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Hepatopulmonary fusion in congenital diaphragmatic hernia: successful management of a lethal variant
  1. Revathy Menon1,
  2. Rahul Saxena1,
  3. Manish Pathak1 and
  4. Taruna Yadav2
  1. 1 Paediatric Surgery, AIIMS Jodhpur, Jodhpur, Rajasthan, India
  2. 2 Diagnostic and Interventional Radiology, All India Institute of Medical Sciences Jodphur, Jodhpur, Rajasthan, India
  1. Correspondence to Dr Rahul Saxena; drrahulsaxena{at}gmail.com

Abstract

Congenital diaphragmatic hernia (CDH) is a congenital anomaly involving the herniation of intra-abdominal contents into the thoracic cavity. Hepatopulmonary fusion (HPF), an exceedingly rare subtype mainly associated with right-sided CDH, presents unique diagnostic and therapeutic challenges. This case report describes a male infant with right-sided CDH complicated by HPF. The intricate anatomical anomaly involved the fusion of the right lung to the liver, posing challenges during surgical separation. The patient experienced postoperative complications, including prolonged ventilation, tracheostomy and pulmonary issues, which led to a prolonged hospital stay. Intraoperative challenges stem from the absence of demarcation between lung and liver tissues and abnormal vascular structures. In summary, managing HPF in right-sided CDH necessitates a customised, multidisciplinary approach to optimise patient outcomes, highlighting the need for ongoing research to refine understanding and treatment strategies.

  • Paediatric intensive care
  • Congenital disorders
  • Paediatric Surgery

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Footnotes

  • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: RM, RS, TY and MP. The following authors gave final approval of the manuscript: RM, RS, TY and MP.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.