[Angiosarcoma of the aorta]. / Angiosarcome épithélioïde de l'aorte.

Primary aortic tumors are extremely rare. A 73-year-old woman presented with a 8 kg weight loss associated with abdominal pain. Physical examination was normal. Laboratory tests disclosed increased acute phase reactants. Thoracic and abdominal CT scan showed diffuse splenic and renal hypodense lesions with thrombotic feature of the thoracic aorta extending on 9 cm length. Transesophageal echocardiography showed a large and heterogeneous floating mass advocating a thrombus developed on atheroma. Because of the high risk of embolism the patient underwent surgical replacement of the thoracic aorta. Histopathology revealed an epithelioid angiosarcoma of the aorta. A primary tumor of the aorta should be suspected in the presence of an intra-aortic process presenting features of thrombosis.

[Late complications of a pseudo aneurysm of the left ventricle: thrombus infection and purulent pericarditis]. / Complications tardives d'un faux anévrisme du ventricule gauche: infection du thrombus et péricardite purulente.

Pseudoaneurysm is a rare complication of left ventricle myocardial infarction. Rupture with tamponade and sudden death is the usual outcome. Surgical intervention remains the treatment of choice. Long term survival cases without surgery are rare. Infection of the thrombus is also a possible event. We report the case of a patient with postinfarction left ventricular pseudoaneurysm complicated by infection of the thrombus and purulent pericarditis involving a peptostreptococcus. Infection must be considered a potential complication of left ventricular pseudoaneurysms.

[Endografts (or stent-grafts) and diseases of the descending thoracic aorta]. / Endoprothèses (ou stent-grafts) et pathologies de l'aorte thoracique descendante.

The endovascular treatment of aorta diseases with S-Graft is considered as an alternative to surgery, especially interesting in patients with severe comorbidities. Indeed, the mid-term morbidity and mortality are comparable to surgery in relatively large series, and S-Graft implantation appeared as a safe, less invasive and efficient treatment for different affections of the thoracic aorta. This article reviews technical aspects, indications and results of endovascular repairs of thoracic aorta lesions. We will also assess the advantages and limitations of S-Graft therapy.

Acute traumatic aortic rupture: a comparison of surgical and stent-graft repair.

OBJECTIVE: The study's objective was to comparatively evaluate surgery and stent-graft repair of acute or subacute traumatic aortic rupture. METHODS: A total of 76 patients (14-76 years old; mean, 37 years; male/female ratio, 63/11) with a traumatic aortic injury were admitted to our hospital between 1981 and 2003. Six patients died within 1 to 9 days of another associated severe traumatic lesion. The 70 remaining patients were divided according to the type of rupture repair. In group 1, 35 patients were treated surgically: 28 with immediate repair and 7 with delayed repair (average time interval 66 days, 5-257 days). In group 2, 29 patients were treated with stent grafting of the aortic isthmus. In group 3, 6 patients with minor aortic lesions were treated medically with a close follow-up. RESULTS: In the 28 patients treated surgically in the emergency department, the mortality and paraplegia rates were 21% and 7%, respectively. No death or paraplegia was observed in the group with delayed surgical repair. With stent grafting, complete exclusion of the pseudoaneurysmal sac was observed in all patients. Except for 1 iliac rupture treated during the same procedure, there was no major morbidity or mortality during the mean follow-up of 46 months (13-90 months). No major complication was observed in group 3. CONCLUSIONS: In stable rupture of the aorta, initial conservative treatment is safe and allows management of the major associated lesions. Stent grafting of the aortic isthmus is a valuable therapeutic alternative to surgical repair, especially in patients considered high risk for conventional thoracotomy.

Hypomandibular faciocranial dysostosis: another case and review.

We report on a third case of hypomandibular faciocranial dysostosis and review the literature. Manifestations include craniosynostosis, prominent eyes, deficient midface and zygomatic arches, short nose with anteverted nares, protruding lower face, minute oral aperture, persistent buccopharyngeal membrane, and severe mandibular hypoplasia. In contrast to coronal synostosis found in the 2 earlier cases, our patient had multiple sutural synostosis. The 2 affected sibs reported earlier suggest the possibility of autosomal recessive inheritance. However, gonadal mosaicism for a dominant mutation or an undetected microdeletion must also be considered at this early stage in the delineation of this disorder.

