Quality of life and morbidity in children after repair of congenital diaphragmatic hernia
Authors:
P. Rounová; D. Zemková 1; J. Šulc 1; K. Pýcha; Z. Straňák 2; T. Svobodová 1; M. Kynčl 3; J. Šnajdauf; M. Rygl
Authors‘ workplace:
Klinika dětské chirurgie UK 2. LF a FN Motol, subkatedra dětské chirurgie IPVZ, Praha
přednosta prof. MUDr. J. Šnajdauf, DrSc.
; Pediatrická klinika UK 2. LF a FN Motol, Praha
přednosta prof. MUDr. J. Lebl, CSc.
1; Ústav pro péči o matku a dítě UK 3. LF, Praha
přednosta doc. MUDr. Z. Straňák, CSc.
2; Klinika zobrazovacích metod UK 2. LF a FN Motol, Praha
přednosta doc. MUDr. M. Roček, CSc.
3
Published in:
Čes-slov Pediat 2012; 67 (6): 368-375.
Category:
Original Papers
Overview
Aim of the study:
The aim of this study is to assess the health status and quality of life in children operated on after birth for congenital diaphragmatic hernia (CDH).
Methods:
A retrospective-prospective study of health status and quality of life in children operated in 1997–2009 for congenital diaphragmatic hernia. Analyzed data were obtained from medical records, questionnaires filled in by parents and clinical examination in 57 children after CDH repair (33 boys, 24 girls), mean age at examination was 6.5 years (11 months to 14.5 years).
Results:
The overall quality of life was assessed by the parents as good in 43 children (77%), satisfactory in 11 children (20%), unsatisfactory in 2 children (3%). None of the parents rated the quality of life as bad. Seventeen children (30%) have respiratory problems, and asthma was confirmed in 6 patients (11%). Clinical symptoms of gastroesophageal reflux (GER) occurred in 15 children (26%), in 16 children (28%) GER was sonographically detected. Two patients (4%) required Nissen fundoplication. Nonspecific digestive problems occurred in 25% of children. Scoliosis was found in 5%, poor posture in 33% and pectus excavatum in 42% of patients. Compared to the population norm CDH children had a significantly lower body height SDS (mean -0.39, p<0.05), weight SDS (mean –0.75, p<0.001) and BMI SDS (mean -0.68, p<0.001). Neurological problems were observed in 16 children (28%). The children mostly have neurodevelopmental delay (81%) especially in the first months of life which is normalised in the most of the patients during the first years of life. Social adaptation was evaluated in 33 older children (mean age 9.7 years). Thirty children (91%) attended primary school, 2 children special primary school, one child is in a special kindergarten. Twenty seven children (82%) of 33 children competed in sports or participate in gym class without any problems.
Conclusion:
Despite a greater incidence of problems when compared to healthy population, the health status and quality of life in children after surgery of CDH seems to be satisfactory.
Key words:
congenital diaphragmatic hernia, anthropometric examination, gastroesophageal reflux, long-term morbidity, quality of life
Sources
1. Rygl M, Pycha K, Stranak Z, et al. Congenital diaphragmatic hernia: onset of respiratory distress and size of the defect: analysis of the outcome in 104 neonates. Pediatr Surg Int 2007; 23 (1): 27–31.
2. Lally KP, Lally PA, Lasky RE, et al. Defect size determines survival in infants with congenital diaphragmatic hernia. Pediatrics 2007; 120 (3): e651–657.
3. Golombek SG. The history of congenital diaphragmatic hernia from 1850s to the present. J Perinatol 2002; 22 (3): 242–246.
4. Doyle NM, Lally KP. The CDH Study Group and advances in the clinical care of the patient with congenital diaphragmatic hernia. Semin Perinatol 2004; 28 (3): 174–184.
5. Chiu P, Hedrick HL. Postnatal management and long-term outcome for survivors with congenital diaphragmatic hernia. Prenat Diagn 2008; 28 (7): 592–603.
6. Chen C, Jeruss S, Chapman JS, et al. Long-term functional impact of congenital diaphragmatic hernia repair on children. J Pediatr Surg 2007; 42 (4): 657–665.
7. Downard CD. Congenital diaphragmatic hernia: an ongoing clinical challenge. Curr Opin Pediatr 2008; 20 (3): 300–304.
8. Peetsold MG, Heij HA, Kneepkens CM, et al. The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity. Pediatr Surg Int 2009; 25 (1): 1–17.
9. Trachsel D, Selvadurai H, Bohn D, et al. Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia. Pediatr Pulmonol 2005; 39 (5): 433–439.
10. Jancelewicz T, Vu LT, Keller RL, et al. Long-term surgical outcomes in congenital diaphragmatic hernia: observations from a single institution. J Pediatr Surg 2010; 45 (1): 155–160; discussion 160.
11. Ijsselstijn H, Tibboel D, Hop WJ, et al. Long-term pulmonary sequelae in children with congenital dia-phragmatic hernia. Am J Respir Crit Care Med 1997; 155 (1): 174–180.
12. Bagolan P, Morini F. Long-term follow up of infants with congenital diaphragmatic hernia. Semin Pediatr Surg 2007; 16 (2): 134–144.
