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A diaphragmatic hernia occurs when abdominal contents migrate into the thoracic cavity through a defect in the diaphragm. This condition may be congenital, resulting from developmental abnormalities, or acquired due to trauma or surgical complications. This course reviews the types of diaphragmatic hernia, including congenital diaphragmatic hernias, which frequently cause respiratory distress in neonates, with high morbidity and mortality despite medical advancements, and acquired diaphragmatic hernias, which vary in presentation, from subtle symptoms to severe complications like intestinal strangulation, requiring urgent surgical repair. Diagnostic imaging and treatment, typically including resuscitation, surgical intervention, and, in some cases, minimally invasive techniques or mesh reinforcement for large defects, are also discussed. Participants also gain an in-depth understanding of the clinical presentations and implementation of individualized surgical repair strategies. This activity for healthcare professionals is designed to enhance the learner's competence in identifying diaphragmatic hernias, performing the recommended evaluation, and implementing an appropriate interprofessional approach when managing this condition to improve outcomes. Objectives: Screen high-risk populations, including neonates and trauma patients, for potential diaphragmatic hernias using appropriate tools. Assess diagnostic imaging findings to confirm the severity of a diaphragmatic hernia. Implement resuscitation measures for patients presenting with severe clinical symptoms. Apply interprofessional team strategies to improve care coordination and outcomes in patients with diaphragmatic hernias. Access free multiple choice questions on this topic.
A diaphragmatic hernia is a protrusion of abdominal contents into the thoracic cavity due to a defect within the diaphragm. Diaphragmatic hernia is commonly a congenital phenomenon; however, acquired cases also occur.[1] The reported incidence of diaphragmatic hernia is approximately 0.8 to 5 out of 10,000 live births. Acquired diaphragmatic hernias typically occur following blunt or penetrating trauma, which results in a rupture of the diaphragm, accompanied by herniation of abdominal content; however, acquired diaphragmatic hernias can also occur spontaneously or due to iatrogenic causes.[2] Acquired diaphragmatic hernia is rare but can be life-threatening, resulting in bowel incarceration and strangulation with an overall mortality rate of up to 31%.[3] Congenital diaphragmatic hernia is a developmental defect of the diaphragm that results in the herniation of abdominal viscera into the chest cavity. Neonates with congenital diaphragmatic hernia usually present with respiratory distress early in the first few hours of life. The respiratory distress associated with the congenital diaphragmatic hernia may be mild. Occasionally, the accompanying respiratory distress is so severe that it can be life-threatening. With antenatal diagnosis and improved neonatal care, survival has been remarkably improved, but a notable risk of morbidity and mortality remains in infants with congenital diaphragmatic hernia.[4] Diaphragmatic injury is rare, complicating less than 1% of all patients with trauma.[5][6] However, the incidence includes up to 3% of all abdominal injuries.[7] Isolated diaphragmatic injuries are uncommon and are usually reported in the context of complicated abdominal and thoracic injuries. Notwithstanding the probability of an obvious herniated abdominal viscera in the thoracic cavity in the chest radiographs, the subtle cases should not be underestimated. Therefore, a high index of suspicion is strongly recommended to avoid the debilitating morbidity associated with delayed and missed diagnosis.[3][8]
Congenital Diaphragmatic Hernia The majority of congenital hernias are considered sporadic; however, research indicates that genetics may also be involved in some cases, with potential candidate genes identified.[9] Acquired Diaphragmatic Hernia The most common etiology of acquired diaphragmatic hernia is secondary to trauma, which results in diaphragmatic rupture. Acquired diaphragmatic hernia occurs more often from blunt trauma but can also result from penetrating thoracoabdominal trauma.[10] Diaphragmatic rupture from thoracoabdominal trauma occurs in approximately 0.8% to 3.6% of cases, with the incidence of herniation following such an event being relatively low.[10][11] Iatrogenic causes following surgery are the second most common cause of acquired diaphragmatic hernia; however, they are infrequent, primarily consisting of case studies in the literature. The most common surgeries with which diaphragmatic hernia has been reported occur following pediatric liver transplants and liver resection.[12][13] Other documented episodes of acquired diaphragmatic hernia postsurgery within case studies and systematic reviews include oesophagectomy, Nissen fundoplication, left colectomy, adrenalectomy, laparoscopy-assisted total gastrectomy, nephrectomy, and partial resection of the left lung using thoracoscopic surgery.[14][15][16][17][18][19][20][21] Spontaneous defects have also been noted; in these cases, they may remain asymptomatic for years before becoming symptomatic in the later stages, when the defects can be extensive.[2] Other rare causes documented within case studies include after radio-frequency ablation for hepatocellular carcinoma and during pregnancy.[22][23][24] According to a literature review, including more than 53,000 patients with blunt and penetrating abdominal trauma, diaphragm injury was reported in up to 3% of the cases. Moreover, the reports suggested a 2:1 ratio of penetrating versus blunt traumatic diaphragm injury in the studied population. The National Trauma Data Bank reported similar results (1% to 7% versus 10% to 15%).[7][25]
