Lung Hypoplasia Associated With Ring-Sling Complex Is Usually Right-Sided.

BACKGROUND: Pulmonary artery sling (PAS) is usually associated with long-segment congenital tracheal stenosis (LSCTS). This combination of abnormalities can also be associated with lung hypoplasia abnormalities (hypoplasia, aplasia, or agenesis). This study analyzed the association of lung hypoplasia abnormalities with combined PAS and LSCTS and its influence on its surgical outcomes. METHODS: All patients (0 to 18 years) who underwent surgical procedures for both PAS and LSCTS from 1995 to 2019 were analyzed retrospectively for mortality, ventilation days, and intensive care unit days by dividing them into those with normal lungs (group 1) and hypoplastic lungs (group 2). RESULTS: Included were 75 patients (30 girls [40%]), who were a median age of 5.7 months (interquartile range [IQR], 2.9-13.3 months), median weight of 5.5 kg (IQR, 4.1-7.9 kg), and had a median follow-up of 99.8 months (IQR, 54.5-152.0 months); of these, 8 patients (10.7%) had hypoplastic right lung, comprising hypoplasia in 7 (87.5%), aplasia in 1 (12.5%), and agenesis in 0 (0%). There was a significant difference in mortality (group 1, 9.0%; group 2, 50%; P = .007) but no significant difference in median ventilation days (group 1, 9.0; group 2, 9.0; P = .89) or in median intensive care unit days (group 1, 14.0; group 2, 11.5; P = .44). CONCLUSIONS: Lung hypoplasia associated with PAS and LSCTS is usually right-sided. As a result of severe airway obstruction and single-lung physiology, there is a high requirement of preoperative cardiorespiratory support and a significant association with adverse surgical outcomes.

Bronchial Dieulafoy's Disease in Children: A Case Report and Review of Literature.

Dieulafoy's disease is a rare vascular lesion characterized by presence of large aberrant arteries within the submucosa of gastrointestinal tract or respiratory tract with a potential to cause life-threatening hemorrhage. Treatment includes bronchoscopy ablation, angiographic embolization or surgery. We report management of 7-year old girl with Dieulafoy's disease in the airway who presented with recurrent hemoptysis. Bronchial angiography revealed multiple feeding vessels to the lesion. Considering the potential risk of recurrence with embolization, sleeve resection of bronchus offered complete resolution. This case demonstrates the usefulness of bronchial angiography as part of multi-faceted approach before surgery in the management of Dieulafoy's disease.

Staging of Surgical Procedures in Comorbid Congenital Tracheal Stenosis and Congenital Cardiovascular Disease.

BACKGROUND: Comorbid long segment congenital tracheal stenosis and congenital cardiovascular abnormalities in children pose significant challenges with regard to repairing these abnormalities simultaneously or in stages. The aim of this study was to explore whether this combination of abnormalities needs a staged approach for surgical repairs. METHODS: All children who underwent both tracheal and cardiac surgical procedures at a tertiary hospital from 1995 to 2018 were analyzed retrospectively for mortality, ventilation days, postoperative intensive care unit days, mediastinitis, and unplanned reoperation by dividing them into simultaneous repairs (group 1), staged repairs within the same admission (group 2), and staged repairs during different admissions (group 3). RESULTS: Of 110 patients included in the study (group 1, 74; group 2. 10; and group 3, 26 patients), there was no significant difference in mortality (P = .85), median ventilation days (P = .99), median intensive care unit days (P = .23), unplanned airway reoperation (P = .36), and unplanned cardiac reoperation (P = .77). There was a significant difference in the rate of mediastinitis (group 1, 3%; group 2, 10%; and group 3, 19%; P = .02). There was no significant difference in 5-year survival (group 1, 86.2%; group 2, 77.8%; and group 3, 85.1%; P = .86). A higher STAT category was identified to be a risk factor for mortality in multivariate Cox regression analysis (relative risk, 5.45). CONCLUSIONS: Combined tracheal and cardiac abnormalities need a stratified approach to facilitate better clinical outcomes. Although the trajectory of care is often based on the clinical presentation, establishing a management protocol will be helpful, for which setting an international database will be useful.

Factorial switching linear dynamical systems applied to physiological condition monitoring.

