Management pathway for infants requiring chronic care in neonatal units-a scoping review of practices.

Improvements in diagnostic and therapeutic modalities of antenatal care has meant that the neonatal intensive care units (NICU) need to care for infants with complex congenital conditions who need ongoing care post discharge. This, along with improved survival of the extreme preterm infants, provides the neonatal team with a cohort of infants who require long-standing multi-disciplinary input. However, provision of dedicated teams looking after infants with complex medical needs is not commonly reported in NICU workforce structure. The objective of this study is to perform a scoping review of literature on the presence of structured and dedicated complex care management teams in neonatal intensive care unit and their outcomes on care delivery. A scoping review was undertaken to identify all reported literature on existence of chronic care teams in NICU searching medical databases. A narrative synthesis of results was prepared.   Conclusions: The review identified very few clinical models reported with no robust data on clinical outcome measures. What is Known: • Neonatal units are increasingly providing care for infants who require chronic ongoing input from multiple specialties or allied health professionals. A majority of this cohort is formed by extreme preterm infants who require long term respiratory support or nutritional rehabilitation. • There are examples of dedicated clinical teams looking after complex patients in adult and pediatric intensive care outfits leading to improvements in outcome. What is New: • This is first literature review in the authors' knowledge on the use of complex care teams in neonatal intensive care unit. • This article provides a narrative synthesis of the clinical models that have been used by complex care teams in neonatal intensive care unit and whether they have demonstrated tangible benefits in patient outcome.

Weaning of invasive ventilation in the neonatal intensive care: Towards standardising practice: A narrative review.

To present a physiological algorithmic approach to weaning of invasive ventilation in the neonatal intensive care, focusing mainly on the preterm infant. Review of literature, consensus guidelines, expert commentaries and practice guidelines from individual units. Weaning of ventilation for extubation in the neonatal population must follow certain criteria but needs individualising based on disease process, timeline and physiology. Algorithms based on scenarios and modes of ventilation used might aid the clinician on the floor for decision-making process.

Histogram analysis for bedside respiratory monitoring in not critically ill preterm neonates: a proposal for a new way to look at the monitoring data.

Despite robust evidence in favour of maintaining optimal oxygen saturation targets in the preterm infants, the titration of oxygen is largely dependent on manual observations and transcription. Similarly, notwithstanding the gaining popularity of non-invasive modalities like high-flow nasal therapy, the practices of weaning and escalating support are largely individualized and based on point of care observations. These are often erroneous and lack objectivity. Histogram analysis from patient monitors is an easy and objective way of quantifying vital parameters and their trends. We review the technology and evidence available behind this practice.Conclusions: Though there are no randomized controlled trials on this practice solely, we identify several quality improvement studies implementing this into practice with benefit. We also cite studies which have implemented histogram analysis in methodology, thus concluding that this is a useful clinical tool worth incorporating into clinical practice to reduce manual errors and bring more objectivity into decisions. What is Known: • The data from NeOProM (Neonatal Oxygenation Prospective Meta-analysis Collaboration study protocol) indicates that optimal saturation targets for preterm infants born < 28 weeks should be between 91 and 95%. • The most "failsafe" way of maintaining strict compliance to these limits is automated oxygen titration but this is not widely used or available and manual transcription and monitoring are susceptible to error and fatigue. What is New: • Histogram analysis from patient monitors can provide intelligent data on respiratory monitoring and can be incorporated into algorithm to decide on weaning or escalation of respiratory support. • With appropriate training, histogram monitoring by nursing staff can limit fatigue of manual recording of data.

Outcome Analysis of Congenital Diaphragmatic Hernia Cohort before and after Implementation of Standardized Protocol in a Tertiary Neonatal Unit.

Despite evolving evidence and increased understanding, there is a strong argument that best outcomes in managing congenital diaphragmatic hernia (CDH) patients are achieved in centers which have a high admission rate of such patients and follow standardized operating protocols of management. Pneumothorax and air leak syndromes are one of the main causes of pre- and postoperative morbidity in these patients and experts believe that delivery room sedation and gentle ventilation strategies can minimize this. We observed a significant drop in incidence of pneumothorax and reduction of mortality following implementation of standardized guidelines at the neonatal unit at Southampton which is a tertiary-level neonatal care in the southern United Kingdom and a regional referral center for CDH patients.

Respiratory syncytial virus-neutralizing serum antibody titers in infants following palivizumab prophylaxis with an abbreviated dosing regimen.

