AUTHOR AND EDITOR INFORMATION

Section 1 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

INTRODUCTION

Section 2 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

History of the Procedure

In 1679, Lazarus Riverius (1589-1655) recorded the first reported case of a congenital diaphragmatic hernia (CDH); this was following postmortem examination of a 24-year-old male (Irish, 1996). The first attempt at surgical repair for CDH was by Nauman of Sweden in 1888; the 19-year-old patient presented with acute respiratory distress and an acute abdomen, and a laparotomy was performed. In 1889, J. O'Dwyer, MD, carried out the first repair of CDH in an infant. The first successful repair occurred in 1905. The patient was aged 9 years, and Heidenhain (at the Municipal Hospital for Worms, Germany) reduced the hernia and closed the diaphragmatic defect through a midline laparotomy incision. Approximately 20 years later, Carl Hedbolm reported a 58% mortality rate for patients undergoing surgical intervention for CDH.

In 1940, William Ladd and Robert Gross based their diagnosis of CDH on history, physical examination, and chest radiograph with or without a barium meal. They advocated early surgical intervention (within the first 48 h). Gross also described a 2-staged closure of the abdominal wall in difficult cases—closure of skin and subcutaneous fascia at the initial surgery and closure of the abdominal wall 5-6 days later. In 1950, C. Everett Koop and Julian Johnson suggested the transthoracic approach as a means of closing the defect under more direct vision.

As surgical expertise improved, innovative strategies were developed to address large diaphragmatic defects and agenesis of the hemidiaphragm. Techniques included rotational muscle flaps, perirenal fascia, and, more recently, prosthetic materials.

Instrumental in improving the survival rate in infants with CDH were the exponential elucidation of the pathophysiology of CDH, the investigation of innovative therapies, improved management of associated cardiac anomalies. CDH was no longer primarily considered a surgical disease but rather a disease associated with pulmonary hypoplasia, pulmonary hypertension, pulmonary immaturity, and an increased susceptibility of the lungs to ventilation-induced lung injury. This led to a delayed approach to surgical repair and to a gentle but more ingenious respiratory support. Current investigations also focus on prenatal therapies, such as antenatal steroids, in utero repair of the defect, and tracheal occlusion in utero.

Problem

Ten to fifty percent of patients with CDH have associated anomalies, which confer a 2-fold relative risk of mortality when compared with patients with isolated CDH (Tonks, 2004). Frequently associated anomalies include cardiac defects, chromosomal anomalies (ie, trisomies 21, 18, and 13), renal anomalies, genital anomalies, and neural tube defects.

Frequency

CDH occurs in 1 per 3000 live births (Torfs, 1992).

Mortality: The Congenital Diaphragmatic Hernia Study Group recorded a 63% survival rate in 1995-1996 based on data from 62 centers in North America, Europe, and Australia (Clark, 1998). Survival rates are 60-90% for patients who present within the first few hours of life (Downard, 2003) (see Image 1).

Etiology

Relevant embryology

The diaphragm is derived from 4 embryonic structures: the septum transversum, the pleuroperitoneal membranes, mesoderm of the body wall, and esophageal mesenchyme. Following folding of the fetal head at 4-5 weeks' gestation, the septum transversum comes to lie as a semicircular shelf, which separates the heart from the liver. The septum transversum does not completely separate the thoracic cavity from the peritoneal cavity but allows pericardioperitoneal canals to exist on either side of the esophagus.

During the fifth week of gestation, the pleuroperitoneal membranes develop along a line connecting the root of the 12th rib with the tips of the 7th to 12th ribs. The pleuroperitoneal membranes grow ventrally to fuse with the posterior margins of the septum transversum and the dorsal mesentery of the esophagus. Hence, at 6-7 weeks' gestation, the pleuroperitoneal canals are closed; the left closes after the right. The mesentery of the esophagus condenses to form the left and right crura of the diaphragm, and the mesoderm of the body wall forms the outer rim of diaphragmatic muscle.

The posterolateral diaphragmatic defect is postulated to result from failure of closure of the pleuroperitoneal canals. The canal remains open when the intestines return to the abdomen at 10 weeks' gestation. Some intestine and other viscera enter the thorax and lead to compression of the developing lung at the crucial pseudoglandular stage and shifting of the mediastinum to the contralateral side. This causes compression of the heart and the contralateral lung as well.

