A comparison of respiratory symptoms and inflammation in sudden infant death syndrome and in accidental or inflicted infant death

Upper respiratory infection and pulmonary inflammation are common in sudden infant death syndrome, but their role in the cause of death remains controversial. Controlled studies comparing clinical upper respiratory infection and inflammation in sudden infant death syndrome with sudden infant deaths caused by accidents and inflicted injuries (controls) are unavailable. Our aim was to compare respiratory inflammation and upper respiratory infection within 48 hours of death and postmortem culture results in these two groups. A retrospective analysis of upper respiratory infection and pathologic variables in the trachea and lung of 155 infants dying of sudden infant death syndrome and 33 control infants was undertaken. Upper respiratory infection was present in 39% of sudden infant death syndrome cases and 40% of control cases. Upper respiratory infection was more likely to have occurred in association with more severe lymphocytic interstitial pneumonitis when sudden infant death syndrome cases and control cases were combined ( P=.04). Proximal and distal tracheal lymphocytic infiltration was more severe in control cases than in sudden infant death syndrome cases ( P=.01 and.01, respectively). Lymphocytic infiltrations of the bronchi, bronchioles, and pulmonary interstitium were similar between groups. Bronchial associated lymphoid tissue was more prominent in control cases ( P=.04). Cultures were positive in 80% of sudden infant death syndrome cases, 78% of which were polymicrobial. Among control cases, 89% were positive, with 94% being polymicrobial. This study confirms that microscopic inflammatory infiltrates in sudden infant death syndrome are not lethal.     

Lympho-monocytic enteroviral myocarditis: traditional, immunohistological and molecularpathological methods for diagnosis in a case of suspected sudden infant death syndrome (SIDS)

A 7-month-old boy was found lifeless in his bed by his grandfather. Near the mouth there were some blood and chyme on the bedlinen. His mother reported a chill during the last 8 days. Autopsy revealed multiple petechia under the capsule of the thymus, subepicardially and subpleurally, the cause of death was tentative supposed to be a sudden infant death. Histologically parts of the myocardium showed a lympho-monocytic myocarditis according to the DALLAS-criteria. Immunohistological investigations demonstrated an enhanced expression of MHC class II antigens, an increased number of leucocytes as well as T-lymphocytes. In situ hybridization and immunohistochemistry revealed myocardial enterovirus infection. Considering that there were regions in the myocardium without the traditional findings of myocardial necrosis and inflammation but with distinct expression of the above mentioned immunohistologic markers, immunohistological investigations turn out to be an appropriate tool to examine infant fatalities with uncertain cause of death for early stages of myocarditis.     

Sudden death of an infant with 'an early epileptic encephalopathy'.

This article reports an autopsy case of sudden death of an infant with an infrequent encephalopathy involving epileptic episodes. The infant was a 1-year and 10-month-old boy, who had a history of the first convulsive seizures in the third month after birth. The clinical diagnosis was described as 'an early infantile epileptic encephalopathy with suppression-bursts' (Ohtahara syndrome). On a winter day, he was collapsed following a high fever and was already dead on the arrival at a hospital. The body was small for the age and poorly nourished. The autopsy and postmortem magnetic resonance imaging scan (MRI) of formalin-fixed brain revealed advanced unsymmetric brain atrophy with cortical dysplasia, which were prominent in the left temporal and right occipital lobes, and sclerotic atrophy of the parahippocampal gyri, additionally showing a feature of the olivo-ponto-cerebellar atrophy. However, the cause of death was pathologically and microbiologically determined as bacterial bronchopneumonia following pulmonary infection of the influenza A virus. In sudden death cases of physically handicapped infants, the investigation of viral infection in consideration of an epidemiological survey is important even when the death can be pathomorphologically explained.     