Congenital diaphragmatic hernia is a severe and uncommon congenital defect occurring at a rate of 2.3 per 10,000 live births. Congenital diaphragmatic hernia primarily arises from the incomplete development of the fetal diaphragm during pregnancy, allowing abdominal organs to herniate into the thoracic cavity, leading to abnormal lung development and persistent pulmonary hypertension.[26] Acquired diaphragmatic hernia is also rare. Diaphragmatic rupture from thoracoabdominal trauma occurs in approximately 0.8% to 3.6% of cases, with the incidence of herniation following this being relatively low.[10][11] The number of patients affected by acquired diaphragmatic hernia from other causes is not documented and is most commonly noted within case studies. Currently, no schema exists based on timing. However, most of the literature identifies an acute phase for the first 24 hours following injury and then describes patients presenting in the following 24 hours as delayed. If treatment is delayed, abdominal organs can herniate through the new opening into the thoracic cavity, leading to respiratory distress and ischemia of the affected organs. Traumatic diaphragmatic rupture, when not immediately identified, can present months or even years after the initial trauma. The diagnosis is often challenging due to nonspecific clinical symptoms and radiological signs.[27] Isolated diaphragmatic injuries are uncommon, typically occurring alongside other pathologies in 80% to 100% of cases. D’Souza et al observed intra-abdominal injuries in 77.9% of 127 cases involving penetrating diaphragmatic ruptures.[28] In one study's results, additional organ injuries were found in 86.7% of cases, with the lung being the most frequently affected extra-abdominal organ and the spleen the most common intra-abdominal organ.[29] The incidence of diaphragmatic hernia varies between blunt and penetrating traumas, with reported rates of 94% and 15.1%, respectively. Overall, a diaphragmatic hernia was present in 47.26% of diaphragmatic rupture cases, with the stomach and omentum being the most frequently herniated organs.[30]
Acquired Diaphragmatic Rupture An acquired diaphragmatic hernia due to trauma to the diaphragm is likely to occur at areas of potential weakness along embryological fusion points.[31] This condition is thought to be produced by a sudden increase in the pleuroperitoneal pressure gradient due to the trauma.[31] Multiple factors may result from iatrogenic causes depending on the type of surgery, patient-related factors, and technique used to close the diaphragm.[13] Acquired diaphragmatic hernia and diaphragmatic rupture tend to occur more commonly on the left side of the diaphragm, likely due to the right hemidiaphragm being protected by the size of the liver beneath it.[32] Right-sided ruptures and herniations are very rare and associated with higher mortality and morbidity rates.[31] The abdominal contents that can herniate into the diaphragm vary, but documented cases include herniation of the stomach, small intestine, mesentery, spleen, and pancreas.[3] Penetrating diaphragmatic trauma Anatomical landmarks of the diaphragm clarify the association between penetrating trauma and the potential for traumatic diaphragmatic injury. Anatomical differences during inspiration and expiration should also be considered. The diaphragm rises during expiration up to the fourth and fifth thoracic dermatomes on the right and the left hemithorax, respectively. However, the diaphragm descends to as low as the eighth thoracic dermatome during deep inspiration. The lowest level of the lateral costophrenic angle attachment is the twelfth rib. Therefore, any penetrating object that passes through the torso from T4 through T12 may injure the diaphragm. The higher rate of left traumatic diaphragmatic injury with stab wounds is presumably because of the higher prevalence of right-handed assailants.[33] Blunt diaphragmatic trauma
Anatomical landmarks of the diaphragm clarify the association between penetrating trauma and the potential for traumatic diaphragmatic injury. Anatomical differences during inspiration and expiration should also be considered. The diaphragm rises during expiration up to the fourth and fifth thoracic dermatomes on the right and the left hemithorax, respectively. However, the diaphragm descends to as low as the eighth thoracic dermatome during deep inspiration. The lowest level of the lateral costophrenic angle attachment is the twelfth rib. Therefore, any penetrating object that passes through the torso from T4 through T12 may injure the diaphragm. The higher rate of left traumatic diaphragmatic injury with stab wounds is presumably because of the higher prevalence of right-handed assailants.[33] Blunt diaphragmatic trauma Blunt diaphragmatic trauma from motor vehicle collisions is responsible for most blunt diaphragmatic ruptures in almost 90% of cases. Falling and crushing injuries imply the remaining etiologies of blunt diaphragmatic trauma. A significant increase in intra-abdominal pressure is required to diagnose diaphragmatic rupture in the aforementioned clinical scenarios.[34] During inspiration, with diaphragmatic contraction, the diaphragm flattens. The flattened diaphragm increases the thoracic cavity's volume; accordingly, the pleuroperitoneal pressure gradient can increase from +7 to +20 cm H2O in the supine position to +100 cm H2O. The blunt force trauma to the torso can result in pleuroperitoneal gradients of up to 200 cm H2O. The remarkable increase in the pleuroperitoneal gradient exceeds the diaphragmatic tensile strength, leading to diaphragmatic rupture or avulsion.[35] The diaphragm, a critical skeletal muscle for breathing, has a bidomed shape with dense collagen fibers arranged diagonally from the lateral to medial direction. The diaphragm's mechanical behavior under tension is comparable to the tensile stress-strain behavior of other skeletal muscles.
