Corrado Minutillo

Growth and developmental outcomes of infants with gastroschisis at one year of age: A retrospective study

BACKGROUND The aim of the study was to describe the physical growth and developmental outcomes of babies born with gastroschisis. METHODS We retrospectively reviewed all cases of gastroschisis in Western Australia born between 1997 and 2010. RESULTS In the 128 pregnancies with fetal gastroschisis, 117 babies were live born. 112 (95.7%) survived to one year. 19% had z scores of<-1.28 for weight at birth (<10th centiles) compared with 30% at one year. Neurodevelopmental data were available in 88/112 (79%) of survivors (Griffiths scores in 67; reports of ages and stages questionnaire (ASQ) in 21). The mean GQ at 12 months was 99 (SD 9.8). Suboptimal neurodevelopmental outcomes were noted in eight. Complex gastroschisis (present at birth) and acquired gut related complications were associated with adverse long term outcomes. The incidence of acquired gut complications was least (5%) in those who underwent silo reduction as the primary management. However, on univariate and multivariate analysis, the type of primary reduction did not significantly influence the outcome. CONCLUSIONS A large proportion of infants with gastroschisis exhibit suboptimal weight gain during the first year. The incidence of adverse developmental outcomes appears to be low.

The long-term neurodevelopmental and psychological outcomes of gastroschisis: A cohort study.

OBJECTIVES Previous gastroschisis specific neurodevelopmental studies have focused on the first 3years of life. The aim of this study was to assess the intellectual, behavioral and neurological outcomes of older children and adolescents born with gastroschisis. STUDY DESIGN Of 99 gastroschisis survivors born in Western Australia, 1992 to 2005, and who were at least 5years old, 42 agreed to take part in this study. The study assessed: intellectual ability, with age appropriate Wechsler intelligence scales; neurological status; hearing; vision; behavioral status with the Strengths and Difficulties Questionnaire (SDQ); and parenting style with the Parenting Relationship Questionnaire (PRQ). All results were compared to normative means. RESULTS Median age at follow-up was 10years (range 5-17). No child had evidence of cerebral palsy or hearing loss; 1 child had amblyopia. Psychometric tests were completed in 39 children: mean full scale IQ was 98.2 (standard deviation [SD] 10.7); the working memory index was the only subscale to show a significant decrease from the normative mean (mean 95.5, SD 12.4, p=0.038). The mean SDQ behavioral scores were significantly lower for 3 of 5 domains and the Total Difficulties score. PRQ scores were significantly abnormal for 4 of 7 domains: Communication, Discipline, Satisfaction with School and Relational Frustration. CONCLUSIONS Overall intellectual abilities were within a normal range. The decrease in working memory index and the behavioral and parenting relationship impairments could be an effect of perinatal factors, gastroschisis management and complications or the complexity of the socio-economic environment.

Cuffed endotracheal tubes for neonates and young infants: a comprehensive review

Traditionally, uncuffed endotracheal tubes (ETTs) have been used for artificial ventilation of infants and children. More recently, newer designed high-volume low-pressure (HVLP) cuffed ETTs are being used with increasing frequency in infants from birth. Considering that many paediatric anaesthetists and intensivists are already using cuffed ETTs in infants >3 kg from birth, should neonatologists be doing the same? This review examines the reasons behind the traditional use of uncuffed ETTs and the problems associated with their use; newer HVLP cuffed ETTs and what they can potentially offer neonates; and reviews evidence from studies comparing the use of cuffed and uncuffed ETTs in neonates and small infants.