BACKGROUND: Monthly injections of palivizumab during the respiratory syncytial virus (RSV) season in at-risk infants reduces RSV-associated hospitalizations. However, the additive effect of naturally acquired immunity remains unclear. The objective of this study was to assess total neutralizing serum antibodies (NAb) against RSV in at-risk infants who had received an abbreviated course of palivizumab prophylaxis. METHODS: Serum samples were collected from infants enrolled in the RSV Immunoprophylaxis Program in British Columbia, Canada over 2 consecutive RSV seasons (2013 to 2015). Infants in this program had received an abbreviated course of palivizumab in accordance with the provincial guidelines. Data were compared to adults and infants less than 12 months of age who did not receive palivizumab. Anti-RSV NAb titers were measured using an RSV microneutralization assay. FINDINGS: Infants who received palivizumab had anti-RSV NAb titers at the end of the RSV season that persisted beyond what is expected from the pharmacokinetics of palivizumab alone. Moreover, 54% of the control infants who did not receive palivizumab and all tested adults had protective anti-RSV NAb titers. CONCLUSIONS: Based on our observations, we hypothesize that naturally acquired NAb provide additive protection, which may significantly reduce the need for additional doses of palivizumab in infants at risk of severe RSV infections.

Multiple Successful Angioembolizations for Refractory Cardiac Failure in a Preterm with Rapidly Involuting Congenital Hemangioma.

Rapidly involuting congenital hemangiomas (RICH) are the commonest variety of congenital hemangioma, often diagnosed antenatally as high-flow arteriovenous shunts causing hemodynamic compromise to the fetus. The postnatal management of such patients is often challenging. We present the case of an infant boy who was delivered prematurely at 29 weeks of gestation due to fetal compromise by a RICH, with features of high-output cardiac failure and major systemic hemodynamic steal from peripheral organs. Two early angioembolizations were required to manage his high-output cardiac failure. To our knowledge, this infant is the smallest and earliest newborn case of successful angioembolization for a complex, life-threatening vascular anomaly. We discuss the interventional dilemmas regarding the optimal timing of delivery and early embolization.

Fatal congenital hypertrophic cardiomyopathy and a pancreatic nodule morphologically identical to focal lesion of congenital hyperinsulinism in an infant with costello syndrome: case report and review of the literature.

Costello syndrome is characterized by constitutional mutations in the proto-oncogene HRAS, causing dysmorphic features, multiple cardiac problems, intellectual disability, and an increased risk of neoplasia. We report a male infant with dysmorphic features, born prematurely at 32 weeks, who, during his 3-month life span, had an unusually severe and ultimately fatal manifestation of hypertrophic cardiomyopathy and hyperinsulinemic hypoglycemia. Molecular studies in this patient demonstrated the uncommon Q22K mutation in the HRAS gene, diagnostic of Costello syndrome. The major autopsy findings revealed hypertrophic cardiomyopathy, congenital myopathy, and a 1.4-cm pancreatic nodule that was positive for insulin expression and morphologically identical to a focal lesion of congenital hyperinsulinism. Sequencing of KCNJ11 and ABCC8, the 2 most commonly mutated genes in focal lesion of congenital hyperinsulinism, revealed no mutations. While hyperinsulinism is a recognized feature of RASopathies, a focal proliferation of endocrine cells similar to a focal lesion of hyperinsulinism is a novel pathologic finding in Costello syndrome.

Caudal duplication syndrome.

Caudal duplication syndrome is a rare entity in which structures derived from the embryonic cloaca and notochord are duplicated to various extents. Its prevalence at birth is less than 1 per 100,000. The term caudal duplication encompasses a spectrum of anomalies and is often used to describe incomplete separation of monovular twins or referred to as part of the spectrum of anomalies associated with conjoined twinning. It usually includes multiple rare malformations and duplications of distal organs derived from the hindgut, neural tube, and caudal mesoderm. It was postulated that the disorder is related to misexpression of one or more of the distal HOX genes, potentially HOX10 or HOX11, leading to abnormal proliferation of caudal mesenchyme. The malformations are usually diagnosed by anomaly scan in the second trimester. Here we report the case of a baby presenting on the first day of life with complete duplication of caudal structures below the dorsolumbar level.

Congenital Sternal Cleft along with Persistent Left-Sided Superior Vena Cava: A Rare Presentation.

Congenital sternal cleft is a rare abnormality resulting from fusion failure of sternum. It occurs in isolation or along with defects of abdominal wall, diaphragm, pericardium, and heart. Early surgical correction is required to protect the underlying structures for risk of cardiac compression. Here we report a case of 20-day female child presenting with congenital sternal cleft associated with multiple congenital heart disease and left-sided superior vena cava. She was operated by the cardiothoracic surgical team successfully and is doing well on followup. We discuss this rare case, imaging studies, and surgical strategy.

Left sided oesophageal lung: a diagnostic challenge.

Bronchopulmonary foregut malformations (BPFMs) include a wide variety of malformations such as intralobar or extralobar pulmonary sequestration, foregut duplication cysts, and diverticula of the gastrointestinal or pulmonary tree (Srikanth et al., 1992). Those anomalies in which a tract between the respiratory and alimentary systems exists are termed communicating bronchopulmonary foregut malformations (CBPFMs). Most infants with CBPFMs suffer from respiratory distress, and an accurate diagnosis may be difficult to make at the patient's initial presentation. Herein we report such a case which posed a diagnostic challenge to us. This baby however survived and is doing well on a 2-year followup.