The remarkable increase in the pleuroperitoneal gradient exceeds the diaphragmatic tensile strength, leading to diaphragmatic rupture or avulsion.[35] The diaphragm, a critical skeletal muscle for breathing, has a bidomed shape with dense collagen fibers arranged diagonally from the lateral to medial direction. The diaphragm's mechanical behavior under tension is comparable to the tensile stress-strain behavior of other skeletal muscles. When at rest, the muscle fibers appear randomly oriented. However, under load, they align parallel to the direction of the tension. Initially, elastin fibers stretch linearly, but as the load increases, collagen fibers reorient to bear more load, occurring in the toe region of the stress-strain curve. With increased load, the muscle undergoes a transition from low to high stiffness, known as strain stiffening, during which overstretched fibers eventually rupture and fail.[36] Diaphragm ruptures occur about 10 times more frequently on the left side in cases of blunt trauma, primarily because the diaphragm's left medial and posterolateral sections are less robust during embryonic development. Additionally, the liver provides a protective barrier on the right side, thereby reducing the incidence of rupture.[37] Congenital Diaphragmatic Hernia The embryologic development of the diaphragm facilitates understanding of the pathophysiology of congenital diaphragmatic hernia. The diaphragm originates from the following 4 main structures: Septum transversum: The development of the septum initiates in the fourth gestational week to separate the pericardial and peritoneal cavities. Though initially located next to the cervical vertebrae, the septum transversum grows to the level of the first lumbar vertebrae. Subsequently, dorsal fusion with the esophageal mesentery and pleuroperitoneal membranes occurs. Esophageal mesentery: The esophageal mesentery similarly develops in the fourth gestational week and eventually forms the diaphragm's central portion and crura. Two paired pleuroperitoneal membranes: These develop later in the fifth week of gestation, parallel to the abdominal wall, with the later medial extension. Finally, pleuroperitoneal membranes will fuse with the dorsal borders of the esophageal mesentery and septum transversum.
Esophageal mesentery: The esophageal mesentery similarly develops in the fourth gestational week and eventually forms the diaphragm's central portion and crura. Two paired pleuroperitoneal membranes: These develop later in the fifth week of gestation, parallel to the abdominal wall, with the later medial extension. Finally, pleuroperitoneal membranes will fuse with the dorsal borders of the esophageal mesentery and septum transversum. Chest wall muscular structures: The complete fusion results in a discrete separation of the thoracic cavity from the abdominal cavity during the eighth gestational week.[38] Failure of these developmental steps and incomplete fusion with adjacent structures may result in congenital diaphragmatic hernia.[39]
Acquired Diaphragmatic Hernia Most acquired diaphragmatic hernia cases follow either blunt or penetrating trauma to the thorax or abdomen. A clear history of trauma should prompt appropriate imaging to determine the extent of the injury, which may then demonstrate the presence of an acquired diaphragmatic hernia. For other causes of acquired diaphragmatic hernia, consideration of the patient's prior surgical history is crucial, as these procedures may lead to a diaphragmatic hernia. Patients can present with acquired diaphragmatic hernia in a variety of ways. Respiratory, abdominal, and, in some cases, cardiac symptoms are predicted.[40] The pressure from abdominal organs in the thorax can cause shortness of breath and chest pain. Abdominal symptoms may also be present, including recurrent abdominal pain, postprandial fullness, vomiting, and obstructive gastrointestinal symptoms.[31] In some spontaneous hernia cases, a diaphragmatic hernia may remain asymptomatic and be detected only incidentally on imaging studies. Examination of the patient may demonstrate a scaphoid abdomen, absent breath sounds over the lower chest on the affected side, and bowel sounds over the lower chest on the affected side.[41] Congenital Diaphragmatic Hernia Congenital diaphragmatic hernia is characterized by a diaphragmatic muscle defect leading to a combination of global embryopathy and mechanical compression, resulting in a pathophysiologic triad of pulmonary hypertension, pulmonary hypoplasia, and cardiac dysfunction. These complex clinical challenges necessitate intensive critical care from the moment of birth. Advances in the management of congenital diaphragmatic hernia, including pressure-limited ventilation, extracorporeal life support, and neonatal critical care expertise, have significantly improved outcomes.
