理基础是血小板减少和弥漫性血管内凝血(DIC),与巨大血管肿瘤密切相关.治疗方案主要包括纠正DIC和血小板减少,根据具体情况选择外科治疗(手术切除、栓塞等)或内科药物治疗(糖皮质激素、α-干扰素、免疫抑制剂等)去除血管肿瘤.     

Haemangiomatous polyp of the posterior urethra in a boy

Urethral haemangiomas are rare and usually involve the entire urethra or are localized to the anterior urethra. Posterior urethral polyps are uncommon. A posterior urethral polyp was removed from a 3-year-old boy which proved to be haemangioma. In the histological classification of posterior urethral polyps, haemangioma should therefore be included.     

Hepatic haemangioma of infancy: role of propranolol

A newborn girl with a haemangioma of the liver failed to respond to cortico-steroid therapy. Ultrasonic evidence of the haemangioma disappeared after 2 months of treatment with propranolol. Propranolol, a non-selective beta-blocker, is a promising therapy in the management of haemangiomas.     

Cardiopulmonary bypass for resection of low tracheal haemangioma.

A 3 month old girl, weighing 4000 g, presented with a capillary haemangioma obstructing the lower trachea and left main bronchus; it was not responsive to steroids. Using cardiopulmonary bypass to maintain oxygenation the tumour was excised. We are not aware that this technique has been used before for the resection of such an obstructive haemangioma.     

PHACE syndrome: MRI of intracerebral vascular anomalies and clinical findings in a series of 12 patients

Background  

PHACE (posterior fossa defects, haemangioma, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities) syndrome describes a constellation of abnormalities that can occur in association with segmental craniofacial infantile haemangioma.     

Intraosseous haemangioma of the ilium

Intraosseous haemangioma, an uncommon benign vascular tumour, is most commonly seen in adults and tends to involve the vertebrae and the skull. Lesions of flat bones are rare and the imaging findings in these patients are non-specific. We report a unique case of intraosseous haemangioma in the ilium of a 7-year-old girl studied by US, radiography, scintigraphy, CT and MRI.     

Cavernous Haemangioma of the Jejunum

ABSTRACT. A 15-year-old boy who had been examined for almost lifelong iron deficiency anaemia caused by occult gastrointestinal bleeding was eventually, by abdominal surgery, found to have a cavernous haemangioma of the jejunum. A diagnostic approach is described and the importance of systematic evaluation and subsequent diagnosis in such patients is stressed.     

Development of a testicular haemangioma after interferon therapy for hepatic haemangiomas: a case report

We report a 2-month-old Japanese boy presenting with large multiple haemangiomas invading his liver. He was treated with daily subcutaneous injection of interferon alfa (IFN-α)-2a with progressive reduction of the hepatic haemangioma. He developed a scrotal mass 2 months after discontinuation of IFN, and this mass eventually required surgical management. Resected tumour was a juvenile haemangioma. The escape of this haemangioma from IFN therapy may be correlated to the quite low level of injected IFN in testis. Conclusion IFN therapy may not be curative for testicular haemangioma although it is effective in shrinking haemangiomas of the liver and skin. Received: 27 August 1996 / Accepted: 28 March 1997     

Activation of the coagulation system occurs within rather than outside cutaneous haemangiomas

Haemangiomas are the commonest tumours of infancy. They can become even more serious if followed by consumption coagulopathy and even life-threatening in cases of Kasabach-Merritt syndrome, with thrombocytopenia and haemorrhage. Data exist concerning systemic coagulation abnormalities in children with haemangiomas but to our knowledge there are no data on local consumption coagulopathy in haemangioma per se . We examined blood coagulation and fibrinolysis parameters in blood withdrawn from haemangioma blood vessels and blood withdrawn from the systemic vein in 14 children with cutaneous haemangiomas (3M, 11F; age range 3 mo to 10 y). Compared with controls, significant decreases in fibrinogen levels, FVII activity, antithrombin and plasmin inhibitor levels and increases in international normalized ratio (INR) and D-dimer levels were observed in the blood samples withdrawn directly from haemangioma blood vessels. Fibrinogen and antithrombin levels in samples withdrawn from systemic veins were reduced in relation to control values whilst INR values increased, but within normal ranges. D-dimer levels were increased in peripheral blood. The fibrinogen level was significantly lower and the INR and D-dimer levels were significantly higher in blood samples from haemangiomas compared to systemic blood. Clinical signs of systemic disseminated intravascular coagulation were not observed.

