Jan Sperhake

Shaken baby syndrome: a common variant of non-accidental head injury in infants

BACKGROUNDnRecent cases of child abuse reported in the media have underlined the importance of unambiguous diagnosis and appropriate action. Failure to recognize abuse may have severe consequences. Abuse of infants often leaves few external signs of injury and therefore merits special diligence, especially in the case of non-accidental head injury, which has high morbidity and mortality.nnnMETHODSnSelective literature review including an overview over national and international recommendations.nnnRESULTSnShaken baby syndrome is a common manifestation of non-accidental head injury in infancy. In Germany, there are an estimated 100 to 200 cases annually. The characteristic findings are diffuse encephalopathy and subdural and retinal hemorrhage in the absence of an adequate explanation. The mortality can be as high as 30%, and up to 70% of survivors suffer long-term impairment. Assessment of suspected child abuse requires meticulous documentation in order to preserve evidence as well as radiological, ophthalmological, laboratory, and forensic investigations.nnnCONCLUSIONSnThe correct diagnosis of shaken baby syndrome requires understanding of the underlying pathophysiology. Assessment of suspected child abuse necessitates painstaking clinical examination with careful documentation of the findings. A multidisciplinary approach is indicated. Continuation, expansion, and evaluation of existing preventive measures in Germany is required.

Pathology of fatal traumatic and nontraumatic clostridial gas gangrene: a histopathological, immunohistochemical, and ultrastructural study of six autopsy cases

We prospectively investigated six fatal cases of clostridial gas gangrene using autopsy, histology, immunohistochemistry, microbiology, and scanning electron microscopy. The causative pathogen was Clostridium perfringens in four cases, C. sordellii in one case, and a mixed infection with both C. perfringens and C. sordellii in one case. According to the previous medical history and autopsy findings, clostridial infection was related to trauma in three cases. Characterized by extensive tissue necrosis and total absence of an accompanying leukocyte infiltration and tissue inflammatory response, the histopathological picture of clostridial gas gangrene is distinctly different from other bacterial infections. In medicolegal casework, the proof of the source of infection and the portal of entry of the responsible pathogen is not always an easy task, especially in the absence of trauma.

Cytokines and sudden infant death

BackgroundIt has been hypothesised that inflammatory reactions could play an important role in the pathway(s) leading to sudden and unexpected death in infancy. On a molecular level, these reactions are regulated by various cytokines.MethodsTo characterise the role of IL-1ß, IL-6 and TNFα more precisely, the concentrations of these cytokines were determined quantitatively using specific ELISA techniques in serum and cerebrospinal fluid (CSF) in 119 cases of sudden infant death. The infants were grouped into four categories (SIDS, SIDS with infection, natural death due to infection and unnatural death).ResultsA good correlation was found between CSF and serum for IL-6 (Spearman correlation coefficients (SCC), 0.73) and also for TNFα (SCC, 0.57), although the CSF concentrations were lower than that from the serum. There were no significant differences between the categories of death for any of the serum or CSF cytokines. Compared with normal values, increased serum concentrations of IL-1ß, IL-6 and TNFα were found in 70%, 69% and 38% of the cases respectively, indicating possible agonal or post-mortem changes of cytokine concentrations. In three cases very high cytokine concentrations were found (mainly for IL-6). This may have contributed to the mechanism of death (cytokine storm) in two of the cases.ConclusionsIn a small group of patients, very high cytokine concentrations are a possible explanation for the cause of death (“cytokine storm”).