Congenital diaphragmatic hernia is characterized by a diaphragmatic muscle defect leading to a combination of global embryopathy and mechanical compression, resulting in a pathophysiologic triad of pulmonary hypertension, pulmonary hypoplasia, and cardiac dysfunction. These complex clinical challenges necessitate intensive critical care from the moment of birth. Advances in the management of congenital diaphragmatic hernia, including pressure-limited ventilation, extracorporeal life support, and neonatal critical care expertise, have significantly improved outcomes. Additionally, a deeper understanding of the underlying pathophysiology and the development of innovative interventions are opening new therapeutic possibilities. While overall survival rates for patients with congenital diaphragmatic hernia range from 65% to 80% and are influenced by various factors, the potential for further improvement may be realized through evidence-based, protocol-driven management strategies. However, congenital diaphragmatic hernia encompasses a spectrum of disease severity, and the most challenging, high-risk cases still have a survival rate of around 50%.[42] Patients may initially exhibit no symptoms following a diaphragmatic injury; however, over time, the defect can expand, potentially leading to significant herniation of abdominal organs.[43]
Congenital Diaphragmatic Hernia Diagnostic Studies The diagnostic measures and required evaluations for congenital diaphragmatic hernia are classified into prenatal and postnatal forms. Prenatal diagnosis in up to 60% of neonates with congenital diaphragmatic hernia is described by routine antenatal ultrasound screening during 18 to 22 weeks gestation. Nonspecific sonographic findings, including polyhydramnios or hydrops, are often detected on antenatal ultrasonography. However, the presence of abdominal organs in the fetal chest would characterize the diagnosis of congenital diaphragmatic hernia. Large diaphragmatic defects will optimize the diagnostic potential of the sonography.[44][45] Prenatal congenital diaphragmatic hernia Left-sided congenital diaphragmatic hernia is characterized by a heterogeneous prenatal lesion in the left hemithorax and right mediastinal shift. The heterogeneous mass implies the herniated intestines. The fluid within the mentioned mass and, more importantly, peristalsis assist in differentiating congenital diaphragmatic hernia from other prenatal intrathoracic lesions. The different diagnostic marker of congenital left-sided congenital diaphragmatic hernia is the absence of the stomach in the abdominal cavity. The displaced stomach is usually identified near the left pericardial border or the posterior cardiac space. A homogeneous, hypoechoic mass in the chest, close to the heart, suggests that Doppler sonography can further characterize the herniated liver and the intrahepatic vascular anatomy.[46] Right-sided congenital diaphragmatic hernia is typically suggested by a homogeneous mass in the right chest on imaging studies. The herniated liver may or may not cause the left mediastinal shift. The sonographic appearance of the liver is quite similar to that of the lungs and, therefore, may not serve as a reliable diagnostic marker.[47] The presence of pleural fluid and intestines is another potential characteristic of the right-sided congenital diaphragmatic hernia. The cardiac left shift is a reliable marker for differentiating the right-sided congenital diaphragmatic hernia. Postnatal congenital diaphragmatic hernia
Right-sided congenital diaphragmatic hernia is typically suggested by a homogeneous mass in the right chest on imaging studies. The herniated liver may or may not cause the left mediastinal shift. The sonographic appearance of the liver is quite similar to that of the lungs and, therefore, may not serve as a reliable diagnostic marker.[47] The presence of pleural fluid and intestines is another potential characteristic of the right-sided congenital diaphragmatic hernia. The cardiac left shift is a reliable marker for differentiating the right-sided congenital diaphragmatic hernia. Postnatal congenital diaphragmatic hernia Postnatal congenital diaphragmatic hernia should be excluded in any term infants presenting with symptoms of respiratory distress. Chest radiography indicating herniation of the abdominal organs, usually intestines, characterized by air- or fluid-containing structures, into either the right or left hemithorax with minimal visible aerated lung on the affected side, suggests the diagnosis of congenital diaphragmatic hernia.[48] Further evidence supporting the diagnosis of congenital diaphragmatic hernia includes the contralateral cardiac displacement, impression, and mass effect over the contralateral lung, and reduced abdominal girth. The diagnosis can be confirmed by a chest radiograph demonstrating the placement of a feeding tube within the thoracic cavity or mediastinal left shift in right-sided congenital diaphragmatic hernia. Occasionally, the herniated liver is the only sign suggesting a right-sided congenital diaphragmatic hernia; in such cases, a large right thoracic solid mass without the normal abdominal shadow supports the diagnosis.[49] Acquired Congenital Diaphragmatic Hernia Diagnostic Studies