In 1984, Iritani proposed a different concept of diaphragmatic development. He suggested that a posthepatic mesenchymal plate develops between the septum transversum and the pericardioperitoneal canals (Iritani, 1984). Lateral growth of this plate leads to closure of the pericardioperitoneal canals, and CDH results from a disturbance in growth of the posthepatic mesenchymal plate.

Causes

• Genetic factors: The initiating factor responsible for the development of CDH is unknown. Wide variations have been noted in the reported prevalence of chromosomal abnormalities (7-31%) in patients with CDH. The prevalence is higher in cases of CDH associated with other defects (Torfs, 1992). Familial occurrence has been noted in fewer than 2% of CDH cases.
• Other causes: The role of drugs and environmental chemicals in the development of CDH is uncertain, but nitrofen, quinine, thalidomide, phenmetrazine, and polybrominated diphenyls have been used to induce CDH in various species.

Pathophysiology

The pathophysiology of CDH involves pulmonary hypoplasia, pulmonary hypertension, pulmonary immaturity, and potential deficiencies in the surfactant and antioxidant enzyme system.

Because of bowel herniation into the chest during crucial stages of lung development, airway divisions are limited to the 12th to 14th generation on the ipsilateral side and to the 16th to 18th generation on the contralateral side. Normal airway development results in 23-35 divisions. Because airspace development follows airway development, alveolarization is similarly reduced.

Development of the pulmonary arterial system parallels development of the bronchial tree, and, therefore, fewer arterial branches are observed in CDH. Abnormal medial muscular hypertrophy is observed as far distally as the acinar arterioles, and the pulmonary vessels are more sensitive to stimuli of vasoconstriction (Ting, 1998). Pulmonary hypertension resulting from these arterial anomalies leads to right-to-left shunting at atrial and ductal levels. This persistent fetal circulation leads to right-sided heart strain or failure and to the vicious cycle of progressive hypoxemia, hypercarbia, acidosis, and pulmonary hypertension observed in the neonatal period.

The surfactant system is demonstrably deficient in the lamb model of CDH (Glick, 1992). Postnatal administration of surfactant in these lambs is associated with dramatic increases in gas exchange, lung compliance, and pulmonary blood flow. In human neonates, however, reports on the status of the surfactant system are inconsistent (Janssen, 2003; Lotze, 1994).

The infant with CDH also has an impairment of the pulmonary antioxidant enzyme system and is more susceptible to hyperoxia-induced injury.

In addition, a left ventricular smallness and hypoplasia are observed with CDH. This is believed to arise from decreased in utero blood flow to the left ventricle, the mechanical compression of the herniated viscus similar to that observed in the lungs, and/or a primary yet unidentified developmental defect that simultaneously causes the diaphragmatic hernia and previously described lung problems.

Clinical

Prenatal

The diagnosis of CDH is frequently made prenatally prior to 25 weeks' gestation.

CDH is usually detected in the antenatal period (46-97%), depending on the use of level II ultrasonography techniques (Adzick, 1998). Ultrasonography reveals polyhydramnios, an absent intra-abdominal gastric air bubble, mediastinal shift, and hydrops fetalis. Ultrasonography demonstrates the dynamic nature of the visceral herniation observed with CDH. The visceral hernia has moved in and out of the chest in several fetuses (Adzick, 1998).

Differential diagnoses on prenatal ultrasonography are as follows:

• Congenital cystic adenomatoid malformation
• Pulmonary sequestration
• Mediastinal cystic processes (eg, cystic teratoma, thymic cysts, foregut duplication cysts)
• Neurogenic tumors

Postnatal

History and clinical findings vary with the presence of associated anomalies and the degree of pulmonary hypoplasia and visceral herniation. In the infant presenting in the neonatal period without prenatal diagnosis, variable respiratory distress and cyanosis, feeding intolerance, and tachycardia are noted.

In the physical examination, the abdomen is scaphoid if significant visceral herniation is present (see Image 2).

On auscultation, breath sounds are diminished, bowel sounds may be heard in the chest, and heart sounds are distant or displaced.

Late presentation

Patients may present outside of the neonatal period with intestinal obstruction, bowel ischemia, and necrosis following volvulus.

