Pediatric Giant Cell Myocarditis and Orbital Myositis

Giant cell myocarditis is a rare but often fatal form of myocarditis that often requires cardiac transplantation and has been associated with autoimmune diseases. We describe a 14-year-old female who developed painful proptosis and was diagnosed clinically and histologically with orbital myositis that improved with corticosteroid therapy. Approximately 2 months later, she developed abdominal pain, vomiting, weight gain, and fatigue. She was diagnosed with congestive heart failure and cardiomyopathy, and endomyocardial biopsy revealed giant cell myocarditis. She was treated with immunosuppressive agents and has responded well, without the need for cardiac transplantation. Three previous case reports have described an association between giant cell myocarditis and orbital myositis, but we present the first pediatric case report. We conclude that if orbital myositis is diagnosed in a patient, regardless of age, cardiac function should be closely monitored to detect myocarditis, which may affect the overall outcome.     

CARDIOVASCULAR CAUSES FOR SUDDEN INFANT DEATH

Although sudden infant death syndrome (SIDS) is a cause for sudden infant death, other causes should be ruled out before diagnosing SIDS. Cardiac causes for sudden infant death include viral myocarditis, congential heart disease particularly congential aortic stenosis, endocardial fibroelastosis, and anomalous origin of the left coronary artery from the pulmonary artery. Other cardiac conditions that may result in sudden death include rhabdomyomas of the heart in tuberous sclerosis and conduction system disorders. The most frequent conduction system disorders resulting in sudden death include histiocytoid cardiomyopathy, congential heart block that may be associated with maternal lupus erythematosus, arrhythmogenic right ventricular dysplasia, noncompaction of the left ventricle, and long QT syndromes.     

Distinguishing sudden infant death syndrome from child abuse fatalities

Fatal child abuse has been mistaken for sudden infant death syndrome. When a healthy infant younger than 1 year dies suddenly and unexpectedly, the cause of death may be certified as sudden infant death syndrome. Sudden infant death syndrome is more common than infanticide. Parents of sudden infant death syndrome victims typically are anxious to provide unlimited information to professionals involved in death investigation or research. They also want and deserve to be approached in a nonaccusatory manner. This clinical report provides professionals with information and suggestions for procedures to help avoid stigmatizing families of sudden infant death syndrome victims while allowing accumulation of appropriate evidence in potential cases of infanticide. This clinical report addresses deficiencies and updates recommendations in the 2001 American Academy of Pediatrics policy statement of the same name.     

Incomplete and Atypical Kawasaki Disease: A Clinicopathologic Paradox at High Risk of Sudden and Unexpected Infant Death

Incomplete Kawasaki disease (IKD) and atypical Kawasaki disease (AKD) represent rare conditions. Two cases of unexpected or sudden infant death are reported. The diagnosis for a 3-month-old girl was determined by echocardiography, and the child unexpectedly died despite appropriate treatment, whereas autopsy determined the diagnosis of AKD for a 4-month-old boy. In both patients, giant coronary artery aneurysms with thrombosis and vasculitis, myocarditis, and coagulative necrosis were shown at autopsy. These rare forms of IKD and AKD carry a poor prognosis and represent a paradox between the severe cardiovascular damage and the clinical presentation that mimics common and usually self-limiting exanthematic infectious disease in infancy.     

A case of botulism in a 11-month-old infant

We report a case of botulism in a 11 month-old infant. Hypotonia complicated by progressive bulbar paralysis revealed the disease. Botulism B toxin was present in serum on the 8th day of the disease. On the occasion of this case report the clinical, diagnostic, epidemiological and pathophysiologic aspects of infant botulism are reviewed. The relationship between sudden infant death and botulism is discussed.     