Postnatal congenital diaphragmatic hernia should be excluded in any term infants presenting with symptoms of respiratory distress. Chest radiography indicating herniation of the abdominal organs, usually intestines, characterized by air- or fluid-containing structures, into either the right or left hemithorax with minimal visible aerated lung on the affected side, suggests the diagnosis of congenital diaphragmatic hernia.[48] Further evidence supporting the diagnosis of congenital diaphragmatic hernia includes the contralateral cardiac displacement, impression, and mass effect over the contralateral lung, and reduced abdominal girth. The diagnosis can be confirmed by a chest radiograph demonstrating the placement of a feeding tube within the thoracic cavity or mediastinal left shift in right-sided congenital diaphragmatic hernia. Occasionally, the herniated liver is the only sign suggesting a right-sided congenital diaphragmatic hernia; in such cases, a large right thoracic solid mass without the normal abdominal shadow supports the diagnosis.[49] Acquired Congenital Diaphragmatic Hernia Diagnostic Studies Diagnosis of an acquired diaphragmatic hernia relies on imaging studies. Various imaging modalities are used to diagnose or support the diagnosis of a diaphragmatic hernia, including chest radiographs, ultrasonography, and magnetic resonance imaging, with computed tomography (CT) being the modality of choice.[41] Diagnostic confirmation with laparoscopy or thoracoscopy is recommended in patients with equivocal diagnoses. Moreover, open surgical exploration has been introduced to diagnose highly suspicious cases with equivocal imaging results. Air fluid levels in the chest demonstrate pleural effusions, which may be present in patients presenting with delayed presentation of blunt trauma due to bleeding caused by rib fractures or their subsequent injury to the lung or complications from herniated abdominal organs.[50] In delayed presentation, the pleural effusion may be massive, leading to a mediastinal shift to the opposite side.
Diagnosis of an acquired diaphragmatic hernia relies on imaging studies. Various imaging modalities are used to diagnose or support the diagnosis of a diaphragmatic hernia, including chest radiographs, ultrasonography, and magnetic resonance imaging, with computed tomography (CT) being the modality of choice.[41] Diagnostic confirmation with laparoscopy or thoracoscopy is recommended in patients with equivocal diagnoses. Moreover, open surgical exploration has been introduced to diagnose highly suspicious cases with equivocal imaging results. Air fluid levels in the chest demonstrate pleural effusions, which may be present in patients presenting with delayed presentation of blunt trauma due to bleeding caused by rib fractures or their subsequent injury to the lung or complications from herniated abdominal organs.[50] In delayed presentation, the pleural effusion may be massive, leading to a mediastinal shift to the opposite side. An acquired variant of traumatic diaphragmatic hernia often occurs through a contusive mechanism, resulting in diaphragmatic strains or ruptures. The symptoms of this condition are diverse and depend on factors, including the hernia's location, the size of the defect, the abdominal organs involved, the degree of organ migration, and the presence of volvulus, ischemia, or obstruction. The clinical presentation is frequently mild, and the condition is discovered incidentally, with most digestive symptoms. Severe digestive complications (eg, strangulation, volvulus, and perforation) are rare but can lead to severe shock, typically following several nonspecific digestive symptoms. Diagnosis involves imaging techniques, including plain radiography (see Image. Acquired Diaphragmatic Hernia), contrast studies, CT scans (see Image. Traumatic Diaphragmatic Hernia), and endoscopic evaluations.[51]
Treatment of acquired diaphragmatic hernia in the acute setting requires appropriate patient resuscitation followed by surgical correction.[2] Traditionally, the procedure would be carried out using an open abdominal approach; however, with increased expertise in minimally invasive surgery for stable patients with isolated injury, a laparoscopic approach should be considered the preferred option.[8] In an unstable patient, an open repair approach should be undertaken. In trauma cases of a very unstable patient, consideration of damage control surgery instead of repair should be considered.[8] A few cases of robot-assisted repairs have been documented and could also be an option for a stable patient with an experienced surgeon and team.[52][53] The repair should be attempted with primary closure.