Pneumothoraces As a Fatal Complication of Congenital Diaphragmatic Hernia in the Era of Gentle Ventilation

Abstract

Introduction Pneumothorax remains a life-threatening complication that occurs in congenital diaphragmatic hernia (CDH), even under respiratory management with gentle ventilation. The aim of this study was to evaluate the prevalence of pneumothoraces as a fatal complication during the management of CDH based on the results of a nationwide Japanese survey conducted in the era of gentle ventilation.

Materials and Methods A retrospective cohort study was performed as part of a nationwide Japanese survey of CDH. A total of 510 neonates with isolated CDH born between 2006 and 2010 were included in this study. The patients were divided into four groups according to operative findings related to the diaphragmatic defect size and operability, which represents the disease severity: defects less than 25%, defects more than 25% but less than 75%, defects more than 75%, and a patient group that was unable to undergo surgery. The prevalence of pneumothorax and the survival rate were compared with respect to each disease severity group. Each case was evaluated to determine whether the development of a pneumothorax was the primary cause of death.

Results Of the 510 neonates with isolated CDH, 69 patients developed a pneumothorax before and/or after surgical intervention. Of the 69 patients 38 patients died, and only 26 patients were discharged from the hospital without any major morbidity that requires home treatment (“intact discharge”). The prevalence of pneumothorax increased and the survival rate and intact discharge rate decreased as the severity of the disease worsened. The number of patients whose pneumothorax was presumed to be the primary cause of death also increased as the severity of the disease worsened. The survival rate of the patients with pneumothorax was significantly lower than that of the patients without pneumothorax among the groups with 25 to 75% defects and 75% or more defects.

Conclusions Pneumothoraces was found to more likely occur in neonates with CDH associated with a large defect of the diaphragm. The survival rate and intact discharge rate decreased as the severity of the disease worsened, especially among the patients who developed pneumothorax accompanied by large diaphragmatic defects. No other risk factors related to pneumothorax occurrence were found, except for the severity of the disease itself, thus suggesting that pneumothorax was associated with a lethal outcome in neonates with CDH associated with a large defect of the diaphragm.

Keywords

congenital diaphragmatic hernia - pneumothorax - gentle ventilation - lung injury - pulmonary hypoplasia

• References

• 1 Azarow K, Messineo A, Pearl R, Filler R, Barker G, Bohn D. Congenital diaphragmatic hernia—a tale of two cities: the Toronto experience. J Pediatr Surg 1997; 32 (3) 395-400
• 2 Sakurai Y, Azarow K, Cutz E, Messineo A, Pearl R, Bohn D. Pulmonary barotrauma in congenital diaphragmatic hernia: a clinicopathological correlation. J Pediatr Surg 1999; 34 (12) 1813-1817
• 3 Bos AP, Hussain SM, Hazebroek FWJ, Tibboel D, Meradji M, Molenaar JC. Radiographic evidence of bronchopulmonary dysplasia in high-risk congenital diaphragmatic hernia survivors. Pediatr Pulmonol 1993; 15 (4) 231-234
• 4 Vanamo K, Rintala R, Sovijärvi A , et al. Long-term pulmonary sequelae in survivors of congenital diaphragmatic defects. J Pediatr Surg 1996; 31 (8) 1096-1099 , discussion 1099–1100
• 5 Bagolan P, Casaccia G, Crescenzi F, Nahom A, Trucchi A, Giorlandino C. Impact of a current treatment protocol on outcome of high-risk congenital diaphragmatic hernia. J Pediatr Surg 2004; 39 (3) 313-318 , discussion 313–318
• 6 Finer NN, Tierney A, Etches PC, Peliowski A, Ainsworth W. Congenital diaphragmatic hernia: developing a protocolized approach. J Pediatr Surg 1998; 33 (9) 1331-1337
• 7 Frenckner B, Ehrén H, Granholm T, Lindén V, Palmér K. Improved results in patients who have congenital diaphragmatic hernia using preoperative stabilization, extracorporeal membrane oxygenation, and delayed surgery. J Pediatr Surg 1997; 32 (8) 1185-1189
• 8 Kays DW, Langham Jr MR, Ledbetter DJ, Talbert JL. Detrimental effects of standard medical therapy in congenital diaphragmatic hernia. Ann Surg 1999; 230 (3) 340-348 , discussion 348–351
• 9 Wung JT, Sahni R, Moffitt ST, Lipsitz E, Stolar CJH. Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration, and no chest tube. J Pediatr Surg 1995; 30 (3) 406-409
• 10 Al-Hathlol K, Elmahdy H, Nawaz S , et al. Perioperative course of pulmonary hypertension in infants with congenital diaphragmatic hernia: impact on outcome following successful repair. J Pediatr Surg 2011; 46 (4) 625-629
• 11 Boloker J, Bateman DA, Wung JT, Stolar CJH. Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous respiration/elective repair. J Pediatr Surg 2002; 37 (3) 357-366
• 12 Migliazza L, Bellan C, Alberti D , et al. Retrospective study of 111 cases of congenital diaphragmatic hernia treated with early high-frequency oscillatory ventilation and presurgical stabilization. J Pediatr Surg 2007; 42 (9) 1526-1532
• 13 Waag KL, Loff S, Zahn K , et al. Congenital diaphragmatic hernia: a modern day approach. Semin Pediatr Surg 2008; 17 (4) 244-254
• 14 Nagata K, Usui N, Kanamori Y , et al. The current profile and outcome of congenital diaphragmatic hernia: a nationwide survey in Japan. J Pediatr Surg 2013; 48 (4) 738-744
• 15 Kitano Y, Okuyama H, Saito M , et al. Re-evaluation of stomach position as a simple prognostic factor in fetal left congenital diaphragmatic hernia: a multicenter survey in Japan. Ultrasound Obstet Gynecol 2011; 37 (3) 277-282
• 16 Usui N, Kitano Y, Okuyama H , et al. Prenatal risk stratification for isolated congenital diaphragmatic hernia: results of a Japanese multicenter study. J Pediatr Surg 2011; 46 (10) 1873-1880
• 17 Drummond WH, Gregory GA, Heymann MA, Phibbs RA. The independent effects of hyperventilation, tolazoline, and dopamine on infants with persistent pulmonary hypertension. J Pediatr 1981; 98 (4) 603-611
• 18 Wung JT, James LS, Kilchevsky E, James E. Management of infants with severe respiratory failure and persistence of the fetal circulation, without hyperventilation. Pediatrics 1985; 76 (4) 488-494
• 19 Hayakawa M, Ito M, Hattori T , et al; Japanese Congenital Diaphragmatic Hernia Study Group. Effect of hospital volume on the mortality of congenital diaphragmatic hernia in Japan. Pediatr Int 2013; 55 (2) 190-196
• 20 Tsao K, Lally KP. The congenital diaphragmatic hernia study group: a voluntary international registry. Semin Pediatr Surg 2008; 17 (2) 90-97
• 21 Congenital Diaphragmatic Hernia Study Group, Lally KP, Lally PA. , et al. Defect size determines survival in infants with congenital diaphragmatic hernia. Pediatrics 2007; 120 (3) e651-e657
• 22 Sebald M, Friedlich P, Burns C , et al. Risk of need for extracorporeal membrane oxygenation support in neonates with congenital diaphragmatic hernia treated with inhaled nitric oxide. J Perinatol 2004; 24 (3) 143-146