Outcomes following prosthetic patch repair in newborns with congenital diaphragmatic hernia.

BACKGROUND: The anatomical defect in congenital diaphragmatic hernia (CDH) can usually be closed primarily but prosthetic patch repair may be required in newborns with a deficient diaphragm. High rates of patch failure and hernia recurrence (up to 50 per cent) have been reported. This study evaluated contemporary outcomes following patch repair of CDH at a UK paediatric surgical centre. METHODS: Medical records of newborns undergoing surgery for CDH between 1 February 1990 and 1 November 2010, and attending a multidisciplinary follow-up clinic, were examined. Operative details and patch utilization are reported. RESULTS: Of 118 newborns with CDH, 37 required a patch to the diaphragmatic defect. Gore-Tex® patches were used in 35 and biological Surgisis® patches in two. Eight babies additionally required an abdominal wall patch. Seven infants had an abdominal patch alone with primary diaphragm repair. A total of 102 infants (86·4 per cent) survived after surgery. Two early recurrences were both related to the use of biological patches, leading to revisional surgery with Gore-Tex® patch reconstruction. Diaphragmatic patch use was associated with a greater requirement for intensive cardiovascular and respiratory support, although there was no significant difference in mortality between patch versus primary diaphragm repair. The mortality rate was significantly higher among infants requiring abdominal wall patching (with or without a diaphragmatic patch): 40 per cent (6 of 15) versus 9·7 per cent (10 of 103) (P = 0·006). Postoperative survival rates for infants with a diaphragmatic patch alone, abdominal wall patch alone, and both abdominal and diaphragmatic patches were 86 per cent (25 of 29), 57 per cent (4 of 7) and 63 per cent (5 of 8) respectively. CONCLUSION: Prosthetic diaphragmatic hernia repair at this centre has a good outcome and low rate of recurrence (5 per cent). The recognition of an inadequate abdominal domain prenatally may additionally prove to be a useful marker for predicting increased mortality in newborns with CDH.

Monitoring response to conventional treatment in children with XLH: Value of ALP and Rickets Severity Score (RSS) in a real world setting.

INTRODUCTION: X-linked hypophosphataemia (XLH) is conventionally managed with oral phosphate and active vitamin D analogues. OBJECTIVES: To evaluate long term treatment response by assessing biochemical disease activity [serum alkaline phosphatase (ALP)], radiological rickets severity score (RSS), growth and morbidity in patients with XLH on conventional therapy and assess the correlation between serum ALP and RSS. METHODS: XLH patients from 3 UK tertiary centres with ≥3 radiographs one year apart were included. Data was collected retrospectively. The RSS was assessed from routine hand and knee radiographs and ALP z scores were calculated using age-specific reference data. RESULTS: Thirty-eight (male = 12) patients met the inclusion criteria. The mean ± SD knee, wrist and total RSS at baseline (median age 1.2 years) were 2.0 ± 1.2, 1.9 ± 1.2 and 3.6 ± 1.3 respectively; and at the most recent clinic visit (median age 9.0 years, range 3.3-18.9) were 1.6 ± 1.0, 1.0 ± 1.0 and 2.5 ± 1.5 respectively. The mean ± SD serum ALP z scores at baseline and the most recent visit were 4.2 ± 2.3 and 4.0 ± 3.3. Median height SDS at baseline and most recent visit were -1.2 and -2.1 (p = 0.05). Dental abscess, craniosynostosis, limb deformity requiring orthopaedic intervention and nephrocalcinosis were present in 31.5%, 7.9%, 31.6% and 42.1% of the cohort respectively. There was no statistically significant (p > 0.05) correlation between ALP z scores and knee (r = 0.07) or total (r = 0.12) RSS. CONCLUSIONS: Conventional therapy was not effective in significantly improving biochemical and radiological features of disease. The lack of association between serum ALP and rickets severity on radiographs limits the value of ALP as the sole indicator of rickets activity in patients receiving conventional therapy.

Lung function measurement in prematurely born preschool children with and without chronic lung disease.