Condition monitoring often involves the analysis of systems with hidden factors that switch between different modes of operation in some way. Given a sequence of observations, the task is to infer the filtering distribution of the switch setting at each time step. In this paper, we present factorial switching linear dynamical systems as a general framework for handling such problems. We show how domain knowledge and learning can be successfully combined in this framework, and introduce a new factor (the "X-factor") for dealing with unmodeled variation. We demonstrate the flexibility of this type of model by applying it to the problem of monitoring the condition of a premature baby receiving intensive care. The state of health of a baby cannot be observed directly, but different underlying factors are associated with particular patterns of physiological measurements and artifacts. We have explicit knowledge of common factors and use the X-factor to model novel patterns which are clinically significant but have unknown cause. Experimental results are given which show the developed methods to be effective on typical intensive care unit monitoring data.

Disturbingly high fracture rate of STRATOS bars in pectus corrections.

OBJECTIVES: Several procedures have been described to correct pectus excavatum and carinatum. We have used a modified Ravitch procedure (STRATOS titanium bars) for patients who were unsuitable for the minimally invasive Nuss procedure. The operation produced excellent cosmetic results, but we have noted several fractures and displacements of the STRATOS bars. METHODS: We reviewed all our STRATOS patients since first use in 2009 until 2014. We collated the following data of each patient: diagnosis, severity of pectus deformity, comorbidity, previous operations for pectus, age at bar implantation, the number of bars implanted and duration of implantation. Patients with severe comorbidity were excluded. RESULTS: Between 2009 and 2014, 39 patients were treated for pectus deformities using the STRATOS bars. Twelve of 39 patients (31%) were identified as having bar(s) broken before removal. There were no significant differences between patients with fractured bars and those without, except for the number of bars implanted (P = 0.016). CONCLUSIONS: This incidence of bar fracture is unexpectedly high, although the literature on this topic is sparse. The high fracture rate is alarming, because of its unpredictability and lack of clear aetiological factors. The operation is done (largely) for psychosocial and cosmetic reasons, and therefore, we must have complete confidence in the quality of the bars used in teenage pectus patients. We raise the issue to warn other units and encourage them to report bar fracture rates.

Pathogenesis of retinopathy of prematurity and possible preventive strategies.

Retinopathy of Prematurity (ROP) occurs when premature birth interrupts normal retinal vascular development. Postnatal tissue oxygen levels are significantly higher than those present in utero. Oxygen therapy further increases oxygen levels in the developing retina. Hypoxia driven, VEGF mediated, retinal endothelial cell proliferation is reduced. Low IGF-1 levels may also contribute to delayed retinal vascular development. The neural structures of the peripheral avascular retina continue to develop, and become more metabolically active. Complex, as yet poorly understood abnormalities of structural and molecular interactions between immature endothelial cells and immature astrocytes at the anterior "leading edge" of retinal vascular development leads to the development of an ROP ridge. VEGF produced by the hypoxic peripheral retina, along with structural abnormalities of cell relationships within, and at the vitreoretinal interface of the ROP ridge, results in extraretinal angiogenesis - stage 3 ROP. Stage 3 ROP may resolve spontaneously, or may progress to traction retinal detachment and blindness.

Apolipoprotein E e4 and its prevalence in early childhood death due to sudden infant death syndrome or to recognised causes.

BACKGROUND: Specific genetic polymorphisms have been shown to be more common in unexplained infant death. The APOE genotype exhibits opposite effects at the extremes of age with protective effects of e4 on perinatal mortality but detrimental effects as age progresses. OBJECTIVE: To determine whether the APOE e4 allele is associated with early childhood (1 week-2 years) unexplained death ('sudden infant death syndrome', SIDS) or with recognised causes (non-SIDS) and to compare these cohorts with published perinatal and adult data. METHODS: DNA was extracted from spleen tissue of children dying in South East Scotland between 1990 and 2002. APOE alleles (e2, e3, e4) were determined using PCR. Comparisons of allele frequencies between groups were made. RESULTS: There were 167 SIDS cases and 117 non-SIDS cases. Allele distributions of SIDS cases were similar to healthy newborns. Allele distributions of non-SIDS cases were more similar to adults than to healthy newborns. The percentage of children with at least one e4 allele was significantly lower in non-SIDS compared to SIDS (p = 0.016). Non-SIDS cases had a higher frequency of e3 compared to SIDS cases (p = 0.01) and to healthy newborns (0.005). CONCLUSIONS: Children dying from identified causes have different APOE allele distributions from SIDS cases, but are similar to adults. Children dying from SIDS have an allele distribution comparable to healthy newborns. The prevalence of e4 in SIDS is not of an order to contribute significantly to the age-related decline in e4.

Prematurity and neonatal noxious events exert lasting effects on infant pain behaviour.

