Considerations for prenatal counselling of patients with cardiac rhabdomyomas based on their cardiac and neurologic outcomes.

Cardiac rhabdomyomas are benign cardiac tumours with few cardiac complications, but with a known association to tuberous sclerosis that affects the neurologic outcome of the patients. We have analysed the long-term cardiac and neurological outcomes of patients with cardiac rhabdomyomas in order to allow comprehensive prenatal counselling, basing our findings on the records of all patients seen prenatally and postnatally with an echocardiographic diagnosis of cardiac rhabdomyoma encountered from August, 1982, to September, 2007. We analysed factors such as the number and the location of the tumours to establish their association with a diagnosis of tuberous sclerosis, predicting the cardiac and neurologic outcomes for the patients.Cardiac complications include arrhythmias, obstruction of the ventricular outflow tracts, and secondary cardiogenic shock. Arrhythmias were encountered most often during the neonatal period, with supraventricular tachycardia being the commonest rhythm disturbance identified. No specific dimension or location of the cardiac rhabdomyomas predicted the disturbances of rhythm.The importance of the diagnosis of tuberous sclerosis is exemplified by the neurodevelopmental complications, with four-fifths of the patients showing epilepsy, and two-thirds having delayed development. The presence of multiple cardiac tumours suggested a higher risk of being affected by tuberous sclerosis. The tumours generally regress after birth, and cardiac-related problems are rare after the perinatal period. Tuberous sclerosis and the associated neurodevelopmental complications dominate the clinical picture, and should form an important aspect of the prenatal counselling of parents.

Low levels of human leukocyte antigen donor-specific antibodies detected by solid phase assay before transplantation are frequently clinically irrelevant.

Since new technologies based on solid phase assays (SPA) have been routinely incorporated in the transplant immunology laboratory, the presence of pretransplantation donor-specific antibodies (DSA) against human leukocyte antigen (HLA) molecules has generally been considered as a risk factor for acute rejection (AR) and, in particular, for acute humoral rejection (AHR). We retrospectively studied 113 kidney transplant recipients who had negative prospective T-cell and B-cell complement-dependent cytotoxicity (CDC) crossmatches at the time of transplant. Pretransplantation sera were screened for the presence of circulating anti-HLA antibody and DSA by using highly sensitive and HLA-specific Luminex assay, and the results were correlated with AR and AHR posttransplantation. We found that approximately half of our patient population (55/113, 48.7%) had circulating anti-HLA antibody pretransplantation. Of 113 patients, 11 (9.7%) had HLA-DSA. Of 11 rejection episodes post-transplant, only two patients had pretransplantation DSA, of whom one had a severe AHR (C4d positive). One-year allograft survival was similar between the pretransplantation DSA-positive and -negative groups. Number, class, and intensity of pretransplantation DSA, as well as presensitizing events, could not predict AR. We conclude that, based on the presence of pretransplantation DSA, post-transplantation acute rejections episodes could not have been predicted. The only AHR episode occurred in a recipient with pretransplantation DSA. More work should be performed to better delineate the precise clinical significance of detecting low titers of DSA before transplantation.

Group selection and kin selection: two concepts but one process.

In a recent paper, Traulsen and Nowak use a multilevel selection model to show that cooperation can be favored by group selection in finite populations [Traulsen A, Nowak M (2006) Proc Natl Acad Sci USA 103:10952-10955]. The authors challenge the view that kin selection may be an appropriate interpretation of their results and state that group selection is a distinctive process "that permeates evolutionary processes from the emergence of the first cells to eusociality and the economics of nations." In this paper, we start by addressing Traulsen and Nowak's challenge and demonstrate that all their results can be obtained by an application of kin selection theory. We then extend Traulsen and Nowak's model to life history conditions that have been previously studied. This allows us to highlight the differences and similarities between Traulsen and Nowak's model and typical kin selection models and also to broaden the scope of their results. Our retrospective analyses of Traulsen and Nowak's model illustrate that it is possible to convert group selection models to kin selection models without disturbing the mathematics describing the net effect of selection on cooperation.

Syndrome d'hyperstimulation ovarienne [The syndrome of ovarian hyperstimulation].

The ovarian hyperstimulation syndrome (SHO) can be defined as an iatrogenic pathology induced by active substances administered for controlling follicular maturation and ovulation. The etiology, the physiopathology, the diagnostic and therapeutic methods available are discussed. A theoretical model, based on clinical data, allows identification of a set of criteria which should help determining prospectively the chances of development of such a pathology.

Trapeziectomy and tendon suspension with or without a mitek anchor fixation in the thumb basal joint osteoarthritis.