[20] When primary closure with nonabsorbable sutures is not feasible due to the size of the defect, mesh repair may be an alternative.[31] If the diagnosis is delayed, a thoracic approach is generally preferred to reduce visceral-pleural adhesions and intra-thoracic visceral perforation.[31] In some delayed cases, a combined thoracic-abdominal approach may be appropriate. A laparoscopic approach may be feasible and an option for repair, depending upon experience.[20] Acquired Diaphragmatic Hernia For treatment of acquired diaphragmatic hernias following trauma, preoperative antibiotic therapy with first-generation cephalosporins is recommended. Moreover, in patients with high suspicion of synchronized intestinal trauma, antibiotic therapy is used to cover the anaerobes. Prophylactic antibiotic therapy should be repeated in patients with greater than 1500 mL of blood loss and in the uncommon circumstance of prolonged surgery lasting 2 to 3 hours.
For treatment of acquired diaphragmatic hernias following trauma, preoperative antibiotic therapy with first-generation cephalosporins is recommended. Moreover, in patients with high suspicion of synchronized intestinal trauma, antibiotic therapy is used to cover the anaerobes. Prophylactic antibiotic therapy should be repeated in patients with greater than 1500 mL of blood loss and in the uncommon circumstance of prolonged surgery lasting 2 to 3 hours. Following adequate primary resuscitation, if an open appoach is choosen a long-midline laparotomy incision from the xiphoid to the pubis is used. Primary evaluation of the 4-quadrant abdominal cavities and retroperitoneum should be undertaken. Bleeding and gastrointestinal spillage control should be prioritized. According to some literature reviews, in a selected group of patients with documented pure traumatic diaphragmatic injuries without other organ injuries, a primary upper midline incision might be performed. However, extending the incision for an appropriate exposure in equivocal circumstances after laparotomy should be considered.[54] Next, the bulk of the trimmed diaphragmatic edges is held with Allis clamps. The edges are retracted caudally to provide adequate, versatile exposure while avoiding accidental injury to intrathoracic organs. The thorax should be examined in its entirety to ensure a bloodless, clean field. In case of contamination, copious irrigation is recommended to avoid the risk of later empyema.[55] A thorough evaluation of the diaphragmatic surface should be included during the primary abdominal evaluation. Accordingly, the falciform ligament should be ligated and divided. Downward hepatic traction will improve assessment of the right hemidiaphragm. Additional exposure with mobilizing the liver from the coronary ligaments has not been described. Gastric and splenic traction should be performed to optimize visualization of the left hemidiaphragm. The diaphragmatic central tendon and adequate firm attachments to the lumbar vertebrae should be confirmed.
A thorough evaluation of the diaphragmatic surface should be included during the primary abdominal evaluation. Accordingly, the falciform ligament should be ligated and divided. Downward hepatic traction will improve assessment of the right hemidiaphragm. Additional exposure with mobilizing the liver from the coronary ligaments has not been described. Gastric and splenic traction should be performed to optimize visualization of the left hemidiaphragm. The diaphragmatic central tendon and adequate firm attachments to the lumbar vertebrae should be confirmed. In the presence of abdominal visceral herniation, gentle reduction to the abdominal cavity should be undertaken. In rare circumstances, splenic injury during the reduction could complicate the procedure. In these selected cases, a meticulous extension of the defect is recommended. Chronic herniations differ from acute traumatic cases, where a hernia sac and severe dense adhesions are predicted. The hernia sac should be dissected, followed by the abdominal visceral reduction. Any unintentional injury, including serosal injuries, should be repaired promptly. A transthoracic approach is traditionally used to address a diaphragmatic hernia with delayed presentation (see Image. Delayed Diaphragmatic Hernia). The classic approach via a seventh- or eighth-intercostal thoracotomy provides excellent visualization and access to the diaphragm. This approach facilitates the identification of diaphragmatic ruptures, the release of herniated abdominal organs from potential adhesions to the lung or chest wall, and their proper reduction back into the abdomen. Additionally, thoracotomy allows for the treatment of any obstructive complications, eg, necrosis or perforation. In more complex procedures, enlarging the diaphragmatic laceration can help reduce the amount of viscera in the abdomen.[56] The diaphragmatic repair is then undertaken with either permanent or absorbable sutures; repairs performed in running or interrupted fashion have been described in the literature.