Cardiac rhabdomyoma presenting as sudden infant death syndrome

A case of cardiac rhabdomyoma presenting as sudden infant death in a four-and-one-half month-old infant is reported. The child was the product of an essentially uncomplicated pregnancy and enjoyed good health before his unexpected, sudden death. Autopsy examination revealed the presence of multiple cardiac lesions which histologically were diagnosed as rhabdomyomas. Death was attributed to fatal cardiac arrhythmia caused by the tumor. To the authors' knowledge this represents the first reported case in the forensic science literature of death as a result of cardiac rhabdomyoma presenting as sudden infant death syndrome (SIDS).     

Acute deaths in nonpregnant adults due to invasive streptococcal infections

Streptococcal organisms, part of the normal human bacterial flora, occasionally become infectious pathogens responsible for a wide array of clinical syndromes, ranging from mild pharyngitis to death. Notably, infections due to group A and group B beta-hemolytic strains are well known for causing invasive disease leading to death. These deaths, while often suspected clinically, occasionally are not diagnosed until autopsy. We present 3 rapid deaths, with very different presentations, due to streptococcal infection. Two decedents experienced sudden deaths due to pneumonia and severe meningoencephalitis caused by group B beta-hemolytic streptococcal infection, a common cause of neonatal meningitis but only rarely reported in nonpregnant adults. The final case involves a 69-year-old male who presented to the emergency room with a complaint of shoulder pain but over the next several hours developed signs of necrotizing fasciitis, became septic, and died. While antemortem cultures were negative, owing to antibiotic administration, postmortem cultures of bone and deep soft tissue were positive for group A beta-hemolytic Streptococcus sp. Acute and sudden deaths due to infectious etiology represent an uncommon yet well-documented occurrence. The importance of appropriate postmortem cultures in these situations and a review of the literature will be discussed.     

Denied their ‘natural nourishment’: religion,causes of death and infant mortality in the Netherlands, 1875–1899

At the end of the nineteenth century, infant mortality rates started to fall rapidly in the Netherlands. Unfortunately, not all regions benefited from this development. High infant death in the Roman Catholic provinces of North-Brabant and Limburg has often been ascribed to a growing reluctance of Catholic mothers to breastfeed their infants after 1870. This was supposedly caused by the combination of a strict, prudish Roman Catholic norm prohibiting women from baring their breasts and a refusal to accept new medical insights into healthy childcare. The food given to weaned children was generally of such poor quality that many infants succumbed to gastrointestinal diseases. Consequently, infant mortality rates caused by water- and food-borne infectious diseases would have been higher amongst weaned babies. By using recently digitised municipal cause-of-death registration statistics, it is possible to see if there are, indeed, indications of a shift in breastfeeding patterns after 1870. First, the authors look at infant deaths from all causes to see whether Roman Catholic municipalities underwent a rise in the mortality of children under the age of one. Second, the authors do the same for cause-specific infant mortality from typhus, typhoid fever, diarrhoea, dysentery, acute diseases of the digestive system and cholera. Based on the outcomes, there was no homogenous rise in infant mortality in all Roman Catholic municipalities. Furthermore, there is no indication that infant mortality due to digestive diseases increased uniformly in all Roman Catholic communities between 1875 and 1899. Either some communities were able to counteract the negative effects of a shift towards weaning or changes in breastfeeding patterns were not a specific Roman Catholic phenomenon at all.     

