Outcomes of babies with surgical anomalies

Neonatologists basically invented the field of outcomes research; many of my colleagues from around the world have closely examined the outcomes of very premature and extremely premature infants for many years: well before people in other fields were doing so. Outcome research for other babies that we look after has been more recently performed; here are a few recent studies about surgical congenital anomalies.

Bojanic K, et al. Cardiopulmonary exercise performance is reduced in congenital diaphragmatic hernia survivors. Pediatr Pulmonol. 2016. In this study performed at 5 to 20 years of age, among 27 the survivors of CDH repair, reduced exercise tolerance and other respiratory symptoms were present in about half. VO2 max was much lower but equally variable (which means in relative terms, more variable) than a control group. The results are not too surprising for a condition which is nearly universally lethal without intervention, but the implication is that testing like this could lead to an intervention (that is, exercise training) that might improve outcomes in some individuals. Of course, exercise is good for everyone who can do it. I wonder if it is possible that part of the effect is due to over-protection of children with “weak lungs” and whether one intervention should be just to re-assure parents that exercise is good, and getting tired after exercise is normal.

Calzolari F, et al. Outcome of infants operated on for congenital pulmonary malformations. Pediatr Pulmonol. 2016. In this study the authors looked at outcomes of children who had had congenital cystic adenomatoid malformations (apparently they are now called congenital pulmonary airway malformations CPAM; but it seems a bit redundant to put pulmonary and airway in the same acronym) or sequestrations (which they call broncho-pulmonary sequestrations BPS) or congenital lobar emphysema (which they now call congenital alveolar overdistension CAO). These patients biggest problem in follow up is that nobody knows what problem they had because the acronyms have all changed! There is a lot of uncertainty about what to do with children who have these lesions but who do not have symptoms. Apparently, in the Ospedale Bambino Gésu (I just wanted to type that mellifluous name) in Rome, they operate on all diagnosed cases, which may or may not be the right thing to do, or at least that is what they say in the methods, in the results, they note that there were 8 non-operated infants, who were excluded from the study. They finished with 68 patients aged 4 to 9 years, and compared them to a group of kids who had inguinal hernia surgery. 40% of the patients and 12% of controls had recurrent wheezing, and needed inhaled bronchodilators. There were only 5 patients with congenital lobar emphysema, but they all had recurrent wheeze, the other 2 groups were similar to each other. Many of the patients (20%) had had respiratory tract infections, and none of the controls. The patients who had a lobectomy were more at risk, and babies who needed post-operative assisted ventilation. As they got older the symptoms improved.

Roberts K, et al. Outcomes of oesophageal atresia and tracheo-oesophageal fistula repair. J Paediatr Child Health. 2016. A review article examining the long-term impacts of the conditions in the title. The long-term function of the oesophagus is impaired, with frequent reflux and prolonged, even life-long dysphagia. 50% to 90% of adolescents and adults have at least occasional dysphagia, with many avoiding certain foods, and up to 20% have daily symptoms. Recurrent wheeze is common, many have restrictive defects on lung function testing, but most adults have normal exercise tolerance. Developmental testing has been less frequent, but there do seem to be developmental delays, especially in the language scores of the Bayley scales, version 2, the delays seem to be greater among infants with malnutrition during infancy, underweight being common in the first year, usually improving afterward. Quality of life scores overall are similar to the general population, but Health related quality of life is reduced, mostly because of dysphagia and reflux. A very complete, and clearly written review.

Giudici L, et al. Babies born with gastroschisis and followed up to the age of six years faced long-term morbidity and impairments. Acta Paediatr. 2016;105(6):e275-80. This study from Buenos Aires followed 54 survivors of gastroschisis at 1 year, 34 at 3 years and 117 at 6 years. I don’t recognize the tests that were used to evaluate neurological and developmental progress, but there were frequent delays, with only 64 babies in the normal range at 1 year, 50% at 3 years and 35% at 6 years. Language delays were frequent, but that tended to improve with time, and intervention. 20% of the infants had growth below the 10th percentile, throughout the study.

Sterken C, et al. Neurocognitive Development After Pediatric Heart Surgery. Pediatrics. 2016;137(6). This study followed 107 infants who had cardiac surgery in early life, they were eligible for the study if they surgery at less than a year of age, many were newborns, and the median age of having surgery was 2 months. They then tested 100 of the children again 3 years later, as well as most of the controls. The heart surgery patients had IQ scores that were about 12 points lower than controls at 4 years of age, and 7 points lower at 7 years of age. They also had deficits in visuo-motor integration, alertness, motor coordination, and psychosocial functioning, and these deficits also stayed stable.

Now that we are getting much better information about the long-term problems that these children may have. We need to do the next thing, and study how to help them improve. Whether or not the same strategies will be effective for children in each of these diagnostic groups is uncertain.

About Keith Barrington

I am a neonatologist and clinical researcher at Sainte Justine University Health Center in Montréal
This entry was posted in Neonatal Research and tagged , , , , , . Bookmark the permalink.

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