HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Bradley M. Rodgers Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mallik, K. Articles by McGahren, E. D. Search for Related Content PubMed PubMed Citation Articles by Mallik, K. Articles by McGahren, E. D. Related Collections Related Article

Ann Thorac Surg 1995;60:1331-1335
© 1995 The Society of Thoracic Surgeons

---

Original Articles: General Thoracic

Congenital Diaphragmatic Hernia: Experience in a Single Institution From 1978 Through 1994

Division of Pediatric Surgery, Department of Surgery, University of Virginia Health Sciences Center, Charlottesville, Virginia

	Abstract

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Background. Congenital diaphragmatic hernia continues to be a difficult management problem. Essentially all information on the condition has been compiled in a retrospective manner due to the individualized care that each infant must undergo. We contribute a review of our patients to add to the current fund of knowledge and to assess our experience before and since the introduction of extracorporeal membrane oxygenation in our institution.

Methods. This is a review of records of infants with congenital diaphragmatic hernia treated from 1978 through 1994. Repair has generally been accomplished early with only one repair being accomplished with an infant placed on extracorporeal membrane oxygenation preoperatively.

Results. Overall survival was 63%. Survival was 42% before extracorporeal membrane oxygenation becoming available in our region in 1986, and 75% afterward. Since 1986, 16 of 33 (48%) infants have required extracorporeal membrane oxygenation and 73% have survived.

Conclusions. Overall survival in our series is comparable with that of other reported series. There appears to be an improvement in survival since the introduction of extracorporeal membrane oxygenation. Our present practice of early repair, and postrepair extracorporeal membrane oxygenation if needed, results in a survival rate comparable with that of currently available series reports regardless of the method of treatment reported.

	Introduction

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
See also page 1335.

Congenital diaphragmatic hernia of the Bochdalek variety in infants continues to be a very difficult management problem. In the earlier days of modern pediatric surgery, diaphragmatic hernia was considered a surgical emergency. Gross' series of 7 patients in 1946 certainly advanced this view [1]. In that series, the earliest presenting patient was 22 hours old. We now know that those infants had essentially selected themselves for survival by not exhibiting marked effects of lung hypoplasia or pulmonary hypertension at birth. In 1953, Campanale and [2] recognized that lung hypoplasia was associated with diaphragmatic hernia. In 1971, Murdock and colleagues [3] and Rowe and Uribe [4] subsequently recognized that pulmonary hypertension was also a component of this condition. It became clear that management of an infant with diaphragmatic hernia required a focus on managing these conditions. In 1973, Haphaely and Downes [5] recognized that infants who suffered high preoperative and postoperative alveolar–arterial oxygen gradients, died despite intensive medical and respiratory aid. In essence, they were describing infants who would have likely met today's extracorporeal membrane oxygenation (ECMO) criteria and enjoyed a better chance for survival.

In recent years, infants who in the past would have been stillborn or who would have died at birth without a diagnosis, have been diagnosed either at birth or prenatally [6, 7]. Thus, a larger number of sicker infants is constantly being identified, continuously challenging pediatric surgeons to modify their approaches to this disease.

Recent evidence suggests that operation may initially hinder lung function. Theoretically then, delayed operation may allow time for preoperative medical therapy, and perhaps, preoperative ECMO, thereby improving postoperative lung function and survival [8–11].

The pediatric surgical community's understanding of the pathophysiologic processes underlying diaphragmatic hernia, the number and severity of illness of infants presenting with the condition, and treatment options have been undergoing constant change. Thus, prospective randomized studies to determine predictors of survival and optimal treatment have been impossible to perform. Essentially, all of our data have been generated by retrospective analysis. We reviewed our series of infants with diaphragmatic hernia from 1978 through 1994 as this time period represents the bulk of our experience in the modern era. We introduced ECMO in our center in 1992 and felt it was time to assess our experience to help guide our future directions in the care of diaphragmatic hernia as well as to contribute to the fund of information about this disease.

