[Unusual fetal teratoma presenting as a posterior mediastinal cyst]. / Présentation atypique d'un tératome fœtal du médiastin postérieur.

Mediastinal cysts are uncommon prenatal findings. As isolated and non-compressing entities, they don't compromise the course of gestation. Massive lesions can compress vital structures, resulting in fetal demise. Thus, close follow-up with sonographic monitoring is recommended until birth. Non-hydroptic fetuses can be managed expectantly. Definitive etiology is known after surgical resection only. We present the first case of posterior mediastinal teratoma associated with severe vertebral abnormalities. After CT scan and fetal MRI, medical termination of pregnancy was decided. Histological examination revealed an immature teratoma. With this unique case report, we discuss the optimal prenatal management of mediastinal cysts.

Management of blunt tracheobronchial trauma in the pediatric age group.

INTRODUCTION: Tracheobronchial rupture (TBR) due to blunt chest trauma is a rare but life-threatening injury in the pediatric age group. The aim of this study was to propose a treatment strategy including bronchoscopy, surgery and extracorporeal membrane oxygenation (ECMO) to optimize the emergency management of these patients. METHODS: We reviewed a series of 27 patients with post-traumatic TBR treated since 1996 in our pediatric trauma center. RESULTS: Seven cases had persistent and large volume air leaks. Flexible bronchoscopy was performed in cases of persistent or large volume air leaks. It permitted accurate visualization of the rupture and its extent. It allowed for a clear-cut positioning of the endotracheal tube. Five were managed operatively. Four cases were considered to be life-threatening because of the combination of severe respiratory distress with hemodynamic instability. One of them had severe tracheal laceration and died. Another one had bilateral bronchi disconnection. Based on clinical and endoscopic findings, surgical repair was performed using extracorporeal membrane oxygenation as a ventilatory support. It provided quick relief from the injury, which was previously expected to result in a fatal issue. CONCLUSIONS: Prompt diagnosis and accurate management of surviving patients admitted to emergency rooms are necessary. Bronchoscopy remains a critical diagnosis step. Surgery is warranted for large tracheobronchial tears and ECMO could be beneficial as supportive therapy for selected cases.

[Pulmonary sequestration and enteric cyst, 2 expressions of the same abnormality]. / Séquestration pulmonaire et kyste entérique, 2 expressions d'une même anomalie. Illustration par un cas.

We report the case of an 18-month-old boy operated on for a right lower lobe bronchopulmonary sequestration. At the immediate postoperative check-up, a septic right thoracic effusion appeared, connected to a cystic mediastinal retrocrural formation on CT. After a drainage attempt and medical therapy, the abdominal lesion was resected. Histological examination showed that the cyst cavity was lined with pseudostratified non-ciliated epithelium, without cartilage, consistent with an enteric cyst. Regardless of the embryological theory, a literature review confirmed that in presence of one of these two lesions, one should systematically look for the other.

Long-term outcomes following repair or replacement in degenerative mitral valve disease.

BACKGROUND: We studied whether mitral valvuloplasty (MVP) was superior to mitral valve replacement (MVR) in patients with degenerative mitral regurgitation (MR), and analyzed the independent risk factors for survival and reoperation. METHODS: 326 patients with degenerative MR underwent MVP (n = 241), mitral valve replacement (MVR) (n = 78) or emergent MVR due to failure of repair (EMVR). Clinical data were analyzed retrospectively. RESULTS: Thirty-day mortality was lower after MVP (2.5 %) compared to MVR (9.0 %) ( P < 0.05). Late survival at 1 and 5 years in the MVP group was 94.4 % and 84.3 % versus 80.4 % and 64.6 % in the MVR group ( P < 0.05), respectively. After adjusting the baseline characteristics by the propensity score method, a significant survival benefit was found for patients who underwent MVP. Multivariable analysis showed that MVR was an independent predictor of thirty-day mortality and survival. There was no significant difference in thirty-day mortality and survival between the EMVR and MVR groups. The need for reoperation was not significantly different between the MVP and MVR groups. In the MVP group, the risk factors for survival and reoperation were identified. CONCLUSIONS: MVP is superior to MVR for the treatment of degenerative MR despite the impact of repair failure. Age less than 60 years, ring size to body surface area greater than 19.0, absence of a prosthetic ring and residual MR at the end of surgery (≥ 1/4) reduce the durability of MVP.

Liver transplantation with reconstruction of the inferior vena cava for hepatocarcinoma on chronic Budd-Chiari: a case report.

Management of Budd-Chiari syndrome, from simple medical treatment to liver transplantation, depends on the acute and chronic evolution of the disease and on the degree of hepatic insufficiency. Herein we have reported the case of a man who underwent transplantation after evolution of a Budd-Chiari syndrome with membranous obstruction of the vena cava and developed 2 lesions of hepatocellular carcinoma. Surgery was difficult due to previous procedures requiring reconstruction of the supra-hepatic vena cava. This case emphasized the timing of liver transplantation versus other treatments to decrease the operative risk.

[Combined surgery of acute type A aortic dissections by ascending aorta replacement and bare stent]. / Chirurgie combinée des dissections aiguës de type A, par remplacement de l'aorte ascendante et endoprothèse non couverte.