Additionally, thoracotomy allows for the treatment of any obstructive complications, eg, necrosis or perforation. In more complex procedures, enlarging the diaphragmatic laceration can help reduce the amount of viscera in the abdomen.[56] The diaphragmatic repair is then undertaken with either permanent or absorbable sutures; repairs performed in running or interrupted fashion have been described in the literature. Using mesh repair should not be considered the standard repair method. However, in uncommon circumstances where the primary repair is not entirely feasible due to significant tissue loss, using a mesh might be considered. Nonabsorbable prosthetic materials, eg, polytetrafluoroethylene and polyethylene, can be used in these patients. In cases of gastrointestinal spillage or contamination, adequate abdominal irrigation and autologous flaps are recommended. The latter flaps are provided using either omental or latissimus dorsi flaps.[57] Congenital Diaphragmatic Hernia When an infant with a congenital diaphragmatic hernia is delivered, a few critical steps are necessary to stabilize the child and confirm the diagnosis. In these high-risk patients with significant morbidity and mortality, following a strict delivery protocol ensures the best possible outcome. This protocol includes early recognition, a risk-stratified approach, lung-protective ventilation, and early consideration of extracorporeal life support for patients who are suitable candidates. Study results suggest that planned delivery after 39 weeks of gestation at a high-volume tertiary center, whether by cesarean section or vaginal delivery, offers the best outcomes, with no difference in outcomes based on delivery method. After birth, initial stabilization involves measuring heart rate and pre- and post-ductal saturations. Immediate intubation is recommended for high-risk infants, though exceptions may be made for those with good predicted lung development and strong postnatal respiratory effort.[42]
When an infant with a congenital diaphragmatic hernia is delivered, a few critical steps are necessary to stabilize the child and confirm the diagnosis. In these high-risk patients with significant morbidity and mortality, following a strict delivery protocol ensures the best possible outcome. This protocol includes early recognition, a risk-stratified approach, lung-protective ventilation, and early consideration of extracorporeal life support for patients who are suitable candidates. Study results suggest that planned delivery after 39 weeks of gestation at a high-volume tertiary center, whether by cesarean section or vaginal delivery, offers the best outcomes, with no difference in outcomes based on delivery method. After birth, initial stabilization involves measuring heart rate and pre- and post-ductal saturations. Immediate intubation is recommended for high-risk infants, though exceptions may be made for those with good predicted lung development and strong postnatal respiratory effort.[42] Extracorporeal life support is an artificial method of supporting lung or heart function, allowing recovery from reversible respiratory issues; this is used in approximately 30% of infants with congenital diaphragmatic hernia. Infants who require extracorporeal life support are considered "high risk." Factors influencing extracorporeal life support candidacy include karyotype abnormalities, syndromic features, the presence of congenital diaphragmatic hernia, left and right ventricular function and proportions, clinical status and trajectory, liver herniation, observed-to-expected lung-to-head ratio, and percent predicted lung volume.[58]
Extracorporeal life support is an artificial method of supporting lung or heart function, allowing recovery from reversible respiratory issues; this is used in approximately 30% of infants with congenital diaphragmatic hernia. Infants who require extracorporeal life support are considered "high risk." Factors influencing extracorporeal life support candidacy include karyotype abnormalities, syndromic features, the presence of congenital diaphragmatic hernia, left and right ventricular function and proportions, clinical status and trajectory, liver herniation, observed-to-expected lung-to-head ratio, and percent predicted lung volume.[58] Understanding the estimated risk for infants with congenital diaphragmatic hernia both before and after birth allows families and the medical team to plan appropriately. This planning includes postnatal interventions like extracorporeal life support and, more recently, exploring antenatal interventions and novel pharmacologic treatments. Fetal lung development occurs in overlapping stages, and any disruption can hinder proper pulmonary vascular development, leading to the significant ventilation-perfusion mismatch seen in infants with congenital diaphragmatic hernia. The fetoscopic endoluminal tracheal occlusion procedure involves occluding the trachea to prevent the regression of pulmonary fluid, thereby forcing the pulmonary tissue and vasculature into a hyperplastic state. This promotes the growth and expansion of the alveoli and vasculature.[59]