Pulmonary siderophages and unexpected infant death

It has been proposed that the presence of siderophages in the lungs of infants who die unexpectedly should be considered a marker of a previous hypoxic event, which may preclude a diagnosis of sudden infant death syndrome. The authors retrospectively reviewed all infant deaths (<1 year old) going to autopsy at the Denver Office of the Medical Examiner from January 1999 to January 2001. Lung sections were stained with Prussian blue, and siderophages were counted in 20 high-power fields per lobe sampled. Cell counts were performed by two independent pathologists who were blinded to history and cause of death, with good reproducibility. Iron stain results were then categorized by average number of siderophages per 20 high-power field (category 1 = <5, category 2 = 5-100, category 3A = 100-500, category 3B = >100 in a single lobe, category 4 = >500). The results were subsequently correlated to case history, autopsy findings, and cause/manner of death. Forty-three cases were reviewed. The causes of death included sudden infant death syndrome (16), asphyxia (5), undetermined (6), and other (16). Those deaths were categorized by the above criteria as follows: category 1. (32), category 2. (6), category 3. (4), and category 4. (1). All sudden infant death syndrome deaths were in category 1. Categories 1 and 2 also included deaths in which hypoxia might have been present before death because of such factors as pneumonia and congenital heart disease. Categories 3 and 4 included a known homicidal asphyxia in which repeated episodes of intentional smothering were documented, 2 probable asphyxias, 1 nonaccidental trauma, and 1 undetermined. All 5 cases had questionable circumstances surrounding the death of the infant. Pulmonary siderophages were described in only 1 of the 43 autopsy reports. It was concluded that pulmonary siderophages can be markedly increased in cases of repeated asphyxia. Siderophages may also be increased in cases where hypoxia may have been present for another reason, but not to the same degree. Siderophages are not increased in sudden infant death syndrome. Because iron-laden macrophages often are not recognized on routine examination with hematoxylin and eosin staining, iron stains may be helpful in the evaluation of infant deaths. If siderophages are present in increased amounts without an obvious explanation, further investigation is warranted.     

Coexistence of Subdural Hematoma and a Rare Cardiopathy in an Infant: Etiological and French Medicolegal Discussion

During legal proceedings following the unexpected death of an infant, the magistrate calls on an expert to clarify the causes of death and to decide on the course to be taken. A report was made following the death of a 7‐month‐old baby girl after recovery from cardiac arrest when investigations of the cause revealed a subdural hematoma (SDH). We discuss the interconnection of these two entities and their role in the lethal process. In this infant, two distinct lesions with medicolegal implications were simultaneously present: on the one hand histiocytoid cardiopathy, and on the other hand SDH, which could result from ill‐treatment. The case of this infant reminds us that the role of the pediatrician is to report the suspicion, whereas the role of the medical expert is to inform the magistrate as to the reality of abuse and its implication in the lethal process.     

Frequency of pulmonary hemosiderosis in Eastern North Carolina

Pulmonary hemosiderosis has been attributed to airborne fungi in water-damaged homes in studies of a cluster of cases in infants and children in Cleveland, Ohio, in 1997. We have searched for such emerging infectious agents in the Coastal Plain of North Carolina, which is subject to intermittent flooding. Pulmonary tissue from 206 infants and young children whose deaths had been investigated from October 1978 to September 1996 was retained at East Carolina University School of Medicine. Ages ranged from premature newborns to 49-months. One hundred and ten deaths were attributed to sudden infant death syndrome (SIDS). New sections were cut and analyzed using hematoxylin and eosin, Prussian blue for iron, and Gomori methenamine silver for fungal organisms. Twenty-three infants and children had iron-containing macrophages. Sixteen of these had underlying illnesses, but 4 were originally diagnosed as SIDS. Only one of these had sufficient hemosiderosis to be considered as having pulmonary hemosiderosis and no fungi were present. One case without iron had fungi and was not originally diagnosed as SIDS. The single case of pulmonary hemosiderosis found in this rural area is not similar to the cluster in Cleveland. The study identifies no fungal organisms as emerging infectious diseases in this area.     

Virological analysis in the diagnosis of sudden children death: a medico-legal approach

Infections are considered to be an important cause of unexpected death in children. It has also been assumed that respiratory viruses are involved in the genesis of sudden infant death syndrome (SIDS). The Spanish National Institute of Toxicology and Forensic Sciences act as the forensic reference centre for Spain. We analyse the experience of this centre in the virological study of 64 cases of sudden children death where viral serology, virological cultures, herpesviruses polymerase chain reaction (PCR) and electron microscopy were performed. According to pathological findings, death could only be attributed to an adenovirus infection in one amygdalitis with upper airways stenosis and asphyxia. Human herpes virus 6 (HHV-6) was detected by PCR in one case with pathological findings characteristic of SIDS. Recent infection by respiratory syncytial virus (RSV), Epstein-Barr virus (EBV) and cytomegalovirus (CMV) were also detected. Meanwhile, 85.9% of the cases yielded negative viral results. Twenty-eight infants were finally categorised as SIDS. Pathological findings of infection were detected in 12 patients despite the negativity of viral analyses. Although viral infection is an uncommon cause of sudden children death, a complete microbiological investigation will help to solve the puzzle of SIDS. Definitive guidelines for microbiological analyses need to be updated whilst new pathogens are discovered or new techniques are implemented in order to clarify unsolved cases.     