	Patients and Methods

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
The records of 52 infants with congenital diaphragmatic hernia treated at our institution from 1978 through 1994 were reviewed. Complete follow-up was available for 51 infants. Preoperative, operative, and postoperative data were collected for each infant.

Preoperative data included documentation of a prenatal diagnosis, gestational age at birth, sex, birth weight, Apgar scores at 1 and 5 minutes, presenting symptomatology, initial arterial blood gas, best preoperative arterial blood gas, associated anomalies, and time from birth to operative repair. Operative data included the side of the hernia, the presence of the stomach in the chest, and the type and location of prostheses used. Postoperative data included whether or not ECMO was used, complications, survival, and long-term sequelae.

All operative repairs were accomplished through a transverse or oblique abdominal incision on the side of the defect. The diaphragm was closed primarily when possible and with a Gore-Tex patch (W. L. Gore & Assoc, Flagstaff, AZ) if there was insufficient muscle for a primary closure. The abdomen was also closed primarily when possible. If closure was not possible, or prevented adequate diaphragmatic excursion, a silicone or Gore-Tex prosthesis was placed and removed at a later time.

Comparison of data for survivors and nonsurvivors was analyzed using Student's t test, one-tailed, for unpaired data. Comparison of data between pre-ECMO and post-ECMO patients was analyzed using a normal approximation for a test of proportions.

	Results

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
The series of infants treated for congenital diaphragmatic hernia comprised 52 patients. Follow-up was available for 51 patients. One patient presented to and was operated on at our institution but was subsequently transferred to another center for ECMO before the introduction of ECMO at our center and was lost to follow-up.

Overall there were 32 male (62%) and 20 female (38%) infants. Thirty-eight infants (73%) had left-sided defects, and 14 (27%) had right-sided defects. Of the 52 infants, 34 (65%) were transferred from outside institutions.

Five infants (10%) carried a prenatal diagnosis of congenital diaphragmatic hernia based on ultrasound examination. Only 1 infant was diagnosed earlier than 24 weeks of gestation. Three other infants were diagnosed at 28, 30, and 32 weeks. One infant was documented as having ``multiple ultrasounds'' showing the presence of a hernia. Only the infant diagnosed at 32 weeks died. Thirty-nine infants (75%) without prenatal diagnosis presented with respiratory distress at birth or within the first few hours of life and were diagnosed at that time. Eight infants (15%) were ``late'' presenters. Seven of these presented with significant feeding difficulties including vomiting. An eighth infant in this group presented with respiratory insufficiency and was intubated at birth. A chest radiograph did not reveal any anatomic defect. She was extubated after 5 days, but there appeared to be a right diaphragmatic eventration on a follow-up chest radiograph. She was taken to the operating room at 15 days of life and found to have the dome of the liver protruding through a right-sided diaphragmatic defect. This was repaired primarily and the infant had an uneventful postoperative course. Significant associated anomalies in the overall series are as follows: pectus excavatum, 3 infants; ventricular septal defect, 2; atrial septal defect, 2; patent ductus arteriosus, 2; tricuspid insufficiency, 2; pulmonary sequestration, 2; hypoplastic left heart, 1; Meckel's diverticulum, 1; Cornelia de Lange syndrome, 1; and trisomy 18, 1 infant.

Of the 52 infants in this series, 49 (94%) underwent operative repair of their diaphragmatic defect. Three infants (6%) died without attempted repair. Of these, 1 infant had a hypoplastic left heart, 1 had trisomy 18, and 1 died of respiratory failure immediately after birth. Of 44 infants diagnosed before or at birth, the time from birth to operation was available for 40. Twenty-eight infants underwent repair before 10 hours of life, 8 underwent repair from 10 to 24 hours of life, and 3 infants underwent repair at 26, 33, and 49 hours of life, respectively. One infant was placed on ECMO within hours of birth due to her extremely poor condition. Her hernia was then repaired on ECMO at 7 days of age. The time from birth to operation had no impact on outcome in our series.