INDICATIONS

Section 3 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

There is no ideal time for repair of CDH, but the authors suggest that the window of opportunity is 24-48 hours after birth to achieve normal pulmonary arterial pressures and satisfactory oxygenation and ventilation on minimal ventilator settings.

RELEVANT ANATOMY

Section 4 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

The diaphragm is a musculotendinous structure that separates the thoracic cavity from the abdominal cavity. It is composed of a central nonmuscular portion (central tendon) surrounded by a muscular rim in addition to the right and left diaphragmatic cura. The right and left diaphragmatic cura are 2 muscular bands that originate from vertebral bodies L1-L3 and L1-L2 respectively. These muscular bands insert into the dorsomedial diaphragm.

Most diaphragmatic defects are posterolateral, with 85-90% of these occurring on the left. The label "posterolateral" may be a misnomer because, frequently, much larger areas of the diaphragm are missing and only a posterior rim of muscle can be found. A hernial sac is present in 10-20% of cases.

The Morgagni defect occurs posterior to the sternum and results from failure of sternal and costal fibers to fuse at the site where the superior epigastric artery crosses the diaphragm. Morgagni defect is rare, and it is rarely a cause for surgery in the newborn.

CONTRAINDICATIONS

Section 5 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

The association of congenital diaphragmatic hernia with lethal congenital abnormalities is a relative contraindication to repair of the diaphragmatic defect.

WORKUP

Section 6 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

Lab Studies

• Antenatal
• • Amniocentesis for karyotype analysis should accompany a diagnosis of congenital diaphragmatic hernia (CDH).
  • Maternal serum alpha-fetoprotein may be low in cases of CDH.
• Postnatal
• • Assess arterial blood gases.
  • Hypoxemia, hypercarbia, and respiratory or metabolic acidosis depend on the degree of pulmonary hypoplasia, persistent pulmonary hypertension of newborn (PPHN), right-to-left shunting, and ventricular function.

Imaging Studies

• Level III ultrasonography and echocardiography should accompany a diagnosis of CDH. Prenatal echocardiography may identify cardiac anomalies (more commonly, ventricular hypoplasia, atrial septal defects, and ventricular septal defects) (Fauza, 1994).
• Chest radiography
• • An early chest radiograph is obtained to confirm the diagnosis of CDH.
  • Findings include loops of bowel in the chest, mediastinal shift, paucity of bowel gas in the abdomen, and presence of the tip of a nasogastric tube in the thoracic stomach (see Image 3). Repeated chest radiographs may reveal a change in the intrathoracic gas pattern.
  • Right-sided lesions are difficult to differentiate from diaphragmatic eventration and lobar consolidation.
• Echocardiography
• • Further investigations should include early echocardiography, which may reveal cardiac defects, decreased left ventricular mass, poor ventricular contractility, pulmonary and tricuspid valve regurgitation, and right-to-left shunting.
  • Repeated echocardiography is recommended to measure changes in the pulmonary artery pressure, left-to-right shunt, and flow across the ductus arteriosus.

TREATMENT

Section 7 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

Medical therapy

In contrast to historic management patterns, which focused on the actual repair of the diaphragmatic hernia, the contemporary management of congenital diaphragmatic hernia (CDH) places emphasis on the management of pulmonary hypoplasia and persistent pulmonary hypertension. Current management uses a variety of gentle alveolar recruitment strategies and a nonurgent approach to the operative treatment of CDH (Irish, 1996; Bagolan, 2004).

Immediately following delivery, the infant is intubated (bag and mask ventilation is avoided). A nasogastric tube is passed to decompress the stomach and to avoid visceral distention.

Adequate assessment involves continuous cardiac monitoring, arterial blood gas and systemic pressure measurements, urinary catheterization to monitor fluid resuscitation, and both preductal (radial artery) and postductal (umbilical artery) oximetry.

Pressure limited ventilation should be used, allowing the lowest airway pressures compatible with staying on the steep side of the pressure volume loop and preductal oxygen saturations greater than 90%. Peak inspiratory pressures (PIP) should be less than 30 cm H₂O. Hypercarbia is allowed as long as the pH can be buffered (Chess, 2004).

