Sudden infant death syndrome in Japan

Two autopsied cases are presented, one involving a 5-month-old infant, and a 6-month-old infant both of whom died suddenly and unexpectedly. The incidence of sudden infant death syndrome in Japan is 1.2 per 1,000 babies live births. Among all cases autopsied in the departments of legal or forensic medicine in 78 universities or colleges of Japan, the incidence was 15 (0.5%) per 3,329 in 1984 and 20 (0.6%) per 3,150 in 1985.     

Sudden infant death syndrome: a subject of medicolegal research

During the last decade, much attention has been paid to the risk factors of sudden infant death syndrome (SIDS). Many researchers have demonstrated that infant-care practices are linked to the risk of SIDS. Prone sleeping, bed sharing, maternal substance abuse, and cigarette smoking have been reported to be significant potentially modifiable risk factors for SIDS. Despite the reports that the incidence of SIDS has decreased by 38% in the United States, it remains the leading cause of death in the first year of life. Deaths resulting from child abuse or neglect inflicted or permitted by their caretakers being second only to SIDS in infant mortalities and some recommendations regarding the differentiation of SIDS and child abuse have generated speculation that some cases of infanticide were misdiagnosed as SIDS. To reach a proper conclusion as to the cause and manner of death of an infant who died suddenly and unexpectedly, investigation must be thorough and professional.     

Sudden infant death with periventricular leukomalacia

Periventricular leukomalacia (PVL) is a form of cerebral infarction occurring in neonates, particularly in low-weight and premature infants. PVL is well-known to neonatologists, but generally considered nonfatal. Many infants with PVL die in the hospital with multiple medical problems. Those infants with PVL who survive because of intensive care will have serious motor and sensory deficits, but these problems are rarely recognized before one year of age. When infants with PVL die at home, death seems sudden and unexpected. However, it is important to distinguish death caused by PVL from the Sudden Infant Death Syndrome because the implications for the family are quite different. This case report emphasizes that PVL may be fatal.     

Sudden infant death due to asplenia syndrome

An apparently healthy 80-day-old boy died suddenly for no apparent reason. The autopsy revealed that the patient had had congenital asplenia, extensive cardiovascular anomalies, and other organ malformations, including trisegmented lungs, hypoplasia of the corpus callosum and cranial bones, a symmetrical liver, accessory hepatic tissue in the adrenal glands, malrotation of the intestine, and hypoplasia of the greater omentum.     

Sudden infant death syndrome (SIDS): a burgeoning medicolegal problem.

This Article presents a summary analysis of the administrative and statutory bases for the documented, prevalent mismanagement of Suddern Infant Death Syndrome (SIDS) cases by a majority of local death investigation agencies in the United States. Herein, Alan P. Cleveland, J.D. advances the theory that the unsatisfactory handling of cases of SIDS by the medicolegal community is the inevitable outgrowth of state laws that expressly require investigative agencies to approach a sudden, unexplained death from the direction of determining first whether or not a criminal act has occurred. In so doing, most statutorily mandated autopsy procedures are socially counterproductive since, in ignoring an acute medical need for supportive family counselling, they often constitute an insuperable obstacle to the effective management of SIDS as a public health problem. The author recommends that a requisite first step in implementing an SIDS management program at the state level is to insulate surviving family members form criminal investigative procedures by appropriate amendment of state laws governing local death investigation systems.     

Sudden infant death with congenital cytomegalic inclusion disease

Cytomegalic inclusion disease was found in a 5-week-old infant who died unexpectedly. Myocarditis was observed; it was believed to be the cause of this unexpected death.     

Sudden infant death and liver phosphoenolpyruvate carboxykinase analysis

In an effort to substantiate the impaired gluconeogenesis-terminal hypoglycemia hypothesis of sudden infant death syndrome (SIDS), 52 infants ranging from 3 weeks to 7 months of age which had been brought to autopsy were studied. The stomach contents, vitreous humor glucose concentrations, hepatic glycogen content and hepatic phospho-enolpyruvate carboxykinase (PEPCK) activity were measured as part of the laboratory component of the postmortem investigation. The stomach contents, vitreous humor glucose concentrations and liver glycogen content were similar in SIDS/and non-SIDS victims. PEPCK activity was, however, significantly lower in SIDS (p < 0.001) victims and in SIDS with other findings (p < 0.01) victims when compared to non-SIDS victims. Despite the fact that SIDS victims had lower hepatic PEPCK activity and hence potentially lower gluconeogenic capacity, terminal hypoglycemia could not be demonstrated in this group as compared to the SIDS with other findings and the non-SIDS infants. The impaired gluconeogenesis-terminal hypoglycemia hypothesis thus could not be substantiated.     

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.     

Sudden infant death: lingual thyroglossal duct cyst versus environmental factors

An 8-month-old female baby was found collapsed in the prone position 30 min after being positioned under soft-bedding. She was taken to the emergency room with cardiopulmonary arrest. Her heartbeat was recovered after resuscitation and continued for 20 h under artificial respiration, at which point the child died. At autopsy, the child showed no significant pathological abnormalities apart from a thyroglossal duct cyst of 2.0 cm diameter, therefore, it seemed that the cyst, which was close to the epiglottis, had caused asphyxia through airways occlusion. However, the child had shown no respiratory problems before death, and the risk of airway occlusion as a result of lingual cysts is more likely in a supine rather than a prone position. A small amount of evidence suggested that the child died as a result of suffocation from being covered by soft-bedding, which could have caused fatal asphyxia; it is also possible that a hypoxic state induced by airway obstruction might have been enhanced by being covered with bedding. It seemed reasonable to assume that death was caused by a combination of the lingual thyroglossal duct cysts and asphyxia caused by being covered in bedding, though the main factor appeared to be the large cyst.     