Of 49 infants undergoing repair, 28 (57%) had their diaphragm closed primarily. Six (12%) infants required a diaphragmatic patch, 9 (18%) required an abdominal wall patch, and 6 patients (12%) required both an abdominal wall and diaphragmatic patch.

Extracorporeal membrane oxygenation became available in our region in 1986, and in our center in 1992. Before 1986, 19 infants were operated on for diaphragmatic hernia. Subsequently, 33 more infants have been treated, with 30 undergoing operation. Sixteen of these 33 infants (48%) have received ECMO support, the last 7 at our institution. Only 1 infant was placed on ECMO before repair.

Overall, 32 of 51 infants (63%) survived their perioperative course. Nineteen infants (37%) died in the perioperative period with the longest course being 40 days. Two infants (4%) died beyond their perioperative course (Table 1)tab 1. One patient suffered necrotic bowel from adhesions and subsequently died at 2 months of age. Another suffered respiratory failure at 9 months of age. Twenty-two of 31 male infants (70%) survived their perioperative course. The two late deaths noted above were male infants. Ten of 20 female infants (50%) survived. Twenty-three of 37 infants (62%) with left-sided defects survived, whereas 9 of 14 infants (64%) with right-sided defects also survived. There was one late death in each of these two groups.

Twenty infants were noted to have the stomach in the chest at the time of operation. Eleven (55%) survived (Table 1).

Before the institution of ECMO in our region in 1986, 8 of 19 infants treated for diaphragmatic hernia survived (42%) (Table 2).tab 2 From 1986 through 1994, 24 of 32 infants (75%) survived. Sixteen infants (48%) required ECMO support. Eight were sent to outside centers before 1992. Follow-up was available for 7 infants, and 6 (88%) survived. Beginning in 1992, 8 infants have received ECMO support, all but 1 postoperatively, at our institution with 5 (63%) survivors. Overall, of 15 infants requiring ECMO for whom follow-up is available, 11 (73%) have survived. Since 1986, 17 babies have not required ECMO, and 13 (76%) have survived. There were two late deaths in this group.

Long-term follow-up has revealed instances of dysfunctional feeding, gastrointestinal reflux, reactive airway disease, developmental delay, and hearing loss in a minority of patients. However, it is likely that past documentation of these types of problems has not been as comprehensive as it now appears it needs to be in this population of children. This is particularly true with regard to infants who survive after ECMO therapy.

	Comment

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Our approach to the treatment of children with diaphragmatic hernia has been to attain medical stabilization at the time of diagnosis, and then attempt repair as soon as feasible. All but 1 of the infants in our series who received ECMO treatment, did so after their diaphragms were repaired. The infant who underwent preoperative ECMO presented in such extremis that operative repair would not have been possible before ECMO. Only 2 infants could be identified in this review who underwent an intended prolonged period of medical stabilization before repair. One was operated on at 49 hours of age and subsequently died, whereas another who was operated on at 34 hours of age, survived. Our overall survival rate, and particularly our survival rate since the institution of ECMO in our region, compares favorably with that of current series that report data on infants repaired soon after birth versus those repaired after a longer period of stabilization, with or without ECMO [12–16].

With a limited number of delayed repairs in our series, we cannot make a comparison between early and delayed repair. Attempts to do so in the literature have primarily been retrospective. In addition, the specific medical therapy administered to individual infants undergoing early or delayed repair varies from institution to institution, and indeed, from patient to patient within each institution. The data generated have yet to show convincingly that there is any advantage in delaying repair as far as survival is concerned. However, there does not seem to be any disadvantage to delay either [14, 16, 17].