Alternative means of support (eg, high-frequency oscillatory ventilation [HFOV], extracorporeal membrane oxygenation (ECMO), and inhaled nitric oxide [iNO]) should be considered for the patient who fails to stabilize on conventional ventilation.

HFOV is recommended for infants with hypercarbia and hypoxemia resistant to conventional ventilation or requiring high PIP (>30 cm H₂O) (Reyes, 1998). HFOV uses an oscillating diaphragm to create a sinusoidal column of air within the airways. The diaphragm oscillates at a high frequency and improves gas exchange without increased ventilatory pressures. Increased gas exchange leads to elimination of carbon dioxide, which decreases the stimulus for pulmonary vasoconstriction and decreases pulmonary hypertension. At some institutions, HFOV is chosen as the primary means of ventilation.

Surfactant rescue or prophylactic therapy is associated with an improvement in oxygenation in some neonates with CDH (Bos, 1991; Glick, 1992). Surfactant used as rescue therapy is administered within 24 hours of birth in neonates with CDH and a poor prognosis. As prophylactic therapy, surfactant (50-100 mg/kg of Infasurf R) is administered prior to the first breath in neonates with CDH who were given a poor prognosis antenatally. Prophylactic surfactant therapy and natural surfactants are thought to be more efficacious. There is, as yet, no definitive evidence of a surfactant deficiency in human neonates, and surfactant as rescue therapy has not been shown to improve outcome (Colby, 2004).

iNO has proven to be a highly selective pulmonary vasodilator and has been used as rescue therapy in infants with PPHN. iNO produces pulmonary vasodilatation, decreases the ventilation-perfusion mismatch, and reverses the ductal shunting observed in PPHN. Limited success has been gained in the use of iNO in patients with CDH, but efficacy of iNO improves following surfactant therapy (Karamanoukian, 1995).

The selection criteria for ECMO eligibility in CDH are the standard criteria used for other neonates with respiratory failure, as follows: a pH less than 7.15, oxygenation index greater than 40, and failure to respond to maximal medical treatment. ECMO should be reserved for patients who fail to respond to the alternative therapies if the extent of pulmonary hypoplasia is not considered to be lethal and when acute deterioration occurs in the postoperative period. ECMO in these cases provides respiratory support without additional barotrauma or oxygen toxicity. It allows time for the transition from fetal circulation, as well as the maturation of the pulmonary parenchyma (see Image 4).

Surgical therapy

No ideal time for repair of CDH exists, but the authors suggest that the window of opportunity is 24-48 hours after birth to achieve normal pulmonary arterial pressures and satisfactory oxygenation and ventilation with minimal ventilator settings. However, surgical repair can be safely delayed in stable patients, and the operation can be scheduled on a semi-elective basis. Emphasize that urgent surgical repair is almost never necessary and that it may worsen the pulmonary hypertension.

Preoperative details

The priority of the preoperative care is focused on the ventilatory management of the newborn and determining if the patient has any other associated congenital anomalies, particularly cardiac abnormalities. An echocardiogram should always be obtained prior to surgical repair.

