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Portal cavernoma is a venous vascular anomaly characterized by the formation of a network of veins whose caliber is increased and carrying portal blood. It is due to a thrombotic and always chronic occlusion of the extra-hepatic portal venous system. This is one of the most common causes of portal hypertension in children. Its severity is mainly associated with an high risk of gastrointestinal haemorrhage. Very few cases have been described mainly in African literature. We report the case of a 4-year old boy admitted with very abundant haematemesis, melena and dizziness associated with anemic syndrome on examination. Laboratory tests showed severe microcytic hypochromic anemia with normal renal and hepatic function. Gastrointestinal endoscopy showed esophageal varices (grade III) with red signs. Abdominal ultrasound showed portal vein formation resulting in the classic "spiderweb", in favor of a cavernoma. Abdominal CT scan confirmed portal cavernoma associated with portal hypertensive syndrome and vascular anomaly like an ectopic splenic vein anastomosis with the trunk formed by the gonadal vein and the inferior mesenteric vein. Therapeutic approach was based on blood transfusion and beta-blocker treatment. Portal cavernoma can be a major complication of vascular malformations often unknown. In case of gastrointestinal haemorrhage in children, diagnosis should be suspected. Its management requires early treatment and should be adapted to the patient's condition in order to prevent a fatal evolution., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts.

Introduction: Hemangioma are rare benign vascular tumors. Histologically, there are several types including capillary, cavernous and mixed hemangioma. We present a very rare mixed hemangioma of the nasal septum., Case Report: A 30-year-old woman consulted for unilateral epistaxis and nasal obstruction. CT scan revealed a tumor filling the rhinopharynx, strongly enhanced after injection of contrast medium. It was pedicled on the posterior and superior nasal septum. Arteriography revealed a vascular blush. Tumor exeresis was performed by paralateronasal approach and endoscopic surgery. The histological examination identified a mixed hemangioma., Discussion: Capillary and cavernous hemangiomas are rare and benign vascular tumors developing in the skin and subcutaneous tissues of the head and neck, and/or oral and nasal mucosa. No mixed hemangioma exclusively involving the nasal septum had been previously reported. Complete surgical resection is the treatment of choice, providing a favorable prognosis with low risk of recurrence., (Copyright © 2012. Published by Elsevier Masson SAS.)

The hemangioma of the adrenal gland is an adrenal gland lesion rare, benign and usually asymptomatic. Discovered incidentally during an abdominal imaging study, it is part of incidentalomas. Imagery is the best to characterise these silent adrenal masses (computed tomography [CT], Magnetic Resonance Imaging [MRI]± Positron Emission Tomography [PET scan] with 18F-FDG). The main risks of the hemangioma are ignorance of malignancy, bleeding and abdominal mass syndrome. The analysis of the literature shows the importance of laparoscopy. A multidisciplinary discussion on this type of lesion appears indispensable both diagnostic and therapeutic., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Vascular tumors of the orbit include capillary hemangioma, cavernous hemangioma, hemolymphangioma, hemangiopericytoma and a few rare tumors. Capillary hemangioma and hemolymphangioma, occurring mainly in children, are covered in the chapter devoted to childhood tumors. In this chapter, cavernous hemangioma and hemangiopericytoma are discussed as well as rare vascular tumors. Although orbital varix is not a tumor, it is also considered because of the diagnostic problems and the close correlation of orbital varix with a true tumor: hemolymphangioma., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

Intraosseous cavernous hemangioma is rarely located in the calvarium. This benign tumor, arising from the intrinsic vasculature of bone, grows slowly over a period of years before it causes symptoms such as headaches or localized compression. We report here the case of a 27-year-old man, who presented with multiple calvarial cavernous hemangiomas associated with several hepatic hemangiomas. The patient had a family history of the disease, and is only the second case of multiple calvarial cavernous hemangiomas reported in the literature. The clinical, radiological and therapeutic aspects of this rare entity are also discussed.

The treatment of cavernous sinus meningiomas remains controversial. However, at present, the general consensus is that only tumors with extracavernous extensions should be operated on and that resection is performed only on the extracavernous portions. Since these tumors are deep-seated and in the vicinity of vital and highly functional neurovascular structures, surgery remains difficult and dangerous. To achieve the gross total removal of the extracavernous extensions with minimal sequelae, the operative strategy must take into account the location of the tumor extension, not only in the basal cisterns but also into the skull base structures, the vascularization of the tumor with its feeders, as well as the venous drainage pathways. These anatomical and surgical data are collected from preoperative imaging, which includes CT-scan with bony windows and consecutive fine sections, MRI, angio-MR, and DSA angiography performed with selective arteriography including late venous phases. The operative plan must define the type of craniotomy and complementary osteotomy, the usefulness of an anterior clinoidectomy with skeletonization of the optic nerve, and determine whether extensive exposure of the cerebral middle fossa is necessary to ensure substantial devascularization of the tumor and proximal control of the internal carotid artery at its intrapetrosal portion. In addition, the possible need for an extra-intracranial bypass prior to tumor removal should be evaluated. The high number of neuroimaging investigations is the guarantee of surgical accuracy and safety.