Partial trapeziectomy with suspension ligamentoplasty is a commonly performed treatment of thumb osteoarthritis. Nevertheless, the post-operative recovery remains long and critical reason for which different modifications of the surgical technique have been proposed. To compare two suspension ligamentoplasty techniques, one with a mitek anchor and another without, a retrospective study of 55 consecutive operated patients was performed. A detailed clinical analysis of pain, function and a radiologic assessment of the trapeziometacarpal space were performed. Mitek anchor fixation was associated with a shorter convalescence period. However, in spite of an improved radiological maintenance of the scaphometacarpal space, mitek anchor fixation was associated with an impaired postoperative function and residual pain when compared with the conventional suspension ligamentoplasty procedure. Patient's satisfaction was comparable in both groups. In our series stabilization of the suspension ligamentoplasty procedure by the insertion of a mitek anchor did not bring the hoped benefits to the patients with a trapeziometacarpal arthritis.

Management of hepatitis C virus (HCV) infection in drug substitution programs.

BACKGROUND: In Switzerland, intravenous drug use (IDU) accounts for 80% of newly acquired hepatitis C virus (HCV) infections. Early HCV treatment has the potential to interrupt the transmission chain and reduce morbidity/mortality due to decompensated liver cirrhosis and hepatocellular carcinoma. Nevertheless, patients in drug substitution programs are often insufficiently screened and treated. OBJECTIVE/METHODS: With the aim to improve HCV management in IDUs, we conducted a cross sectional chart review in three opioid substitution programs in St. Gallen (125 methadone and 71 heroin recipients). Results were compared with another heroin substitution program in Bern (202 patients) and SCCS/SHCS data. RESULTS: Among the methadone/heroin recipients in St. Gallen, diagnostic workup of HCV was better than expected: HCV/HIV-status was unknown in only 1% (2/196), HCV RNA was not performed in 9% (13/146) of anti-HCV-positives and the genotype missing in 15% (12/78) of HCV RNA-positives. In those without spontaneous clearance (two thirds), HCV treatment uptake was 23% (21/91) (HIV-: 29% (20/68), HIV+: 4% (1/23)), which was lower than in methadone/heroin recipients and particularly non-IDUs within the SCCS/SHCS, but higher than in the, mainly psychiatrically focussed, heroin substitution program in Bern (8%). Sustained virological response (SVR) rates were comparable in all settings (overall: 50%, genotype 1: 35-40%, genotype 3: two thirds). In St. Gallen, the median delay from the estimated date of infection (IDU start) to first diagnosis was 10 years and to treatment was another 7.5 years. CONCLUSIONS: Future efforts need to focus on earlier HCV diagnosis and improvement of treatment uptake among patients in drug substitution programs, particularly if patients are HIV-co-infected. New potent drugs might facilitate the decision to initiate treatment.

Athletic injuries of the extensor carpi ulnaris subsheath: MRI findings and utility of gadolinium-enhanced fat-saturated T1-weighted sequences with wrist pronation and supination.

OBJECTIVE: To report the magnetic resonance imaging (MRI) findings in athletic injuries of the extensor carpi ulnaris (ECU) subsheath, assessing the utility of gadolinium-enhanced (Gd) fat-saturated (FS) T1-weighted sequences with wrist pronation and supination. METHODS: Sixteen patients (13 male, three female; mean age 30.3 years) with athletic injuries of the ECU subsheath sustained between January 2003 and June 2009 were included in this retrospective study. Initial and follow-up 1.5-T wrist MRIs were performed with transverse T1-weighted and STIR sequences in pronation, and Gd FS T1-weighted sequences with wrist pronation and supination. Two radiologists assessed the type of injury (A to C), ECU tendon stability, associated lesions and rated pulse sequences using a three-point scale: 1=poor, 2=good and 3=excellent. RESULTS: Gd-enhanced FS T1-weighted transverse sequences in supination (2.63) and pronation (2.56) were most valuable, compared with STIR (2.19) and T1-weighted (1.94). Nine type A, one type B and six type C injuries were found. There were trends towards diminution in size, signal intensity and enhancement of associated pouches on follow-up MRI and tendon stabilisation within the ulnar groove. CONCLUSION: Gd-enhanced FS T1-weighted sequences with wrist pronation and supination are most valuable in assessing and follow-up athletic injuries of the ECU subsheath on 1.5-T MRI.

Helicopter rescue operations involving winching of an emergency physician.