Using the table in the Swedish review on shaken baby syndrome will not help courts deliver justice

1.Department of Forensic Medicine, Section on Forensic Pediatrics, Netherlands Forensic Institute, The Hague, The Netherlands 2.Institute of Legal Medicine/University Hospital of Cologne, Cologne, Germany 3.Department for Pediatric and Adolescent Medicine, Child Protection Center, Klinikum Kassel, Germany 4.Department of Forensic Medicine, Netherlands Forensic Institute, The Hague, The Netherlands 5.Forensic Pathology, University Medical Center, Maastricht, The Netherlands 6.Forensic (Paediatric) Radiology, Amsterdam Medical Center, Amsterdam, The Netherlands 7.Department of Legal Medicine, University Medical Center, Hamburg-Eppendorf, Germany 8.Forensic Pathology and Clinical Forensic Medicine, Oslo University Hospital and Institute of Clinical Medicine, University of Oslo, Oslo, Norway

Current recommendations on infants’ sleeping position are being followed—initial results of a population-based sentinel study on risk factors for SIDS, 1996–2006, in Hamburg, Germany

Sudden infant death syndrome (SIDS) is a target for public health care in Germany. The aim of this study was to monitor data on risk-related behavior in the population of Hamburg, Germany, in order to respond to changes quickly and to estimate the effectiveness of prevention activities. Data have been gathered using the sentinel system with repeated surveys (1996, 1998, 2001, and 2006) in pediatric practices, thus allowing an estimate of the prevalence of risk factors in an urban population, both transversally and vertically. From 1996 to 2007, the SIDS rate in Hamburg fell from 0.9/1,000 live births to 0.1. The prevalence of infants sleeping prone declined from 8.1% in 1996 to 3.5% in 2006. In this small subgroup, up to 81.7% (2006) of the caretakers were well aware of the risk of sleeping prone. The prevalence of infants sleeping on their sides fell from 55.3% in 1998 to 10.6% in 2006. The sentinel setting is suitable for gathering risk-related data on SIDS. Despite the fact that, so far, no nationwide back-to-sleep campaign has been instituted in Germany, local campaigns have proved successful in reducing prone sleeping for infants. Moreover, the substantial reduction of side sleeping within a short time span going along with a reduced SIDS rate is an indicator of the effectiveness of prevention activities on a local basis.

Estimation of the postmortem interval by analyzing potassium in the vitreous humor: could repetitive sampling enhance accuracy?

AbstractThe relationship between the postmortem interval and the potassium concentration in the vitreous humor has been described by different authors. A limitation of most previous studies might be that all of the vitreous humor has been extracted in 1 sample from different subjects and that the sample was therefore inhomogeneous. In 32 traumatic deaths with known postmortem intervals, small quantities of vitreous humor have been sampled repetitively in 3-hour interval. The bodies were stored at 20°C. The average concentrations of potassium in relation to the postmortem interval were 6.11 to 14.46 mmol/L. An equation that allows accurate prediction of the postmortem interval was established: postmortem interval (h) = 2.749 × [K+] − 11.978.

Pathological Features of Waterhouse-Friderichsen Syndrome in Infancy and Childhood

Between 1997 and 2002, five cases of fatal Waterhouse—Friderichsen syndrome (WFS) that occurred in infancy or childhood were investigated in our institute. The diagnosis of WFS was based on the following clinical as well as morphological criteria: (a) fulminant sepsis, (b) patchy purpura of the skin as a result of disseminated intravascular coagulation (DIC), and (c) bilateral hemorrhagic necroses of the adrenals. All cases had a very rapid clinical course of the disease (about 1 day or less). In two cases, postmortem microbiological examinations yielded meningococci as the infective agent. In both cases, the children examined underwent autopsy very early after death (5 hours and 24 hours, respectively) and did not receive antibiotics prior to death. In two other cases, meningococci were cultured in antemortem blood samples. Macroscopically, the leptomeninges looked inconspicious in all five cases investigated. However, histology revealed meningitis to a mild or moderate degree in four cases. A previously clinically undiagnosed interstitial myocarditis was diagnosed in three cases by histological means, of which two cases showed Gram-negative diplococci in the myocardial lesions. For the pathologist investigating cases of WFS in infancy and childhood, the following points have to be emphasized: (a) when performing a medicolegal autopsy in a case of suspected WFS, the postmortem interval should be as short as possible (not longer than 24 hours) to provide the opportunity for an accurate microbiological examination of postmortem swabs; (b) in cases of WFS, meningitis does not seem to play a leading role for the clinical course of the disease or the actual cause of death; however, histologically a mild to moderate degree of meningitis is a frequent finding; (c) the presence of Gram-negative diplococci in myocardial lesions by histological means suggests that invasion of meningococci might be a causative factor for myocarditis in WFS; and (d) in accordance with the literature, myocarditis is often present in cases of WFS and therefore might be of importance for the clinical course. It is not certain yet if all children die from shock or DIC or rather, if myocarditis, leading to complete heart block, forward failure of the heart, or arrhythmia, is the actual cause of death.

