Congenital Diaphragmatic Hernia

Chapter 84 Congenital Diaphragmatic Hernia



T.A. Rothenbach, MD, A. Alfred Chahine, MD



INTRODUCTION


Congenital diaphragmatic hernias (CDHs) occur in approximately 1 out of every 2500 live births. The true incidence is likely higher because some fetuses do not survive to birth. The two most common types of CDH are Bochdalek (posterolateral) and Morgagni (anterior). The development of the diaphragm defect is first noticeable in the 8th week of gestation. Bochdalek-type defects tend to occur more commonly on the left than the right. The actual pathophysiology of the CDH lies not in the physical defect, but in the resultant pulmonary hypoplasia and hypertension that occur. The exact mechanism of these physiologic responses to the CDH remains poorly understood.


CDH can be diagnosed by fetal sonogram. Once diagnosed, a work-up should ensue to rule out concomitant anomalies. This should include a chromosomal analysis as well as evaluation of the cardiac, gastrointestinal, and genitourinary systems.


Most infants present shortly after birth with respiratory distress. Immediate care includes endotracheal intubation and placement of a nasogastric tube and central access. Diagnosis is confirmed by plain film. Depending on the degree of physiologic compromise caused by the pulmonary hypoplasia and hypertension, interventions such as nitric oxide administration, high-frequency oscillating ventilation, and extracorporeal membrane oxygenation (ECMO) may be necessary.


Timing for repair is determined by the infant’s physiologic condition and response to interventions. As previously stated, the pulmonary hypertension and degree of pulmonary hypoplasia—not the presence of abdominal viscera in the chest—are the source of physiologic derangement. These realizations have led to a shift from urgent repair of CDH to delayed repair when the infant is stable from a pulmonary hypertension standpoint. The timing of repair of infants who require ECMO intervention remains controversial.


The standard approach for repairing a CDH in neonates remains the open approach. Laparoscopic and thoracoscopic repairs have been reported but have been fraught with high conversion rates, long operative times, and high recurrence rates.1–4 Yang and coworkers2 suggested anatomic (presence of the stomach in the abdomen) and physiologic (minimal ventilator requirements and no evidence of pulmonary hypertension) criteria for successful thoracoscopic repair in neonates.



INDICATION



• Presence of a CDH


OPERATIVE STEPS



Step 1 Infant is placed on operating table in supine position under warming lights with small roll placed under infant’s flank on affected side

Step 2 Prepare patient to have access to both thoracic cavities

Step 3 Gentle reduction of abdominal contents from chest to allow visualization of diaphragmatic defect

Step 4 Inspection for extralobar pulmonary sequestration

Step 5 Inspection for, and excision of, hernia sac

Step 6 Creation of tension-free closure

Step 7 Placement of chest tube

Step 8 Monitor pulmonary tidal volume while closing abdominal wall


OPERATIVE PROCEDURE



Anesthetic Preparation


Small, critically ill patients require close monitoring throughout the procedure. Appropriate presurgical preparation includes increasing the temperature in the operating room, placing overhead warming lights on the infant, placing an arterial line for monitoring of preductal blood gases, nasogastric tube placement, and Foley catheter placement.



Skin Preparation of Both Thoracic Cavities


Occasionally, these infants may develop a contralateral pneumothorax, severely compromising pulmonary function. This will require rapid placement of the chest tube on the contralateral thorax.



Gentle Reduction of the Abdominal Viscera from the Thoracic Cavity


After making a left subcostal incision, gentle downward retraction is placed on the viscera in the thoracic cavity.



Injury to Solid Organs or Hollow Viscus Organ



• Consequence



Inappropriate retraction of the small or large intestine can lead to intramural hematomas. More commonly, aggressive reduction of the liver and spleen can lead to hemorrhage.

Grade 2–4 complication


• Repair



If hematomas of small and large bowel are identified, they should be closely inspected. If there is concern for a full-thickness injury to the involved intestine, resection should be carried out with a primary anastomosis in cases in which the infant is stable. The larger concern is for potential injury to the liver and spleen. When encountered, control of liver bleeding in a neonate is best achieved with compression of the liver and topical hemostatic agents. Further interventions are often more harmful than helpful. Initial management of splenic or renal lacerations should be handled in a similar fashion.


• Prevention



Gentle distraction of the bowel, either manually or with the use of atraumatic forceps, will help prevent bowel or mesenteric injury. When reducing the stomach, care must be taken to avoid excessive stretch on the short gastric vessels. With careful manual reduction of the solid viscera, injury can usually be avoided. In order to maintain the intra-abdominal position of each organ as it is reduced, placement of laparotomy pads under retractors can be helpful in avoiding solid organ injury.

Related posts:

  1. Isolated Limb Perfusions and Extremity Amputations
  2. Lateral Pancreaticojejunostomy (Puestow) Procedure
  3. Left Colectomy: Open and Laparoscopic
  4. Bronchial and Vascular Sleeve Lobectomy

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Jun 21, 2017 | Posted by in GENERAL SURGERY | Comments Off on Congenital Diaphragmatic Hernia

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