Intraoperative details

• A subcostal incision is made. The abdominal viscera are examined, and the hernia is reduced by gentle traction. A hernia sac is sought and excised if found. Following careful dissection of the posterior leaf of the diaphragm, primary repair can be accomplished in a single layer using nonabsorbable sutures. If the diaphragmatic defect is large enough to preclude primary closure, a Gore-Tex patch, or rotational muscle flaps (Scaife, 2003) or fascial flaps (Okazaki, 2005), can be used. If the patient is stable, the malrotation is corrected and Ladd bands lysed. The transthoracic repair of a left- and right-sided diaphragmatic hernia has been reported. However, this approach is not commonly used.
• If abdominal closure may interfere with chest wall or diaphragmatic compliance or lead to abdominal compartment syndrome, then a temporary silo with delayed primary closure of the fascia or skin can be safely accomplished.
• The use of chest tubes is controversial, as is the use of suction. The authors prefer to use a chest tube but limit suction to 5 cm H₂O. Most authors in North America suggest avoiding the use of suction to minimize mediastinal shift.
• The patient with a right-sided defect and an intrathoracic liver presents unique problems to the surgeon. The neonatal liver is extremely friable, and kinking of the hepatic veins and the inferior vena cava can accompany the return of the liver to the abdomen. Careful manipulation of the liver into the abdomen must be accompanied by hemodynamic monitoring. Occasionally, a 2-cavity (right chest and abdomen) approach may be necessary to reduce the viscera. Another well-described technique is to repair the diaphragmatic hernia using thoracotomy. Such approach typically allows for reduction of the liver and viscera back into the abdomen with excellent exposure of the diaphragm.
• Surgical repair while the patient is on ECMO was initially associated with an increased mortality rate, surgical site hemorrhage, and intracranial hemorrhage (Wilson, 1994). To decrease the hemostatic complications, associated ECMO platelet counts are now maintained above 150,000/µL³, and the activated clotting times (ACT) are decreased to 160-180 seconds.
• Use of aminocaproic acid in the perioperative period decreases the fibrinolysis associated with use of the ECMO circuit and leads to decreased hemorrhagic complications. Intraoperative and postoperative blood loss is decreased with the following:
  • Use of electrocautery for skin incision
  • No dissection of the posterior leaf if primary repair is unlikely
  • Use of prosthetic patch repair
  • Limited blunt and sharp dissection
  • Judicious use of electrocautery
  • Application of topical thrombin to the suture line
• Repairing the diaphragmatic hernia after decannulation from ECMO avoids the hemostatic complications associated with ECMO. This leads to recurrent pulmonary hypertension in some patients. The authors prefer repair on ECMO when the patient is ready for decannulation. Therefore, the patient tolerates decannulation if bleeding occurs.

Follow-up

Continued care is provided for survivors of CDH by a multidisciplinary team consisting of a social worker, nutritionist, physiotherapist, pediatrician/neonatologist, neurologist, and pediatric surgeon. The following screening tests could be performed prior to discharge: chest radiography, arterial blood gas, brain stem auditory evoked potentials, head CT scanning or head ultrasonography, and a developmental evaluation. In the outpatient clinic, chest radiography, pulmonary function tests, nutritional and developmental assessments, and repeated auditory, ophthalmology, and neurology evaluations are performed.

COMPLICATIONS

Section 8 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

• Complications observed in the early postoperative period include recurrent pulmonary hypertension and deterioration in respiratory mechanics and gaseous exchange (Adzick, 1998).
• Less commonly observed complications are the disruption of the suture line, recurrence of the diaphragmatic hernia, leakage of peritoneal fluid and blood into the thorax, and development of an ipsilateral hydrothorax.
• Small-bowel obstruction may occur secondary to adhesions or volvulus.

OUTCOME AND PROGNOSIS

Section 9 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

Long-term outcomes and prognosis are as follows:

• Long-term pulmonary disease depends on the degree of pulmonary hypoplasia, barotrauma, and volutrauma sustained in the neonatal period. Bronchopulmonary dysplasia and restrictive and/or obstructive lung disease may be observed in congenital diaphragmatic hernia (CDH) survivors.
• Failure to thrive is often observed in the presence of optimal feeding regimes.
• Functional and anatomic esophageal abnormalities in CDH survivors are sometimes associated with gastroesophageal reflux, requiring surgical management.
• The use of ECMO, hyperventilation treatment, and ototoxic medication places this population at a higher risk for sensorineural hearing loss as well as neurodevelopmental abnormalities (ie, cognitive and developmental delay, cerebral palsy, seizure disorders, impaired vision).
• Altered musculoskeletal development results in thoracic scoliosis, pectus deformities, and a decreased thoracic cavity on the affected side.

For excellent patient education resources, visit eMedicine's Esophagus, Stomach, and Intestine Center. Also, see eMedicine's patient education article Hiatal Hernia.

FUTURE AND CONTROVERSIES

Section 10 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

Liquid ventilation uses perfluorocarbon, which is an inert compound with low surface tension and greater solubility for respiratory gases than blood. In partial liquid ventilation (PLV), the lungs are filled with perfluorocarbon to the functional residual capacity, and conventional ventilation is superimposed. PLV is associated with improved oxygenation and decreased PIP requirements. This may be due to recruitment of atelectatic lungs and decreased ventilation-perfusion mismatch. Theoretically, PLV decreases the requirements for ventilation and so decreases barotrauma- and hyperoxia-induced pulmonary injury associated with congenital diaphragmatic hernia (CDH).