The imaging features of rectal tumors on CT and MRI are reviewed. MRI is the imaging modality of choice for loco-regional staging, and may provide clues as to the histological nature of some tumors.

Aims: To establish the correlation between the clinical manifestations of hearing and balance disturbance and the anatomical site within the pons of cavernous angiomas, and to describe their clinical features, and the findings on ABR and MRI., Materials and Methods: Two clinical cases of cavernous angioma with cochlear and vestibular manifestations underwent audiometric evaluation, with VNG and ABR as well as CT and MRI scans., Conclusion: Cavernous angiomas are rare (less than 2% of intra-cranial space-occupying lesions). Their most common topographical site is midline in the pons, and may give rise to symptoms in attacks (due to episodes of bleeding within the tumour) which may mimic symptoms of peripheral origin (sudden deafness, fluctuating hearing loss, Menière-like vertigo). There may be ectopic remnants of the embryonic blood vascular system. They may be associated with other malformations (intra-cerebral venous angiomas, cavernous angiomas at other sites). Their development is often by sudden progression which may be dramatic, and have accompanying neurological features. For investigating the hearing and balance, ABR is an excellent test, giving a typical picture of a retro-cochlear lesion that necessitates progression to MRI, the investigation of choice. This will demonstrate the typical rosette-like appearance with a heterogeneous T2 image (a less dense peripheral ring, with a denser central signal). There is no specific medical treatment for this condition, and surgery is indicated only exceptionally. There is a contraindication to anticoagulant therapy, to platelet-dispersing medication and to violent sports activities. Two cases of cavernous angioma are reported; one was more anteriorly situated, and had mainly vestibular features, and the other was more posterior near the floor of the IVth ventricule, with mainly auditory features. The correlation of the anatomical and clinical findings with those of the MRI are discussed, especially in relation to our understanding of the auditory and vestibular pathways within the brainstem (the auditory striae, the crossed auditory pathways and the nuclei of the corpus trapezoideum for the auditory effects, and the paramedian reticular nucleus and crossed inter-commissure pathways to the cerebellum and vestibular nuclei, together with the proximity of the direct midline vestibulo-spinal tract, for the vestibular effects).

We report the surgical results in a series of 47 patients with cerebral cavernous malformation who had undergone surgery between 1973 and 1994, with a follow up ranging from 12 months to 24 years (mean: 4 years). They were divided in there groups according to their initial clinical presentation: epilepsy (31 cases), hemorrhage (11 cases) and neurological deficit (5 cases). Surgery consisted of cavernoma resection only (11 cases) or its extension to surrounding gliotic tissue (36 cases). Results are satisfactory: no surgical mortality, low morbidity (4 cases), no recurrent hemorrhage, seizures disappearance with anticonvulsant therapy stop (4 cases) or alleviation (20 cases). Only one patient died far from surgery (6 months) consequently to his initial bleeding, while all the others lead a normal active life. The therapeutic management, compared to the literature, pleads in favour of intentionally surgical attitude and gliotic tissue removal as often as reasonably possible.

A case of an extra-axial cavernous angioma of the cisterna magna is described. The clinical presentation, radiographic features, treatment of that unusual location in the posterior fossa are discussed. The patient is a 31 years old man admitted for the sudden onset of occipital headache and instability. A CT scan demonstrated a slightly calcified lesion, minimally enhanced after intravenous injection of a contrast agent, located within the posterior fossa. A T2-weighted MR image showed a well circumscribed mass, located between medulla oblongata and left cerebellar tonsil, of mixed signal intensity surrounded by a rim of decreased signal intensity related to the presence of hemosiderin. Cerebral arteriography was normal. A medial suboccipital craniectomy was performed, with the patient in the ventral position, in which a 1.5 x 1 cm solid dark-red mass was encountered arising from the cisterna magna and was easily removed "en bloc". The operative extra-axial aspect of that lesion was evident excluding a medulla oblongata tumor protruding into the cisterna magna. Histologically, the lesion was a cavernous angioma consisting of numerous vascular channels with collagen fibrous walls filled with fibrin thrombi and hemosiderin. Few elastic fibers were found in vascular walls. The patient's post-operative course was unremarkable.

