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Congenital diaphragmatic hernia

 

Congenital diaphragmatic hernia

Congenital diaphragmatic hernia
Morgagni hernia seen on a chest radiograph.
Classification and external resources
ICD-10 Q79.0
ICD-9-CM 756.6
OMIM 142340 222400
DiseasesDB 32489
MedlinePlus 001135
eMedicine ped/2603
MeSH D006548

Congenital diaphragmatic hernia (CDH) is a chest cavity, hindering proper lung formation.

CDH is a life-threatening pathology in infants, and a major cause of death due to two complications: pulmonary hypoplasia and pulmonary hypertension.[1] Experts disagree on the relative importance of these two conditions, with some focusing on hypoplasia, others on hypertension.[2] Newborns with CDH often have severe respiratory distress which can be life-threatening unless treated appropriately.

Contents

  • Types of congenital diaphragmatic hernia 1
    • Bochdalek hernia 1.1
    • Morgagni hernia 1.2
    • Diaphragm eventration 1.3
  • Pathophysiology 2
  • Morbidity and mortality 3
  • Presentation and diagnosis 4
  • Treatment 5
  • See also 6
  • References 7
  • External links 8

Types of congenital diaphragmatic hernia

Bochdalek hernia

The Bochdalek hernia, also known as a postero-lateral diaphragmatic hernia, is the most common manifestation of CDH, accounting for more than 95% of cases. In this instance the diaphragm abnormality is characterized by a hole in the postero-lateral corner of the diaphragm which allows passage of the abdominal viscera into the chest cavity. The majority of Bochdalek hernias (80-85%) occur on the left side of the diaphragm, a large proportion of the remaining cases occur on the right side. To date, it carries a high mortality and an active area of clinical research.

Morgagni hernia

CT of the chest demonstrates a Morgagni hernia (red arrow)

This rare anterior defect of the diaphragm is variably referred to as Morgagni, retrosternal, or parasternal hernia. Accounting for approximately 2% of all CDH cases, it is characterized by herniation through the xiphoid process of the sternum.[3]

Diaphragm eventration

The diagnosis of congenital diaphragmatic eventration is used when there is abnormal displacement (i.e. elevation) of part or all of an otherwise intact diaphragm into the chest cavity. This rare type of CDH occurs because in the region of eventration the diaphragm is thinner, allowing the abdominal viscera to protrude upwards.

Pathophysiology

It involves three major defects:

Morbidity and mortality

Congenital diaphragmatic hernia has a mortality rate of 40-62%,[4] outcomes being more favorable in the absence of other congenital abnormalities. Individual rates vary greatly dependent upon multiple factors; size of hernia, organs involved, additional birth defects or genetic problems, amount of lung growth, age and size at birth, type of treatments, timing of treatments, complications such as infections and lack of lung function.

Presentation and diagnosis

Congenital diaphragmatic hernia: coronal obstetric ultrasound (head to right of image, thorax centre, abdomen left) shows the stomach and heart both within the thorax.

This condition can often be diagnosed before birth and fetal intervention can sometimes help, depending on the severity of the condition.[5] Infants born with diaphragmatic hernia experience respiratory failure due to both pulmonary hypertension and pulmonary hypoplasia. The first condition is a restriction of blood flow through the lungs thought to be caused by defects in the lung. Pulmonary hypoplasia or decreased lung volume is directly related to the abdominal organs presence in the chest cavity which causes the lungs to be severely undersized, especially on the side of the hernia.

Survival rates for infants with this condition vary, but have generally been increasing through advances in neonatal medicine. Work has been done to correlate survival rates to ultrasound measurements of the lung volume as compared to the baby's head circumference. This figure known as the lung to head ratio (LHR).

A small percentage of cases go unrecognized into adulthood.[6]

Treatment

First step in management is orogastric tube placement and securing the airway (intubation). The baby will usually be immediately placed on a ventilator.