Sudden death in an 8-week-old infant with Beckwith-Wiedemann syndrome

The authors report a case of a 2-month-old girl diagnosed with Beckwith-Wiedemann syndrome (BWS) who was born prematurely and died suddenly in the hospital just before being discharged. BWS is a malformation syndrome associated with an increased risk of childhood tumors. The major features of BWS are macroglossia, abdominal wall defects, and visceromegaly, frequently leading to premature birth. Due to complex inheritance patterns, a predominance of nonfamilial cases, and the variability in expression of the features (termed incomplete penetrance), the risk of delayed diagnosis is evident. Secondary to hyperplastic pancreatic islands, hypoglycemia occurs frequently, and if not anticipated, adequate measures for prevention of hypoglycemic episodes may be delayed, resulting in possible intellectual deficits. The infant presented here died of natural causes: immaturity of the lungs resulting in marginal respiratory function and compounded by increased risk for asphyxia secondary to the enlarged tongue. The clinical history and findings in this infant are discussed in respect to the genetic syndrome with their relevance to medicolegal examination and the causes and manner of death.     

Sudden death in an infant caused by rupture of a basilar artery aneurysm

Ruptured aneurysms of the cerebrovasculature in infancy and early childhood, except for "giant" aneurysms and arteriovenous malformations, are rare. Seizures, loss of consciousness, and apnea are the usual presenting signs in infancy; symptoms such as headache or visual disturbances and signs such as cranial nerve compression or meningeal irritation commonly found in older children or adults are absent in infants. However, the morphologic findings (i.e., subarachnoid and retinal hemorrhage, and occasionally subdural hemorrhage) may be mistaken for inflicted trauma, especially if the aneurysm is not identified. Sudden death caused by rupture of a cerebral aneurysm has not been previously described in an infant. This report outlines the investigation and autopsy findings in a 7-month-old infant who died unexpectedly as a result of rupture of a complex basilar artery aneurysm.     

Sudden infant death syndrome: Histological studies on adrenal gland and kidney

Histological observations were made on the adrenal glands and kidneys in ten cases of sudden infant death syndrome (SIDS). The amount of fetal cortex was excessive in the adrenal glands and many glomeruli of fetal form were observed in the kidneys. These findings suggest that the adrenal glands and the kidneys in SIDS cases are more or less immature in development. The immature development in these organs, especially in the adrenal glands, was considered to play a role in the cardiac or respiratory mechanisms in SIDS.     

Sudden infant death syndrome: diagnostic practices and investigative policies, 2004

Using a 2004 population-based survey of all US medical examiner and coroner offices, we examined the characteristics of offices accepting an infant death case and calculated the percentage of offices that had death scene investigation or autopsy policies for the investigation of sudden unexpected infant death (SUID). We also calculated the percentage of offices that used and did not use sudden infant death syndrome (SIDS) as a cause of death, and we compared differences in characteristics among those offices.Of medical examiner and coroner offices, 52% did not report an infant death in 2004. Of the 7957 infant deaths reported, 43% occurred in jurisdictions that experienced 1 or 2 infant deaths. Of the offices that used SIDS as a classification, 34% did not have policies for conducting death scene investigations and autopsies for SUID. At least 5% of offices that reported an infant death did not use SIDS as a cause of death classification. These findings have important implications for understanding recent trends in SIDS and SUID. Supporting the implementation of national standards for investigating and certifying infant deaths could provide guidelines for consistent practices in medical examiner and coroner offices.     

Sudden death in an infant caused by rupture of a basilar artery aneurysm.

Ruptured aneurysms of the cerebrovasculature in infancy and early childhood, except for "giant" aneurysms and arteriovenous malformations, are rare. Seizures, loss of consciousness, and apnea are the usual presenting signs in infancy; symptoms such as headache or visual disturbances and signs such as cranial nerve compression or meningeal irritation commonly found in older children or adults are absent in infants. However, the morphologic findings (i.e., subarachnoid and retinal hemorrhage, and occasionally subdural hemorrhage) may be mistaken for inflicted trauma, especially if the aneurysm is not identified. Sudden death caused by rupture of a cerebral aneurysm has not been previously described in an infant. This report outlines the investigation and autopsy findings in a 7-month-old infant who died unexpectedly as a result of rupture of a complex basilar artery aneurysm.     

Sudden unexpected infant death due to fibroma of the heart

A 7-month-old previously healthy female infant was found dead in her crib by her mother shortly after having been laid down to sleep following the noontime feeding. Because the child did not suffer from an acute illness and no other evidence pointed to a cause of death, it was initially assumed by the police that she had died of sudden infant death syndrome. At autopsy, however, the cause of death was determined to be cardiac arrhythmia secondary to fibroma of the heart.     

Sudden infant death from atelectasis due to amniotic fluid aspiration

An infant girl, whose hands showed lobster-claw deformity, was found dead in her bed at 17 days of age. Macroscopic and microscopic examination of the lungs showed fatal atelectasis. The alveolar spaces were filled with fluid, epithelial cells, and squamous debris. These were the constituents of amniotic fluid aspirated before birth. The present case suggested that some sudden unexpected deaths in early infancy are delayed deaths caused by amniotic fluid aspiration.