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Congenital Diaphragmatic Hernia (CDH)

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Summary

This on-demand session led by Milind Chitnis, part of the International Paediatric Surgery Teaching Series 2023, delves deeply into the complexities of congenital diaphragmatic hernia. This medical professional teaching series addresses the management challenges, mortality and morbidity of this condition, whilst also providing thorough information on the different hernia types, embryological causes and pathophysiology. A prime focus is on antenatal and postnatal presentation and the differential diagnosis. Attendees will grasp the understanding of pre-op monitoring, ventilatory strategies, stabilization procedures, surgical management options, and possible long-term morbidities and sequalae. The session explores the role of minimally invasive surgery, diaphragmatic replacement options, risk stratification and fetal therapy. This comprehensive session highlights key takeaways including the importance of stabilisation and lung protective ventilation and emphasizes that surgical repair is not emergent. A must for those involved in pediatric surgery.

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Description

These slides will take you through Congenital Diaphragmatic Hernia (CDH) from an International Pediatric Surgery Teaching Series in 2023, presented by Milind Chitnis. It covers the following key areas:

Types and Embryology of CDH: CDH primarily presents as a posterolateral defect (Bochdalek hernia) in 75% of cases, followed by an antero-medial defect (Morgagni hernia) in 23%, and hiatus hernia in 2%. The condition arises from developmental defects in the closure of the pleuroperitoneal folds and muscularization, leading to lung hypoplasia.

Clinical Presentation and Diagnosis: CDH can be detected antenatally through ultrasound and MRI, and postnatally presents with respiratory distress, scaphoid abdomen, and mediastinal shift. Diagnostic imaging typically reveals hypoplastic lungs, stomach, and bowel shadows in the chest.

Preoperative Management: Effective preoperative strategies include monitoring overall perfusion, pulmonary hypertension, and cardiac function, alongside ventilatory support through pressure-controlled ventilation and permissive hypercarbia. Stabilization with minimal stimulation, fluid optimization, and possible use of ECMO are critical.

Surgical Management: Surgical repair, ideally performed after stabilization, may involve laparotomy or thoracotomy, with careful reduction of herniated contents and consideration for the use of synthetic patches for large defects. Minimally invasive surgery is an option for experts, with pros like less pain and shorter hospital stays but cons like higher recurrence rates.

Long-term Morbidity and Outcomes: Long-term complications can include pulmonary, neurodevelopmental, gastrointestinal, musculoskeletal, and surgical issues. Risk stratification is important, considering defect size, associated anomalies, and the volume of cases managed by the center. Despite advancements, CDH remains associated with high morbidity and mortality.

Learning objectives

  1. By the end of the session, learners should be able to explain the embryological development of congenital diaphragmatic hernia, including the development of the pleuroperitoneal folds and lung hypoplasia.
  2. Participants should be able to identify the presentation and diagnostic signs of congenital diaphragmatic hernia in both antenatal and postnatal patients, and differentiate between different types of hernias.
  3. Learners should be able to comprehend the preoperative monitoring measures for patients with congenital diaphragmatic hernia, and discuss the ventilatory strategies and stabilization techniques before surgery.
  4. After the teaching session, participants are expected to be proficient in understanding the surgical management of congenital diaphragmatic hernia, including the steps of open surgery, role of minimally invasive thoracoscopic surgery, and choice of diaphragmatic replacement materials.
  5. Learners are expected to reflect on the long-term morbidity and outcomes of congenital diaphragmatic hernia, opine on risk stratification, and discuss the potential role of fetal interventions in its management.
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Congenital diaphragmatic hernia Case to Case: International Paediatric Surgery Teaching Series 2023 Milind Chitnis Developmental defect Lung hypoplasia + pulmonary HT Introduction Management is still a challenge Significant mortality & morbidityCDH- types • Posterolateral defect (Bochdalek hernia) 75% • Antero-medial (Morgagni hernia) 23% • Hiatus hernia 2%Embryology of diaphragm CDH- Embryology • Failure of closure of pleuroperitoneal folds • Failure of muscularization • Lung hypoplasia as primary causeCDH- Embryology- of lung Bilateral lung hypoplasiaCDH- pathophysiologyCDH- presentation- antenatal- US & MRICDH- presentation- postnatal Respiratory distress +/- cyanosis Scaphoid abdomen ↓breath +/- bowel sounds in chest Heart shifted to rightCDH- investigations- diagnostic • Absent stomach bubble in abdomen • Stomach (NG tube) & • bowel shadows in chest • Mediastinal shift • Hypoplastic lungsCDH- differential diagnosis Overall perfusion & severity of pulmonary hypertension/hypoplasia Cardiac function CDH- pre-op Preductal oxygen saturation monitoring Acid–base balance Oxygenation–ventilation status Conventional ventilation- pressure controlled Prevention of barotrauma- gentle ventilation CDH- preop ventilatory Permissive hypercarbia strategies Avoid neuromuscular paralysis High frequency oscillatory ventilation Minimal environmental stimulation Fluid optimization Vasopressor agents Pre-op Surfactant- only for prematurity stabilisation Pulmonary vasodilators • iNO= no proven benefit • Sidenifil- promising ECMO Need for risk stratification Surgery after optimum stabilisation CDH- surgical management- Laparotomy v/s thoracotomy • Reduce contents carefully open surgery • Resect sac • Avoid respiratory compromise • ICD v/s no ICD • Need for patch * Pros: • Less postoperative pain CDH- role of • Shorter hospitalizations • Avoidance of thoracotomy-associated MIS- complications thoracoscopic- • Less chance of adhesive bowel obstruction for experts * Cons: only • CO₂ absorption • High recurrence rate * No standardisation of technique For large defects Synthetic patches • PTFE/ Gore-Tex Marlex ® Diaphragmatic Absorbable Biosynthetic Patches ® ®/ ® replacement • Surgisis (SIS)/ Permacol AlloDerm Dome/cone shaped patch Local muscle flaps Tissue engineered patches Defect size CDH- risk Associated congenital heart disease stratification Associated anomalies & outcomes Prematurity Volume of centres Pulmonary CDH- long Neurodevelopmental term Gastrointestinal morbidity Musculoskeletal & sequalae Surgical complications Open foetal surgery- abandoned CDH-foetal therapy endoscopic insertion of an occlusive balloon into the fetal trachea (FETO) - on trial basis onlyCDH- take home message •Stabilization and lung protective ventilation- key •Surgical repair is not an emergency •Foetal interventions, ECMO are costly, not easily available & don’t reduce mortality •High morbidity & mortality