Survival and health in liveborn infants with transposition of great arteries--a population-based study.

OBJECTIVE: To describe treatment, survival, and morbidity for liveborn infants with isolated transposition of great arteries (TGA). DESIGN: Population-based data from 7 European registries of congenital malformations (EUROCAT). RESULTS: Ninety-seven infants were diagnosed with isolated TGA and livebirth prevalence was 2.0 per 10,000 livebirths. The majority of infants were treated with prostaglandins (83%) and 57% had a catheter atrial septostomia performed. Arterial switch surgery was performed in 78 infants, other or unknown type of surgery was performed in 3 cases, and for 6 infants there was no information on surgery. At 1 year of age 69 infants were alive (71%) and 24 (25%) were dead (4 unknown). There were 10 deaths before surgery and 58% of all deaths took place during the first week. There was no statistically significant regional difference in mortality. Eight infants diagnosed prenatally all survived to 1 year and only 71% of infants diagnosed after birth survived (P = 0.08). Data on morbidity at 1 year of age was available for 57 infants. Fifty-one infants were reported with normal health and development. CONCLUSIONS: In this population-based study survival for liveborn infants with TGA is lower than in studies published from tertiary centers. Outcome for survivors at 1 year of age seems favorable.

Local transient myocardial liposomal gene transfer of inducible nitric oxide synthase does not aggravate myocardial function and fibrosis and leads to moderate neovascularization in chronic myocardial ischemia in pigs.

Microcirculation (2010) 17, 69-78. doi: 10.1111/j.1549-8719.2010.00002.x Abstract Background: This study was designed to explore the effect of transient inducible nitric oxide synthase (iNOS) overexpression via cationic liposome-mediated gene transfer on cardiac function, fibrosis, and microvascular perfusion in a porcine model of chronic ischemia. Methods and Results: Chronic myocardial ischemia was induced using a minimally invasive model in 23 landrace pigs. Upon demonstration of heart failure, 10 animals were treated with liposome-mediated iNOS-gene-transfer by local intramyocardial injection and 13 animals received a sham procedure to serve as control. The efficacy of this iNOS-gene-transfer was demonstrated for up to 7 days by reverse transcriptase-polymerase chain reaction in preliminary studies. Four weeks after iNOS transfer, magnetic resonance imaging showed no effect of iNOS overexpression on cardiac contractility at rest and during dobutamine stress (resting ejection fraction: control 27%, iNOS 26%; P = ns). Late enhancement, infarct size, and the amount of fibrosis were similar between groups. Although perfusion and perfusion reserve in response to adenosine and dobutamine were not significantly modified by iNOS-transfer, both vessel number and diameter were significantly increased in the ischemic area in the iNOS-treated group versus control (point score: control 15.3, iNOS 34.7; P < 0.05). Conclusions: Our findings demonstrate that transient iNOS overexpression does not aggravate cardiac dysfunction or postischemic fibrosis, while potentially contributing to neovascularization in the chronically ischemic heart.

Decrease in hemoglobin levels following surgery influences the outcome in head and neck cancer patients treated with accelerated postoperative radiotherapy.

AIM: To assess the influence of hemoglobin (Hb) levels in locally advanced head and neck cancer (LAHNC) patients treated with surgery and postoperative radiotherapy (PORT). MATERIAL AND METHODS: Pre- and postoperative Hb levels were collected in 79 patients treated with surgery followed by accelerated PORT for LAHNC. Median follow-up was 52 months (range 12-95 months). RESULTS AND DISCUSSION: Four-year overall survival (OS) rate was 51%. Neither pre- nor postoperative Hb level (&lt;120 or 130 g/l in women or men, respectively) influenced the outcome. However, when Hb decrease between pre- and postoperative Hb values was taken into account, 4-year OS was significantly higher in patients with Hb difference less than 38 g/l (quartile value) compared with those with Hb decrease 38 g/l or more (61% versus 16%, P = 0.008). CONCLUSION: Decrease in Hb level by more than 38 g/l after surgery secondary to blood loss influences the outcome when postoperative RT is indicated.

Contrôle de qualité en urologie [Quality assessment in urology].

