The role of radiosurgery in the acute management of fourth ventricle compression due to brain metastases.

BACKGROUND: Approximately 20-30% of all intracranial metastases are located in the posterior fossa. The clinical evolution hinges on factors such as tumor growth dynamics, local topographic conditions, performance status, and prompt intervention. Fourth ventricle (V4) compression with secondary life-threatening obstructive hydrocephalus remains a major concern, often requiring acute surgical intervention. We have previously reported on the application of adaptive hypofractionated Gamma Knife Radiosurgery in the acute management of critically located metastases, a technique known to us as rapid rescue radiosurgery (3R). We report the results of 3R in the management of posterior fossa lesions and ensuing V4 decompression. CASE DESCRIPTIONS: Four patients with V4 compression due to posterior fossa metastases were treated with 3R by three separate gamma knife radiosurgical sessions (GKRS) over a period of seven days. Mean V4 volume was 1.02 cm3 at GKRS 1, 1.13 cm3 at GKRS 2, and 1.12 cm3 at GKRS 3. Mean tumor volume during the week of treatment was 10 cm3 at both GKRS 1 and 2 and 9 cm3 at GKRS 3. On average, we achieved a tumor volume reduction of 52% and a V4 size increase of 64% at the first follow-up (4 weeks after GKRS 3). Long-term follow-up showed continued local tumor control, stable V4 volume, and absence of hydrocephalus. CONCLUSION: For this series, 3R was effective in terms of rapid tumor ablation, V4 decompression, and limited radiation-induced toxicity. This surgical procedure may become an additional tool in the management of intractable posterior fossa metastasis with V4 compression.

[Severe aneurysmal dilatation of the left atrium. Apropos of a case]. / Importante dilatation anévrysmale de l'auricule gauche. A propos d'un cas.

The authors report the case of an eleven year old child with aneurysmal dilatation of the left atrial appendage and underline the diagnostic value of Doppler echocardiography in this affectation. The two signs which led to investigation of the patient and the diagnosis of this rare congenital abnormality were bulging of the left heart border on chest X-ray and atrial fibrillation. In addition to visualising a left para-cardiac chamber communicating with the left atrium on transthoracic and transoesophageal echocardiography, colour Doppler confirmed an exchange of blood between the two chambers. This aneurysm was particularly voluminous and contained echos of spontaneous contrast, a possible source of thrombosis. Thoracic CT scan and angiocardiography did not provide any further useful information. The surgical findings confirmed the presence of a large aneurysm of the left atrial appendage and excluded partial agenesis of the pericardium, the main differential diagnosis. The aneurysm was excised under cardiopulmonary bypass, and, in particular, atrial fibrillation did not recur after surgery.

[Evaluation of the degree of mitral stenosis : reassessment of the M mode echocardiographic index]. / Appréciation du degré de la sténose mitrale : réévaluation des index échocardiographiques en mode M.

Fifty consecutive patients (32 female and 18 male) with mitral stenosis aged 11 to 60 years underwent cardiac catheterisation and echocardiography to determine the value of M mode in assessing the degree of stenosis. Mitral stenosis was pure in 47 cases; isolated in 31 cases; associated with minimal aortic regurgitation in 11 cases, with mild mitral incompetence in 3 cases and with tricuspid incompetence in 5 cases (all patients underwent aortography and left ventriculography). Mitral valve surface area (MSA) calculated from the Gorlin formula correlated well with the anatomical mitral valve area (r = 0.88) in the 30 operated patients and enabled the patients to be divided into three subgroups : Group I : 36 patients with severe mitral stenosis; MS less than 1.3 cm2 including 29 with very severe stenosis : MS less than 1.0 cm2; Group II : 9 cases of moderate stenosis (1.3 cm2 less than MS less than or equal to 1.8 cm2), and Group III : 8 cases of mild mitral stenosis (MS greater than 1.8 cm2). The indices correlating with haemodynamic MSA were, in decreasing order of significance : EF slope of early diastolic closure of the anterior leaflet (r = 0.74); maximal EE' diastolic separation of the two leaflets (r = 0.57); the ratio of left atrium/aortic root dimensions (r = 0.39) and the Q-mitral closure interval (r = 0.31). The left atrial emptying index, the mitral valve closure index and changes in the rapid phase of left ventricular filling did not correlate with the degree of stenosis. An EF slope of less than 15 mm/sec had a sensitivity of 77% and a specificity of 93% and was a satisfactory method for distinguishing patients in Group I from those in Group II.(ABSTRACT TRUNCATED AT 250 WORDS)

[Study of cor triatriatum. Apropos of 6 cases]. / Contribution à l'étude du coeur triatrial. A propos de six observations.

The authors report 6 cases of triatrial heart with various and non-specific manifestations and attribute the difficulty of its clinical identification above all to the frequency of associated lesions, which were present in 5 of the 6 cases reported here. An atrial septal defect and left superior vena cava were each seen twice while a ventricular septal defect, sub-valvular aortic stenosis, mitral insufficiency and transposition of the great vessels were each seen once. It was routine echocardiography which led to the discovery of triatrial heart in each of these 6 cases. The value of this investigation in the positive diagnosis of triatrial heart proved, in these patients, to be greater than that of cardiac catheterisation.

[Angina and myocardial ischemia related to a fistula between the anterior interventricular artery and the pulmonary artery in a 56-year-old man]. / Angor et ischémie myocardique liés à une fistule entre l'interventriculaire antérieure et l'artère pulmonaire chez un homme de 56 ans.

