1,721,352 research outputs found
Sudden Intrauterine Unexplained Death (SIUD) «Gray Zone» or borderline
No full textThe author reports the histopathological findings of 9 fetuses (3 females and 6 males, ranging in age from 34 to 41 gestational weeks) that died suddenly and unexpectedly in utero. They presented brainstem and cardiac conduction system lesions together with abnormalities of the fetal adnexa. A complete autopsy was performed, including detained investigation of the brainstem and cardiac conduction system on serial sections, as well as of the fetal adnexa, according to our guidelines. Histological examination of the fetal adnexa disclosed the presence of chorioamnionitis (7 cases), an abnormally short umbilical cord (1 case), and placental infection by parvovirus (1 case). These lesions were associated with brainstem lesions, i.e., hypoplasia of the arcuate nucleus (6 cases), inflammatory infiltrates in the brainstem (2 cases), hypoplasia of the raphe obscurus nucleus (2 cases), hypoplasia of the parabrachial Kölliker-Fuse complex (1 case), hypoplasia of the pre-Bötzinger complex (1 case), agenesis of the facial/parafacial complex (1 case), as well as conduction system lesions, i.e., dispersion or septation of the atrio-ventricular junction (9 cases), islands of the conduction system inside the central fibrous body (5 cases) resorptive degeneration (4 cases), cartilaginous meta-hyperplasia (2 cases), Mahaim fibers (1 case). Each SIUD victim presented at least one of these brainstem and/or cardiac conduction abnormalities, more than one change being present in the same fetus. The SIUD «gray zone», or borderline cases, are hereby described as those cases in which the lesions of the fetal adnexa alone might not have accounted for the sudden deaths, had it not been for the concomitant presence of brainstem and cardiac conduction lesions representing the morphological substrates for SIUD, as well as for SIDS. Our 9 cases are consistent with the triple-risk model, a hypothesis introduced for SIDS postulating an underlying biological vulnerability to exogenous stressors or triggering factors in a critical developmental period. In conclusion, we are convinced that there is a continuum between SIUD and SIDS and the triple-risk model is herein considered for the first time also for SIUD “gray zone” victims
Role of post-mortem investigations in determining the cause of sudden unexpected death in infancy
No full textPost-mortem bacteriological examination is of great importance, but it should be stressed that post-mortem investigations in every case of sudden unexpected death in infancy should primarily focus on the possibility of pathological findings in the brainstem and the cardiovascular system. Based on careful evaluation of recent anatomo-pathological studies on sudden unexpected death in infancy, there is now sound evidence to rely on for a new approach to this thorny problem
Sudden unexplained neonatal deaths
No full textWhile several works have stressed the importance of post-mortem examination in every case of suspected sudden infant death syndrome (SIDS), little, if any, attention has been given to the mandatory need to apply the same investigational protocol also in all cases of sudden perinatal unexplained death, i.e., sudden neonatal unexplained death (SNUD) and sudden intrauterine unexplained death (SIUD). First of all, it should be underlined that there is a clear continuum between unexplained perinatal death and SIDS, as developmental abnormalities have been detected to be common to both, particularly in the cardiac conduction system and in the brainstem centers regulating vital functions. From the analysis of the conducting tissue, the following pathological findings emerged: accessory atrio-ventricular pathways, mostly Mahaim fibers, cartilaginous hypermetaplasia, abnormal resorptive degeneration, junctional islands, persistent fetal dispersion, hypoplasia of the cardiac conduction system or of the central fibrous body, splitting of the atrio-ventricular node or of the His bundle, and the Zahn node. All of these cardiac conduction findings may be isolated incidents, but they are frequently associated with autonomic nervous system alterations of the brainstem
Crib death - Sudden infant death syndrome (SIDS). Sudden infant and perinatal unexplained death: The pathologist's viewpoint
No full textThis book describes systematic studies of the cardiovascular system and autonomic nervous system carried out in a large number of infants, newborns, and fetuses who have died suddenly and unexpectedly, as well as in age-matched control cases. The cardiovascular and neuropathological findings are presented in detail, and the relationship between crib death and unexplained perinatal death is discussed. This monograph will aid pathologists, forensic pathologists, pediatricians, obstetricians, and neonatologists in recognizing all potential morphological substrata. It puts forward a well-researched standardized postmortem protocol to be applied in all cases of sudden unexpected infant and perinatal death
Sudden deaths in term infants within 24 hours of birth
No full textSudden Deaths and Severe Apparent Life-Threatening Events in Term Infants Within 24 Hours of Birth, Anette Poets, Renate Steinfeldt, and Christian F. Poets, Pediatrics 2011; 127:4 e869-e873; published ahead of print March 28, 2011, doi:10.1542/peds.2010-2189 The post-mortem examination is important in every case of sudden unexpected infant and perinatal death, i.e., sudden neonatal unexplained death (SNUD) and sudden intrauterine unexplained death (SIUD). There is not a clear separation between unexplained perinatal death and sudden infant death syndrome (SIDS), because a continuity of histopathological and genetic developmental abnormalities have been detected in the cardiac conduction system and in the cardio-respiratory and arousal regulating centers of the brainstem. Prevention of sudden unexplained perinatal death and SIDS is the object of laws and bills in some countries, with the aim to enhance the anatomo- pathological, genetic, and clinico-epidemiological research. It should be underlined that the primary risk factors for both sudden unexplained perinatal death and SIDS include exposure to cigarette smoke that, starting in uterus, acts as a causative agent and triggering factor in vulnerable infants with developmental abnormalities in the cardiac conduction system and/or autonomic nervous system detected at necropsy examinations
Histopathological study of the cardiac conduction system in systemic lupus erythematosus
Full text linkSystemic lupus erythematosus (SLE) is a chronic multisystem inflammatory connective disease characterized by the production of auto-antibodies and immuno-complexes. SLE can affects all organs including heart.