The impact of ECMO on survival in our series seems to be a favorable one. The survival rate since ECMO became available is significantly better than before that time (Table 2). If one presumes a mortality of 80% without ECMO for those infants placed on ECMO, survival in our series from 1986 through 1994 would have been 48% and therefore, not significantly different from survival before 1986. It is likely, but not provable, that before 1986 infants who died could have benefitted from ECMO. Although ECMO appears to be making a positive impact on overall survival in our institution, reports in the literature have yet to show an impact from ECMO on overall survival in diaphragmatic hernia [13–17]. However, it is widely believed that a greater number of infants with diaphragmatic hernia are being diagnosed. More important, sicker infants are being diagnosed. Thus, the numerator of survivors is expanding at the same time as the denominator of presenting infants. The ELSO registry indicates that from 1978 through 1994, 1,886 infants were placed on ECMO for treatment of congenital diaphragmatic hernia, and 1,102 survived (58%). If the survival rate of infants placed on ECMO is compared to a predicted mortality of 80% to 95%, depending on each institution's ECMO criteria, then it appears that infants who otherwise would have died are being saved.

Infants in our series who presented late usually had gastrointestinal symptoms. Their outcome was uniformly good with all but 1 of the infants surviving. The 1 nonsurvivor had Cornelia de Lange syndrome. This favorable outcome reflects the experience of other investigators as long as there are not anatomic complications compromising gastrointestinal viability at presentation [18, 19].

Attempts have been made to determine characteristics of infants with diaphragmatic hernia that might predict their likely survival or demise. For example, factors such as carbon dioxide tension versus ventilatory index (Bohn's criteria), alveolar–arterial oxygen gradient, oxygenation index, best postductal carbon dioxide tension, symptomatology within 6 hours of birth, diagnosis from prenatal ultrasound, the need for a patch in closing a defect, and the position of the stomach have all been cited as indicators of outcome [20–25]. Again, difficulty arises in that these data have been generated retrospec-tively in an uncontrolled manner. In some instances, such as in the case of Bohn's criteria for 100% mortality, subsequent data have indicated significant exceptions to the predictions [26].

In the present series, we generated data addressing outcome after diaphragmatic and abdominal patch placement, outcome in infants discovered to have the stomach in the chest, as well as limited data concerning prenatal ultrasound. Our series indicates that where only one patch is needed, either diaphragmatic or abdominal, survival can be expected to reflect the outcome of diaphragmatic hernia repair in general. This contradicts other reports [20, 21]. Poor outcome for infants needing diaphragmatic patches reported in the literature may be partly attributable to some of these infants having total agenesis of the diaphragm. This appears to behave as a more severe form of diaphragmatic hernia [20]. In addition, there are no objective criteria to determine when a patch should or should not be placed. Therefore, there is no real indication of how ill infants have been who have received patches.

Our series indicates that when the stomach is found in the chest in left-sided hernias, outcome is not compromised. This is contrary to data in the literature [24]. We also have not found that prenatal ultrasound portends a poor outcome. Our series of infants diagnosed prenatally is small, however, and most of the diagnoses were made relatively late in gestation. The literature is mixed on this subject [7, 27–30].

Our series does indicate that survivors have higher birth weights, 5-minute Apgar scores, and best preoperative oxygen tension than nonsurvivors. However, in both the survivor and nonsurvivor groups, there were multiple exceptions to these findings.

There have been attempts in the literature to stratify infants to determine which infants should be offered ECMO, or even operation. It is still difficult to predict the outcome of any one particular infant with diaphragmatic hernia. At the present time, we continue to initially stabilize infants with diaphragmatic hernia, but then pursue surgical correction as soon as possible so as to have the operation completed before ECMO cannulation should it be necessary. Our experience, and that of others, has indicated the potential for significant bleeding complications when major surgical procedures are performed during ECMO therapy [31]. Consistent randomized prospective data have yet to be generated, although a recent prospective report indicates that extended preoperative medical therapy has no impact on survival or the need for ECMO [32]. Therefore, for the present, without consistent evidence that extended medical therapy or ECMO before operation provides a better outcome, and with the favorable survival generated in our center, we believe that our current approach to the management of infants with diaphragmatic hernia is justifiable, but should be scrutinized continuously as new data emerge.

	Footnotes

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Presented at the Thirty-First Annual Meeting of The Society of Thoracic Surgeons, Palm Springs, CA, Jan 30–Feb 1, 1995.

Address reprint requests to Dr McGahren, Division of Pediatric Surgery, Department of Surgery, University of Virginia Health Sciences Center, PO Box 181, Charlottesville, VA 22908.

