eMedicine Specialties > Pediatrics: Surgery > General Surgery

Diaphragmatic Hernias: Follow-up

Author: Nicola Lewis, MBBS, FRCS, Specialist Registrar, Department of Surgery, Birmingham Children's Hospital, UK
Coauthor(s): Philip Glick, MD, MBA, Professor, Departments of Surgery, Pediatrics, and Gynecology and Obstetrics, Vice-Chairperson for Research and Development, Department of Surgery, State University of New York at Buffalo
Contributor Information and Disclosures

Updated: Oct 27, 2008

Outcome and Prognosis

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 patients who survive congenital diaphragmatic hernias (CDHs).
  • Failure to thrive is often observed in the presence of optimal feeding regimes.
  • Functional and anatomic esophageal abnormalities are associated with significant gastroesophageal reflux in 40% of survivors; less than half of these patients require antireflux surgery in the first 6 months of life.9
  • The use of extracorporeal membrane oxygenation (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

Liquid ventilation uses perfluorocarbon (PFC), 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 PFC to the functional residual capacity, and conventional ventilation is superimposed. PLV is associated with improved oxygenation and decreased peak inspiratory pressure (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-induced and hyperoxia-induced pulmonary injury associated with congenital diaphragmatic hernia (CDH).

Preliminary clinical trials were conducted on infants with congenital diaphragmatic hernias 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 H2 O. 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 congenital diaphragmatic hernia 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 using minimal access surgery (see Media file 5). 

The immature lung in fetuses with congenital diaphragmatic hernia 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. Clinical trials using late prenatal steroids have failed to demonstrate improved survival, length of stay, and duration of ventilation.27

Thoracoscopic repair of congenital diaphragmatic hernia 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 or delayed presentation are more likely to undergo a successful thoracoscopic repair.28

 


More on Diaphragmatic Hernias

Overview: Diaphragmatic Hernias
Workup: Diaphragmatic Hernias
Treatment: Diaphragmatic Hernias
Follow-up: Diaphragmatic Hernias
Multimedia: Diaphragmatic Hernias
References
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References

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Further Reading

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Keywords

congenital diaphragmatic hernia, CDH, posterolateral diaphragmatic hernia, Bochdalek hernia, retrosternal hernia, Morgagni's hernia, respiratory distress, pulmonary hypoplasia, pulmonary hypertension, pulmonary immaturity, neural tube defects, polyhydramnios, hydrops fetalis, cystic adenomatoid malformation, cystic teratoma, thymic cysts, foregut duplication cyst, neurogenic tumors, feeding intolerance, tachycardia, intestinal obstruction, bowel ischemia, necrosis, volvulus, ventricular hypoplasia, atrial septal defects, ventricular septal defects, metabolic acidosis, persistent-newborn pulmonary hypertension

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Contributor Information and Disclosures

Author

Nicola Lewis, MBBS, FRCS, Specialist Registrar, Department of Surgery, Birmingham Children's Hospital, UK
Disclosure: Nothing to disclose.

Coauthor(s)

Philip Glick, MD, MBA, Professor, Departments of Surgery, Pediatrics, and Gynecology and Obstetrics, Vice-Chairperson for Research and Development, Department of Surgery, State University of New York at Buffalo
Philip Glick, MD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Thoracic Society, Association for Academic Surgery, Association for Surgical Education, Central Surgical Association, Federation of American Societies for Experimental Biology, Medical Society of the State of New York, Phi Beta Kappa, Physicians for Social Responsibility, Royal College of Surgeons of England, Sigma Xi, Society for Pediatric Research, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, and Society of University Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Robert K Minkes, MD, PhD, Professor of Surgery, University of Texas Southwestern; Chief of Surgical Services, Children's Medical Center of Dallas-Legacy
Robert K Minkes, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Andre Hebra, MD, Chief, Division of Pediatric Surgery, Medical University of South Carolina; Professor of Surgery and Pediatrics, Medical University of South Carolina
Andre Hebra, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, Association for Academic Surgery, Society of Laparoendoscopic Surgeons, South Carolina Medical Association, Southeastern Surgical Congress, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

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