Overall, the prevalence of cardiac involvement is estimated to affect more than 50% of SLE cases. All portions of the heart can be involved: pericardium, myocardium, cardiac conduction system, as well as coronary arteries. Pericarditis is the most common finding, while endocarditis is characterized by small nonbacterial vegetations along the valve leaflets known as Libman Sacks endocarditis.
The involvement of the cardiac conduction system in SLE has been less commonly described but should always be taken into account.
SLE affects particularly young women and the passive acquisition of maternal IgG antibodies during pregnancy cause neonatal lupus which is often related to congenital heart block.
Pre- or perinatal death from heart block due to severe autoimmune lesions of the atrioventricular junction has been reported with emphasis to the possible lethal association between maternal auto-antibodies and QT-prolongation. Recently, we reported a case of sudden unexpected intrauterine death of a term fetus in a anti-cardiolipin positive mother. The findings of the postmortem examination including the study of the cardiac conduction system and brainstem on serial sections ruled out the clinically suspected atrio-ventricular block due to the anti-cardiolipin antibodies, and disclosed severe bilateral hypoplasia of the arcuate nucleus which is an important chemoreceptor center for the control of breathing activity, located on the medullary ventral surface.
As the volume of data on new morphological and functional alterations of the cardiac conduction system increases, it becomes worldwide essential that victims of SLE, especially in cases of sudden deaths in young age, be submitted to an in-depth necropsy examination, focusing particularly on the study of the cardiac conduction system on serial sections. To examine the cardiac conduction system, two blocks of heart tissue should be obtained, for paraffin embedding. The first block contains the junction of superior vena cava and right atrium encompassing the entire area of the sino-atrial node. This sino-atrial block should be cut serially sectioned in a plane parallel to the crista terminalis. The second block contains the atrio-ventricular node (AV), His bundle down to bifurcation and bundle branches, with two centimeters of attached septum above and below. This AV junctional block is serially sectioned in a plane parallel to the two atrioventricular valve rings
Crib death. sudden unexplained death of infants : the pathologists viewpoint
No full textCrib death (SIDS) is the most frequent cause of death for infants during the first year, striking one out of every 700-1,000. Scarce knowledge in the field of SIDS and its pathology has led to a continued and growing concern with finding an explanation, with the goal of being able to either predict or quickly diagnose the infant or term fetus.
A systematic study of the autonomic nervous system and cardiac system has been performed on a large number of infants and fetuses who died suddenly and unexpectedly, as well as in age-matched control cases. The neurological and cardiac findings are described here, and the relationship between SIDS and unexplained fetal death is discussed.
This book helps pathologists, forensic pathologists, pediatricians, obstetrics, and neonatologists in recognizing all potential morphological substrata. It puts forward a well-researched postmortem study for use in a standardized autopsy protocol for use in all cases of sudden unexpected infant and fetal death.
Written for:
Pathologists, forensic pathologists, pediatricians, obstetricians, neonatologists
How to sample the cardiac conduction system.
No full textIn order to examine the cardiac conduction system, the heart is sampled in two blocks for paraffin embedding. The cardiac conduction system is removed in two blocks for paraffin embedding. It is important to "save the pacemaker" by avoiding the customary cut of the right heart margin by driving lengthwise the intercaval bridge. Indeed, by this technique one happens to slash diagonally the sino-atrial node, together with the Crista Terminalis. Block 1 consists of a portion of the right atrial wall including the lateral half of the funnel of the superior vena cava, sulcus and crista terminalis. This first block includes the sino-atrial node, its atrial approaches, the crista terminalis (with the upper 2/3 of the posterior internodal tract, the proximal part of the middle and anterior tracts), and the sino-atrial node’s ganglionated plexus (Fig. 1). Block 2 consists of the lower portion of the atrial septum, the trigonum fibrosus, and anterior contour of the coronary sinus and the upper 2/3 of the ventricular septum. This second block contains the atrio-ventricular node, His bundle, bifurcation, and bundle branches. The two blocks are cut serially
Sudden infants death syndrome (SIDS) : Standardised investigations and classification: Recommendations
No full textThe anatomo-pathological research has been particularly focused to study the cardiac conduction and neuropathological substrates of SIDS and sudden unexplained perinatal death. The pathogenic mechanism leading to death is, in many cases, related to the triggering role of the autonomic reflexes caused by alterations, mostly of a congenital nature, of the autonomic nervous system which regulates vital activities of respiratory, cardiovascular, digestive and arousal type. It is thus essential that victims of infant perinatal death “sine causa” be subjected to an in-depth histopathologic examination focusing particularly on the cardiac conduction and on the autonomic nervous systems regulating the cardiac, respiratory, upper digestive and arousal activities. Cases of SIDS «gray zone» have been reported in which only our further investigations on serial sections successfully identified anatomo-pathological findings likely representing the morphological substrates for sudden death. Such borderline or «gray zone» SIDS cases have been described as those cases in which anatomo-pathological findings alone might not have accounted for the sudden deaths, if it had not been for the location and/or concomitant presence of brainstem abnormalities which could have had a triggering role in causing the infant sudden death
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