Conclusions : Our results suggest a strong local activation and local consumption coagulopathy in haemangioma, along with less conspicuous but observable systemic changes in coagulation and fibrinolysis parameters, although without signs of consumptive coagulopathy. These systemic changes could be a reflection of intra-lesion coagulation activation although there is no evidence to suggest truly systemic disseminated intravascular coagulation.     

Cavernous haemangioma of the jejunum. Long-standing anaemia in a child

A 15-year-old boy who had been examined for almost lifelong iron deficiency anaemia caused by occult gastrointestinal bleeding was eventually, by abdominal surgery, found to have a cavernous haemangioma of the jejunum. A diagnostic approach is described and the importance of systematic evaluation and subsequent diagnosis in such patients is stressed.     

Congenital haemangioma of the parotid gland

Summary A case of haemangioma of the right parotid gland in a female infant without associated skin lesion is presented. The patient was treated by superficial parotidectomy. From the Department of Surgery, Pt. J.N.M. Medical College, Raipur, M.P.     

Giant cutaneous haemangioma with heart failure in a neonate: successful surgical treatment

A large cutaneous haemangioma was identified on the posterior thorax of an 18-weeks' gestation fetus during routine antenatal ultrasonography. The diagnosis was confirmed after birth with computed axial tomography. Intractable heart failure was cured by excision of the haemangioma. Offprint requests to: E. R. Howard     

Urinary matrix metalloproteinases-2/9 in healthy infants and haemangioma patients prior to and during propranolol therapy

The mechanism of therapeutic success of propranolol for severe infantile haemangioma remains unclear. Propranolol was shown to modify matrix metalloproteinase (MMP) levels, which are associated with tumour pathogenesis. We hypothesized that urinary MMP2/9 is higher in patients with infantile haemangioma compared to healthy infants and that propranolol reduces MMP2/9 levels and thus leads to an involution of the haemangioma. In this case, MMP2/9 could be used as a marker of indicated therapy or therapeutic success. Urinary samples were taken before, 2?weeks after, and 2?months after the beginning of propranolol treatment in haemangioma patients and once in healthy controls. Activity of MMP2/9 was determined by commercially available activity kits. Urine of 22 haemangioma patients and 21 control subjects was obtained. Propranolol therapy had significant success in all patients. MMP2/9 was present in most samples, the younger the children the higher the MMP2 levels. Haemangioma patients showed lower levels of MMP2. The MMP2 levels were significantly higher after 2?weeks of propranolol than prior to therapy. There were no differences in MMP9 levels. Conclusions: Presence of MMP2/9 in the urine of infants <1?year can be explained by high rate of physiological tissue remodelling. Unexpectedly, MMP2 was lower in the urine of haemangioma patients and higher 2?weeks after propranolol treatment. Taking this and the diverse results in literature into account, the correlation between MMPs, proliferation, and regression of haemangiomas and propranolol remains unclear.     