Comparative efficacies of erythromycin-sulfisoxazole and cefaclor in acute otitis media: a double blind randomized trial.

A prospective double blind trial compared the fixed combination of erythromycin-sulfisoxazole (E/S) with cefaclor in the treatment of acute otitis media. One hundred nineteen children in six centers across Canada were studied. Diagnostic tympanocentesis of 134 ears yielded 135 bacterial isolates: Streptococcus pneumoniae (42%); Haemophilus influenzae (21%); Branhamella catarrhalis (10%); Streptococcus pyogenes (5%); and other bacteria (22%). Seventy-seven percent of strains of B. catarrhalis and 14% of strains of H. influenzae were beta-lactamase producers. E/S exhibited greater in vitro activity against H. influenzae and B. catarrhalis. Twenty-three patients had bacteriologically sterile middle ear fluid. The overall clinical outcome at Days 10 and 31 was identical in both treatment groups. Otoscopic findings improved more rapidly in the E/S group than in the cefaclor group at 10 and 31 days (P less than or equal to 0.04). In cases where pre-treatment middle ear fluid was negative on routine bacterial culture, complete cure at 10 days was observed in 75% of patients treated with E/S but only in 14% of those treated with cefaclor (P = 0.02). Side effects were infrequent and comparable between the test drugs. E/S is at least as effective as cefaclor in the management of acute otitis media and may be superior, particularly for cases not yielding bacteria on routine culture.

Long-term effects of dynamic aortomyoplasty.

Aortomyoplasty consists of wrapping the latissimus dorsi muscle (LDM) around the ascending aorta and electrostimulating it during diastole. The ascending aorta will act as an ectopic neo-ventricle compressed during diastole, thus reproducing the effects of long-term diastolic counterpulsation. In 5 goats, the right LDM was transferred to the thoracic cavity after removal of the second rib. The ascending aorta was enlarged by a pericardial patch and wrapped with the LDM. Postoperative electrostimulation was delivered in a counterpulsating manner. Hemodynamic studies were performed at 12 and 24 months postoperatively. Percent increase in the subendocardial viability index (diastolic pressure-time index/systolic tension-time index) was calculated using unassisted and assisted cardiac cycles with the stimulator off versus the stimulator on at a 1:1 ratio in the basal state and after acute heart failure was induced by the administration of high doses of propranolol hydrochloride. Diastolic counterpulsation of the ascending aorta resulted in significant improvement in the subendocardial viability index long term, both in basal state conditions and after induced cardiac failure. During heart failure, aortomyoplasty increased the cardiac output and decreased systemic vascular resistance. Histopathologic studies up to 24 months showed preservation of the histologic structure of the aortic wall and no evidence of thromboembolism. Tight adhesions developed between the aortic wall (including the pericardial patch) and the LDM. The diameters of the enlarged aortas showed no significant differences compared with diameters immediately postoperatively. In conclusion, aortomyoplasty produces chronic diastolic augmentation with preservation of aortic structure. After induction of heart failure, aortomyoplasty offers efficient circulatory support.

Linear nevus sebaceous of Jadassohn.

Though the described lesion is uncommon, it is nevertheless important to recognize for several reasons. Its rapid growth during certain phases makes early recognition and surgical excision mandatory to preclude cosmetic deformity. In addition, its significant tendency for malignant degeneration makes early excision the preferred treatment.

Protection of the spinal cord during surgery of thoraco-abdominal aortic aneurysms.