  • Jackson Fetal Therapy Institute. Jackson Memorial Hospital, Miami, FL. Congenital Diaphragmatic Hernia Treatment Center
  • CDH UK - A registered leading CDH charity in the United Kingdom offering information, advice and support to everyone affected by Congenital Diaphragmatic Hernia
  • DHREAMS (Diaphragmatic Hernia Research & Exploration; Advancing Molecular Science) Congential Diaphragmatic Hernia (CDH) Program at Columbia University Medical Center, New York
  • Congenital Diaphragmatic Hernia Program at Children's Hospital Boston
  • CHERUBS - The Association of Congenital Diaphragmatic Hernia Research, Awareness and Support, International Non-profit organization for families and medical professionals
  • GeneReviews: Congenital Diaphragmatic Hernia Overview
  • Congenital Diaphragmatic Hernia: The University of Michigan Fetal Diagnosis & Treatment Center
  • Congenital Diaphragmatic Hernia: The Center for Fetal Diagnosis and Treatment at The Children's Hospital of Philadelphia
  • Cincinnati Fetal Center: Congenital Diaphrahmatic Hernia / CDH
  • Fetal Care Institute, Cardinal Glennon Children's Medical Center, St Louis
  • Real Hope for CDH: Hope and Support for parents facing CDH
  • Hope, Patience, Trust: The Journey of having a baby with CDH
  • Global CDH - Aid for Congenital Diaphragmatic Hernia
  • CDH Rainbows - Informational website for those affected by the birth defect known as congenital diaphragmatic hernia

External links

  1. ^ Gaxiola A, Varon J, Valladolid G (April 2009). "Congenital diaphragmatic hernia: an overview of the etiology and current management". Acta Paediatrica (Oslo, Norway : 1992) 98 (4): 621–7.  
  2. ^ Migliazza L, Bellan C, Alberti D, Auriemma A, Burgio G, Locatelli G, Colombo A (September 2007). "Retrospective study of 111 cases of congenital diaphragmatic hernia treated with early high-frequency oscillatory ventilation and presurgical stabilization". Journal of Pediatric Surgery 42 (9): 1526–32.  
  3. ^ Arráez-Aybar, L. A., González-Gómez, C. C., & Torres-García, A. J. (2009). Morgagni-Larrey parasternal diaphragmatic hernia in the adult. Rev Esp Enferm Dig, 101(5), 357-366.
  4. ^ Pediatric Congenital Diaphragmatic Hernia at eMedicine
  5. ^ "Deadly hernia corrected in womb - Surgeons have developed an operation to repair a potentially fatal abnormality in babies before they are born.". BBC news. 2004-07-26.  - report of new operation, pioneered at London's King's College Hospital which reduced death rates in the most at risk by 50%
  6. ^ Swain F, Klaus A, Achem S, Hinder R (2001). "Congenital Diaphragmatic Hernia in Adults". Surgical Innovation 8 (4): 246–255.  
  7. ^ Tiruvoipati R, Vinogradova Y, Faulkner G, Sosnowski AW, Firmin RK, Peek GJ (2007). "Predictors of outcome in patients with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation". J. Pediatr. Surg. 42 (8): 1345–50.  
  8. ^ Logan JW, Rice HE, Goldberg RN, Cotten CM (2007). "Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies". Journal of perinatology : official journal of the California Perinatal Association 27 (9): 535–49.  
  9. ^ personal experience and talking with doctors, nurses, and surgeons at Primary Children's Medical Center in Salt Lake City, UT
  10. ^ Becmeur F, Talon I, Schaarschmidt K; et al. (2005). "Thoracoscopic diaphragmatic eventration repair in children: about 10 cases". J. Pediatr. Surg. 40 (11): 1712–5.  

References

Breath of Hope

See also

Diaphragm eventration is typically repaired thoracoscopically, by a technique called plication of the diaphragm.[10] Plication basically involves a folding of the eventrated diaphragm which is then sutured in order to “take up the slack” of the excess diaphragm tissue.

[9]

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