UNLABELLED: Aim. After surgical treatment of type A aortic dissections a long segment of these aortas often remain dissected. Our goal was to analyse feasibility and first clinical and pathophysiological results of a combined treatment by ascending aorta replacement and stenting of the arch or descending aorta with Djumbodis(R) bare stents. PATIENTS AND METHODS: Twenty two cases from two centres were analyzed (Universitary Hospital of Parma and Rangueil Universitary Hospital of Toulouse). RESULTS: All the stents have been implanted with short times of circulatory arrest. Average follow-up was 278 days (0-2005). There were two peroperative deaths (9.1%). One year cumulate survival rate was 72.7%. Postoperative complications were mainly respiratory and renal. We have shown a reduction in number of perfused false lumen for aortic arches, more often stented, than for descending aortas (p=0.0104), and for dissected and stented segments versus dissected unstented segments (p=0.0083). CONCLUSION: Our study demonstrates feasibility of this combined procedure and its positive effect on pathophysiologic evolution. Long term results have to be evaluated, but we think promising to extend this treatment to the whole dissected aorta.

[Transposition of the great arteries with pulmonary valvular stenosis and intact interventricular septum. Discussion of management strategies with respect to one case]. / Transposition des gros vaisseaux avec sténose valvulaire pulmonaire à septum interventriculaire intact. Discussion des enjeux thérapeutiques à propos d'un cas.

The authors report the case of a neonate with transposition of the great arteries (TGA) with pulmonary stenosis (PS) and intact Interventricular septum. The child was born at full term without a prenatal diagnosis with cyanosis developing at the 12th hour. After emergency atrioseptostomy, detransposition was performed on the 6th day. Commissurotomy and shaving of the much thickened bicuspid pulmonary valve was also performed. The postoperative course was complicated by a non-compliant left ventricle. After two months, myocardial ischaemia and persistent obstruction of the outflow tract led to severe cardiac failure and the death of the child. TGA with PS and intact interventricular septum is a very rare cardiac malformation. The prognosis is closely related to the anatomy of the pulmonary valve and the impact on the left ventricle. The therapeutic options are complex and are discussed in relation to this case.

Optimal pacing in congenital complete atrioventricular block of immunological origin: interest of multisite stimulation.

An infant with a congenital auriculoventricular block (CAVB) of immunological origin was diagnosed prenatally. The mother had Gougerot-Sjögren disease with positive anti-Sjogren's Syndrome A (SSA) and Sjogren's Syndrome B (SSB) serologies. Cardiac pacing was necessary and the epicardial route was chosen. Considering the left ventricular (LV) dilatation, bi-ventricular (BiV) stimulation was preferred to the usual DDD mode, presumed to have a deleterious long-term effect. Echographic parameters were better with BiV stimulation: the asynchronism induced by mono-RV stimulation was corrected and the QRS complexes were narrower. BiV pacing of a CAVB with LV dilation looks clinically and echographically attractive but needs to be validated in the long term.

Epicardial catheter ablation of ventricular tachycardia using surgical subxyphoid approach.

We report the case of a patient presenting with a previous inferior myocardial infarction complicated by incessant monomorphic ventricular tachycardia resistant to antiarrhythmic drugs. Because endocardial catheter ablation failed and because of focal endocardial activation arising from the left ventricular inferior wall, an epicardial location of the reentry circuit was suspected. Catheter mapping of the pericardial space through a surgical subxyphoid approach performed in the electrophysiological laboratory confirmed the epicardial location of the arrhythmogenic substrate and allowed us successfully to ablate and cure the patient. Surgical subxyphoid approach can be performed in the electrophysiological laboratory when epicardial ablation is needed in case of inadvisable, difficult, or failed non-surgical percutaneous access.

Endovascular treatment of thoracic dissection.

Type A aortic dissection remains fatal if untreated. Although classical medical therapy for type B dissection is considered the therapy of choice in uncomplicated cases, the paradigm is changing as greater experience is accrued with endovascular treatments and technical advances improve the long-term outlook. Diagnosis is also becoming more sophisticated, allowing greater appreciation of the anatomy of dissections and improving the knowledge base as their natural history is assessed.

[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.

[Cardiac cellular therapy: from cells to the first clinical uses]. / Thérapie cellulaire cardiaque: des cellules aux premières indications cliniques.

Despite the improvement in revascularisation techniques, coronary artery disease remains the principal aetiology of cardiac failure in developed countries. The therapeutic management of cardiac failure has been improved over recent years, yet cardiac failure is still associated with significant morbidity and mortality. As cardiac transplantation lacks donors, techniques that allow myocardial regeneration represent an attractive alternative. To date, several types of cells are under study and are suitable for implantation into infarcted myocardium (myoblasts, medullary stem cells...). Following good preclinical study results, the first human cell therapy trials, using the intramyocardial route, have begun, in the course of aorto-coronary bypass surgery in patients with chronic ischaemic cardiopathy and little altered left ventricular function, and then in those with ventricular dysfunction. Different modes of administration of these cell therapy products are under study and could be envisaged in clinical situations such as just after infarction in order to improve ventricular remodelling with an intracoronary injection technique. As for every new treatment, there are numerous problems to resolve, from understanding the relevant mechanisms of cellular transplantation, to the secondary effects that it could entail. Nevertheless, cardiac cellular transplantation is expanding rapidly and with the evolution of techniques it allows a glimpse of a new field of treatment for cardiac failure.