OBJECTIVE: To assess the risk of ischemic cord injury, we have retrospectively studied the 115 patients who underwent a replacement of the thoracic descending or thoraco-abdominal aorta between January 1980 and December 1994. METHODS: In 72 patients the aortic lesion was located above the diaphragm. The aortic replacement was performed with the aid of extracorporeal circulation in all but 2 patients (97.2%). Only two cases of postoperative paraplegia were observed (2.7%). In 43 patients (10 females and 33 males aged from 26 to 69 years), the occurrence of postoperative paraplegia was considered as a major risk, because of the extension of the aortic lesions (Crawford types I, II and III). Twenty-six patients (60.4%) suffered from chronic dissection and 17 patients had atheromatous aneurysms. Sixteen patients (37.2%) had Marfan syndrome. Twelve patients (27.9%) had already undergone aortic replacement. A preoperative study of the spinal cord vascularization was carried out in 36 patients (83.6%) and the Adamkiewicz artery was visualized in 28 patients (77.8%). In 17 patients (39.5%, group I), the surgical procedure was performed without the aid of extracorporeal circulation. In the remaining 26 patients (60.5%, group II), the surgical procedure was carried out with the aid of cardiopulmonary bypass and profound hypothermic circulatory arrest. Sequential unclamping of the aorta was used in all patients. The cord vascularization was surgically restored in 32 patients (74.4%). When the Adamkiewicz artery was identified, the critical intercostal artery was reimplanted together with the two pairs of adjacent intercostal arteries (25 patients). When the origin of the Adamkiewicz artery remained unknown, the two or three most important patent pairs of intercostal arteries were reimplanted (7 patients). In 8 patients (18.6%) there were no patent intercostal arteries. RESULTS: Hospital mortality accounted for 37.2% (16 patients, including 5 patients with paraplegia). On univariate analysis, extension of the aortic lesions, emergency and redo surgery were the only significant risk factors of mortality (P = 0.05). Cord ischemia was observed in 9 patients (21%): permanent paraplegia in 7 patients (16.2%) and transient medullar disturbance in 2 patients (4.6%). The occurrence of paraplegia was reduced, though not significantly, in group II (16%) vs group I (29%) and in patients with preoperative assessment of the cord vascularization (18% vs 38%). CONCLUSIONS: In our experience: 1) The risk of paraplegia is related to the extension and the type of the aortic lesions. 2) The preoperative study of the medullar vascularization and the use of extracorporeal circulation with deep hypothermia and sequential aortic unclamping, reduce the risk of severe cord ischemia, and 3) Occurrence of postoperative paraplegia depends on several factors and cannot be totally prevented by the surgical technique.

Dominant recurrent ataxia and vertigo of childhood.

Four families are described with an autosomal dominant illness characterized by the childhood onset of recurrent attacks of prolonged ataxia, server vertigo, and vomiting. The attacks often begin in infancy. On the average, attacks occur monthly, and last between one hour to more than a week. Variations in severity occur within families. During an attack, consciousness is unaltered, but severe vertigo makes walking impossible and vomiting is frequent and severe. An attack is marked by horizontal and vertical jerk nystagmus, accompanied by vertigo which is sometimes worsened by position; however, there is no muscular weakness. During an attack, blood gases, ammonia, and amino acid studies are normal. Between attacks patients manifest combinations of slight horizontal or vertical jerk nystagmus or mild clumsiness. Cochlear and labyrinthine studies and neurologic investigations were noncontributory. Conventional therapies for vertigo, epilepsy, and migraine were ineffective, but acetazolamide (250-500 mg/day) stopped the attacks.

Long-term of patients receiving an aorto-bi-femoral prosthesis for atherosclerotic occlusive disease of the aortic bifurcation.

Over a 14-year period (1975-1989), 547 patients with a mean age of 57 years received an aorto-bi-femoral prosthesis for atherosclerotic occlusive disease (AOD) and underwent regular follow-up over a 13-year period. Immediate post-operative mortality was 2.5% with few deaths due to cardiovascular causes (0.5%). The long-term mortality was significantly elevated at 5 years (10% higher than controls) and at 10 years (16%). These deaths were due to the AOD and its surgical treatment (10%), other cardiovascular disease (30%) and cancer (41%). Approximately 5% of patients required an immediate post-operative, or delayed amputation, compared to 33% which had a threatened limb before the operation. Thrombosis of the prosthesis was relatively rare. At 5 years, 81% of the survivors remained largely improved; at 10 years, 67% and at 13 years, 62%. The benefit of this intervention is therefore unquestionable with regards to the functional state and survival. Also, in avoiding immobility due to the disabling intermittent claudication or amputation, it considerably improves the quality of life which largely compensates for the low mortality rates and subsequent complications of the prosthesis.

[Long-term results of aorto-bifemoral prostheses in the surgery for atheromatous stenosis of the aortic bifurcation]. / Résultats à long terme des prothèses aorto-bifémorales dans la chirurgie des sténoses athéromateuses du carrefour aortique. 700 malades suivis pendant 20 ans.