Preliminary clinical trials were conducted on infants with CDH and a high predicted mortality rate; while these infants were on extracorporeal life support, their lungs were filled with PFC and continuous positive airway pressure was maintained at 7-10 cm H₂O. Accelerated growth of the ipsilateral lung, improved gas exchange, and improved survival were observed after one week.

Experimental fetal surgery has been expanding rapidly over the last 2 decades. The fetus with CDH most likely to benefit from in utero intervention has lethal pulmonary hypoplasia and no coexisting other lethal congenital anomalies. To date, no prenatal parameter has been able to reliably predict the occurrence of lethal pulmonary hypoplasia. Hence, selection criteria for in utero intervention remain controversial. Current trends in fetal surgery for severe congenital diaphragmatic hernia focus on the manipulation of lung growth by temporary occlusion of the fetal trachea utilizing minimal access surgery (see Image 5).

Theoretically, fetuses with CDH should benefit from antenatally administered corticosteroids. In the fetal lamb model, corticosteroid administration at 24 and 48 hours prior to delivery was associated with significant increases in lung compliance. Antenatal steroids are currently used at some centers.

Thoracoscopic repair of CDH in the neonatal period is now being attempted. This is associated with increased complication rates and longer operating times. As with most minimally invasive techniques, patient selection criteria prove to be the determining factor in successful thoracoscopic repair. Patients who require minimal ventilation support or those with an intra-abdominal stomach are more likely to undergo a successful thoracoscopic repair.

The revelation of lethal pulmonary hypoplasia with antenatal MRI or ultrasonography has been disappointing. This may be because other significant factors, such as lung maturity and the development of persistent pulmonary hypertension, determine outcome following delivery.

MULTIMEDIA

Section 11 of 12  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

Media file 1:  Graph illustrating the concept of the hidden mortality of congenital diaphragmatic hernia. Image courtesy of Michael Harrison, MD.
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Media file 2:  Photograph of a one-day-old infant with congenital diaphragmatic hernia. Note the scaphoid abdomen. This occurs if significant visceral herniation into the chest is present.
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Media file 3:  Radiograph of an infant with congenital diaphragmatic hernia. Note shift of the mediastinum to the right, air-filled bowel in the left chest, and the position of the orogastric tube.
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Media file 4:  Newborn baby with congenital diaphragmatic hernia on venoarterial extracorporeal membrane oxygenation (ECMO). Note the arterial and venous cannulas connected to the bedside cardiovascular bypass machine.
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Media type:  Photo

Media file 5:  Diagram illustrating the sheep model of PLUG, the trachea used for the fetal management of congenital diaphragmatic hernia. Image courtesy of Michael Harrison, MD.
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Media type:  Image

