Congenital Diaphragmatic Hernia Treatment & Management

Updated: Oct 08, 2019
  • Author: Daniel S Schwartz, MD, MBA, FACS; Chief Editor: Jeffrey C Milliken, MD  more...
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Treatment

Approach Considerations

The diaphragm is the major muscle of respiration and the second most important muscle after the heart. When a decrease in diaphragmatic function occurs, a concomitant respiratory dysfunction generally always accompanies the functional decrease. Although the body has many compensatory mechanisms in the setting of decreased diaphragmatic function, little can be done to prevent respiratory compromise if excursion of the diaphragm is moderately diminished or simply absent. Appropriate treatment is essential in cases of congenital diaphragmatic hernia (CDH).

Some reports exist of increases in mortality with early surgical intervention for CDH in infants. Many authors suggest that the patient be stabilized (often with the use of extracorporeal membrane oxygenation [ECMO]) and that repair be delayed until the infant is better prepared to survive the operation.

Research is under way into the use of inhaled nitric oxide (iNO) or partial liquid ventilation and the possibility of lung transplantation for infants born with severe hypoplasia. [18]  Minimally invasive repair techniques for latent CDH are being explored at a number of institutions.

Guidelines for the management of CDH have been published. [23]

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Medical Therapy

Resuscitation with ventilatory support is of prime importance in patients born with a CDH. There has been a trend toward switching from conventional mechanical ventilation to high-frequency oscillatory ventilation (HFOV). HFOV serves to minimize airway pressure and, in conjunction with permissive hypercapnia, helps those with CDH suffer less traumatic lung injury and fewer long-term complications. Mortality has been shown to decrease from 49% to 20% when HFOV is used early in the treatment course. [24]

ECMO has been shown to decrease the mortality of CDH significantly but is currently reserved for individuals whose condition fails to improve with both HFOV and conventional mechanical ventilation. The decision to utilize ECMO is made early in the disease process, usually within 24 hours of birth.

With the addition of HFOV, more reports exist of decreased morbidity and mortality with early surgical intervention. However, there remains some controversy regarding this practice. The classical teaching is that patients need to be stabilized (often with the use of ECMO) and that repair should be delayed until the infant is better prepared to survive the operation. [25]

Maternal antenatal corticosteroid administration has also been employed in an effort to improve fetal lung growrth and maturity, but the available evidence is insufficient to support its routine use. [26]

Prostaglandin E1 (PGE1) has been used to treat severe pulmonary hypertension in patients with CDH; a study by Lawrence et al found it to be well tolerated in this setting and to be associated with improvements in B-type natriuretic peptide (BNP) and echocardiographic indices of pulmonary hypertension. [27]

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Surgical Therapy

With regard to treatment of CDH in the perinatal period, significant issues remain, and many questions are yet to be answered. However, the possibility of maternal-fetal surgical treatment of CDH is on the horizon. [28, 29]

For treatment of CDH in the neonatal period, a subcostal incision is used. An attempt should be made to carry out a primary repair of the hernia. The abdominal contents are reduced, and the edges of the diaphragm are then approximated with nonabsorbable suture. In some cases, such as when the defect is large or the repair is being made while the patient is on ECMO, a prosthetic material such as expanded polytetrafluoroethylene (ePTFE) or polypropylene is used.

For treatment of latent CDH, the typical surgical approach has been through a thoracotomy or laparotomy. Most surgeons approach via a laparotomy so that abdominal contents can be inspected adequately. In children, prosthetic mesh is typically used; in adults, direct suture technique using nonabsorbable suture material is commonly employed.

For CDH related to traumatic rupture, [30, 31, 32]  the surgical approach depends on the timing of the diagnosis with the surgical intervention. [29]

Minimally invasive approaches (eg, involving video-assisted thoracoscopy

[33]

or laparoscopy

[34]

) are being explored. Putnam et al found that a minimally invasive approach was associated with a shorter hospital stay and reduced small-bowel obstruction but with higher recurrence rates.

[35]

Criss et al found that in low-risk patients born with small or moderate-sized defects, a thoracoscopic approach was associated with a shorter hospital stay, reduced mechanical ventilation days, and decreased time to feeding but also with a trend toward higher recurrence rates.

[36]

 Robotic approaches are being explored as well.

[37]

Fetoscopy

Some centers use intrauterine fetal surgery (fetoscopy) to repair diaphragmatic defects in an attempt to prevent the hypoplastic lung problems encountered with large hernias. [18]  (See Fetal Surgery for Congenital Diaphragmatic Hernia.)

Because the number of cases performed is still relatively small and very few centers are capable of performing intrauterine surgery, this practice has been controversial. A 2015 Cochrane review concluded that there was not sufficient evidence to recommend in-utero intervention for fetuses with CDH (including both maternal antenatal corticosteroid administration and fetoscopic tracheal occlusion) as a part of routine clinical practice. [26]  

Preterm rupture of membranes remains the weak link of fetoscopy. Refinement of the technique of fetoscopy and technologic advances such as partial amniotic carbon dioxide insufflation will help correct this problem and may allow the application of fetoscopy to other pathologies in the future. [38, 29, 28]

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Complications

It is extremely important to keep in mind that there is an increased risk of concomitant congenital defects in an infant with CDH. If a diaphragmatic hernia is diagnosed in utero, a high index of suspicion for concomitant abnormalities must be maintained. It should always be remembered that infants with CDH are at increased risk for postnatal respiratory failure, incarceration or strangulation of bowel, and pulmonary hypoplasia. [39]

In cases of traumatic or adult repaired CDH, recurrence of the hernia is possible; thus, follow-up with chest radiography is important.

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Long-Term Monitoring

Once an anatomic defect has been corrected, periodically assessing pulmonary function and obtaining chest radiographs is important. Although spontaneous recurrence of a repaired diaphragmatic hernia is low, small defects in the repair site have been reported; accordingly, surveillance is essential.

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