Necrotizing fasciitis: a fatal outcome following minor trauma. Case report and literature review

The death of a young healthy man, 11 days after he sustained minor trauma, illustrates the potential disaster of undiagnosed necrotizing fasciitis. Difficulty in early diagnosis often arises because the skin is initially spared and only later becomes secondarily involved. In this case, because of the lack of external manifestations, the diagnosis of necrotizing fasciitis was not considered until the postmortem examination. Necrotizing fasciitis should be considered a potentially serious problem that can follow minor trauma and eventually lead to death. The need for a high index of suspicion to make this diagnosis, along with the medicolegal implication of a diagnosis of necrotizing fasciitis, are both addressed in this report. Also of note is the fact that this patient was treated with nonsteroidal antiinflammatory drugs for pain and inflammation, which has recently been reported to be associated with an increased incidence of fulminant evolution of necrotizing fasciitis.     

Postmortem diagnostics using MSCT and MRI of a lethal streptococcus group A infection at infancy: a case report

Postmortem cross-sectional imaging using multislice computed tomography (MSCT) and magnetic resonance imaging (MRI) was considered as a base for a minimal invasive postmortem investigation in forensic medicine such as within the Virtopsy approach. We present the case of a 3-year-old girl with a lethal streptococcus group A infection and the findings of postmortem imaging in this kind of natural death. Postmortem MSCT and MRI revealed an edematous occlusion of the larynx at the level of the vocal cords, severe pneumonia with atelectatic parts of both upper lobes and complete atelectasis of both lower lobes, purulent fluid-filled right main bronchus, enlargement of cervical lymph nodes and pharyngeal tonsils, and additionally, a remaining glossopharyngeal cyst as well as an ureter fissus of the right kidney. All relevant autopsy findings could be obtained and visualized by postmortem imaging and confirmed by histological and microbiological investigations supporting the idea of a minimal invasive autopsy technique.     

A case of Ludwig angina: a case report and review of the literature

Ludwig angina is a rapidly progressing submaxillary, submandibular, and sublingual necrotizing cellulitis of the floor of the mouth that can have lethal consequences due to airway obstruction. Various aerobic and anaerobic microorganisms, and less often fungi, have been implicated to cause Ludwig angina, including oral flora such as streptococci and staphylococci. Early recognition and the use of parenteral antibiotics can prevent mortality and morbidity. We report a case of a 25-year-old white man who was admitted to the hospital by his dentist after being diagnosed with Ludwig angina secondary to periodontal abscesses involving teeth #17 and #32. Although antibiotics were administered, while in the hospital, the decedent had difficulty swallowing and was drooling. He suddenly began to have seizure-like activity thought to be anoxic myoclonus. The decedent was aggressively resuscitated and taken to the operating room for neck exploration and a tracheostomy. Neck exploration revealed severe necrotizing acute inflammation of the deep soft tissues and musculature of the neck. He remained on life support for 7 days until he was declared brain dead. Ludwig angina is a progressive cellulitis that often results in death by asphyxia. Ludwig angina can be complicated by subsequent deep neck infection. The underlying etiologies and common scenarios are examined, and significant autopsy findings and dissecting procedures are discussed. The pathophysiology of Ludwig angina is studied with a review of the current literature.     

Toxicologic analysis in cases of possible sudden infant death syndrome: a worthwhile exercise?