REFERENCES

Section 12 of 12

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Multimedia
• References

• Bagolan P, Casaccia G, Crescenzi F, et al. Impact of current treatment protocol on outcome of high risk congenital diaphragmatic hernia. Journal of Pediatric surgery. 2004;39:313-318. [Medline].
• Bohn DJ, Pearl R, Irish MS, Glick PL. Postnatal management of congenital diaphragmatic hernia. Clin Perinatol. Dec 1996;23(4):843-72. [Medline].
• Bos AP, Tibboel D, Hazebroek FW, et al. Surfactant replacement therapy in high-risk congenital diaphragmatic hernia. Lancet. Nov 16 1991;338(8777):1279. [Medline].
• Chess PR. The effects of gentle ventilation on survival in congenital diaphragmatic hernia. Pediatrics. 2004;113:917. [Medline].
• 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. Jul 1998;33(7):1004-9. [Medline].
• Colby CE, Lally KP, Hintz SR, et al. Surfactant replacement therapy on ECMO does not improve outcome in neonates with congenital diaphragmatic hernia. Journal of Pediatric Surgery. 2004;39(11):1632-7. [Medline].
• Downard C, Jaksic T, Garza J, et al. Analysis of an improved survival rate for congenital diaphragmatic hernia. Journal of Pediatric Surgery. 2003;38:729-732. [Medline].
• Fauza DO, Wilson JM. Congenital diaphragmatic hernia and associated anomalies: their incidence, identification, and impact on prognosis. J Pediatr Surg. Aug 1994;29(8):1113-7. [Medline].
• Glick PL, Leach CL, Besner GE, et al. Pathophysiology of congenital diaphragmatic hernia. III: Exogenous surfactant therapy for the high-risk neonate with CDH. J Pediatr Surg. Jul 1992;27(7):866-9. [Medline].
• Glick PL, Pohlson EC, Resta R, et al. Maternal serum alpha-fetoprotein is a marker for fetal anomalies in pediatric surgery. J Pediatr Surg. Jan 1988;23(1 Pt 2):16-20. [Medline].
• Irish MS, Holm BA, Glick PL. Congenital diaphragmatic hernia. A historical review. Clin Perinatol. Dec 1996;23(4):625-53. [Medline].
• Iritani I. Experimental study on embryogenesis of congenital diaphragmatic hernia. Anat Embryol (Berl). 1984;169(2):133-9. [Medline].
• Janssen DJ, Tibboel D, Carnielli VP, et al. Surfactant phosphatidylcholine pool size in human neonates with congenital diaphragmatic hernia requiring ECMO. J Pediatr. Mar 2003;142(3):247-52. [Medline].
• Karamanoukian HL, Glick PL, Wilcox DT, et al. Pathophysiology of congenital diaphragmatic hernia. VIII: Inhaled nitric oxide requires exogenous surfactant therapy in the lamb model of congenital diaphragmatic hernia. J Pediatr Surg. Jan 1995;30(1):1-4. [Medline].
• Karamanoukian HL, O''Toole SJ, Glick PL. "State-of-the-art" management strategies for the fetus and neonate with congenital diaphragmatic hernia. J Perinatol. Mar-Apr 1996;16(2 Pt 2 Su):S40-7. [Medline].
• Koop CE, Johnson J. Transthoracic repair of diaphragmatic hernia in infants. Ann Surg. Dec 1952;136(6):1007-11. [Medline].
• Ladd WE, Gross RE. Congenital diaphragmatic hernia. N Engl J Med. 1940;223:917-925.
• Lotze A, Knight GR, Anderson KD, et al. Surfactant (beractant) therapy for infants with congenital diaphragmatic hernia on ECMO: evidence of persistent surfactant deficiency. J Pediatr Surg. Mar 1994;29(3):407-12. [Medline].
• Okazaki T, Masegava S, Urushihara N, et al. Toldt's fascia flap: a new technique for repairing large diaphragmatic hernias. Pediatric Surgery International. 2005;21:64-67. [Medline].
• Reyes C, Chang LK, Waffarn F, et al. Delayed repair of congenital diaphragmatic hernia with early high-frequency oscillatory ventilation during preoperative stabilization. J Pediatr Surg. Jul 1998;33(7):1010-4; discussion 1014-6. [Medline].
• Scaife ER, Johnson DG, Meyers RL, et al. The split abdominal wall muscle flap. a simple mesh free approach to repair of large diaphragmatic defects. Journal of Pediatric Surgery. 2003;38:1748-1751. [Medline].
• Sreenan C, Etches P, Osiovich H. The western Canadian experience with congenital diaphragmatic hernia: perinatal factors predictive of extracorporeal membrane oxygenation and death. Pediatr Surg Int. Mar 2001;17(2-3):196-200. [Medline].
• Ting A, Glick PL, Wilcox DT, et al. Alveolar vascularization of the lung in a lamb model of congenital diaphragmatic hernia. Am J Respir Crit Care Med. Jan 1998;157(1):31-4. [Medline].
• Tonks A, Wyldes M, Sommerset DA, et al. Congenital malformations of the diaphragm: findings of the West Midlands congenital anomaly register 1995 to 2000. prenatal diagnosis. 2004;24:596-604. [Medline].
• Torfs CP, Curry CJ, Bateson TF, Honore LH. A population-based study of congenital diaphragmatic hernia. Teratology. Dec 1992;46(6):555-65. [Medline].
• Wilson JM, Bower LK, Lund DP. Evolution of the technique of congenital diaphragmatic hernia repair on ECMO. J Pediatr Surg. Aug 1994;29(8):1109-12. [Medline].
• Wung JT, Sahni R, Moffitt ST, et al. Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration, and no chest tube. J Pediatr Surg. Mar 1995;30(3):406-9. [Medline].

Article Last Updated: May 22, 2006