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Biomedical subjects

P Bret

Publications and source records attributed to P Bret.

At least 55 records · Page 3Linked to original sources

[Renal and adrenal involvement in von Hippel-Lindau disease: clinical features and therapeutic strategies].

OBJECTIVES: We report our experience of renal and/or adrenal manifestations of von Hippel-Lindau disease and propose a practical approach. METHODS: Eight patients (mean age: 43 years) presented with predominant renal and adrenal lesions in 6 cases and 2 cases, respectively. RESULTS: All patients are alive with a mean follow-up of 8.1 years. A local recurrence after partial nephrectomy was observed in two cases. Two patients are in renal failure and are treated by dialysis and two patients require hormone replacement therapy for adrenal insufficiency. CONCLUSIONS: The predegenerative nature of simple renal was not observed. Conservative renal surgery is adapted to small renal tumours, with a low cytological grade and without any distant lesions in the same kidney. Radical nephrectomy is reserved for large lesions (greater than 5 cm) with a high cytological grade. The presence of pheochromocytoma must be systematically excluded. Preservation of the adrenal gland in the case of homolateral renal surgery for cancer is recommended. The reliability of the genetic test allows early diagnosis of this disease.

Adrenal Gland Diseases↗

Chronic ("normal pressure") hydrocephalus in childhood and adolescence. A review of 16 cases and reappraisal of the syndrome.

"Normal pressure" hydrocephalus (NPH) is generally considered to be a disorder of the adult and geriatric population. Only a few reports have described the possible occurrence of this condition in children. A series of 16 patients aged less than 20 years forms the basis of the present report. Among these 16 patients, 11 had a clearly identified etiologic factor and 7 had had a shunt previously implanted. The majority of patients exhibited at least two elements of the adult's triad of psychomotor retardation (14 cases) and/or psychotic-like symptoms (4 cases), gait anomalies (8 cases), and sphincter disturbances (3 cases). Six patients had their intracranial pressure (ICP) monitored. ICP values were estimated to be within the normal limits for age. All the 16 patients underwent shunting or shunt revision. Surgical results were as follows (mean follow-up 20 +/- 17.2 months): a good response to shunting was obtained in 12 cases ("cured": 5, improved: 7), while the other 4 patients failed to improve. It seems likely that associated parenchymal disorders have played a major role in therapeutic failures. In children showing ventricular dilation on computed tomographic (CT) analysis and a clinical picture of subtle psychomotor deterioration, it may be difficult to distinguish an active disorder of the CSF dynamics from "arrested hydrocephalus." Since intracranial manometry cannot be undertaken as a routine procedure, less invasive methods such as cerebrospinal fluid (CSF) tap test, psychometric, or urodynamic tests deserve special attention as reliable predictors of outcome after shunting. Because most patients undergo shunting without prior assessment of their CSF pressure, the term "chronic hydrocephalus" is proposed as an alternative designation to "NPH," since there is little argument for maintaining an instrumentally based definition of the syndrome.

Adolescent↗

Dural arteriovenous fistula of the posterior fossa draining into the spinal medullary veins--an unusual cause of myelopathy: case report.

In a 31-year-old male patient suffering from progressive myelopathy, a right carotid angiography disclosed an intracranial tentorial arteriovenous fistula (AVF) draining intrathecally into the spinal medullary veins. An embolization via the afferent meningohypophyseal artery was not technically feasible, and a microsurgical excision of the AVF was accomplished via a suboccipital approach, resulting in the angiographic cure of the fistula and progressive relief of the myelopathy. Fourteen reported cases of intracranial dural AVF draining intrathecally are reviewed. In most patients, authors encountered diagnostic difficulties similar to those noted in this case. Surgical and/or endovascular therapeutic methods have provided disappointing results, likely attributable to a prolonged course of spinal cord dysfunction. Pathophysiologically, a mechanism of venous congestion of the cord seems to be involved, as acknowledged by several magnetic resonance and angiographic studies. In patients who appear to have a clinical and myelographic picture of "vascular" myelopathy and who exhibit negative spinal angiography, a four-vessel cerebral angiography should be undertaken, aiming at the recognition of an intracranial AVF.

Adult↗

[Sphenoido-nasopharyngeal craniopharyngioma. A case with radical excision by Le Fort I-type maxillotomy].