OBJECTIVE: We sought to study the epidemiologic and medical aspects of alpine helicopter rescue operations involving the winching of an emergency physician to the victim. METHODS: We retrospectively reviewed the medical and operational reports of a single helicopter-based emergency medical service. Data from 1 January 2003 to 31 December 2008 were analysed. RESULTS: A total of 921 patients were identified, with a male:female ratio of 2:1. There were 56 (6%) patients aged 15 or under. The median time from emergency call to helicopter take-off was 7 min (IQR = 5-10 min). 840 (91%) patients suffered from trauma-related injuries, with falls from heights during sports activities the most frequent event. The most common injuries involved the legs (246 or 27%), head (175 or 19%), upper limbs (117 or 13%), spine (108 or 12%), and femur (66 or 7%). Only 81 (9%) victims suffered from a medical emergency, but these cases were, when compared to the trauma victims, significantly more severe according to the NACA index (p<0.001). Overall, 246 (27%) patients had a severe injury or illness, namely, a potential or overt vital threat (NACA score between 4 and 6). A total of 478 (52%) patients required administration of major analgesics: fentanyl (443 patients or 48%), ketamine (42 patients or 5%) or morphine (7 patients or 1%). The mean dose of fentanyl was 188 micrograms (range 25-750, SD 127). Major medical interventions such as administration of vasoactive drugs, intravenous perfusions of more than 1000 ml of fluids, ventilation or intubation were performed on 39 (4%) patients. CONCLUSIONS: The severity of the patients' injuries or illnesses along with the high proportion of medical procedures performed directly on-site validates emergency physician winching for advanced life support procedures and analgesia.

Mortality, complications and loss of pulmonary function after pneumonectomy vs. sleeve lobectomy in patients younger and older than 70 years.

Retrospective single institution analysis of all patients undergoing sleeve lobectomy or pneumonectomy between 2000 and 2005. Seventy-eight patients underwent pneumonectomy (65 patients <70 years, 13 patients >70 years) and 69 sleeve lobectomy (50 patients <70 years, 19 patients >70 years). Pre-existing co-morbidity, surgical indication and induction therapy was similarly distributed between treatment by age-groups. In patients <70 years, pneumonectomy and sleeve lobectomy resulted in a 30-day mortality of 3% vs. 0 and an overall complication rate of 26% vs. 44%, respectively. In patients >70 years, pneumonectomy and sleeve lobectomy resulted in a 30-day mortality of 15% vs. 0 and an overall complication rate of 23% vs. 32%. In both age groups, pneumonectomy was associated with more airway complications (NS) and a significantly higher postoperative loss of FEV(1) than sleeve lobectomy (P<0.0001, P<0.03). Age per se did not influence the loss of FEV(1) and DLCO for a given type of resection. Sleeve lobectomy may have a therapeutic advantage over pneumonectomy in the postoperative course of elderly patients.

Expérience de dix ans de réanimation chirurgicale d'un centre hospitalier universitaire. Détermination d'un critère d'identification des patients à risque de décéder de coagulopathie irréversible [10 years experience in surgical resuscitation at a university hospital center. Determination of a criterion for identifying patients at risk for fatal irreversible coagulopathy]

The authors evaluated ten years of surgical reanimation in the University Centre of Lausanne (CHUV). Irreversible coagulopathy (IC) is the predominant cause of death for the polytraumatized patient. Acidosis, hypothermy, and coagulation troubles are crucial elements of this coagulopathy. The authors looked for a criterion allowing the identification of dying of IC. In a retrospective study, laboratory results of pH, TP, PTT, thrombocyte count and the need for blood transfusion units were checked for each major step of the primary evaluation and treatment of the polytraumatized patients. These results were considered as critical according to criteria of the literature (30). The authors conclude that the apparation of a third critical value may be useful to identify the polytraumatized patient at risk of dying of IC status. This criterion may also guide the trauma team in selecting a damage control surgical approach (DCS). This criterion was then introduced into an algorithm involving the Emergency Department, the operating room and the Intensive Care Unit. This criterion is a new tool to address the patient at the crucial moment to the appropriate hospital structure.

Congenital high airway obstruction syndrome and airway reconstruction: an evolving paradigm.

OBJECTIVES: To refine the classic definition of, and provide a working definition for, congenital high airway obstruction syndrome (CHAOS) and to discuss the various aspects of long-term airway reconstruction, including the range of laryngeal anomalies and the various techniques for reconstruction. DESIGN: Retrospective chart review. PATIENTS: Four children (age range, 2-8 years) with CHAOS who presented to a single tertiary care children's hospital for pediatric airway reconstruction between 1995 and 2000. CONCLUSIONS: To date, CHAOS remains poorly described in the otolaryngologic literature. We propose the following working definition for pediatric cases of CHAOS: any neonate who needs a surgical airway within 1 hour of birth owing to high upper airway (ie, glottic, subglottic, or upper tracheal) obstruction and who cannot be tracheally intubated other than through a persistent tracheoesophageal fistula. Therefore, CHAOS has 3 possible presentations: (1) complete laryngeal atresia without an esophageal fistula, (2) complete laryngeal atresia with a tracheoesophageal fistula, and (3) near-complete high upper airway obstruction. Management of the airway, particularly in regard to long-term reconstruction, in children with CHAOS is complex and challenging.