A case of a cavernous haemangioma associated with an hepatic haemangioma is reported. It presented in the form of a hyperechogenic mass on ultrasound imaging. On the Computed Tomography scan with contrast, the splenic tumour became progressively hyperdense: this last characteristic is observed in 6 out of 9 cases reported in the literature. MR imaging seems to allow an accurate preoperative diagnosis; nevertheless, splenectomy is often indicated because of the risk of rupture, in which case histological examination removes any doubt concerning an exceptional malignant form.

The authors report on a right fronto-singular cavernous angioma which appeared, in a 46 year-old woman, four years after the excision of a right rolandic localization. Diagnosis suggested on radiological findings was confirmed by histological examination. After a review of the literature the authors discuss possible mechanisms of such delayed appearance of intra-cerebral cavernous angiomas. They suggest the possibility of infraradiologic stage corresponding to a micro-malformation characterized by abnormal capillaries, which has not been yet histologically modified by hemorrhages, thrombosis, fibrosis, gliosis and calcifications.

In a poorly equipped tropical setting, limb amputation is often considered as the only therapeutic solution for malignant tumors or large benign tumors. However it can be avoided by bone replacement using an avascular autologous fibular graft. This method was used in a young girl from Tchad presenting a giant cavernous hemangioma of the humerus. This technique allowed salvage of the extremity but resulted inevitably in radial paralysis. The time needed for bone healing was normal. This technique required minimum facilities and was perfectly biocompatible. It can be used in tropical settings where allograft techniques and prosthetic replacement are not available.

Eighteen cases of cerebral cavernous angiomas are reported, and their histopathological, clinical and radiological features are reviewed. MR complements TC in characterizing cavernomas and in distinguishing them from similar-appearing lesions. However, when such lesions are seen with atypical features, discrimination from hemorrhagic tumors remains problematic. More malignant lesions present, on MRI, a much larger area of abnormality and make themselves obvious in a shorter period. Gadolinium administration may allow a more specific interpretation of small lesions.

The incidence of abdominal tuberculosis represents less than 1% of all forms of tuberculosis. We report the case of a portal vein thrombosis associated with tuberculous adenopathy of the hepatic hilum discovered in a young woman free of symptoms, without any risk factors, who had recovered from a cavity pulmonary tuberculosis after a classical anti-tuberculous treatment. This case suggests that abdominal tuberculosis often remains undiagnosed. Reemergence of tuberculosis is likely because of the increase in the incidence of the acquired immunodeficiency syndrome.

We described the case of a 56 year-old man with cirrhosis due to hemochromatosis and who had undergone a surgical terminolateral portocaval shunt 17 years previously. The patient was admitted for jaundice. Ultrasonography initially suggested multiple intrahepatic tubular structures, interpreted as enlarged intrahepatic bile ducts. Pulsed Doppler ultrasonography demonstrated that this spectral waveform corresponded to the intrahepatic extension of a diffuse portal cavernoma. Cavernomatous transformation of portal vein should be suspected when intrahepatic tubular structures are associated with long standing portal vein thrombosis.

The authors report a retrospective series of 10 cases of intracerebral haematomas consecutive to cavernomas and studied with MRI. The factors that led to the MRI study were the patients' age and clinical history, the site of the haematoma, the persistence of CT images and a negative arteriography. MRI showed that the haematoma was in contact with the cavernoma in 4 cases and at a distance from it in one case. In 5 cases only the cavernoma was seen at the site of the haematoma. Three patients had multiple cavernomas. In all cases, the diagnosis of cavernoma was confirmed at surgical excision.

On four occasions since 1978, this 53 year-old woman presented with a right hemicorporal hypotonia, symptomatic of a hemispheric cerebellar syndrome. In 1981, she experienced the progressive development of a cervical dystonia. CT scan and RM scan showed a cavernous angioma in the right cerebellar hemisphere. The 18F-2-fluoro-2-deoxy-glucose PET scan revealed a right cerebellar and a contralateral cortical and striatal hypometabolism. This crossed cerebello-cortical diaschisis can be interpreted as a functional interruption of the cerebello-cerebral pathways. This case raises the question of the role played by a cerebellar lesion in the development of a focal dystonia.

Solitary hemangioma located in the skeleton of the hand is a rare tumor, very different from diffuse angiomatosis or benign, hemangioma, located in the two distal phalanges of the right fourth finger. Due to articular stiffness, pain, and cosmetic problem, the treatment consisted of central ray resection. The pathological examination revealed a true benign skeletal hemangioma with marked bone resorption of the phalangeal skeleton. The soft tissues ant the periosteum were not involved.