13. Grethel EJ, Cortes RA, Wagner AJ, et al. Prosthetic patches for congenital diaphragmatic hernia repair: Surgisis vs Gore-Tex. J Pediatr Surg 2006; 41 (1): 29–33; discussion 29–33.
14. Koivusalo AI, Pakarinen MP, Lindahl HG, et al. The cumulative incidence of significant gastroesophageal reflux in patients with congenital diaphragmatic hernia – a systematic clinical, pH-metric, and endoscopic follow-up study. J Pe-diatr Surg 2008;43(2):279-282.
15. Martin R, Saller K. Lehrbuch der Anthropologie. Stuttgart: G. Fisher Verlag, 1957.
16. Fetter V, Prokopec M, Suchý J, et al. Antropologie. Praha: Academia, 1967.
17. Bláha P, Vignerová J, Riedlová J, et al. 6. celostátní antropologický výzkum dětí a mládeže 2001, Česká republika. Základní tělesné charakteristiky 0–19 let, percentilové grafy 0–18 let, rozměry hlavy dětí 0–6 let. Praha: Státní zdravotní ústav, 2005. ISBN 80-7071-251-1.
18. Bláha P, Vignerová J, Paulová M, et al. Vývoj tělesných parametrů českých dětí a mládeže se zaměřením na rozměry hlavy (0–16 let), I. a II. díl. Praha: Státní zdravotní ústav, Univerzita Karlova Praha, 1999.
19. Vignerová J, Bláha P. Sledování růstu českých dětí a dospívajících. Norma, vyhublost, obezita. Praha: Státní zdravotní ústav, Univerzita Karlova Praha, 2001.
20. Krásničanová H, Lesný P. Kompendium pediatrické auxologie. CD-ROM. Novo Nordisk, 2005.
21. van den Hout L, Sluiter I, Gischler S, et al. Can we improve outcome of congenital diaphragmatic hernia? Pediatr Surg Int 2009; 25 (9): 733–743.
22. Diamond IR, Mah K, Kim PC, et al. Predicting the need for fundoplication at the time of congenital dia-phragmatic hernia repair. J Pediatr Surg 2007; 42 (6): 1066–1070.
23. Guner YS, Elliott S, Marr CC, et al. Anterior fundoplication at the time of congenital diaphragmatic hernia repair. Pediatr Surg Int 2009; 25 (8): 715–718.
24. Muratore CS, Kharasch V, Lund DP, et al. Pulmonary morbidity in 100 survivors of congenital diaphragmatic hernia monitored in a multidisciplinary clinic. J Pediatr Surg 2001; 36 (1): 133–140.
25. Vanamo K, Peltonen J, Rintala R, et al. Chest wall and spinal deformities in adults with congenital dia-phragmatic defects. J Pediatr Surg 1996; 31 (6): 851–854.
26. Okuyama H, Kubota A, Kawahara H, et al. Correlation between lung scintigraphy and long-term outcome in survivors of congenital diaphragmatic hernia. Pediatr Pulmonol 2006; 41 (9): 882–886.
27. Hayward MJ, Kharasch V, Sheils C, et al. Predicting inadequate long-term lung development in children with congenital diaphragmatic hernia: an analysis of longitudinal changes in ventilation and perfusion. J Pediatr Surg 2007; 42 (1): 112–116.
28. Šulc J, Zikán J, Kredba V, et al. Funkční vyšetření plic u nespolupracujících dětí. Část I. Kazuistiky v alergologii, pneumologii a ORL 2011; 8 (1):17–25.
29. Gappa M, Pillow JJ, Allen J, et al. Lung function tests in neonates and infants with chronic lung disease: lung and chest-wall mechanics. Pediatr Pulmonol 2006; 41 (4): 291–317.
30. Godfrey S, Bar-Yishay E, Avital A, et al. What is the role of tests of lung function in the management of infants with lung disease? Pediatr Pulmonol 2003; 36 (1): 1–9.
31. Godfrey S, Springer C, Bar-Yishay E. Evaluating the lung function of infants. Isr Med Assoc J 2009;11 (8): 492–497.
32. Cortes RA, Keller RL, Townsend T, et al. Survival of severe congenital diaphragmatic hernia has morbid consequences. J Pediatr Surg 2005; 40 (1): 36–45; discussion 45–36.
33. Friedman S, Chen C, Chapman JS, et al. Neurodevelopmental outcomes of congenital diaphragmatic hernia survivors followed in a multidisciplinary clinic at ages 1 and 3. J Pediatr Surg 2008; 43 (6): 1035–1043.
34. Rygl M, Pýcha K, Šnajdauf J, et al. Rekonstrukce bránice polytetrafluoretylenovou záplatou u novorozenců s vrozenou brániční kýlou. Rozhl Chir 2001; 80 (12): 628–632.
35. Clark RH, Hardin WD Jr, Hirschl RB, et al. Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg 1998; 33 (7): 1004–1009.
36. Sydorak RM, Hoffman W, Lee H, et al. Reversed latissimus dorsi muscle flap for repair of recurrent congenital diaphragmatic hernia. J Pediatr Surg 2003; 38 (3): 296–300; discussion 296–300.
37. Valfre L, Braguglia A, Conforti A, et al. Long term follow-up in high-risk congenital diaphragmatic hernia survivors: patching the diaphragm affects the outcome. J Pediatr Surg 2011; 46 (1): 52–56.
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Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2012 Issue 6
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