351/1154. The blue rubber bleb nevus syndrome: a case with prominent head and neck findings. The blue rubber nevus syndrome describes a clinical entity that presents with cutaneous and visceral hemangiomas, particularly those found in the gastrointestinal tract. These lesions may appear at birth or in early childhood and can increase in size and frequency with age. Oral cavity hemangiomas occur in 59% to 64% of cases. Successful diagnosis requires a high index of suspicion, and treatment is symptomatic. This report discusses a unique presentation and a review of the literature. ( info) |
352/1154. Splenic hemangiopericytoma and serosal cavernous hemangiomatosis of the adjacent colon. A healthy 31-years-old man presented with a three-year history of abdominal discomfort. Radiological examinations revealed multifocal tumoral lesions in the spleen. The patient underwent splenectomy for differential diagnosis and treatment. During the operation, in addition to the splenic masses, there were also multiple millimetric purpuric-like lesions on the colonic serosal surfaces adjacent to the splenic hilus. One of them was excised. Histologic examination showed hemangiopericytoma of the spleen and cavernous hemangioma of the adjacent colon. This is the first report showing the close association of these two distinct lesions with vascular origin in the literature. Despite not having any apparent evidence, there may be a sequential relationship between the hemangiopericytoma of the spleen and cavernous hemangiomas. ( info) |
353/1154. Craniofacial cavernous hemangioma: succesful treatment with methylprednisolone. Systemic corticosteroid treatment is reported as effective for problematic cutaneous hemangiomas occuring in infancy, and depend on the dose, the duration of treatment, and the age at which the course of drugs is initiated. A 7-month-old female infant with extended cavernous hemangioma on the left part of forehead, face,and neck which appeared 15 days after birth is presented. She was successfully treated with oral methylprednisolone (initial doses of 3 mg/kg/daily and reduced in steps over 6 months) with significant involution of the lesions and with good aesthetic results.The oral corticosteroid treatment is an efficient medical therapy for common extended cavernous infantile hemangiomas with accelerated growth if initiated early in the proliferative phase. ( info) |
354/1154. Diffuse cavernous hemangioma of the rectosigmoid colon. Diffuse cavernous hemangioma of the rectosigmoid colon is an uncommon benign vascular lesion. We report 5 cases of diffuse cavernous hemangioma, focusing on the clinical features, diagnosis procedure and treatment. Five patients have undergone sphincter-saving procedures, 3 cases had coloanal sleeve anastomoses and 1 patient each had pull-through anastomosis and lower anterior resection. During the follow-up, which ranged from 3 to 10 years, 3 patients had no further anal bleeding and 2 patients had minor intermittent anal bleeding. Continence for normal stool was satisfactory in all patients. In conclusion, sphincter-saving procedure is most appropriate and curative approach for the treatment of diffuse cavernous hemangioma. Imaging study plays an important role in the diagnosis, preoperative staging and follow-up. ( info) |
355/1154. A diagnostic pitfall: Angiosarcoma of the brain mimicking cavernous angioma. Primary or secondary angiosarcoma in the central nervous system is rarely reported. We present a rare case of cerebral angiosarcoma, which comprised both poorly-differentiated solid areas and well-differentiated areas that led to the misdiagnosis of cavernous angioma. A 79-year old woman presented with an intracerebral hematoma in the left frontal lobe that was misdiagnosed as a hemorrhage from a cavernous angioma at initial operation. At a second surgery, the lesion was diagnosed as angiosarcoma involving the cerebellum, heart, femur, sacro-iliac bones and other locations. An autopsy suggested that the angiosarcoma of the heart was the primary lesion, which was occult at the time of the initial operation. Angiosarcoma may have areas with different degrees of differentiation and when a cavernous angioma is suspected histopathologically, the specimen should also be carefully explored for poorly-differentiated areas and the diagnosis of primary or secondary angiosarcoma considered. ( info) |
| We report a patient with multiple angiographically occult vascular malformations in the brain and spine. magnetic resonance imaging showed multiple lesions in brain and spine with hypointense areas on both T1 and T2-weighted images. These hypointense areas are usually secondary to hemosiderin deposits consistent with remote bleeding in the lesions. We conclude that when magnetic resonance reveals an intraspinal lesion with signal intensity characteristics consistent with a vascular malformation, an examination of the brain should be performed to rule out associated intracranial lesions. The finding of multiple lesions in the brain with identical signal intensity characteristics reinforces the diagnosis of vascular malformation. ( info) |
357/1154. Cavernous haemangioma in the orbital apex: stereotactic-guided transcranial cryoextraction. A 55-year-old Caucasian woman presented with an orbital cavernous haemangioma superior to the optic nerve in the orbital apex. Preoperative imaging demonstrated a mass involving the superomedial and superolateral quadrants of the posterior orbit. A stereotactic fronto-orbital approach was performed by the neurosurgical team, and cryoextraction of the lesion was accomplished by the ocular plastic surgical team. ( info) |
358/1154. Persistent posterior globe flattening after orbital cavernous haemangioma excision. A 54-year-old man presented with left intraconal cavernous haemangioma resulting in globe compression and hyperopia. The tumour was excised completely via lateral orbitotomy approach. Complete excision of tumour did not result in resolution of globe flattening and hyperopia after 21 months. Persistent globe flattening despite removal of a longstanding orbital tumour may be caused by remodelling of the sclera from long-term compression. ( info) |
359/1154. Blue rubber bleb nevus syndrome: laser photocoagulation of colonic hemangiomas in a child with microcytic anemia. This is a case report and review of the literature on the blue rubber bleb nevus syndrome. The clinical features of the syndrome are discussed, with emphasis on chronic gastrointestinal bleeding and resulting anemia. A new therapeutic modality, colonoscopy with laser photocoagulation, is recommended as a safe, effective, and less invasive method of controlling bleeding from colonic hemangiomas than surgical resection and repeated transfusions. This technique is less helpful for patients with prominent involvement of the small intestine. ( info) |
360/1154. Angiographically occult brainstem vascular malformation: a longitudinal comparison of magnetic resonance imaging (MRI) and multimodal evoked potential (EP) recordings. Over a period of several years, a patient with angiographically occult vascular malformation (AOVM) involving the brainstem was longitudinally studied by means of serial magnetic resonance imaging (MRI) and multimodal Evoked Potential (EP) recordings (visual-VEP, brainstem auditory-BAEP, somatosensory--SEP--by stimulating median and peroneal nerves). MRI did contribute to an accurate definition of AOVM features. In particular, it was able to follow over time the AOVM size, and to discriminate between recent and old bleedings. Multimodal EP recordings displayed different pathological BAEP and peroneal SEP values, which documented a transient segmental brainstem involvement (related to the presence of hemorrhage), along with persistent and probably irreversible signs of diffuse brainstem dysfunction. Thus, MRI and EP assessment is useful in monitoring the clinical course of brainstem occult vascular malformations. ( info) |