BACKGROUND: There is concern that exposure of preterm infants to noxious insults over a prolonged period may have long term effects on their developing nervous system. AIMS: To investigate medium and long term effects of heel pricks in infants over the first year of life. STUDY DESIGN: Study 1-a longitudinal study, 2 days and 4 weeks after heel prick. Study 2-a cross sectional study over the first year of life. SUBJECTS: Study 1-13 healthy preterm (PT) infants. Study 2-63 full term (FT) and 62 PT infants, divided into 3 timed groups (0-20, 21-37 and 38-52 weeks postterm and corrected for prematurity). OUTCOME MEASURES: Threshold responses (flexion withdrawal (FWR) , gross body movements (GBM) and grimace (G)) to increasing mechanical force applied with Von Frey filaments. RESULTS: Study 1-Thresholds were all significantly lower (more sensitive) from the pricked heel compared to the contralateral side at 2 days and 4 weeks. Study 2-There were significant differences in threshold between PT and FT infants at all time points for both FWR (P=0.001, <0.001, <0.001) and GBM (P=<0.001, <0.001, 0.009 respectively), the preterm infants always being lower. The threshold for the FWR in FT infants steadily increased, but the threshold for the PT infants remained the same. GBM thresholds increased during the year in both FT and PT infants, but were always significantly lower in the ex-preterm group (P<0.012). CONCLUSIONS: Either PT birth or repetitive procedures associated with such birth alters the sensitivity threshold of PT infants compared with FT infants for at least the first year of life.

The neuropathology of stillbirth - correlation with apolipoprotein genotype in a Scottish population based study.

BACKGROUND: The neuropathology of stillbirths has been widely studied but rarely on a population basis. Whether foetal apolipoprotein E (APOE) genotype exerts any influence has been little investigated, despite well known effects in adult brains. AIMS: To establish the neuropathology of a population cohort of stillbirths and compare with the APOE genotype. STUDY DESIGN AND SUBJECTS: The brains of 191 stillbirths (≥24weeks of gestation) were recruited from a Scottish population cohort and grouped by clinical history. APOE genotype was available for 97%. RESULTS AND CONCLUSIONS: One or more neuropathological features, most appearing relatively recent, were found in 54% of 157 antepartum singletons, 44% of 9 abruption-associated stillbirths, 85% of 13 in multiple pregnancies but in only 19% of 12 intrapartum stillbirths. White matter injury (WMI) occurred in 36% of preterm and 21% mature stillbirths. Fresh petechial haemorrhages were common in all groups (29%) but germinal matrix haemorrhage (GMH) (7%) and periventricular leucomalacia (1%) were confined to preterm. GMH was significantly associated with WMI (p=0.003). Placental inflammation was common in intrapartum stillbirths (50%), compared with antepartum (15%), multiple pregnancy (23%) and abruption (0%). ß-Amyloid precursor protein (ßAPP) positive axons (36% stillbirths overall) correlated closely with WMI (p<0.0001), justifying future routine inclusion in foetal neuropathological investigation. This study highlights the paucity of brain damage in intrapartum stillbirths. While APOE2 was significantly overrepresented in stillbirths, there was no correlation between APOE genotype and neuropathological findings. We conclude that APOE does not influence neuropathological outcomes in stillbirths.

Cutaneous hypersensitivity following peripheral tissue damage in newborn infants and its reversal with topical anaesthesia.

The flexion reflex threshold has been used as a measure of sensation in a group of premature infants born at 27-32 weeks postmenstrual age. The threshold in an area of local tissue damage created by routine heel lances was half the threshold on the intact heel on the other side. This indicated a hypersensitivity to tissue damage analogous to tenderness or hyperalgesia reported in adults. In a double-blind study, treatment of the damaged area with the topical anaesthetic cream, EMLA, was found to reverse this hypersensitivity or in other words increase the flexion reflex threshold. Treatment with placebo had no effect. The results show that the newborn infant central nervous system is capable of mounting a chronic pain response to local injury which can be reduced by local anaesthetic.

The pain of heel prick and its measurement in preterm infants.

Variability of physiological parameters was used as a measure of stress in the newborn infant. There was a significant increase in variability of the heart rate (P < 0.01) when the stab of the heel prick occurred in addition to the other elements of the procedure (positioning, warming, alcohol swab cleansing and squeezing). This dummy procedure itself caused some increase in variability although this was not significant at the 5% level. There were similar significant increases in variability of the respiratory rate and O2 and CO2 tensions in the blood (P < 0.05) during the stab procedure.

Pain and compassion in the neonatal unit -- a neonatologist's view.