Quality control (QuaCo) in urology is mandatory to standardize or even increase the level of care. While QuaCo is undertaken at every step in the clinical pathway, it should focus on the patient's comorbidities and on the urologist and its complication rate. Resulting from political and economical pressures, comparing QuaCo and outcomes between urologists and institutions is nowadays often performed. However, careful interpretation of these comparisons is mandatory to avoid potential discriminations. Indeed, the reader has to make sure that patients groups and surgical techniques are comparable, definitions of complications are similar, classification of complications is standardized, and finally that the methodology in collecting data is irreproachable.

Thrombosis in paroxysmal nocturnal hemoglobinuria at a glance: a clinical review.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired stem cell disorder, with its primary clinical manifestations being hemolytic anemia, marrow failure and thrombophilia. Chronic hemolysis, failures of the fibrinolytic system, increased leukocyte-derived tissue factor levels in plasma, procoagulant microparticles generated through complement-mediated damage of platelets and venous endothelium are related to the acquired hypercoagulable state. Visceral thrombosis (including hepatic veins and mesenteric veins), cerebrovascular events and pulmonary embolism predict a poor outcome. Thrombosis is also associated with significant morbidity during pregnancy. Depending on the sites of thrombosis, a score-based probability to predict outcome can be assigned. Abdominal vein thromboses account for the majority of morbidity and mortality related to thrombosis, and time-dependent trends suggest that mortality rates tend to decline, with the advent of evolution of therapeutic and diagnostic strategies. In contrast, mortality rates from cerebrovascular events display no significant decline. Prompt diagnosis requires both clinical suspicion and sophisticated imaging techniques, along with multidisciplinary therapeutic intervention. In the eculizumab era, a significant reduction of thrombotic events was observed during therapy, and long-term follow up is needed to establish any benefit in rates and pattern of this complication. However, up to now, only bone marrow transplantation permanently abolishes the coagulation defect.

Congenital diaphragmatic hernia: current status and review of the literature.

Treatment of congenital diaphragmatic hernia (CDH) challenges obstetricians, pediatric surgeons, and neonatologists. Persistent pulmonary hypertension (PPHT) associated with lung hypoplasia in CDH leads to a high mortality rate at birth. PPHT is principally due to an increased muscularization of the arterioles. Management of CDH has been greatly improved by the introduction of prenatal surgical intervention with tracheal obstruction (TO) and by more appropriate postnatal care. TO appears to accelerate fetal lung growth and to increase the number of capillary vessels and alveoli. Improvement of postnatal care over the last years is mainly due to the avoidance of lung injury by applying low peak inflation pressure during ventilation. The benefits of other drugs or technical improvements such as the use of inhaled nitric oxide or extracorporeal membrane oxygenation (ECMO) are still being debated and no single strategy is accepted worldwide. Despite intensive clinical and experimental research, the treatment of newborn with CDH remains difficult.

Endocavitary contact radiation therapy for ultrasonographically staged T1 N0 and T2 N0 rectal cancer.

BACKGROUND: The purpose of this study was to determine the long-term outcomes of patients undergoing endocavitary contact radiation therapy (ECR) for stage I rectal cancer. METHODS: A database of patients treated with ECR for biopsy-proven rectal adenocarcinoma from July 1986 to June 2006 was reviewed retrospectively. Only patients with primary, non-metastatic, ultrasonographically staged T1 N0 and T2 N0 cancer who had no adjuvant treatment were included. Patients received a median of 90 (range 60-190) Gy contact radiation, delivered transanally by a 50-kV X-ray tube in two to five fractions. RESULTS: Of 149 patients, 77 (40 T1, 37 T2) met the inclusion criteria. Median age was 74 (range 38-104) years, and median follow-up 69 (range 10-219) months. ECR failed in 21 patients (27 per cent) (persistent disease, four; recurrence, 17), of whom ten remained disease free after salvage therapy. The estimated 5-year disease-free survival rate was 74 (95 per cent confidence interval 63 to 83) per cent after ECR alone, and 87 (76 to 93) per cent when survival after salvage therapy for recurrence was included. CONCLUSION: ECR is a minimally invasive treatment option for early-stage rectal cancer. However, similar to other local therapies, ECR has a worse oncological outcome than radical surgery.

Métastases pulmonaires: quelle place pour la chirurgie [Management pulmonary metastases: when operate?].