Pseudomembranous colitis with fatal outcome in a 43-year-old man

Pseudomembranous colitis is a life-threatening complication of broad spectrum antibiotic therapy caused by Clostridium difficile. Untreated, the disease can lead to severe and in many cases fatal complications such as peritonitis due to colonic wall perforation, shock as a consequence of volume depletion, toxic megacolon and massive lower gastrointestinal haemorrhage. Fatal complications mostly occur in elderly people with a high degree of comorbidity. We report the case of a 43-year-old patient with AIDS who was admitted to hospital with abdominal pain of unknown origin and died before the correct diagnosis could be established. Autopsy and postmortem stool cultures revealed severe pseudomembranous colitis due to C. difficile with toxic cardiac and circulatory failure as cause of death.

The prone sleeping position and SIDS. Historical aspects and possible pathomechanisms

The incidence of SIDS decreased during the previous 25xa0years significantly. This is mainly due to epidemiological research identifying important risk factors such as prone sleeping position and subsequent campaigns to reduce this risk factor.Originally, the prone sleeping position for babies had been strongly recommended in the sixties and seventies despite previous publications pointed to the associated risk. Worldwide, many infants died of SIDS whose deaths could have been avoided. Today, the recommendation that infants should sleep in supine position has been scientifically verified. In supine sleeping position, pathophysiological mechanisms can be avoided which may lead to hypoxia and death in prone position. Such mechanisms could be occlusion of airways (in particularly associated with face-down position), elevated diaphragm, positional cerebral hypoxia caused by constriction of arteries, rebreathing CO2, and overheating.Irrespective of the specific pathomechanism leading to death in individual cases, it has been established that the prone position is the most important risk factor for SIDS and therefore should be incorporated in the definition of the term SIDS.

Early time-related course of image findings in postmortem MRI: Typical findings and observer agreement in a porcine model

PURPOSEnTo assess early time-related image findings in postmortem magnetic resonance imaging (MRI) and to evaluate observer agreement in a porcine model.nnnMATERIAL AND METHODSnThree pigs were examined by MRI at 15 different time points in the first 36h after death. Two observers independently recorded the postmortem interval (PMI) to the appearance of fluid collection (pleural space, interlobar and interlobular pulmonary fissures and hepatic interlobar fissures), gas accumulation (intrahepatic and intracardial) and blood clotting (aorta and intrahepatic venous vessels). Cohens Kappa statistics and intraclass correlation coefficient (ICC) were used to evaluate intra- and interobserver agreement.nnnRESULTSnIntrahepatic gas was evident after 12, 17 and 30h. Also, intracardiac gas was present in all animals. However, no chronological appearance has been observed. Blood clotting in the hepatic vein was observed within 3min, aortic clotting with a delay of 2-12h after death. Interlobular pulmonary fluid (median PMI: 2h, range 2-2h), pleural effusion (median PMI: 10.5h, range 9-12h) and hepatic interlobar fissure fluid (median PMI: 11.5h, range 11-12h) displayed a chronological appearance pattern. Qualitative and quantitative detection of most postmortem findings showed excellent intra- and interobserver agreement with Kappa values>0.8 or ICC>0.75.nnnCONCLUSIONnPulmonary and hepatic fissural fluid collection as well as intravasal gas accumulation are early time-related image findings in postmortem MRI. The chronological appearance of these findings can be assessed with excellent observer agreement in a porcine model.