Patient Presentation
A term female infant with prenatally diagnosed left congenital diaphragmatic hernia was born by normal spontaneous vaginal delivery.
She developed respiratory distress at the time of birth that was treated with nasopharyngeal continuous positive airway pressure.
On day of life 4 she underwent a repairlater of a Bochdalek-type congenital diaphragmatic hernia with opening of the left chest cavity. The procedure went well. On day of life 5, she had a chest x-ray that revealed some atelectesis.
She was intubated on day of life 6 for increased tachypnea and work of breathing.
A few hours her pertinent physical exam showed an intubated infant with a clean surgical scar. Her respiratory rate was 84, heart rate was in the 150-180s with normal blood pressure. She had a weight of 3450 grams that was increased from a birth weight of 3260 grams.
Her heart examination had a normal S1, S2 with no murmur. Her lung examination revealed decreased breath sounds on the left relative to the right side which had been equal prior.
She had some paradoxical breathing. The rest of her examination was normal.
The laboratory evaluation of a complete blood count, C-reactive protein and blood cultures were eventually normal. The radiologic evaluation of a chest radiograph showed a large left pleural effusion with a shift of the heart to the right side with no bowel gas in the chest. Thoracentesis revealed increased triglycerides and lymphocytes in the fluid.
The diagnosis of a chylothorax was made. A chest tube was placed for drainage and the infant was placed on formula containing medium chain fatty acids.
She stabilized and was doing well 2 days later.
Figure 78 – AP radiographs of the chest taken immediately after a left-sided congenital diaphragmatic hernia repair (above) and then 2 days later (below). The radiograph taken immediately post-operatively shows the hypoplastic left lung bud in the apex of the lung, and there is no pleural fluid. The radiograph taken 2 days later shows interval near-total opacification of the left hemithorax secondary to development of a large left pleural effusion. The aerated lung bud in the apex of the left lung is faintly seen. There is slight mediastinal shift to the right and the diaphragmatic hernia has not recurred.
Discussion
Congenital diaphragmatic hernia (CDH) occurs in 1 / 2000-3000 live births.
Its overall mortality has been hard to determine as many infants die in-utero or just after birth before transfer for surgical care.
One study found that ~61% of fetuses with CDH are live born. Many of these fetuses had other congenital anomalies.
Mortality after live birth is ~ 40-62%.
There are 3 major types of CDH:
- Bochdalek hernia which occurs posterolaterally
- Morgagni hernia which occurs anteriorly
- Hiatus hernia
Left sided Bochdalek hernias are the most common (85%). Treatment currently includes fetal surgical repair, post-natal surgical repair and critical care.
Learning Point
Cardiothoracic surgical repairs are critical for treatment of CDH and other congenital heart disease or congenital malformations. These surgeries themselves have their own associated mortalities and morbidities.
The European Association for Cardiothoracic Surgery and The Society of Thoracic Surgeons and other groups published a comprehensive listing of more than 2800 definitions of cardiothoracic surgical complications.
Below is a condensation of this vast listing:
- Arrhythmias requiring drug treatment, defibrillation, cardioversion, or pacemaker
- Cardiac
- Cardiac arrest, dysfunction or failure
- Endocarditis
- Pericardial effusion
- Pulmonary hypertension
- Pulmonary hypertensive crisis
- Pulmonary vein obstruction
- Systemic vein obstruction
- General
- Death
- Readmission
- Multiple organ system failure
- Neurological
- Neurological deficit – transient or persistent
- Paralyzed diaphragm
- Peripheral nerve injury
- Seizure
- Spinal cord injury
- Stroke
- Vocal cord dysfunction
- Operative/Procedures
- Bleeding requiring re-operation
- Sternum left open – planned or unplanned
- Unplanned cardiac re-operation or interventional procedure
- Mechanical support utilization
- Pulmonary
- Chylothorax
- Pleural effusion requiring drainage
- Pneumonia
- Pneumothorax
- Respiratory insufficiency requiring mechanical ventilation for > 7 Days, reintubation or tracheostomy
- Renal failure requiring dialysis
- Sepsis
- Wound
- Infection – superficial, deep, mediastinitis
- Dehiscence
- Other complications not listed in the exhaustive listing
Questions for Further Discussion
1. What are the potential complications of fetal surgeries?
2. How do various specialties work with parents of in-utero fetuses with potential congenital anomalies to plan prenatal and post-natal treatment in your region?
3. What surgical procedures are being performed using robotic surgery?
Related Cases
- Disease: Congential Diaphragmatic Hernia | Hernia | Birth Defects
- Symptom/Presentation: Genetic Disorder | Respiratory Distress
- Specialty: Cardiology / Cardiovascular-Thoracic Surgery | Critical Care | Neonatology | Surgery | Radiology / Nuclear Medicine / Radiation Oncology
- Age: Newborn
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: Hernia and Birth Defects.
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
Jacobs JP. Introduction – Databases and the assessment of complications associated with the treatment of patients with congenital cardiac disease.
Cardiology in the Young, 2008;18 (S2);1-37.
Part IV – the dictionary of definitions of complications associated with the treatment of patients with congenital cardiac disease.
Cardiology in the Young, 2008;18 (S2);282-530.
Steinhorn RH. Congenital Diaphragmatic Hernia. eMedicine.
Available from the Internet at http://emedicine.medscape.com/article/978118-overview (rev. 7/9/2009, cited 7/22/09).
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital
Pediatric Education 
