Liina Kiho

Routine perinatal and paediatric post-mortem radiography: detection rates and implications for practice

BackgroundRoutine perinatal and paediatric post-mortem plain radiography allows for the diagnosis and assessment of skeletal dysplasias, fractures and other bony abnormalities.ObjectiveThe aim of this study was to review the diagnostic yield of this practice.Materials and methodsWe identified 1,027 cases performed in a single institution over a 2½-year period, including babygrams (whole-body examinations) and full skeletal surveys. Images were reported prior to autopsy in all cases. Radiology findings were cross-referenced with the autopsy findings using an autopsy database. We scored each case from 0 to 4 according to the level of diagnostic usefulness.ResultsThe overall abnormality rate was 126/1,027 (12.3%). There was a significantly higher rate of abnormality when a skeletal survey was performed (18%) rather than a babygram (10%; P < 0.01); 90% (665/739) of babygrams were normal. Of the 74 abnormal babygrams, we found 33 incidental non-contributory cases, 19 contributory, 20 diagnostic, and 2 false-positive cases. There were only 2 cases out of 739 (0.27%) in whom routine post-mortem imaging identified potentially significant abnormalities that would not have been detected if only selected imaging had been performed. A policy of performing selected, rather than routine, foetal post-mortem radiography could result in a significant cost saving.ConclusionRoutine post-mortem paediatric radiography in foetuses and neonates is neither diagnostically useful nor cost-effective. A more evidence-based, selective protocol should yield significant cost savings.

Acute fatal upper airway obstruction from an occult cavernous hemangioma of the larynx.

A 42‐year‐old previously well man collapsed while holding his throat. Resuscitation was unsuccessful, and at autopsy, the most significant findings were limited to the larynx, pharynx, and anterior mediastinum where there were multinodular dark blue tumors. Within the larynx, the lower border of the tumor was well demarcated, not extending beyond the vocal cords. A separate large polypoidal tumor mass was attached to the right aryepiglottic fold by a thin fibrous stalk. Histologic examination revealed numerous large, thin‐walled cavernous‐type vascular spaces typical of a multifocal cavernous hemangioma. Death was due to asphyxiation from obstruction of the upper airways by a cavernous hemangioma of the larynx. Adult laryngeal hemangiomas are rare and are usually supraglottic. This case demonstrates that pedunculated laryngeal cavernous hemangiomas may remain occult until the initiation of an obstructive episode with sudden collapse and death. In such instances, the diagnosis must rely upon an autopsy examination.

Sudden Unexpected Death in Infancy Associated with an Epithelial-Type Hepatoblastoma in a 6-Month-Old Infant

Sudden unexpected death in infancy and childhood attributable to undiagnosed neoplasia is rare. Malignant neoplasms are very uncommon in infancy, with an age-standardized incidence rate of 118.3 per million. Primary malignant liver tumors are rare, with hepatoblastoma accounting for up to two thirds of cases. Although hepatoblastoma is the most common malignant neoplasm of the liver in childhood, it only accounts for 3.1% of childhood cancers for infants less than 12 months of age. We describe the first case of sudden death in an apparently healthy 6-month-old infant whose autopsy revealed an epithelial type hepatoblastoma with mixed fetal and embryonal patterns.

Social and behavioural factors in Non-suspicious unexpected death in infancy; experience from metropolitan police project indigo investigation

BackgroundRisk factors for Sudden Unexpected Death in Infancy (SUDI) are well described, and such cases are now investigated according to standard protocols. In London, Project Indigo of the Metropolitan Police provides a unique, detailed framework for such data collection. We investigate such data to provide a contemporary account of SUDI in a large city and further link data to publically available datasets to investigate interactions with social factors.MethodsRetrospective analysis of data routinely collected by the Metropolitan Police Service in all cases of non-suspicious SUDI deaths in London during a six year period.ResultsSUDI deaths are associated with markers of social deprivation in London. A significant proportion of such deaths are associated with potentially modifiable risk factors such as cigarette smoking and co-sleeping, such behaviour also being associated with social factors, including accommodation issues.ConclusionsRoutinely collected data provide valuable insight into patterns and associations of mortality, with SUDI remaining a significant issue in London. Risk factors include social disadvantage, which may manifest in part by affecting behavioural patterns such as co-sleeping and public health interventions to reduce rates require significant social modification.

Forensic Aspects of Perinatal Pathology

Neonaticide occurs throughout the world and is thought to be one of the least preventable crimes. Appropriately, perinatal pathologists can sometimes be asked to lend their expertise to the forensic examination of stillborn or newly born babies who have died in unusual or suspicious circumstances. Such babies are often the product of concealed pregnancies, have been abandoned shortly after delivery, or have been stillborn and their bodies deliberately (often poorly) concealed. Rarely, mummified or skeletonized remains may be brought to our attention by law enforcement agencies. In addition to the usual problems in the identification of the cause of death, various additional considerations are of importance, principally in relation to the potential for homicide/neonaticide. It is crucial that there be a thorough assessment of the apparent age/viability of the neonate and whether or not it can be determined if the baby had been born alive and had an independent existence, separate from the mother.

The Alimentary Tract and Exocrine Pancreas

Gastrointestinal (GI) abnormality is common in both the fetus and the neonate. GI malformations are diagnosed in fetal life with increasing frequency, enabling delivery in units with appropriate facilities. GI malformations, both isolated and syndromic, are encountered at autopsy, although only a small proportion are in themselves fatal. Malformations and postnatally acquired diseases contribute to neonatal morbidity and mortality, comprising most of the histopathological specimens received from neonatal surgical units. An understanding of GI development, the range of pathological abnormalities, and their associations and significance makes an important contribution to clinical management of the affected individual and his family.

The Reproductive System

A basic requirement of the perinatal autopsy is to document the fetal sex. When this is discordant with the sex documented by the clinical team, it should be communicated rapidly. Occasionally, a disorder of sex development (DSD) may be present, and this should be studied carefully so that the parents may be adequately counseled. Well-documented DSDs, when properly investigated, may also shed new light on our understanding of human reproductive system development. Reproductive tract anomalies can also be associated with complex malformations involving other organs; occasionally a DSD may be the cause of fetal or neonatal demise, such as in cases of pulmonary hypoplasia secondary to hydrometrocolpos.