Surgical management of neonates with congenital diaphragmatic hernia

doi:10.1053/j.sempedsurg.2007.01.007

Seminars in Pediatric Surgery

Volume 16, Issue 2, May 2007, Pages 109-114

https://doi.org/10.1053/j.sempedsurg.2007.01.007 Get rights and content

Congenital diaphragmatic hernia (CDH) is one of the most challenging and complex pediatric abnormalities to manage, both medically and surgically. The care of these neonates has seen significant evolution, from previous aggressive ventilation and emergent operation to current permissive hypercapnea, physiologic stabilization, and elective surgical repair, all in less than two decades. These changes have led to many centers reporting survival rates near 80%, a dramatic improvement from the 50% survival reported in the 1970s. This review covers the current principles guiding the surgical management of CDH in the neonate, including preoperative stabilization, operative timing, extracorporeal membrane oxygenation, surgical approach, and management of recurrence. Although many clinical challenges remain, multi-institutional collaboration and ongoing research efforts will hopefully improve the clinical care of these patients.

Section snippets

History of surgical management

Although diaphragmatic hernia was initially described by Ambrose Pare in the late 16th century, and the first published case of congenital diaphragmatic hernia in a child appeared in the early 18th century, the first report of a successful surgical repair was not until the early 20th century.¹ Nearly 25 years later, Hedblom published a large series of patients who underwent surgical repair for diaphragmatic hernia, concluding that early surgery would improve survival.² In 1940, Ladd and Gross

Operative timing

Although considered a surgical emergency from the 1940s³ through the 1980s,⁵ CDH is currently managed with cardiopulmonary stabilization, followed by definitive surgical repair. The paradigm shift from emergent to delayed repair occurred in 1987, when Sakai and coworkers showed that respiratory system compliance frequently deteriorates after CDH repair.⁶ Their report identified multiple factors, including distortion of the repaired diaphragm, increased intraabdominal pressure, and the

Pre-operative stabilization

Optimal initial postnatal resuscitation and management is aimed toward minimizing the physiologic derangements associated with pulmonary hypoplasia and pulmonary hypertension. Following delivery (or postnatal diagnosis of CDH), prompt endotracheal intubation (without high-pressure bag ventilation), nasogastric tube placement, and arterial/venous catheter placement are important initial maneuvers for pulmonary and hemodynamic support.¹¹ Oxygenation and acid-base status should be closely

Extracorporeal membrane oxygenation

In the late 1970s, the first reports of ECMO for infants with CDH¹⁴ provided a potential therapy for these children with severely hypoplastic lungs. Since that time, the strategy for ECMO has undergone continual refinement and critical examination.

Approximately one-third of infants born with CDH will be treated with ECMO during their initial course of management.15, 16 The previously accepted indications for initiation of ECMO include an Oxygenation Index (OI) >40, Paco₂ consistently >12, and

Surgical approach and principles

The traditional approach to repair of the diaphragmatic defect is via a subcostal incision on the ipsilateral side of the hernia. More than 90% of surgeons use this incision, whereas only 6% prefer the thoracic approach.¹³ After reduction of the abdominal viscera from the thorax and evisceration of the bowel to achieve adequate exposure, a true hernia sac (present only 10-20% of the time) should be identified and excised. Depending on the size of the defect, there are three general operative

Minimally invasive surgery

Advances in minimally invasive surgery (MIS) have led to both thoracoscopic and laparoscopic repairs of CDH. Arca and coworkers described the technical development of their minimally invasive approach to 15 children.³⁶ They found laparoscopy to be a better approach to Morgagni defects (Figure 1) and thoracoscopy a better approach to Bochdalek defects (Figure 2). They concluded that MIS was ideal for Morgagni defects, but that Bochdalek repair (via thoracoscopy) should be approached cautiously

Recurrent diaphragmatic hernia

Changes in the medical and surgical management have improved survival and, consequently, have exposed previously uncommon additional morbidity. Recurrence of the diaphragmatic defect is one such complication. Prevention of recurrent herniation requires that the reconstructed diaphragm grow with the patient. This growth is dependent on cellular deposition, proliferation, and organization into tissue. Such tissue organization requires vascular supply. Current theory, given the location of the