A case of infra-sellar (sphenoidal nasopharyngeal) craniopharyngioma is reported. A 16 year-old girl presented with progressive nasal obstruction. CT and MR images showed a heterogeneous tumor originating from the sphenoid bone with no intra-cranial or supra-sellar extension. On a biopsy specimen, the histologic examination was consistent with a craniopharyngioma. The tumor was successfully removed via an inferior route using a Le Fort I maxillotomy. Plates and screws were used to achieve osteosynthesis. The post-operative course was uneventful and there was no residual tumor on post-operative CT. Craniopharyngiomas with strictly infra-sellar development are very rare. Up to now, 19 cases have been reported in the literature. This condition likely results from growth of embryonic remnants lying along the craniopharyngeal duct. Several surgical approaches have been proposed, most of those giving poor exposure to the clival and lateral tumorous extensions. Of particular interest in the authors' experience was the use of a Le Fort I maxillotomy which provided optimal exposure to the skull-base and facilitated radical excision. CT and MR studies have proved of upmost importance in delineating the area of bone to be removed at operation.

Adolescent↗

Transvaginal intratubal methotrexate treatment of ectopic pregnancy.

OBJECTIVE: To evaluate the efficacy of transvaginal intratubal methotrexate (MTX) treatment of tubal ectopic pregnancy (EP). SETTING: Outpatient setting in University Hospital. PATIENTS: Forty women with early EP and rising serum beta-human chorionic gonadotropin (beta-hCG) levels. INTERVENTION: Transvaginal intratubal administration of MTX (1 mg/kg body weight). MAIN OUTCOME MEASURES: Success was defined as declining serum beta-hCG to undetectable levels, no tubal dilatation on ultrasound examination, and no further intervention was required. RESULTS: Treatment was associated with a 70% success rate. No difference was found in the success rate between women with an embryo (76.9%) and those with no embryo in their fallopian tubes (66.7%). The initial serum beta-hCG levels were also not different between patients who were successfully treated and those who failed to respond to the treatment. Despite declining serum beta-hCG levels, tubal rupture occurred in two patients. CONCLUSIONS: Treatment of EP by transvaginal MTX administration is associated with a 70% success rate. This is independent of the presence of an embryo or the initial serum beta-hCG levels. Rupture of EP can still occur despite low and declining serum beta-hCG levels.

Adult↗

Cerebral metastases as first symptom of bronchogenic carcinoma. A prospective study of 37 cases.

Among the patients showing evidence of cerebral metastases without previously known cancer history, lung cancer has been found 37 times as the primary tumor in our institution. There were 34 men and three women and all but two were heavy smokers. Only one presented at diagnosis with thoracic symptoms but the chest radiograph was abnormal in 34. The histologic type of the primary tumor was obtained in 32 cases as a result of thoracic investigations and in five cases from metastatic tumor tissue. The primary tumor appeared to be non-small cell lung carcinoma in 26 cases and small cell lung carcinoma in 11 cases. These results show that patients treated with surgery (20 cases) have a better survival (median 10 months versus 4.5) than the others, and among surgically treated patients only those treated with bifocal resection (eight patients) are long-term survivors. Also, in four of six patients, objective regression of the neurologic symptoms was seen after radiation therapy alone. Central nervous system relapse was seen in 12 patients, but in none of the patients treated with postoperative radiation therapy. Conventional chemotherapy (11 patients) induced objective responses only in the small cell type and proved to be too toxic when used simultaneously with radiation therapy in inoperable patients.

Adult↗

[Meningioma of the apex of the tentorial incisura. Diagnostic and surgical aspects apropos of a series of 7 cases].

