A Twenty-year Review of Early and Late-presenting Congenital Bochdalek Diaphragmatic Hernia: Are They Different Clinical Spectra?
Article Outline
Background
Congenital Bochdalek diaphragmatic hernia (CDH) is easily recognized if it is present shortly after birth. However, cases of delayed CDH presentation are more subtle and therefore less easily diagnosed.
Methods
We retrospectively analyzed 85 patients who were under 18 and diagnosed with CDH between June 1987 and May 2007. Those diagnosed before 1 month of age were categorized as having early-presenting CDH and those diagnosed after 1 month were categorized as having late-presenting CDH.
Results
Of the 85 cases, 68 (80%) were early-presenting and 17 (20%) were late-presenting CDH. Respiratory symptoms were more common in early-presenting CDH, while gastrointestinal complaints were more dominant in the late-presenting group, particularly in patients with left diaphragmatic defects. Late-presenting CDH, which was more likely to be associated with chronic gastrointestinal symptoms, was associated with a lower mortality than acute early-presenting CDH with respiratory symptoms, despite the more prompt diagnosis of the latter. The small bowel was the organ most commonly herniated into the thorax. Congenital heart disease was the most commonly associated malformation, but the presence of associated anomalies did not increase mortality.
Conclusion
Early-presenting CDH shows acute symptoms, is readily diagnosed, and requires prompt intervention to prevent death. In contrast, late-presenting CDH shows more subtle symptoms and is more difficult to diagnose. Although the late-presenting CHD has better prognosis, it still requires surgery intervention to alleviate the symptoms. Clinicians must maintain a high index of suspicion for CDH, regardless of its presentation.
Key Words: Bochdalek , children , congenital diaphragmatic hernia , early-presenting , late-presenting
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References
- . Diaphragmatic hernia . In: Mustard WT , Ravitch MM , Snyder WH , et al. editor. Pediatric Surgery . Chicago: Year Book Medical; 1969;p. 342
- . The CDH Study Group and advances in the clinical care of the patient with congenital diaphragmatic hernia . Semin Perinatol . 2004;28:174–184
- . Congenital posterolateral diaphragmatic hernia past infancy . Am J Surg . 1976;131:748–754
- Hernias of the foramen of Bochdalek in children . Mayo Clin Proc . 1968;43:580–591
- . Congenital diaphragmatic hernia: two case studies with atypical presentations . Neonatal Netw . 2006;25:239–249
- . Outcomes of congenital diaphragmatic hernia: a population-based study in Western Australia . Pediatrics . 2005;116:e356–e363
- . Late-presenting congenital diaphragmatic hernia . J Pediatr Surg . 2005;40:1839–1843
- . Late-presenting congenital diaphragmatic hernia in children: a literature review . Pediatr Radiol . 2005;35:478–488
- . Late-presenting congenital diaphragmatic hernia in children: a clinical spectrum . Pediatr Surg Int . 2004;20:658–669
- . Bochdalek diaphragmatic hernia: not only a neonatal disease . Arch Dis Child . 2003;88:532–535
- . The late-presenting pediatric Bochdalek hernia: a 20-year review . J Pediatr Surg . 1988;23:735–739
- . Presentation of congenital diaphragmatic hernia past the neonatal period . Arch Surg . 1986;121:813–816
- . Delayed presentation of congenital diaphragmatic hernia . Pediatr Surg Int . 2002;18:480–485
- . Late presentation of Bochdalek hernia: clinical and radiological aspects . Pediatr Pulmonol . 2001;31:306–310
- . “Acquired” congenital diaphragmatic hernia . J Pediatr Surg . 1977;12:657–665
- Associated malformations in delayed presentation of congenital diaphragmatic hernia . J Pediatr Surg . 2004;39:1073–1076
- Birth weight and McGoon Index predict mortality in newborn infants with congenital diaphragmatic hernia . J Pediatr Surg . 2006;41:25–28
- Associated malformations in congenital diaphragmatic hernia cases in the last 15 years in a tertiary referral institute . Am J Med Genet A . 2006;140:2298–2304
PII: S1875-9572(10)60006-X
doi:10.1016/S1875-9572(10)60006-X
© 2010 Taiwan Pediatric Association. Published by Elsevier Inc. All rights reserved.
