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

BACKGROUND: Risk 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. METHODS: Retrospective 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. RESULTS: SUDI 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. CONCLUSIONS: Routinely 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.

Ventilated postmortem computed tomography in children: feasibility and initial experience.

OBJECTIVES: Ventilated postmortem computed tomography (vPMCT) is associated with improved pulmonary imaging compared to standard PMCT in adults. We aimed to evaluate the feasibility of performing ventilated PMCT in children. METHODS: Postmortem thoracic CT was performed before (PMCT) and after ventilation (vPMCT). We used a range of mouthpieces, including endotracheal tubes, bag and mask and laryngeal mask airway (LMA). Hounsfield units of the lungs at PMCT were measured for normal and abnormal lung areas, before and after ventilation. All patients underwent full conventional autopsy and histology. RESULTS: Twelve patients underwent ventilated PMCT, median age 52 days (range 3-304 days). Ventilated PMCT provided diagnostic lung images in all 12 cases, compared to only three unventilated PMCT examinations (p < 0.005). In all cases, ventilated PMCT improved the image quality of aerated lungs irrespective of the method used. Average lung Hounsfield units decreased significantly with ventilation from pre-vPMCT values (-134.1 ± 215.1 vs post-vPMCT -531.8 ± 190.1; p < 0.001). LMA with continuous positive pressure ventilation subjectively provided the best results. CONCLUSION: Ventilated PMCT significantly improves lung aeration in children and can aid recognition of areas of abnormality in paediatric lungs. Such advances will improve accuracy and uptake of imaging-assisted autopsies in children.

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.

Reference ranges for organ weights of infants at autopsy: results of >1,000 consecutive cases from a single centre.

BACKGROUND: Infancy is the most common period for childhood death, including both neonatal deaths from obstetric or medical complications and sudden unexpected infant deaths. The weighing of organs at autopsy is an established process and is recommended in current protocols. However, minimal contemporary data is available regarding reference ranges for organ weights of infants. METHODS: Organ weight data for consecutive infant autopsies over a 14 year period performed at a single tertiary centre, including >1,000 cases, were examined in order to provide up to date reference ranges across this age range, using linear regression modelling and the standard LMS method. RESULTS: 1,525 infant autopsies were analysed, of which 1,190 were subsequently used in the creation of linear regression models prior to performance of the LMS method. Organ weight charts were produced for the 5th, 25th, 50th, 75th and 95th centiles for the heart, lungs, liver, spleen, kidneys, pancreas, thymus gland and adrenal glands. CONCLUSION: This study provides the largest single centre contemporary dataset of infant autopsies allowing provision of up-to-date 'normal' ranges for all major organ weights across this age range.

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

BACKGROUND: Routine perinatal and paediatric post-mortem plain radiography allows for the diagnosis and assessment of skeletal dysplasias, fractures and other bony abnormalities. OBJECTIVE: The aim of this study was to review the diagnostic yield of this practice. MATERIALS AND METHODS: We 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. RESULTS: The 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. CONCLUSION: Routine 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.

Neonatal functional intestinal obstruction and the presence of severely immature ganglion cells on rectal biopsy: 6 year experience.

PURPOSE: We report our experience of managing eight babies who presented with neonatal intestinal obstruction and whose rectal biopsies showed severely immature ganglion cells. METHODS: Neonatal unit records were reviewed to detect patients with suspected Hirschsprung's disease or functional intestinal obstruction. Those with intestinal atresia, anorectal malformation, malrotation, cystic fibrosis and prematurity were excluded. RESULTS: We identified 73 patients born at term. Twenty-seven did not need a rectal biopsy. Twenty-one had biopsy proven Hirschsprung's disease, while 17 had a normal rectal biopsy. Eight patients, all of whom presented with severe abdominal distension, showed immature ganglion cells. Seven had failed to pass meconium after birth. X-rays in all patients showed distended loops of bowel. Two neonates underwent an emergency laparotomy and a stoma. A repeat biopsy at 3 months showed maturation of ganglion cells and the stoma was reversed. Rectal biopsy was repeated in two other patients 2-9 months after the first biopsy and showed mature ganglion cells. At follow-up, one patient still suffers from severe constipation. Seven are asymptomatic now, including the two patients who needed a stoma. CONCLUSION: Immature ganglion cells on rectal biopsy may be an indicator of transient functional immaturity of the intestine.

Case Report: Fatal case of disseminated BCG infection in an infant born to a mother taking infliximab for Crohn's disease.

We present the case of a 28 year old lady with refractory Crohn's Disease treated with infliximab throughout her pregnancy. Her baby was born healthy and received a Bacillus Calmette-Guérin (BCG) vaccine aged 3 months. Soon after this the infant became unwell and died aged 4.5 months. At post-mortem the cause of death was attributed to an unusual complication of the BCG vaccine, known as disseminated BCG. BCG vaccination is contraindicated in individuals who are receiving immunosuppressive drugs. We recommend physicians should exercise caution before such vaccines are used in infants born to mothers taking anti-TNF therapies or other potentially immunosuppressive IgG1 antibodies.

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.

Fowler syndrome presenting as a Dandy-Walker malformation: a second case report.

Fowler syndrome, also known as proliferative vasculopathy and hydrocephaly-hydranencephaly, is a lethal condition characterized by hydrocephalus associated with progressive destruction of central nervous system tissue as a result of an unusual and characteristic proliferative vasculopathy. The occurrence of Fowler syndrome in consanguineous families and recurrence in both sexes are suggestive of an autosomal recessive transmission. We present the second case of Fowler syndrome presenting as a Dandy-Walker malformation, in a consanguineous family.

Recurrent placental massive perivillous fibrin deposition associated with polymyositis: a case report and review of the literature.

We present a case of recurrent massive perivillous fibrin deposition (MPVFD) in a patient with polymyositis (PM). Massive perivillous fibrin deposition occurred in 2 consecutive pregnancies: the 1st ended in an intrauterine death at 34 weeks of gestation, while the 2nd pregnancy resulted in a live-born baby at 32 weeks of gestation. The association between MPVFD and PM has been rarely reported in the literature in the form of case reports of single pregnancies.

Maternal pancreatic carcinoma metastatic to the placenta: a case report and literature review.

In this article, we report a case of maternal pancreatic carcinoma metastatic to the placenta. The baby is alive and well, with no evidence of tumor metastasis at the age of 1 year. We also present a comprehensive review of all maternal cancers with metastasis to the placenta and/or baby published in the English language.