Cuffed endotracheal tubes in neonates and infants: a survey of practice

High volume low pressure cuffed endotracheal tubes (ETTs) are being used with increasing frequency in neonates during anaesthesia and in paediatric intensive care units (PICUs). The incidence of use of cuffed ETTs in neonatal intensive care units (NICUs) is unknown. To our knowledge, there are three survey reports on the incidence of use of cuffed ETTs in young children. In a French survey, Orliaguet et al 1 reported that 25% of paediatric anaesthetic respondents used cuffed ETTs ‘routinely’ and 38% used them ‘frequently’. Flynn et al 2 reported that only 5% PICU and 7% anaesthetic respondents ‘routinely’ used cuffed ETTs in neonates and infants in Britain. Nishisaki et al 3 reported that 90% of intubations across 15 PICUs in North America were with cuffed ETTs. In …

Growth and Developmental Outcomes of Infants with Hirschsprung Disease Presenting in the Neonatal Period: A Retrospective Study

OBJECTIVES To describe the presentation and progress over the first year of life of neonates with Hirschsprung disease, to describe their physical and developmental outcomes at 12 months of age, and to compare the outcomes of infants with short- vs long-segment Hirschsprung disease. STUDY DESIGN A retrospective study of neonates born with Hirschsprung disease in Western Australia between January 1, 2001, and December 31, 2010, to review their presentation, progress, growth, and development at 12 months of age. RESULTS Fifty-four infants were identified (40 with short and 11 with long segment and 3 with total colonic aganglionosis); 9 infants had a recognized syndrome and 1 infant died, unrelated to Hirschsprung disease. A primary pull-through procedure was performed in 97% and 21% of neonates with short- and non-short-segment Hirschsprung disease, respectively; 17 (31%) infants developed anal stenosis requiring dilatations. Enterocolitis occurred in 14 (26%) infants. Griffiths Mental Development Scale scores (1 year) were available in 31 of 45 nonsyndromic survivors: mean general quotient (94.2, SD 8.89) was significantly less than the population mean (P = .007), but the number of infants with developmental delay was within the expected range. Physical growth, except length, appeared adequate in nonsyndromic infants. There were no significant differences in the outcomes of infants with short- vs non-short-segment Hirschsprung disease. CONCLUSIONS At 1 year of age, many infants with Hirschsprung disease have ongoing gastrointestinal problems. Their overall growth appears satisfactory, and most infants are developing normally; however, their mean general quotient appears shifted to the left. Longer-term studies will better define developmental outcomes.

Cuffed endotracheal tubes in infants less than 3 kg: A retrospective cohort study

Cuffed endotracheal tubes are being increasingly used in infants; however, current evidence in the literature mostly includes infants ≥ 3‐kg weight.

Severe acquired subglottic stenosis in neonatal intensive care graduates: a case–control study

Objective To analyse current incidence and risk factors associated with severe acquired subglottic stenosis (SASGS) requiring surgical intervention in neonates. Design Retrospective case–control study. Setting Sole tertiary children’s hospital. Participants Patients who underwent surgical intervention for SASGS from January 2006 to December 2014. For each neonatal intensive care unit (NICU) graduate with acquired SASGS, two controls were selected (matched for gestation and year of birth). Main outcomes and measures Incidences were calculated and cases and controls compared using conditional logistic regression analysis to identify risk factors for SASGS. Results Thirty-seven NICU graduates required surgical intervention for SASGS of whom 35 were <30-week gestation at birth. The incidence of SASGS in surviving children who had required ventilation in the neonatal period was 27/2913 (0.93%). Incidence was higher in infants <28-week gestation (24/623=3.8%) compared with infants ≥28-week gestation (3/2290=0.13%; p=0.0001). On univariate analysis, risk factors for SASGS were: higher number of intubations (4 vs 2; p<0.001); longer duration ventilation (16 vs 9.5 days; p<0.001); unplanned extubation (45.7% vs 20.0%; p=0.007); traumatic intubation (34.3% vs 7.1%; p=0.003) and oversized endotracheal tubes (ETTs) (74.3% vs 42.9%; p=0.001). On multivariate analysis, risk factors for SASGS were: Sherman ratio >0.1 (adjusted OR (aOR) 6.40; 95% CI 1.65 to 24.77); more than five previous intubations (aOR 3.74; 95% CI 1.15 to 12.19); traumatic intubation (aOR 3.37; 95% CI 1.01 to 11.26). Conclusions SASGS is a serious consequence of intubation for mechanical ventilation in NICU graduates, especially in preterm infants. Minimising trauma during intubations, avoiding recurrent extubation/reintubations and using appropriate sized ETTs may help prevent this serious complication.