OBJECTIVE: Prematurely born infants often have recurrent wheeze and long-term respiratory morbidity at follow-up. Assessment of airways obstruction in preschool children is feasible using the interrupter resistance (Rint) but has rarely been examined in preterm children with and without chronic lung disease (CLD). The objective of this study was to determine lung function measured by the interrupter technique, its feasibility in the ambulatory setting and respiratory health in prematurely born preschool children with and without CLD. STUDY DESIGN: Preterm children of 2 to 4 years with severe CLD (>30% oxygen at 36 weeks and discharged home receiving supplemental oxygen) (n=43, median gestational age 27 weeks and median birth weight 995 g) and without CLD (n=33, median gestational age 29 weeks and median birth weight 1366 g) attempting lung function test for the first time were enrolled. Respiratory symptoms score was calculated using a questionnaire. A single set of 10 consecutive Rint measurements was obtained using a portable device (MicroRint). Median of at least five occlusions with consistent shape of mouth pressure-time curves was taken to be a Rint measurement. To assess feasibility the children were categorized as 'satisfactory', 'failure' and 'rejected' depending on the outcome of the test. Outcome variables were respiratory symptoms score and Rint. RESULT: Satisfactory Rint measurement was obtained in 46 (61%) children, 9 (36%) 2-year olds, 17 (65%) 3-year olds and 20 (80%) 4-year olds. As compared with the preterm control children (n=18), CLD children (n=28) had significantly higher respiratory symptoms score (18.5 vs 6, P<0.01) and Rint expressed as absolute values (kPa l(-1)) and z-scores (1.33 vs 1.16 and 1.42 vs 1.0, P<0.01), respectively. CONCLUSION: Rint measurement is feasible in prematurely born children of preschool age in the ambulatory setup. Preschool children with severe CLD may be identified from preterm children without CLD by increased Rint that may be used as a screening tool and as an outcome measure for interventions.

Changes in pulmonary artery pressure during the acute phase of respiratory distress syndrome treated with three different types of surfactant.

We studied the changes in acceleration time/right ventricular ejection time ratio (AT/RVET; indicative of changes in pulmonary artery pressure) calculated from Doppler ultrasound examinations performed before and 1, 6, and 12 h after the first and second doses of surfactant following the administration of each of three different surfactants during the acute phase of the respiratory distress syndrome. Maximum fractional inspired oxygen concentration (F(I,O2)) and peak inspiratory pressure (PIP) were recorded during each 4 h period from birth for the first 24 h and subsequently every 24 h until 72 h. Eighty-three infants were studied. Fifty patients weighing > 1 kg received Exosurf (n = 29) or ALEC (n = 21) and 33 weighing < or = 1 kg received Exosurf (n = 22) or Survanta (n = 11). The AT/RVET rose rapidly after administration of all three surfactants. There was no significant difference in the change in AT/RVET between those > 1 kg who received Exosurf and those who received ALEC (a synthetic surfactant). Similarly, there was no difference between those infants < or = 1 kg who received Exosurf and those who received Survanta. The F(I,O2) requirements, but not PIP, were lower in those infants who received Survanta at 12 and 20 h compared with those who received Exosurf. There was no significant difference in the F(I,O2) or PIP requirements between infants > 1 kg who received Exosurf compared with those who received ALEC. The rise in AT/RVET found in this study after administration of ALEC, Exosurf, or Survanta suggests that similar and rapid falls in pulmonary artery pressure occur after all three surfactant administrations, despite the difference in clinical response demonstrated between Exosurf and Survanta.

Changes in pulmonary artery pressure in infants with respiratory distress syndrome following treatment with Exosurf.

The pulmonary artery pressure (PAP) changes were studied using Doppler echocardiography in preterm infants treated with an artificial surfactant (Exosurf) during the acute phase of respiratory distress syndrome (RDS). The ratio of pulmonary artery acceleration time to the right ventricular ejection time (AT:RVET), measured from the Doppler wave form, was determined in 38 infants before the first dose of Exosurf, at one and six hours after the first dose, immediately before the second dose of Exosurf, and at one, six, 12, 36, and 60 hours subsequently. The median AT:RVET ratio corrected for heart rate (AT:RVET(c)) increased significantly an hour after administration of the first dose of Exosurf from 0.330 (0.273-0.410) to 0.380 (0.303-0.445) and similarly an hour after the second dose from 0.426 (0.252-0.495) to 0.440 (0.373-0.500). These changes occurred against a steady increase in the median AT:RVET(c) over the first 72 hours. It is concluded that the reduction in PAP correlates well with clinical parameters of disease severity and raises questions regarding the mode of action of Exosurf.