SIDS and other sleep-related infant deaths: expansion of recommendations for a safe infant sleeping environment

Despite a major decrease in the incidence of sudden infant death syndrome (SIDS) since the American Academy of Pediatrics (AAP) released its recommendation in 1992 that infants be placed for sleep in a nonprone position, this decline has plateaued in recent years. Concurrently, other causes of sudden unexpected infant death occurring during sleep (sleep-related deaths), including suffocation, asphyxia, and entrapment, and ill-defined or unspecified causes of death have increased in incidence, particularly since the AAP published its last statement on SIDS in 2005. It has become increasingly important to address these other causes of sleep-related infant death. Many of the modifiable and nonmodifiable risk factors for SIDS and suffocation are strikingly similar. The AAP, therefore, is expanding its recommendations from being only SIDS-focused to focusing on a safe sleep environment that can reduce the risk of all sleep-related infant deaths including SIDS. The recommendations described in this report include supine positioning, use of a firm sleep surface, breastfeeding, room-sharing without bed-sharing, routine immunization, consideration of a pacifier, and avoidance of soft bedding, overheating, and exposure to tobacco smoke, alcohol, and illicit drugs. The rationale for these recommendations is discussed in detail in this technical report. The recommendations are published in the accompanying "Policy Statement--Sudden Infant Death Syndrome and Other Sleep-Related Infant Deaths: Expansion of Recommendations for a Safe Infant Sleeping Environment," which is included in this issue (www.pediatrics.org/cgi/doi/10.1542/peds.2011-2220).     

Acute myocarditis

Myocarditis is defined as inflammation of the myocardium accompanied by myocellular necrosis. Acute myocarditis must be considered in patients who present with recent onset of cardiac failure or arrhythmia. Often there is a history of an antecedent flu-like illness. Fulminant myocarditis is a distinct entity characterized by sudden onset of severe congestive heart failure or cardiogenic shock, usually following a flu-like illness. Giant cell myocarditis is a rare, frequently fatal disorder of unknown origin characterized by presence of giant cell inflammatory infiltrate in the myocardium. In recent years we have made good progress in understanding the causes, pathogenesis, natural history, diagnosis, and treatment of myocarditis. However, our knowledge is still far from complete. New information that extends our understanding of myocarditis is being reported constantly. This review summarizes recent advances in myocarditis, with an emphasis on the literature during the last year.     

Sudden natural death in later childhood and adolescence.

During a 6-year period 389 children and adolescents aged between 1 and 20 years died violently and 31 died suddenly, naturally, and unexpectedly in an area of southern Sweden. In about half of these 31 cases, death was caused by common infectious diseases--for example broncho-pneumonia, myocarditis, or acute epiglottiditis. In one group death was certainly sudden, but was caused by a known chronic disease--such as epilepsy or bronchial asthma. Three young men died from chronic heart disease, and one died from adrenal failure during or immediately after physical exertion. There were 4 cases of sudden, natural death for which the cause was unknown--that is 0.007 per 1000 live births. This figure is extremely low compared with the incidence of sudden unexplained infant death--that is deaths of infants aged between one week and one year.     

Sudden and Unexpected Death-A Late Effect of Occult Intraesophageal Foreign Body

Acute upper airway obstruction in a 4-month-old male infant who presented as sudden infant death syndrome is described. At autopsy external tracheal compression and tracheobronchitis with plugging of the trachea and bronchi by an abundant mucopurulent exudate were found. The source of the inflammation was the adjacent esophagus where previous impaction of a coin had caused pressure necrosis with mucosal erosion and transmural granulation tissue formation. This case is reported to demonstrate that foreign bodies that remain in the esophagus, having by-passed the larynx, may still result in upper airway obstruction and death in early infancy by this unusual mechanism.     