A modern era comparison of right versus left sided congenital diaphragmatic hernia outcomes

BACKGROUND/PURPOSE This study aims to retrospectively review outcomes, including neurodevelopmental outcomes, of neonatal right sided congenital diaphragmatic hernias (RCDH) compared with left sided congenital diaphragmatic hernias (L-CDH) treated surgically at our institute. METHODS A retrospective review was undertaken of all cases of congenital diaphragmatic hernia (CDH) treated at Princess Margaret Hospital for Children (PMH), Perth, born between 1st January 2002 and 1st August 2012. The outcomes of R-CDH cases were compared with L-CDH cases. We examined duration of ventilatory support, use of patch versus primary closure, the CDH recurrence rates, the number of reoperations and neurodevelopmental follow-up at one year of age. RESULTS Forty-nine cases of CDH were operated on at PMH during the 10-year period. Of these, ten cases were R-CDH with 39 L-CDH cases. Of 49 cases, 34 were diagnosed antenatally, 5 R-CDH versus 29 L-CDH. Only 8/39 cases of L-CDH required patch repair for larger defects, while 5/10 R-CDH required patch repair. Postoperative mortality was 6/49 (1/10 right sided versus 5/39 left sided). Recurrence was observed in 5/10 R-CDH versus 6/39 L-CDH with p=0.03. Thirty-three of 43 surviving patients received one-year follow-up with Griffiths general quotient (GQ) assessment demonstrating a median score of 98 for L-CDH (IQR 86 to 104.25) and 91 for R-CDH (IQR 76.5 to 93). CONCLUSIONS R-CDH required patch repair more commonly than L-CDH because of larger defect size or complete agenesis. The rate of recurrent herniation was the only morbidity significantly higher in the R-CDH group. Survivors of R-CDH did not have a significant difference in neurodevelopmental outcome compared to L-CDH cases, with both groups exhibiting normal median GQ scores at one year of age.

Impact of hypoglycaemia on neurodevelopmental outcomes in hypoxic ischaemic encephalopathy: a retrospective cohort study

Background Low blood glucose levels (BGLs) in infants are known to adversely affect neurodevelopmental outcomes. However, this risk is not well explored in infants with hypoxic ischaemic encephalopathy (HIE) that receive therapeutic hypothermia (TH). Additionally, little information is available on the optimal BGLs to target in infants with HIE. Aim To explore the association between hypoglycaemia and neurodevelopmental outcomes at different BGL thresholds (2.6 and 3.0 mmol/L) in neonates with HIE treated with TH. Methods Retrospective cohort study. Clinical information and 2-year neurodevelopmental data using Bayley Scales of Infant Development, third edition (BSID-III) and disabilities were recorded for infants born in Western Australia with HIE and treated with TH between February 2008 and February 2012. Multivariable logistic regression models explored the association between hypoglycaemia and neurodevelopmental outcomes. Results 122 infants underwent a total of 1616 BGL estimations before and during 72 hours of TH. Hypoglycaemia (BGL<2.6 mmol/L) occurred in 38/122 (31%) infants and 11/122 (9%) had recurrent hypoglycaemia (three or more episodes). Infants with recurrent hypoglycaemia (<2.6 mmol/L) had significantly lower mean BSID-III cognitive, language and socioemotional subscale scores. On multivariable analysis, recurrent hypoglycaemia (<2.6 mmol/L) was associated with increased odds of death or disability (adjusted OR 8.15; 95% CI 1.31 to 50.58; p=0.024). Recurrent hypoglycaemia (<3.0 mmol/L) during the first 12 hours of life was also associated with severe disability among survivors (adjusted OR 11.13; 95% CI 2.06 to 59.89; p=0.005). Conclusions Early recurrent hypoglycaemia was associated with increased risk of death or severe disability in neonates undergoing TH for HIE. Prospective studies are needed to identify the ideal target BGL in this population.