The authors report a case of fistula between the left anterior descending artery (LAD) and the pulmonary artery (PA), in which the only clinical expression was angina associated with electrical signs of myocardial ischaemia on exertion. In the absence of any other signs of left-right shunt related to the fistula, the initial diagnosis was that of atherosclerotic coronary artery disease, especially as this 56-year-old man presented three risk factors. Coronary angiography revealed this anomaly and corrected the diagnosis. Surgical closure of the fistula under cardiopulmonary bypass and via a pulmonary artery approach led to resolution of the angina and exertional ischaemia. Similar cases of LAD-PA fistula responsible for angina as the only clinical manifestation and discovered on coronary angiography have been rarely reported in the literature.

[Results of percutaneous mitral dilatation in 11 cases of poorly tolerated mitral stenosis during pregnancy]. / Résultats de la dilatation mitrale percutanée dans 11 cas de rétrécissement mitral mal toléré durant la grossesse.

The authors report the results of percutaneous mitral dilatation (PMD) during pregnancy in 11 cases of tight and poorly tolerated mitral stenosis (MS). This dilatation was performed on average at 22 +/- 5 weeks of pregnancy, protecting the fetus against radiation and using the double balloon technique. Fluoroscopy time, with a mean of 10.5 +/- 2.2 minutes, was greatly reduced in the last five patients by the elimination of cineangiography. Mitral insufficiency was then sought and estimated by colour Doppler. This also avoided iodine overload and its possible effects on the fetal thyroid. Mitral surface area and hemodynamic parameters were very significantly improved by PMD: mean capillary pressure fell from 25.1 +/- 6.2 to 10.7 +/- 4.4 mmHg, mean transmitral gradient from 18.8 +/- 6.2 to 5.9 +/- 2.9 mmHg and mitral surface area increased from 0.9 +/- 0.2 to 2.1 +/- 0.4 cm2 (p < 0.0001). Pregnancy continued under good hemodynamic conditions until delivery which occurred after 8 and a half months, vaginally, in 10 cases. Cesarean section was performed at 8 months in only one patient because of fetal distress not related to the dilatation and with a satisfactory outcome. The 11 children, now with a mean age of 18 months, are all well and free of any functional or organic abnormality. Measurement of thyroid hormone levels in the first 6 also proved normal. In the light of these results, PMD during pregnancy can be considered as an excellent alternative to surgery when it is possible in terms of the anatomical status of the mitral valve.

[Superior vena cava syndrome disclosing dissection of the aorta]. / Syndrome cave supérieur révélateur d'une dissection de l'aorte.

The authors report a case of dissection of the aorta presenting as a superior vena cava syndrome. Because of the rarity of such a manifestation, this dissection was not suspected before CT scan. A review of the literature confirms the rarity of obstruction of the superior vena cava during dissection and indicates that it results from a compressive hemomediastinum. In this case, the superior vena cava was compressed by the ascending aorta itself, which had become very large but had not ruptured into the mediastinum. This forms the basis of the originality of the case.

[Endomyocardial fibrosis in children]. / Fibrose endomyocardique chez l'enfant.

BACKGROUND: Recent advances in imaging techniques can facilitate the diagnosis of endomyocardial fibrosis. CASE REPORTS: Two cases of endomyocardial fibrosis were diagnosed in two Tunisian children, one aged 3 years and the other 12 years. Both were admitted with severe cardiac failure, predominantly of the right ventricule. Two dimensional echocardiography showed an apical amputation with echogenic material plus a thick, dense endocardial image, particularly at the level of the papillary muscles. The older child who also had cardiac catheterization, died 2 years later and the younger child died 4 years after diagnosis. Post mortem examination showed fibroelastic thickening of the endocardium and areas of fibrosis in the muscles of both children. CONCLUSIONS: The advantage of two-dimensional echocardiography over other imaging techniques is that it can confirm the diagnosis of endomyocardial fibrosis and indicate the extent and degree of fibrosis.

[A rare and little known cause of cyanosis: pulmonary arteriovenous aneurysm]. / Une cause rare et peu connue de cyanose: l'anévrysme artério-veineux pulmonaire.

Cyanosis with digital clubbing and a systolic murmur were found in a five and a half-year-old. The usual causes of cyanosis, including congenital heart disease, were discussed. Correct diagnosis was, however, arteriovenous aneurysm of the lung which was seen on the pulmonary angiogram. The lung density on chest films and lack of echocardiographic evidence of congenital heart disease might have suggested this infrequent malformation. Permanent cure was achieved by removing the lower and middle lobes of the right lung.

[Cardiac involvement in mucopolysaccharidosis. Apropos of an echocardiographic study in 8 cases]. / L'atteinte cardiaque dans la mucopolysaccharidose. A propos d'une étude échocardiographique de huit cas.

Of all the storage diseases, mucopolysaccharidosis is the one whose cardiac manifestations are probably the least well known. Clinical and above all echocardiographic findings of heart involvement were studied in 8 patients with mucopolysaccharidosis, including four with Hunter disease. The paucity of clinical manifestations was in sharp contrast with the highly informative echocardiographic results. Valvular dystrophy, usually of the left side of the heart, was the most common anomaly, with five patients affected. Whereas some valvular lesions had no consequences, others led to stenosis or incompetence. Asymmetrical hypertrophy of the septum was found in one patient. No patient had evidence suggestive of vascular involvement, in particular of the coronary arteries.