Conclusion

The management of CDH has seen steady progress over the last 20 years. Today, overall survival has reached 80% in live-born infants as a direct result of changes in medical and surgical management. Preoperative physiologic stabilization and subsequent elective repair have become the cornerstones of management. In many centers, ECMO is a key component of stabilization. Although elective repair has become routine, optimal timing of operation remains unclear. The role of minimally invasive surgery

References (44)

H.V. Firor
Surgical emergencies in newborns and infants
Surg Clin North Am
(1972)
H. Sakai et al.
Effect of surgical repair on respiratory mechanics in congenital diaphragmatic hernia
J Pediatr
(1987)
M. Nio et al.
A prospective trial of delayed versus immediate repair of congenital diaphragmatic hernia
J Pediatr Surg
(1994)
M.N. de la Hunt et al.
Is delayed surgery really better for congenital diaphragmatic hernia?A prospective, randomized clinical trial
J Pediatr Surg
(1996)
R.H. Clark et al.
Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group
J Pediatr Surg
(1998)
J.C. German et al.
Management of pulmonary insufficiency in diaphragmatic hernia using extracorporeal circulation with a membrane oxygenator (ECMO)
J Pediatr Surg
(1977)
C.D. Downard et al.
Impact of Amicar on hemorrhagic complications of ECMO: a ten-year review
J Pediatr Surg
(2003)
M.R. Langham et al.
Extracorporeal membrane oxygenation following repair of congenital diaphragmatic hernias
Ann Thorac Surg
(1987)
P.J. Davis et al.
Long-term outcome following extracorporeal membrane oxygenation for congenital diaphragmatic hernia: the UK experience
J Pediatr
(2004)
J.M. Wilson et al.
Evolution of the technique of congenital diaphragmatic hernia repair on ECMO
J Pediatr Surg
(1994)

J.M. Wilson et al.

Aminocaproic acid decreases the incidence of intracranial hemorrhage and other hemorrhagic complications of ECMO

J Pediatr Surg

(1993)

J.R. Horwitz et al.

A multicenter trial of 6-Aminocaproic Acid (Amicar) in the prevention of bleeding in infants on ECMO

J Pediatr Surg

(1998)

R.L. Moss et al.

Prosthetic patch durability in congenital diaphragmatic hernia: a long-term follow-up study

J Pediatr Surg

(2001)

S. Loff et al.

Implantation of a cone-shaped double-fixed patch increases abdominal space and prevents recurrence of large defects in congenital diaphragmatic hernia

J Pediatr Surg

(2005)

J.R. Fuchs et al.

Diaphragmatic reconstruction with autologous tendon engineered from mesenchymal amniocytes

J Pediatr Surg

(2004)

U. Samarakkody et al.

Reconstruction of congenital agenesis of the hemidiaphragm by combined reverse latissimus dorsi and serratus anterior muscle flaps

J Pediatr Surg

(2001)

A.F. Conforti et al.

Perinatal management of congenital diaphragmatic hernia

Early Hum Dev

(2006)

J.T. Wung et al.

Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration, and no chest tube

J Pediatr Surg

(1995)

M.J. Arca et al.

Early experience with minimally invasive repair of congenital diaphragmatic hernias: results and lessons learned

J Pediatr Surg

(2003)

E.Y. Yang et al.

Neonatal thoracoscopic repair of congenital diaphragmatic hernia: selection criteria for successful outcome

J Pediatr Surg

(2005)

D.H. Rowe et al.

Recurrent diaphragmatic hernia

Semin Pediatr Surg

(2003)

D.A. Saltzman et al.

Recurrent congenital diaphragmatic hernia: a novel repair

J Pediatr Surg

(2001)

Cited by (39)