	References

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 

1. Gross RE. Congenital hernia of the diaphragm. Am J Dis Child 1946;71:579–92.[Abstract/Free Full Text]
2. Campanale RP, Rowland RH. Hypoplasia of the lung associated with congenital diaphragmatic hernia. Ann Surg 1955;142:176–89.[Medline]
3. Murdock AI, Burrington JB, Snyer PR. Alveolar to arterial oxygen tension difference and venous admixture in newly born infants with congenital diaphragmatic hernia through the foramen of Bochdalek. Biol Neonate 1971;17:161–72.[Medline]
4. Rowe MI, Uribe FL. Diaphragmatic hernia in the newborn infant: blood gas and pH considerations. Surg 1971;70: 758–61.
5. Raphaely RC, Downes JJ. Congenital diaphragmatic hernia: prediction of survival. J Pediatr Surg 1973;8:815–23.[Medline]
6. Nakayama DK, Harrison MR, Chinn DH, et al. Prenatal diagnosis and natural history of the fetus with a congenital diaphragmatic hernia: initial clinical experience. J Pediatr Surg 1985;20:118–24.[Medline]
7. Wilson JM, Fauza DO, Lund DP, Benacerraf BR, Hendren WH. Antenatal diagnosis of isolated congenital diaphragmatic hernia is not an indicator of outcome. J Pediatr Surg 1994;29:815–9.[Medline]
8. Nakayama DK, Motoyama EK, Tagge EM. Effect of preoperative stabilization on respiratory system compliance and outcome in new born infants with congenital diaphragmatic hernia. J Pediatr 1990;8:793–9.
9. Sakai H, Tamura M, Hosokawa Y, Bryan AC, Barker GA, Bohn DJ. Effect of surgical repair on respiratory mechanics in congenital diaphragmatic hernia. J Pediatr 1987;111:432–8.[Medline]
10. Nakayama DK, Motoyama EK, Smith SD, Tagge E. Perioperative ECMO improves respiratory system compliance in newborns with congenital diaphragmatic hernia. Surg Forum 1990;576–9.
11. Heiss K, Manning P, Oldham KT, et al. Reversal of mortality for congenital diaphragmatic hernia with ECMO. Ann Surg 1989;209:225–30.[Medline]
12. Breaux CW, Rouse TM, Cain WS, Georgeson KE. Congenital diaphragmatic hernia in an era of delayed repair after medical and/or extracorporeal membrane oxygenation stabilization: a prognostic and management classification. J Pediatr Surg 1992;27:1192–6.[Medline]
13. Atkinson JB, Ford EG, Humphries D, et al. The impact of extracorporeal membrane support in the treatment of congenital diaphragmatic hernia. J Pediatr Surg 1991;26:791–3.[Medline]
14. West KW, Bengston K, Rescorla FJ, Engle WA, Grosfeld JA. Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia. Ann Surg 1992;216:454–62.[Medline]
15. Lally KP, Paranka MS, Roden J, et al. Congenital diaphragmatic hernia : stabilization and repair on ECMO. Ann Surg 1992;216:569–73.[Medline]
16. Charlton AJ. The management of congenital diaphragmatic hernia without ECMO. Pediatr Anesth 1993;3:201–4.
17. Goh DW, Drake DP, Brereton RJ, Kiely EM, Spitz L. Delayed surgery for congenital diaphragmatic hernia. Br J Surg 1992;79:644–6.[Medline]
18. Manning PB, Murphy JP, Raynour SC, Ashcraft KW. Con-genital diaphragmatic hernia presenting due to gastrointestinal complications. J Pediatr Surg 1992;27:1225–8.[Medline]
19. Malone PS, Brain AJ, Kiely EM, Spitz L. Congenital diaphragmatic defects that present late. Arch Dis Child 1989;64:1542–4.[Abstract/Free Full Text]
20. Eichelberger MR, Kettrick RG, Hoelzer AJ, Swedlow DB, Schnaufer L. Agenesis of the left diaphragm: surgical repair and physiologic consequences. J Pediatr Surg 1980;15:395–7.[Medline]