Research has shown that even extremely premature babies are sufficiently developed, anatomically and physiologically, to be capable of experiencing and responding to pain. All newborn infants and especially those who require intensive care in the first days of life are exposed to some painful procedures. Part of the neonatologist's role is the detection and management of pain in these infants. Difficult challenges come with this role. All medications carry known or potential adverse effects and limited research has been done in this vulnerable population. The benefits and risks of all available pain-relieving measures should be balanced when planning management. Compassion is no excuse for a high incidence of undesirable or dangerous side effects. We must proceed with great care.

Intensive care monitoring: past, present and future.

Monitoring is the serial evaluation of time-stamped data, and the volume of such data in an intensive care unit is huge. Clinical and biochemical data may be available at hourly or more frequent intervals but physiological data are 'continuous'. Although sophisticated monitors display the physiological data in multiple and varied combinations, staff are challenged by the frequency of the false alarms and lack of knowledge of the patterns from which they could predict problems. All these data, together with large amounts of clinical data, lead to information overload. In this paper, the case is made for the development of automatic decision-support system based on statistical and probabilistic analysis of data patterns appropriate for the level of cognition of the user (nurses and juniors at the bedside rather than consultants). Such decision support could both reduce the false-positive alarms that frustrate clinical staff, and improve the early detection of pathophysiological events. We have used the development of a pneumothorax as our paradigm. Our data indicate that the clinical diagnosis of pneumothorax takes a median of 127 minutes, but using short decision algorithms based on routinely available monitoring data, most can be detected within 10-15 minutes of occurrence.

An electronic surveillance system for monitoring the hospital presentation of nutritional vitamin D deficiency in children in Scotland.

BACKGROUND: Routine surveillance would be valuable for vitamin D deficiency as symptomatic vitamin D deficiency may be common in Scotland. AIM: To assess the effectiveness of an electronic surveillance system to determine the current incidence of hospital-based presentation of childhood vitamin D deficiency in Scotland. METHODS: Active surveillance was performed for 2 years as part of an electronic web-based surveillance programme by the Scottish Paediatric Surveillance Unit. Notifications were followed by completion of a questionnaire. To further examine the validity of the system, cases with severe vitamin D deficiency in Glasgow and Edinburgh were identified from the regional laboratory and their clinical details were checked against those identified through the surveillance system. RESULTS: Between September 2009 and August 2011, 109 cases of vitamin D deficiency were notified. The majority of cases (n=82) were reported in Glasgow with an annual incidence of 41 cases per year. Fourteen cases were reported in Edinburgh during the first year of the study and two cases during the second year. At the time of clinical diagnosis, the median age of the children was 2 years (range 3 months-16 years). Cross-validation of data showed that among symptomatic cases that had a measured serum vitamin D of <14 nmol/L, 89% of eligible cases had been reported in Glasgow and 33% of cases had been reported in Edinburgh. CONCLUSION: The incidence of vitamin D deficiency remains high in Scotland. An electronic surveillance system can provide data for studying the epidemiology of vitamin D deficiency but may underestimate the number of positive cases.

The epidemiology of hypernatraemia in hospitalised children in Lothian: a 10-year study showing differences between dehydration, osmoregulatory dysfunction and salt poisoning.

INTRODUCTION: The relative frequencies of the causes of hypernatraemia in children after the neonatal period are unknown. Salt poisoning and osmoregulatory dysfunction are extremely rare and potentially fatal. In this retrospective 10-year study, the incidence, causes and differential biochemistry of hypernatraemia in children is examined. METHODS: Children with hypernatraemia (sodium ≥ 150 mmol/litre) aged >2 weeks to 17 years were identified from laboratory data of two paediatric departments serving the Lothian region of Scotland. A review of patient notes established time of onset and cause. Denominator data were available from the Scottish Health Service. RESULTS: On admission to hospital, 1 in 2288 children (1:1535 admitted as an emergency) had hypernatraemia. This is 1 in 30 563 Lothian children <17 years. Overall 0.04% hospital stays had an episode of hypernatraemia. In 45 children admitted with 64 separate episodes (11 from a case of salt poisoning), the commonest cause was dehydration secondary to either gastroenteritis or systemic infection; 31% had an underlying chronic neurological disorder. A total of 177 further cases developed hypernatraemia after admission. The commonest causes were dehydration secondary to severe systemic infection and postoperative cardiac surgery. Urine sodium:creatinine ratio and fractional excretion of sodium were both much higher in the salt poisoning case than in a child with osmoregulatory dysfunction or children with simple dehydration. CONCLUSIONS: Hypernatraemia after 2 weeks of age is uncommon, and on admission is usually associated with dehydration. Salt poisoning and osmoregulatory dysfunction are rare but should be considered in cases of repeated hypernatraemia without obvious cause. Routine measurement of urea, creatinine and electrolytes on paired urine and plasma on admission will differentiate these rare causes.