Seven cases of meningiomas implanted on the medial portion of the free edge of the tentorium are reported. The denomination "meningiomas of the apex of the tentorial incisura" is proposed. In 4 patients, the clinical presentation was that of an intracranial hypertension without signs of compression of the quadrigeminal plate involvement. In the other 3 patients, the initial symptoms were those of the triad of the adult chronic hydrocephalus (N.P.H.) syndrome. Angiographic evaluation provides useful informations on the arterial supply (which is mainly from the tentorial branches of the internal carotid arteries and occasionally from the middle meningeal arteries) and the relationships of the tumor with the deep venous system. C.T. and M.R.I. facilitate earlier diagnosis and better demonstration of implantation and extension of the tumor, which the selection of a surgical approach is based upon. Four patients have undergone an occipital supratentorial approach and 2 a supra-cerebellar infra-tentorial approach. The meningioma was totally removed in 5 patients, sub-totally in 1 to avoid bilateral lesions of the visual pathways and cortical areas. The 7th patient was treated by C.S.F. shunt alone. No operative mortality occurred. The follow-up is averaging 18 months: 4 patients are symptom-free, 3 exhibit minor sequelae (epilepsy: 1, visual field defects: 2, memory impairement: 2). Six patients of the series have resumed their previous activities. Discussion is focused on the difficulties of surgical removal of this particular type of tentorial meningioma, resulting from volume, implantation and relationships of the tumor with the deep veins.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[A method of barium transit in the small intestine in children].

The authors describe and illustrate their method of barium small bowel examination in children. This method utilizes a rapid repletion of the small bowel with diluted barium, and a careful palpation of each intestinal loop under radioscopic supervision with a Holzknecht's palpator.

Age Factors↗

[Value of barium transit of the small intestine in research on Meckel's diverticulum].

Twelve children were diagnosed as having a bleeding Meckel diverticulum upon laparotomy. Before surgery, barium small bowel examination was performed according to the Bret technique (J Radiol Paris [1980] 61, 753-758) in 8 cases; the Meckel diverticulum being apparent in all 8 cases. By contrast, a classical technique of barium small bowel examination used in 4 cases, and 99m TC pertechnate scan performed in 7 cases, yielded only negative results. We conclude that barium small bowel examination, when performed according to the Bret's technique, is a very effective method for the diagnosis of Meckel diverticulum.

Adolescent↗

[Chronic hydrocephalus in adults].

The aim of this study is to sum up our current knowledge on the so-called "normal pressure hydrocephalus" syndrome, revisited by the authors as the "adult chronic hydrocephalus" syndrome (A.C.H.). It is based upon 1) a review of the literature dealing with the subject (518 references) and 2) an original series of 243 cases summarizing a 10 year experience of 2 french neurosurgical teams (Lyon: 123 cases, Clermont-Ferrand: 120 cases). Part I is devoted to the review of pathologic and pathophysiologic data. Both autopsy and microscopic findings clearly show 2 categories of anomalies in the brains of patients showing this condition: "non specific" lesions may be either causative (leptomeningeal thickening, villositar sclerosis) or secondary to hydrocephalus (ependymal wall disruption, sub-ependymal "edema"). Conversely, "specific lesions" are either degenerative (Alzheimer-type) or ischemic in origin. They seem unrelated to hydrocephalus, but may play a role in the ventricular enlargement by favoring changes in brain elastic properties. Data available from brain biopsies (13 cases) have confirmed the high incidence of such "specific lesions" of the parenchyma. Following the Hakim's initial hypothesis, several mechanisms have been proposed to explain the progressive ventricular dilation despite a fall in C.S.F. pressure. Experimental and clinical data (namely those referring to the pathogenesis of secondary A.C.H.) are reviewed. The sequence of events following acute ventricular obstruction is analysed. The ultimate state of chronic uncompensated hydrocephalus involve multiple factors among which a trans-cerebral mantle pressure gradient may play a major role. The intervention of compensatory mechanisms (reduction of C.S.F. production, establishment of alternative pathways of C.S.F. absorption) is also discussed. Part II is devoted to the presentation of the clinical material. Series of the literature including more than 20 surgical cases are briefly reviewed together with the authors' material and methods. Criteria of selection of patients, methods of clinical evaluation and of statistical analysis of results are exposed. Of the 243 patients of the present series, 225 were managed by C.S.F. shunt (V.A.: 137, V.P.:60, L.P.:28). Results were as follows (mean follow-up:31 mths +/- 28): good results (including excellent results): 128 (52.6%), fair: 52 (21.3%), poor and aggravated: 49 (20%), early deaths: 14 (5.7%), 12 of those post-operatively. The rate of surgical complications was 35% (S.D.H.: 16.8%, sepsis: 4%, seizures: 4%, shunt malfunction: 10.6%) leading to reoperation in 25.7%. Long-term survival rate was 75% (172 alive patients and 57 late deaths).(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