21. Atkinson JB, Poon MW. ECMO and the management of congenital diaphragmatic hernia with large diaphragmatic defects requiring a prosthetic patch. J Pediatr Surg 1992;27:754–6.[Medline]
22. Redmond C, Heaton J, Calix J, et al. A correlation of pulmonary hypoplasia, mean airway pressure, and survival in congenital diaphragmatic hernia treated with extracorporeal membrane oxygenation. J Pediatr Surg 1987;22:1143–9.[Medline]
23. Wilson JM, Lund DP, Lillehei CW, Vacenti JP. Congenital diaphragmatic hernia: predictors of severity in the ECMO era. J Pediatr Surg 1991;26:1028–34.[Medline]
24. Hatch EI, Kendall J, Blumhagen J. Stomach position as an in utero predictor of neonatal outcome in left-sided diaphragmatic hernia. J Pediatr Surg 1992;27:778–9.[Medline]
25. Johnston PW, Liberman R, Gangitano E, Vogt J. Ventilation parameters and arterial blood gases as a prediction of hypoplasia in congenital diaphragmatic hernia. J Pediatr Surg 1990;25:496–9.[Medline]
26. Van Meurs KP, Newman KD, Anderson KD, Short BL. Effect of extracorporeal membrane oxygenation on survival of infants with congenital diaphragmatic hernia. J Pediatr 1990;117:954–60.[Medline]
27. Nakayama DK, Harrison MR, Chinn DH, et al. Prenatal diagnosis and natural history of the fetus with a congenital diaphragmatic hernia: initial clinical experience. J Pediatr Surg 1985;20:118–24.[Medline]
28. Adzick NS, Harrison MR, Glick PL, Nakayama DK, Manning FA, DeLorimier AA. Diaphragmatic hernia in the fetus: prenatal diagnosis and outcome in 94 cases. J Pediatr Surg 1985;20:357–61.[Medline]
29. Benacerraf BR, Adzick NS. Fetal diaphragmatic hernia: ultrasound diagnosis and clinical outcome in 19 cases. Am J Obstet 1987;156:573–6.[Medline]
30. Adzick NS, Vacanti JP, Lillehei CW, O'Rourke PP, Crone RK, Wilson JM. Fetal diaphragmatic hernia: ultrasound diagnosis and clinical outcome in 38 cases. J Pediatr Surg 1989;24:654–8.[Medline]
31. Atkinson JB, Kitagawa H, Hamphries B. Major surgical intervention during extracorporeal membrane oxygenation. J Pediatr Surg 1992;27:1197–8.[Medline]
32. Nio M, Haase G, Kennaugh J, Bui K, Atkinson JB. A prospective randomized trial of delayed versus immediate repair of congenital diaphragmatic hernia. J Pediatr Surg 1994;29: 618–21.[Medline]

This article has been cited by other articles:

				J. Colvin, C. Bower, J. E. Dickinson, and J. Sokol Outcomes of Congenital Diaphragmatic Hernia: A Population-Based Study in Western Australia Pediatrics, September 1, 2005; 116(3): e356 - e363. [Abstract] [Full Text] [PDF]

				T. P. Stevens, P. R. Chess, K. M. McConnochie, R. A. Sinkin, R. Guillet, W. M. Maniscalco, and S. G. Fisher Survival in Early- and Late-Term Infants With Congenital Diaphragmatic Hernia Treated With Extracorporeal Membrane Oxygenation Pediatrics, September 1, 2002; 110(3): 590 - 596. [Abstract] [Full Text] [PDF]

				J. B Zwischenberger and S. K Alpard Artificial lungs: a new inspiration Perfusion, July 1, 2002; 17(4): 253 - 268. [Abstract] [PDF]

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Bradley M. Rodgers Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mallik, K. Articles by McGahren, E. D. Search for Related Content PubMed PubMed Citation Articles by Mallik, K. Articles by McGahren, E. D. Related Collections Related Article