Muscle Flap Technique Safe for On-ECMO Congenital Diaphragmatic Hernia Repair
2024, Journal of Pediatric Surgery
Prosthetic patches (patch) and muscle flaps (flap) are techniques used for repair of congenital diaphragmatic hernia (CDH) with a large defect unamenable to primary closure. We hypothesized that the flap technique for CDH repair while on extra-corporeal membrane oxygenation (on-ECMO) would have decreased bleeding complications compared to patch due to the hemostatic advantage of native tissue.
A single-center retrospective comparative study of patients who underwent on-ECMO CDH repair between 2008 and 2022 was performed.
Fifty-two patients met inclusion criteria: 18 patch (34.6%) and 34 flap (65.4%). There was no difference in CDH severity between groups. On univariate analysis, reoperation for surgical bleeding was lower following flap repair compared to patch (23.5% vs 55.6%, respectively; p = 0.045), 48-h postoperative blood product transfusion was lower after flap repair (132 mL/kg vs 273.5 mL/kg patch; p = 0.006), and two-year survival was increased in the flap repair group compared to patch (53.1% vs 17.7%, respectively; p = 0.036). On multivariate analysis adjusting for CDH side, day on ECMO repaired, and day of life CDH repaired, flap repair was significantly associated with lower five-day postoperative packed red blood cell transfusion amount, improved survival to hospital discharge, and improved two-year survival.
Our experience suggests that the muscle flap technique for on-ECMO CDH repair is associated with reduced bleeding complications compared to prosthetic patch repair, which may in part be responsible for the improved survival seen in the flap repair group. These results support the flap repair technique as a favored method for on-ECMO CDH repair.
Level III.
Surgical Management of Congenital Diaphragmatic Hernia
2022, Clinics in Perinatology
Porcine bladder extracellular matrix as a tissue regenerative strategy in a neonate with omphalocele and diaphragmatic agenesis
2021, Journal of Pediatric Surgery Case Reports
Citation Excerpt :
Commonly used prosthetic mesh include polytetrafluoroethylene (Gore-Tex), polypropylene (Marlex), and Dacron [1,2]. Post-operative complications from repairs with prosthetic mesh include skeletal abnormalities such as pectus deformities and scoliosis, restrictive pulmonary function, and lack of material growth leading to 50 % of patients having a recurrence of hernia [1,4]. There is concern for the durability of prosthetic patches with reports of nearly half of prosthetic patches for congenital diaphragmatic hernia repair showing evidence of reherniation and requiring subsequent revision with a muscle flap approach [5,6].
Porcine bladder extracellular matrix (PEBM) is a biologic membrane that promotes tissue regeneration in neonates with complex surgical disease. PEBM promotes cellular infiltration and capillary ingrowth for site-specific tissue deposition, providing both form and function to the remodeled host tissue. We present a case demonstrating the feasibility of this matrix as a tissue regeneration strategy in a patient presenting with omphalocele and diaphragmatic agenesis. Patient is an ex 36 + 1 week infant with prenatally diagnosed omphalocele containing liver who underwent operative closure on day of life (DOL) 2. During the procedure the posterior half of the right diaphragm was found to be absent. This was subsequently replaced with a 5 × 5cm PEBM patch and 10 × 7cm PEBM sheet over the liver as an underlay to thin fascia with subsequent layered closure. Patient was extubated DOL 5, advanced to goal enteral feeds DOL 18, and discharged home DOL 23. An ultrasound of the chest obtained 6 months post-repair demonstrated normal diaphragmatic motion bilaterally. Patient had no evidence of an abdominal wall defect on exam at 1 year follow up. Chest radiograph at that time also showed normal diaphragmatic contour. Patient is currently 4 years old without clinical or radiographic evidence of recurrence of either defect.
Congenital diaphragmatic hernia repair in patients on extracorporeal membrane oxygenation: How early can we repair?
2019, Journal of Pediatric Surgery
The benefits to early repair (< 72 h postcannulation) of infants with congenital diaphragmatic hernia (CDH) on extracorporeal membrane oxygenation (ECMO) are increasingly recognized. Yet it is not known if even earlier repair (< 24 h) results in comparable or improved patient outcomes. The goal of this study was to compare “super-early” (< 24 h) to early repair (24-72 h) of CDH patients on ECMO.
A retrospective review of infants with CDH placed on ECMO (2004–2017; n = 72) was performed. Data collected on the patients repaired while on ECMO within 72 h of cannulation (n = 33) included pre- and postnatal disease severity stratification variables and postnatal outcomes. Comparison groups were those patients repaired within 24 h of cannulation (n = 14) and those repaired between 24 and 72 h postcannulation (n = 19).