The diagnosis of sudden infant death syndrome (SIDS) is one of exclusion. At the Department of Forensic Medicine, Westmead Hospital, toxicologic analysis is performed as part of the postmortem examination of all apparent SIDS deaths. The results for the 5-year period January 1, 1994, to December 31, 1999, were audited to determine whether such routine testing was worthwhile. During this time there were 117 cases with a history consistent with SIDS. Drugs were detected in 19 (16%) of these cases. In 1 case, death was attributed to the finding of methadone. The presence of methadone was regarded as a possible contributing factor to death in a further 2 cases. The presence of possible methadone toxicity had not been expected from the history given before the examination in these 3 cases. In 114 cases there was a suitable sample for alcohol testing; in no case was alcohol detected. In 13 cases the postmortem examination revealed an anatomic cause of death (including 3 cases consistent with whiplash/shaken baby/impact head injury), which excluded a diagnosis of SIDS. In conclusion, routine toxicologic testing in all possible cases of SIDS death supplements the postmortem examination in excluding cases of non-SIDS.     

An autopsy case of an infant with Joubert syndrome who died unexpectedly and a review of the literature

The present report describes the unexpected death of a 6-month-old female infant who had been clinically diagnosed with Joubert syndrome. This is a relatively rare congenital neurological disorder characterized by hypoplasia/aplasia of cerebellar vermis, which transmits information from the body to the cerebellum, and is associated with respiratory dysfunction, abnormal eye movements, and developmental delay. The infant was found dead in bed and the immediate cause of death was determined as aspiration of vomit which may have been induced by a neurological disorder related to hypoplasia of the cerebellar vermis. These findings, together with a review of previous clinical case reports, suggest that Joubert syndrome should be considered as a predisposition to sudden unexpected death in infants mainly due to aspiration or complicated infection.     

Sudden infant death due to neurofibromatosis type I

Sudden infant death syndrome (SIDS) is the unexpected death of an infant under the age of 1 year, where a complete autopsy, including scene investigation, fails to reveal a cause of death. Although the frequency of SIDS has decreased almost 50% over the past 10 years, it remains the leading cause of death in infants aged 1 to 6 months. SIDS is a diagnosis of exclusion and requires the elimination of a wide range of possible causes, including asphyxia, poisoning, abuse, occult heart disease, and other natural disease processes. In this report, we describe the case of an infant death initially suspected to be a SIDS death in which autopsy revealed an optic pathway glioma (optic glioma or hypothalamic glioma) and other stigmata of neurofibromatosis type I.     

Abnormal heart rate response during newborn sucking behavior study: Subsequent sudden infant death syndrome with cardiac conduction abnormality

This report concerns a four-month-old white female infant who exhibited abnormal feeding behavior and EKG irregularities during a newborn sucking behavior study. The immediate post-birth history showed an irregular heart rate on two occasions, but a cardiac consultation elicited no unusual findings. During sucrose sucking conditions, the heart rate increased with a beat-to-beat variation of 50 beats per minute, noted to be due to premature atrial beats. At 39 days, an EKG showed a marked sinus tachycardia of 156, a PR interval of 0.08, QRS of 0.05 and a QT of 0.26. The infant was diagnosed as a sudden infant death syndrome (SIDS) following an unexpected death at home. Subsequent cardiac pathology revealed an anomalous tract between the right atrium and the atrioventricular (AV) bundle which formed an extensive bypass of the AV node (atrio-His tract), and two accessory AV connections between the left atrium and posterior left ventricle. These findings are consistent with the Wolff-Parkinson-White syndrome type A. Only further studies can determine whether such abnormal feeding behavior with EKG irregularities can be used to identify infants who are at high risk for sudden death.     

Coronal cleft vertebra initially suspected as an abusive fracture in an infant

A case of a four-month-old male infant is described who was found unresponsive at home and brought to the emergency room. He lived for four days. During his hospital admission he was diagnosed by radiography with a fracture of the third lumbar vertebra that was presumed abusive in nature. Autopsy examination failed to confirm a fracture. However, a defect in the development of the vertebral bodies was discovered. He was diagnosed with possible failure of the notochord to regress, a condition with no significant sequelae. The cause of death was certified as sudden infant death syndrome after full investigation, and all autopsy studies were negative.