Angina pectoris with troponin increase in arrhythmogenic right ventricle dysplasia: case article and review of the literature

A 16-year-old Hispanic girl with arrhythmogenic right-ventricle dysplasia (ARVD) presented with angina pectoris and troponin increase on three occasions. There was a family history of sudden cardiac death in a cousin. Her mother was diagnosed with ARVD. The patient herself had a history of nonsustained ventricular tachycardia but did not meet diagnostic criteria for ARVD. Cardiac workup, including serial transthoracic echocardiograms and a coronary angiogram, showed a structurally normal heart without coronary artery stenosis. Results of cardiac magnetic resonance imaging were questionable, but endomyocardial biopsy did not show evidence of viral myocarditis by polymerase chain reaction. Genetic testing confirmed ARVD.     

Sudden and unexpected death--a late effect of occult intraesophageal foreign body

Acute upper airway obstruction in a 4-month-old male infant who presented as sudden infant death syndrome is described. At autopsy external tracheal compression and tracheobronchitis with plugging of the trachea and bronchi by an abundant mucopurulent exudate were found. The source of the inflammation was the adjacent esophagus where previous impaction of a coin had caused pressure necrosis with mucosal erosion and transmural granulation tissue formation. This case is reported to demonstrate that foreign bodies that remain in the esophagus, having by-passed the larynx, may still result in upper airway obstruction and death in early infancy by this unusual mechanism.     

Complete heart block from mycoplasma pneumoniae infection

Summary Complete heart block (CHB) in infants and children is usually congenital. Nonsurgical acquired CHB is rare. Occasionally, transient acquired CHB is seen in association with viral myocarditis. We describe here an unusual case of transient CHB in a 12-year-old boy with endomyocardial biopsy-proven myocarditis and evidence ofMycoplasma pneumoniae infection.     

Thrombosis of superior longitudinal sinus and pulmonary embolism in nephrotic syndrome].

The authors report on the case of a child with the nephrotic syndrome complicated by thrombosis of superior longitudinal sinus, bilateral massive pulmonary embolism resulting in a sudden death. A dramatic deficiency in factor XII was demonstrated; the pathophysiology and management of such an abnormality are discussed.     

Apnoea and seizures.

We report two infants with near miss sudden infant death syndrome events exhibiting seizure disorders after caffeine treatment, suggesting there is an infant subgroup diagnosed as near miss sudden infant death syndrome who have apnoea possibly with seizures whose seizure threshold may be lowered by central nervous system stimulants like caffeine.     

Hydropericardium causing sudden infant death in glycogenosis type I: osmotic injury due to percutaneous silastic catheterization

BACKGROUND: The aim of this case report and the review of the literature is to demonstrate dangers when using peripherial silastic catheters in preterm and term newborns or infants. PATIENT, METHODS AND RESULTS: We report on a female infant with glycogenosis type I a (MIM 232200) due to glucose 6-phosphatase deficiency (homozygosity for R170X) and sudden infant death at the age of 9 months due to a rare catheter complication (hydropericardium with tamponade without perforation). CONCLUSION: We believe that this fatal complication was caused by local osmotic dysbalance due to direct contacts between atrial wall and the catheter tip. There is no relation known between patients with inborn errors of metabolism complicated by metabolic derangement and higher incidences of mechanical or non-mechanical catheter complications.     

Sudden infant death syndrome among Asians in California

We analyzed California linked birth and infant death records for 1978 to 1985 to determine the ethnic-specific incidence of sudden infant death syndrome in five Asian-American subgroups, and to assess the association of sudden infant death syndrome with cultural assimilation. The overall incidence of sudden infant death syndrome for these groups was 1.1/1000 live births (194 cases). The ethnic-specific incidence was statistically associated with the immigration status of each ethnic group, as measured by the proportion of all live births for which the mother was born in the United States, with a higher incidence for groups that have been in the United States for the longest period. A logistic model was used to examine simultaneously the association of sudden infant death syndrome with maternal ethnicity, ethnic homogeneity or heterogeneity of the parents, and maternal birthplace (United States, or elsewhere). The logistic analysis did not yield statistically significant evidence to support cultural assimilation as a factor in the incidence of sudden infant death syndrome for these groups. This finding may be due to small sample size and inadequate indicators of assimilation. It may also be that other factors relating to immigration and selective migration affect the incidence of sudden infant death syndrome among Asian-Americans.     