Patients undergoing “super-early” (< 24 h) repair had an average survival of 71.4% compared to the average survival of 59.7% in the early repair group. Pre- and postnatal variables predicting disease severity were not significantly different between the groups. Mean hospital stays, ventilator days, and cannulation days were statistically similar between the groups.
Repair of patients with CDH patients on ECMO at less than 24 h postcannulation achieves outcomes that are comparable to those of repair between 24 and 72 h. While the present data suggest that there is not a “too early” time point for CDH repair on ECMO, larger multicenter studies are needed to validate our findings and determine the overall benefits.
Retrospective comparative study.
Level III.
The Extremely Premature Infant (Micropremie) and Common Neonatal Emergencies
2019, A Practice of Anesthesia for Infants and Children
The preterm infant, defined as birth before 37 weeks gestation, provides unique medical and surgical challenges to health care providers due to a myriad of anatomical underdevelopments and physiologic derangements. The physiology of prematurity as it relates to anesthesia is of particular importance when preparing for surgery. Anatomic differences of the premature airway and altered respiratory mechanics, such as a smaller airway diameter, increased oxygen consumption, bronchopulmonary dysplasia and apnea place these infants at risk for rapid and profound desaturation and hypoventilation during anesthesia. The immature heart has not had time to develop sufficient muscle fibers for optimal contractility. In addition, persistent pulmonary hypertension of the newborn may develop when right-to-left shunting of blood occurs through a patent ductus arteriosus and/or a patent foramen ovale due to failure of the pulmonary vascular resistance to drop at birth. These factors may combine and lead to significant cardiovascular collapse during surgery. Premature infants are susceptible to metabolic derangements such as hypoglycemia and hypocalcemia due to improper storage and loss of maternal-fetal transfer during gestation. The liver and kidneys are underdeveloped, leading to altered drug metabolism, thus, anesthetic drugs must be tailored accordingly. Neonatal surgical emergencies, such as congenital diaphragmatic hernia, hypertrophic pyloric stenosis, necrotizing enterocolitis and gastroschisis, can present at any time and be life-threatening. Immediate surgical intervention is not always necessary and there is often time for medical optimization. Advances in neonatal care have improved the morbidity and mortality of critically ill newborns.
The Extremely Premature Infant (Micropremie) and Common Neonatal Emergencies
2018, A Practice of Anesthesia for Infants and Children
The preterm infant, defined as birth before 37 weeks gestation, provides unique medical and surgical challenges to health care providers due to a myriad of anatomical underdevelopments and physiologic derangements. The physiology of prematurity as it relates to anesthesia is of particular importance when preparing for surgery. Anatomic differences of the premature airway and altered respiratory mechanics, such as a smaller airway diameter, increased oxygen consumption, bronchopulmonary dysplasia and apnea place these infants at risk for rapid and profound desaturation and hypoventilation during anesthesia. The immature heart has not had time to develop sufficient muscle fibers for optimal contractility. In addition, persistent pulmonary hypertension of the newborn may develop when right-to-left shunting of blood occurs through a patent ductus arteriosus and/or a patent foramen ovale due to failure of the pulmonary vascular resistance to drop at birth. These factors may combine and lead to significant cardiovascular collapse during surgery. Premature infants are susceptible to metabolic derangements such as hypoglycemia and hypocalcemia due to improper storage and loss of maternal-fetal transfer during gestation. The liver and kidneys are underdeveloped, leading to altered drug metabolism, thus, anesthetic drugs must be tailored accordingly. Neonatal surgical emergencies, such as congenital diaphragmatic hernia, hypertrophic pyloric stenosis, necrotizing enterocolitis and gastroschisis, can present at any time and be life-threatening. Immediate surgical intervention is not always necessary and there is often time for medical optimization. Advances in neonatal care have improved the morbidity and mortality of critically ill newborns.

View all citing articles on Scopus

View full text

Surgical management of neonates with congenital diaphragmatic hernia

Section snippets

History of surgical management

Operative timing

Pre-operative stabilization

Extracorporeal membrane oxygenation

Surgical approach and principles

Minimally invasive surgery

Recurrent diaphragmatic hernia

Conclusion

Surg Clin North Am

J Pediatr

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Ann Thorac Surg

J Pediatr

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Early Hum Dev

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Semin Pediatr Surg

J Pediatr Surg