A broad differential diagnosis for acquired diaphragmatic hernias should be considered, given the wide range of associated clinical features. However, before imaging is performed, a diaphragmatic hernia is unlikely to be high on the differential list for causation of the patient's symptoms, except perhaps in trauma cases. After imaging, an alternative diagnosis that should be considered is metastasis, particularly in the presence of minor defects.[40] Congenital causes of a diaphragmatic hernia should always be a consideration, particularly when trauma has not occurred before the presentation.[60]
If the clinician misses the diagnosis of acquired diaphragmatic hernia, then a substantial risk of death is present; this can be due to lung compression with respiratory failure, vascular compromise with gastric or intestinal infarction, or perforation.[61] A variable risk in surgical mortality has been documented for diaphragmatic repair, depending on concomitant injuries, between 5% and 50%.[2] Generally, the outcomes following correction are good, with low recurrence levels.[2] Research indicates that survival rates for isolated congenital diaphragmatic hernia cases are higher than those for cases accompanied by additional anomalies. Key factors influencing outcomes in congenital diaphragmatic hernia include: The presence of associated anomalies, particularly cardiac issues The degree of lung underdevelopment The liver's position Generally, the prognosis for isolated congenital diaphragmatic hernia is more favorable than that of congenital diaphragmatic hernia with multiple anomalies, as evidenced by population-based studies.[1] Pulmonary hypertension is a critical factor in the pathophysiology of congenital diaphragmatic hernia, characterized by sustained, abnormally high pulmonary arterial pressures. This condition leads to dysfunction within the pulmonary circulation, impaired gas exchange, and exacerbated cardiac dysfunction. In congenital diaphragmatic hernia, pulmonary hypertension is caused by abnormal prenatal development of the pulmonary vasculature, marked by hypertrophic smooth muscle cells, vessel thickening, and reduced angiogenesis. These changes result in elevated right heart pressures, circulatory shunting, poor ventilation, and decreased oxygenation after birth. Pulmonary hypertension is a critical element in congenital diaphragmatic hernia's pathophysiology and significantly influences disease outcomes, especially among high-risk patients. Study results have shown that 70% of infants born with congenital diaphragmatic hernia will develop pulmonary hypertension, and of these, 38% will ultimately require extracorporeal life support. This highlights the importance of pulmonary hypertension as a high-risk feature and its critical role in the management and outcomes of congenital diaphragmatic hernia.[62]
Many potential complications can result from an acquired diaphragmatic hernia. Complications reported include diaphragmatic rupture, acute obstructive symptoms, respiratory failure, incarceration, strangulation, and cardiac tamponade.[61][63] Delayed diagnosis would result in irreversible complications related to prolonged herniation. Accordingly, strangulation of abdominal viscera and life-threatening intestinal obstruction, perforation, and necrosis are predictable.
Most cases of acquired diaphragmatic hernia occur in emergencies following trauma; in these situations, surgery is life-saving. If possible, consent for surgery should be obtained with an explanation of the potential complications. These should be balanced with the complications, including death, if the surgery is not performed.
Acquired diaphragmatic hernia is a critical condition requiring urgent surgical intervention, and patient-centered care and optimal outcomes in its management depend heavily on an interprofessional approach. Given the importance of timely and accurate diagnosis, imaging expertise is vital, though it often proves challenging, particularly in patients with concurrent trauma. Effective communication and collaboration between radiologists and surgeons are essential in overcoming diagnostic hurdles, enabling surgeons to make well-informed decisions regarding the surgical approach. General surgeons typically manage these cases, but delayed diagnosis can require cardiothoracic surgical expertise, making interprofessional communication essential. Due to the rarity of acquired diaphragmatic hernias, case studies and shared surgical experiences play a crucial role in guiding complex management decisions. In cases involving neonates with high-risk congenital diaphragmatic hernias, interprofessional teams must engage in comprehensive prenatal imaging and risk stratification to provide families with clear and informed counseling. These neonates often face unpredictable clinical courses and elevated morbidity, necessitating coordinated care goals that adapt as the patient's needs evolve. To meet these demands, clinicians, nurses, pharmacists, and other healthcare professionals collaborate continuously, working together to ensure safety and patient-centered care. Such coordinated, team-based management is critical for optimizing outcomes, maintaining patient safety, and improving overall team performance in cases of acquired and congenital diaphragmatic hernia.