Coexistence of arrhythmogenic right ventricular cardiomyopathy and chronic myocarditis in children

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiomyopathy described in adults as a risk factor in sudden unexpected cardiac death. The disease is of unknown etiology and is characterized by progressive fatty or fibrofatty replacement of the subendocardial layer of the heart muscle, predominantly of the right ventricle. The disease is not associated with primary pulmonary disease or mitral insufficiency. The aim of the present paper is to show that ARVC in children coexists with chronic myocarditis, as was shown with the aid of immunocytochemical staining for markers for T-lymphocytes (CD3, CD4, CD8) B lymphocytes (CD22) and macrophages (CD68), and for adhesion molecules (ICAM-1, VCAM, ELAM) and an endothelial marker (CD31/PECAM-1). Out of 55 endomyocardial biopsy specimens in children with a clinical diagnosis of dilated cardiomyopathy, myocarditis or arrhythmias, ARVD was recognized histologically in 12 patients (21.8%). Nine of these patients had shown immunocytochemical equivalents of persistent myocarditis.     

Risk factors for SIDS in Japan: A record-linkage study based on vital statistics

This study examines the effect of items as reported on birth certificates on sudden infant death. We linked infant death certificates with birth certificates for the infants born in 1989 to residents of the Tohoku, Tokai and Kyushu regions in Japan (n = 409 679), that is, about one-third of the infants born in Japan that year. The mortality rate from sudden infant death, including 88 deaths from sudden infant death syndrome (SIDS) and 17 deaths from instantaneous death, was 25.6 per 100 000 live births. Elevated risk of sudden infant death was associated with low birthweight, late birth order, illegitimacy, male gender and young maternal age. These results correspond to previous studies from Western countries, suggesting a similar pattern for SIDS in Japan.     

Clinicopathological features of paediatric deaths due to myocarditis: an autopsy series.

INTRODUCTION: Myocarditis is a recognised cause of cardiac failure in childhood but the frequency of myocarditis as a cause of sudden unexpected death across the paediatric age range is uncertain. METHODS: A structured review of the results of all autopsies carried out in a single paediatric centre over a 10-year period, including the results of all investigations performed as part of the centre's policy for the post-mortem investigation of paediatric deaths. RESULTS: During the study period there were 1516 autopsies of children aged 0-18 years. Histologically proven myocarditis was present in 28 cases (1.8%, age range 10 days to 16 years, median age 10 months), of which 16 (57%) presented as sudden death. More than half of all cases (54%) occurred in infants less than 1 year of age, accounting for 2% of infant deaths referred for autopsy, compared with around 5% of childhood deaths over the age of 5 years. In almost 40% of cases there were no macroscopic cardiac abnormalities, the diagnosis being entirely dependent on routine histological examination of the heart, and post-mortem heart weight was normal in the majority of cases. Virus was detected in nine (36%) of the 25 cases in whom virological analyses were performed. The histological features were similar in all cases, with an interstitial inflammatory cell infiltrate, predominantly lymphocytic, with focal myocyte necrosis and interstitial oedema. CONCLUSIONS: Myocarditis is a rare cause of death in infancy and childhood, and the majority of cases present as sudden unexpected deaths, which require routine histological sampling of the heart for its detection.     

Grief reaction to sudden unexpected cardiorespiratory arrest in a normal newborn nursery

The authors report two cases of sudden unexpected cardiorespiratory arrest occurring in a normal newborn nursery. They discuss the impact on the families and hospital personnel. The nursing and medical staff demonstrated many of the reactions experienced by families of sudden infant death syndrome (SIDS) victims, including shock, anger, guilt, disbelief, fear, and doubt. The manner in which hospital personnel were supported and counseled is discussed. Specific clinical implications of these cases, including the need to provide for appropriate monitoring and resuscitation in normal newborn nurseries, are presented.