Thirty percent of patients suffering from malignant disease will develop pulmonary metastases. Effective chemotherapy is lacking for many of these tumors. Many studies suggest survival benefit in selected patients when pulmonary metastasectomy allows complete resection. Several operative approach may be offered in order to achieve complete resection and maximal lung sparring. Pre-operative workup must assess the control of the primary tumor and the possibility of performing complete resection. Minimally invasive approaches may offer better quality life and equivalent oncologic outcomes than open approach.

La fibrose rétropéritonéale, une maladie inflammatoire méconnue. Observations cliniques et revue de la littérature [Retroperitoneal fibrosis, an unrecognized inflammatory disease. Clinical observations and review of the literature]

Retroperitoneal fibrosis (RF) is a rare disease, typically with an insidious clinical course. The peak incidence is seen in patients 40 to 60 years of age and mostly in man. The characteristic finding in this disease is a periaortic fibrous mass that often surrounds the ureters. Although usually regarded as an obstructive uropathy, there has been growing recognition of the condition as a generalized disease. It may have a wide variety of manifestations including mediastinitis, thyroiditis and sclerosing cholangitis. The most common mode of presentation remains abdominal or flank pain with uremia, anemia and a high sedimentation rate. Although ultrasound and renal scintigraphy may contribute to the general evaluation of patients with RF, CT-scanner is the preferred imaging method. The multiplanar imaging capability of magnetic resonance may facilitate assessment of disease extent. The pathogenesis of the disease remains unknown. Steroids and, more recently tamoxifen, appear to be effective in the treatment of the RF. In most instances, RF does not lead to long-term morbidity or affect survival. The three cases of RF reported herein illustrate the varied mode of presentation and the response to the treatment.

Donor site morbidity of the posterior conchal region.

BACKGROUND: The perichondral cutaneous graft (PCCG) from the posterior conchal region is an elegant solution for the coverage of facial defects with particular stability requirements. The donor defect can easily be covered with a transposition flap from the postauricular region. Although this region is a common donor site for skin grafts and has an important supporting function for glasses or hearing aids, little is known about long-term morbidity after graft harvest. OBJECTIVE: To assess the morbidity of the posterior concha and the postauricular region in terms of pain, scar formation, and patient satisfaction. MATERIALS AND METHODS: A retrospective study of 16 patients who had a PCCG harvested from the posterior concha. RESULTS: Two patients presented with a postoperative wound dehiscence on the postauricular region and one with a keloid scar on the posterior concha. One case of transitory hyperesthesia and pain when sleeping on the operated site was observed. None had complaints related to wearing glasses or hearing aids. CONCLUSION: Donor site morbidity of the postauricular and posterior conchal region is minimal and associated with high patient satisfaction, excellent aesthetic results, and emotional detachment from the hidden donor site.

Quoi de neuf en chirurgie [Novelties in surgery in 2013?].

Multidisciplinary management of colorectal liver metastases allows an increase of about 20% in the resection rate of liver metastases. It includes chemotherapy, interventional radiology and surgery. In 2013, the preliminary results of the in-situ split of the liver associated with portal vein ligation (ALLPS) are promising with unprecedented mean hypertrophy up to 70% at day 9. However, the related morbidity of this procedure is about 40% and hence should be performed in the setting of study protocol only. For pancreatic cancer, the future belongs to the use of adjuvant and neo adjuvant therapies in order to increase the resection rate. Laparoscopic and robot-assisted surgery is still in evolution with significant benefits in the reduction of cost, hospital stay, and postoperative morbidity. Finally, enhanced recovery pathways (ERAS) have been validated for colorectal surgery and are currently assessed in other fields of surgery like HPB and upper GI surgery.

Intussusception like lesion after fenestration in aortic type B dissection.

We report on a patient with Marfan syndrome who presented a Stanford type B dissection of the descending thoracic aorta in late pregnancy. After a cesarean section, the patient presented a severe obstruction of the mesenteric superior artery. An endovascular fenestration was performed (balloon and guidewire based fenestration). Computed tomography (CT) angiography revealed an intussusception 'like' image of the abdominal aortic layers as a consequence of the fenestration procedure. Because of aneurismal progression in the abdominal aorta, surgical repair of the abdominal aorta and intussusception material removal was achieved six weeks later. The patient is currently in good condition. We conclude that the intussusception could be induced by a guidewire. This fenestration procedure is not recommended in patients with structural aortic disorders.