Superior oblique myokymia: efficacy of medical treatment.

PURPOSE: Superior oblique myokymia (SOM) is an uncommon disorder characterized by episodic monocular oscillopsia. Several medications have been reported to be of benefit for some patients with this condition, but the efficacy of medical treatment has not been well established and little long-term follow-up data are available. The purpose of this study was to better clarify the role of medical therapy in the management of SOM. METHODS: A retrospective review of patients with this disorder seen in an outpatient neuro-ophthalmology clinic. The diagnosis of SOM was based on a history of episodic unilateral oscillopsia with or without torsional diplopia. Twenty-seven patients with SOM were identified. Twenty of these were treated medically and these formed the basis of the study. Follow-up interval ranged from 1 to 12.5 years (mean, 6.5 years). The main outcome measure was relief of oscillopsia. RESULTS: Fifteen of the 18 patients treated with carbamazepine (83%) reported some benefit, 6 of whom continue to do well on medication 9 months to 5 years later. In four patients improvement was only transient and in five others treatment was subsequently discontinued for various reasons. In addition, one patient had sustained benefit from phenytoin, one from propranolol, and one from propranolol plus valproic acid. We found no treatment success with baclofen. Overall, nine patients (45%) enjoy sustained benefit unassociated with adverse side effects. CONCLUSIONS: In contrast to previous reports emphasizing the efficacy of surgery for SOM, our data demonstrate the potential benefits of medical treatment for patients with this disorder.

Value of positron emission tomography in full-thickness chest wall resections for malignancies.

Preoperative imaging for resection of chest wall malignancies is generally performed by computed tomography (CT). We evaluated the role of (18)F-fluorodeoxyglucose (FDG) positron emission tomography (PET) in planning full-thickness chest wall resections for malignancies. We retrospectively included 18 consecutive patients operated from 2004 to 2006 at our institution. Tumor extent was measured by CT and PET, using the two largest perpendicular tumor extensions in the chest wall plane to compute the tumor surface assuming an elliptical shape. Imaging measurements were compared to histopathology assessment of tumor borders. CT assessment consistently overestimated the tumor size as compared to PET (+64% vs. +1%, P<0.001). Moreover, PET was significantly better than CT at defining the size of lesions >24 cm(2) corresponding to a mean diameter >5.5 cm or an ellipse of >4 cm x 7.6 cm (positive predictive value 80% vs. 44% and specificity 93% vs. 64%, respectively). Metabolic PET imaging was superior to CT for defining the extent of chest wall tumors, particularly for tumors with a diameter >5.5 cm. PET can complement CT in planning full-thickness chest wall resection for malignancies, but its true value remains to be determined in larger, prospective studies.

Chronic subdural hematomas treated by burr hole trepanation and a subperiostal drainage system.

OBJECTIVE: Most symptomatic chronic subdural hematomas are treated by subdural drainage. However, a subperiostal (i.e., extracranial) passive closed-drainage system in combination with double burr hole trepanation is used at our institution. Therefore, we wanted to analyze our results and compare them with the alternate treatment strategies reported in the current literature. METHODS: In a retrospective single-center study, we analyzed the data of all patients undergoing double burr hole trepanation with a subperiostal passive closed-drainage system. Data analysis included general patient data, complications, postoperative seizure rate, and outcome. RESULTS: One hundred forty-seven patients underwent surgery for 183 symptomatic chronic subdural hematomas. The perioperative mortality rate was 3.4%. Hematoma persistence or recurrence occurred in 13.1% of the cases. The postoperative seizure rate was 6.6%, and the infection rate was 1.6%, including 3 cases of superficial wound infection and 1 case with deep infection. The reintervention rate was 9.3%, including trepanation in 8.2% of the patients and craniotomy in 1.1%. The overall complication rate was 10.9%. CONCLUSION: Double burr hole trepanation combined with a subperiostal passive closed-drainage system is a technically easy, highly effective, safe, and cost-efficient treatment strategy for symptomatic chronic subdural hematomas. The absence of a drain in direct contact with the hematoma capsule may moderate the risk of postoperative seizure and limit the secondary spread of infection to intracranial compartments.