Seven hundred patients operated consecutively by the same surgical team for atheromatous stenosis of the aortic bifurcation were followed up for 20 years with only 5 patients lost to follow-up. There were 94.5% of men with a mean age of 58 years. The operative mortality was 2.7% with few deaths due to true cardiovascular causes (0.7% of patients). The secondary mortality was very high with two main causes: cancer (39% of patients) and cardiovascular diseases (37%). Other causes were responsible for only 24% of deaths. The principal complications of the prostheses were: infection (0.6% of operated patients), thrombosis (6.7%), pseudo-aneurysm of the aorta (1.57%) and pseudo-aneurysm of Scarpa's triangle (4%). The benefits of surgery are unquestionable both on terms of survival, as amputation, bed confinement and invalidity are avoided, and in terms of function, as amputation was avoided in 84% of the 32% of patients in Stages III or IV before surgery. Only 5% of patients were amputed during the observation period. Moreover, 79% of survivors had a good functional result at 15 years. Improvement of results depends on better hygienic measures, systematic screening for high risk cancers and a better management of the arterial disease with early treatment of other arterial diseases (coronary, carotid) in order to reduce postoperative and mostly medium- and long-term cardiovascular mortality.

[Aortofemoral bypass: gold-standard or outdated?]. / Le pontage aorto-bifémoral: traitement de référence ou intervention du passé?

Although aorto-bifemoral bypass procedures have proven efficacy for the treatment of aortoiliac occlusion, complications have led to a preference for less invasive interventions. A precise knowledge of this morbidity is thus necessary to evaluate and compare outcome with alternative techniques. The purpose of this study was to analyze the course of complications observed in a large group of patients who recently underwent aorto-bifemoral bypass performed by the same team. Between 1975 and 1996, 720 patients underwent aorto-bifemoral bypass procedures for occlusion. Indication for surgery was invalidating claudication in 68%, pain at rest in 28% and trophic disorders in 4%. Mean follow-up was 9.6 years. Twenty-three patients were lost to follow-up. One hundred sixty cases of prosthesis complications were recorded (21.3% of patients): ten infections (1.3%), 14 false aortic aneurysms (2%), 40 false femoral aneurysms (5.5%), 82 prosthetic thrombotic events (11.2%), and 14 femoral anastomotic strictures (1.9%). These complications led to death in nine patients (1.2%) and amputation in 23 (3.2%). Analysis of the results show that late mortality attributable to the prosthesis was minimal compare with other causes of death. Numerous complications occur late after prosthetic repair but their impact on mortality and amputations is limited. Aorto-bifemoral bypass is a safe technique that remains the gold standard for evaluation of other revascularization methods.

[Surgical repair of postinfarction left ventricular lesion type I]. / Riparazione chirurgica della lesione postinfartuale di I tipo del ventricolo sinistro.

Left ventricular rupture is the most frequent cause of death following myocardial infarction after ventricular arrhytmias and cardiogenic shock. Under these circumstances, only a prompt diagnosis and urgent surgical treatment can be lifesaving. A review of the literature is made and a simple surgical technique with GRF glue application is presented.

[Transhepatic percutaneous embolisation of a post-traumatic pseudoaneurysm of hepatic artery]. / Embolisation transhépatique percutanée d'un pseudoanévrisme post-traumatique de l'artère hépatique.

Pseudoaneurysm of the hepatic artery is a rare complication of blunt abdominal trauma. We report a case of post-traumatic pseudoaneurysm diagnosed several months after the initial traumatism in a 18-year-old man who presented recurrent abdominal pain. This pseudoaneurysm was successfully treated by association of both classical endovascular treatment and transhepatic percutaneous embolization.

[Circulatory assistance through cardiomyoplasty and aortomyoplasty. Experience and first clinical results]. / Assistance circulatoire par cardiomyoplastie et aortomyoplastie. Expérience et premiers résultats cliniques.

