Bochdalek Hernia and Partial Diaphragmatic Agenesis: Pedicled Intercostal Muscle Flap and Mesh Repair in a Young

Klein Dantis1, Devendra Kumar Rathore2, Nilesh Gupta3

  • 1Department of Cardiothoracic Surgery, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India.

Surgery Journal (New York, N.Y.)
|December 30, 2021
PubMed

Insights

Congenital Bochdalek hernia (BH) is rare in adults, often presenting unusually. This case highlights a rare adult BH with partial diaphragmatic agenesis and sickle cell disease, a unique presentation not previously reported.

Area of Science:

  • Medical Science
  • Surgical Science
  • Pediatric Surgery

Background:

  • Congenital Bochdalek hernia (BH) is a rare condition, primarily affecting infants with an incidence of 1:3,000 live births.
  • Adult presentation of BH is exceptionally uncommon, with most cases diagnosed in the pediatric age group.
  • Surgical repair is the standard treatment, involving thoracic, abdominal, or thoraco-abdominal approaches, often with diaphragmatic reconstruction for agenesis.

Observation:

  • This report details a rare case of a congenital Bochdalek hernia in a young adult.
  • The patient presented with an unusual manifestation of BH, persisting asymptomatically into adulthood.
  • A significant comorbidity of sickle cell disease was noted in this adult BH patient.

Findings:

  • This case represents one of only 10 reported instances of BH with partial diaphragmatic agenesis.
  • The co-occurrence of adult BH, partial diaphragmatic agenesis, and sickle cell disease is unprecedented in medical literature.
  • The study discusses the rarity, atypical presentation, and management strategies for this complex clinical scenario.

Implications:

  • This case expands the understanding of congenital Bochdalek hernia presentations beyond infancy.
  • It underscores the importance of considering rare diagnoses in adult patients with atypical symptoms.
  • The findings may inform surgical approaches and management protocols for similar complex cases in the future.