Büschelartiges Hämangiom (“tufted angioma”) des Unterarms Fallbericht und Literaturübersicht

Background: Tufted angioma represents a benign vascular lesion of the skin. The knowledge of this distinctive entity is important in distinction of clinically more aggressive and malignant neoplasms respectively. Case report. We report a 8-months-old girl who developed a deep seated tufted haemangioma on her right forearm. Sonography and MRI showed a good vascularized tumor with interseptal spreading. Histological examination of the incisional biopsy revealed a cellular capillary haemangioma with morphological features of rare tufted haemangioma. Discussion. Characteristic morphological features of tufted haemangioma allow accurate diagnosis and differential diagnosis to clinically more aggressive and malignant neoplasms of skin and soft tissues, wheras radiological methods are rather unspecific and do not distinguish reliably between these vascular lesions. It is of special clinical importance that tufted haemangioma represents an enlarging but biologically benign vascular neoplasm.     

Hypergalactosaemia in a patient with portal-hepatic venous and hepatic arterio-venous shunts detected by neonatal screening

Hypergalactosaemia was discovered in a newborn girl during routine metabolic screening. Hereditary enzyme deficiency was ruled out. She had multiple hepatic haemangiomas with portal-hepatic venous and hepatic arterio-venous shunts. Since she showed signs of high-output heart failure due to the arterio-venous shunt, hepatic artery embolization was performed at age 3 months. A galactose tolerance test was performed before and after embolization and when the haemangioma no longer appeared on ultrasonography. Even after embolization, the level of blood galactose was abnormally elevated in the galactose tolerance test, but the blood galactose was eliminated more rapidly than before embolization. When the hepatic haemangioma was no longer detected by ultrasonography, the peak galactose level decreased. We surmise that the hypergalactosaemia was due to these shunts. In cases of hypergalactosaemia of unknown cause; liver haemangioma with portal-hepatic venous shunting should be considered as a possible cause. If a hepatic arteriovenous shunt also exists, this may contribute to the effect of the portosystemic shunting.     

Dynamic MR imaging of bladder haemangioma

Haemangioma is a rare benign mesenchymal tumour of the bladder. We report a case of bladder haemangioma and describe the enhancement kinetics observed on MR images after gadolinium bolus administration, which is similar to that reported in liver haemangioma. Received: 30 January 2001/Accepted: 7 July 2001     

Rapidly involuting congenital haemangioma (RICH) of the liver

Background  

Rapidly involuting congenital haemangioma (RICH) is a benign neoplasm that may occur in many locations in the body. When RICH occurs in the liver, it may be confused with other lesions.     

Nasal glioma presenting as capillary haemangioma

We report the case of a 5-month-old female infant with a congenital nasal tumour originally attributed to a capillary haemangioma. Doppler-flow ultrasound imaging revealed a solid mass surrounded by mildly enlarged vessels which had a flow pattern atypical of haemangioma. Histology showed non-malignant gliomatous cells with low proliferative activity. A diagnosis of nasal glioma was thus established and the patient underwent cranial MRT which excluded intracranial communication of the nasal glioma. Nasal gliomas arise from a skull defect, originating from the defective closure of the anterior neuroporus. They represent encephaloceles which have lost their intracranial connection. Nasal gliomas usually present shortly after birth as an intranasal obstruction or, as in our case, as a mostly extranasal tumour. Conclusion Nasal glioma is often misdiagnosed as a capillary haemangioma. It can be distinguished from the latter by Doppler-flow ultrasonography. Magnetic resonance imaging is required to exclude intracranial communication. Received: 4 March 2000 and in revised form: 19 May 2000 / Accepted: 19 May 2000     

Arteriovenous haemangioma of the joint capsule of the knee in a child

We report a rare case of arteriovenous haemangioma of the knee joint in a girl. This treatable entity should be included in the differential diagnosis of nonspecific recurrent knee swelling or pain in children.     

Computerized tomography appearances of pelvic haemangioma involving the large bowel in childhood

The Computed Tomography (CT) appearance of a pelvic haemangioma involving the rectum in two children, one with the Vater association and one with Klippel-Trenaunay syndrome, is described. Both cases complained of painless rectal bleeding and the diagnosis was suggested by the presence of pelvic phleboliths on the plain abdominal radiograph. CT provides a simple, non-invasive method of diagnosing such lesions and assessing their extent.