The authors report five cases of cavernous hemangioma belonging to two families. In the first family, an 8 year old child was operated on in 1970 for a left frontal location. His mother, aged 39, was operated on in december, 1981, with stereotaxic approach, for two locations: one was frontal location, the other one was in the pineal region. Among the second family, a 21 year old woman was operated on in december 1976, for a cerebellar cavernous hemangioma. Her sister, aged 18, presented with subarachnoid hemorrhage in january 1980. C.T. scan showed a right temporal high attenuation area. This patient was not operated on. The aunt to those two sisters was operated on in october, 1981, for a left temporal cavernous hemangioma. Those five cases represent about half of the intracranial cavernous hemangiomas operated on in the Lille Neurosurgery Department since 1967. Even, unusual, the familial occurrence of this affection must be admitted. It shows a nosological interest, but also allows prevention by searching and surveying the asymptomatic cases, or genetic evaluation of disease risk.

A cavernous hemangioma of spleen in a clinically asymptomatic patient presented in the form of a hyperechogenic mass on ultrasound imaging, becoming progressively hyperdense on the CT scan with contrast. This appearance, similar in all respects to that seen with cavernous hemangioma of liver, is rarely reported in the literature.

A 23 year-old man, with a two month-history of back pain, presented with spastic paraplegia and hypesthesia of both lower limbs. A myelo C.T. revealed a spinal epidural dumb bell-shaped mass extending in the intra and extra vertebral spaces through the enlarged intervertebral foramen. Total recovery was observed three months after surgery. Pathological examination of the tumor showed it to be a cavernous angioma. Primary epidural angioma is rare: in 2 reported dumb bell-shaped spinal epidural cavernous angiomas, radiological findings were also suggesting a spinal neurinoma.

This report presents the spectrum of radiologic findings in a case of cavernous haemangioma of the spleen. Certain sonographic findings, echogenic mass surrounded by a rim of hypoechogenic splenic tissue, were suggestive of this tumor. Computed tomography and delayed-phase angiograms were non specific.

Based on findings for 30 patients, the authors assess the value of CT scans for the diagnosis of cavernous hemangiomas of the liver. Four images were retained, and were graded 0 (absence) or 1 (presence): hypodensity before injection of the contrast material, arteriolar halo after injection, progressive centripetal filling and late hyperdensity. A score of 3 or 4 after the CT scan allows diagnosis. Solitary lesions of at least 3 cm are correctly evaluated. When associated with other smaller lesions, or when smaller than 3 cm, CT scans are less helpful and arteriography is necessary. In all. CT scanning has four limiting parameters: lesion diameter, the number of images requiring exploration, the difficulty in obtaining exactly the same section in various sequences allowing effective scan analysis (thoraco-abdominal position of the liver) and problems specific to individual patients (poor circulation, iodine allergy or intolerance). These limiting factors explain the "wait-and-see" strategy suggested when confronted with solitary hyperechoic nodules smaller than 3 cm.

The difficulty of surgical treatment of cavernomas of the portal vein results from the anatomical conditions created by this type of portal hypertension, in spite of favourable physiological conditions due to the integrity of the hepatic parenchyma. The latter is, in fact, relative after a certain length of history. This erratic course renders difficult assessment of the efficacy of methods of treatment. In the light of 56 cases, including 42 operated cases, the authors present the indications for surgery which depend on the severity and frequency of hemorrhage and also on the patient's age. When repeated bleeding occurs, a by-pass should be attempted whenever possible, if the bleeding impedes social life or growth, or if it threatens life. Mesenterico-caval anastomosis is preferable before the age of 7 years. Spleno-renal anastomosis requires a sufficient size of splenic vein. Ligature of aesophageal varices or disconnection of the oesophagus from the stomach, may be considered in cases of moderate hemorrhage. The varices may be treated by sclerosing injections after failure of a by-pass operation or in inoperable patients. Oesophago-gastrectomy is useful but should be a last resort.

The retinal cavernous hemangioma is a unilateral retinal or prepapillary vascular malformation disposed in clusters, sometimes recovered by a fine glial membrane which can in some cases be complicated by retinal or vitreous hemorrhages. In fundus fluorescein angiography, it is characterized by a delayed and incomplete filling with accumulation inside the angioma in the later phases of the angiogram, but without leakage through its wall. The authors report 7 cases, 5 are pure typical forms and 2 cases present an association of retinal cavernous hemangioma and Leber-Coats angiopathy. These mixed forms set the problems of relationship between these 2 diseases.