The finding that skeletal muscles can be made resistant to fatigue by progressive electrical stimulation has been used as a means of providing circulatory support in cardiac surgery. The first application of this discovery was dynamic cardiomyoplasty, performed for the first time in man in 1985 at the Broussais Hospital, Paris. The latissimus dorsi muscle is transposed into the thorax, then attached around the heart and finally stimulated synchronously with the ventricular systole. So far, more than 200 patients in the whole world (including 57 at the Broussais Hospital) have undergone this operation with results that are increasingly encouraging. In these cases the muscle is used to reinforce or replace the left or right ventricle, but other applications are being studied, such as double cardiomyoplasty (left latissimus dorsi and right pectoralis major muscles), cardiomyoplasty of the right atrium and aortomyoplasty which produces aortic counterpulsation. The development of these techniques underlines the ever growing interest raised by this type of autologous circulatory support.

[Indication of endovascular stent grafts for traumatic rupture of the thoracic aorta]. / Place des endoprothèses dans le traitement des ruptures traumatiques de l'aorte thoracique.

OBJECTIVE: The usual treatment of traumatic aortic rupture (TAR) is surgical. This invasive technique necessitating thoracotomy and ECC is associated with a mortality rate of more than 20% and a paraplegia risk of about 10%. New minimally-invasive techniques (aortic stent-grafting) are emerging as less risky alternatives to surgery. We report our experience in the percutaneous treatment of TAR with stent-graft via a surgical femoral cut-down. PATIENTS AND METHODS: Between 1996 and 2002, 23 patients (16-65-year-old, mean 36 years) were treated by thoracic stent-grafting. An informed consent was obtained for every patients. Thirteen patients had an acute or sub-acute TAR (1-8 months, mean 5 months) and five patients had chronic TAR (13-24 years, mean 17 years). The technique was done under general anaesthesia and each patient received a preoperative blood-pressure reduction treatment. During the procedure, anticoagulation (heparin) was given and hypotension was induced when the stent-graft was deployed. Direct positioning control was obtained by means of TEE. RESULTS: Eighty percent of patients were extubed immediately after the procedure. Bleeding was <150 ml. The primary success rate was 100% with one minor type 2 endoleak that was spontaneously resolved after 2 months. There was no case of mortality or paraplegia. There were three minor complications (17%), two haematomas at the arteriotomy site and one inflammatory syndrome characterised by slight fever, raised biological markers but with negative blood culture. CONCLUSION: Percutaneous aortic stent-grafting for TAR is a minimally-invasive technique, which constitute an interesting alternative to surgery. It only necessitates a femoral surgical cut-down compared to the thoracotomy and ECC associated with surgery. The complication rate is low and no mortality or major complication was encountered in our patients. Eventually, the long-term follow-up will allow a widening of indications.

[Minimal craniocerebral trauma]. / Das minimale Schädelhirntrauma.

The patient with mild head injury is the most frequently hospitalised trauma patient. The costs for this treatment are enormous. Guidelines for managing the patients are changing for the last 20 years. Haematoma rates of 10% have been shown with CT scans in prospective studies for patients with GCS 14/15 and normal neurological examination. One out of ten of these patients had to undergo craniotomy. CT scans have shown to reduce costs if done on all patients with mild head injury and discharged with normal findings. Patients with skull fractures and age over 65 years are at higher risk, but not patients with loss of consciousness and post traumatic amnesia. We suggest CT scans on all patients. If a CT scan is not available we recommend to observe the patient for 24 hours in the hospital. If a patient with GCS 15 is to be discharged, an information leaflet with instructions for surveillance at home should be given to the patients and to the care taker.

Prospective study of a panfungal PCR assay followed by sequencing, for the detection of fungal DNA in normally sterile specimens in a clinical setting: a complementary tool in the diagnosis of invasive fungal disease?

We prospectively analyzed 34 clinical biopsy samples from 23 patients with a suspected invasive fungal infection by fungal culture, histology and a panfungal PCR followed by sequencing. Results were compared to the composite diagnosis according the European Organization for Research and Treatment of Cancer (EORTC) criteria. In 34 samples, culture, histology and panfungal PCR were positive in 35%, 38% and 62%, respectively. On the sample level the panfungal PCR revealed a sensitivity of 69% and a specificity of 62.5% compared to proven IFI according postoperative EORTC criteria. On patient level, the sensitivity of the PCR approach was 100%, specificity 62.5%.