Congenital Diaphragmatic Hernia Overview
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Questions and Answers

What is the most common location for congenital diaphragmatic hernia?

  • Left sided (correct)
  • Morgagni CDH
  • Bilateral
  • Right sided

    • Which embryonic element does NOT contribute to the formation of the diaphragm?

  • Esophageal mesentery
  • Septum transversum
  • Pleuroperitoneal folds
  • Lung buds (correct)

    • What percentage of congenital diaphragmatic hernia cases are associated with other anomalies?

  • 30%
  • 25%
  • 50%
  • 40% (correct)

    • At what gestational week is the fusion of diaphragm components completed?

    <p>7-8 weeks</p> Signup and view all the answers

    Which type of congenital diaphragmatic hernia accounts for 90% of cases?

    <p>Bochdalek CDH</p> Signup and view all the answers

    What is the estimated incidence rate of congenital diaphragmatic hernia?

    <p>1 in 2000 live births</p> Signup and view all the answers

    Congenital diaphragmatic hernia is more commonly observed in which gender?

    <p>Male infants</p> Signup and view all the answers

    Which anomaly is NOT typically associated with congenital diaphragmatic hernia?

    <p>Dermatological anomalies</p> Signup and view all the answers

    What parameter indicates evidence of good tissue perfusion after stabilization?

    <p>Resolution of cyanosis</p> Signup and view all the answers

    Which surgical approach is most commonly used for repairing congenital diaphragmatic hernia?

    <p>Laparotomy</p> Signup and view all the answers

    What occurs during inspiration in a normally functioning diaphragm?

    <p>The diaphragm contracts downward.</p> Signup and view all the answers

    What is the primary feature seen in the diagnosis of Morgagni hernia on a chest radiograph?

    <p>Bowel loops in the retrosternal space</p> Signup and view all the answers

    What is the primary cause of congenital diaphragmatic eventration?

    <p>Incomplete development of the diaphragm.</p> Signup and view all the answers

    Which symptom is commonly associated with Morgagni hernia in children?

    <p>Recurrent respiratory infections</p> Signup and view all the answers

    Which symptom is NOT commonly associated with diaphragmatic eventration in children?

    <p>Persistent fetal circulation.</p> Signup and view all the answers

    What type of tissue repair may be used if the diaphragmatic defect is large?

    <p>Prosthetic mesh</p> Signup and view all the answers

    Which of the following statements about diaphragmatic eventration is true?

    <p>It results in abnormal diaphragm movement during respiration.</p> Signup and view all the answers

    Which imaging technique is primarily used to confirm diaphragmatic eventration?

    <p>Fluoroscopy.</p> Signup and view all the answers

    What surgical procedure is indicated for a large diaphragmatic eventration causing pulmonary impairment?

    <p>Diaphragmatic plication.</p> Signup and view all the answers

    What is the treatment method for Morgagni hernia?

    <p>Operative repair through open or minimally invasive approach</p> Signup and view all the answers

    In which scenario would a hernia sac most likely be present?

    <p>Congenital diaphragmatic hernia</p> Signup and view all the answers

    In which scenario might minimal symptoms of diaphragmatic eventration be observed?

    <p>In small eventration cases.</p> Signup and view all the answers

    What is a common feature of acquired diaphragmatic eventration?

    <p>Injury to the phrenic nerve.</p> Signup and view all the answers

    What result can occur from large congenital diaphragmatic eventration?

    <p>Lung development interference.</p> Signup and view all the answers

    What initial management step is crucial in the resuscitation of newborns with congenital diaphragmatic hernia?

    <p>Resuscitation and stabilization</p> Signup and view all the answers

    Which laboratory test is primarily used to evaluate acid-base status in congenital diaphragmatic hernia?

    <p>Blood gas analysis</p> Signup and view all the answers

    What is a common finding on a chest radiograph that confirms the diagnosis of congenital diaphragmatic hernia?

    <p>Mediastinal shift toward the contralateral side</p> Signup and view all the answers

    Which ventilation method should be avoided in the initial management of congenital diaphragmatic hernia due to its risk of gastric distention?

    <p>Mask and bag ventilation</p> Signup and view all the answers

    What monitoring device is crucial for assessing fluid resuscitation in newborns with respiratory distress from congenital diaphragmatic hernia?

    <p>Urinary catheter</p> Signup and view all the answers

    What is the primary purpose of administering isotonic water-soluble contrast enema in newborns with simple meconium ileus?

    <p>To facilitate meconium evacuation</p> Signup and view all the answers

    What should be administered through the nasogastric tube to help dissolve thick secretions in the upper gastrointestinal tract?

    <p>N-acetylcysteine</p> Signup and view all the answers

    What is a significant risk associated with using high pressure in conventional ventilation for treating congenital diaphragmatic hernia?

    <p>Barotrauma</p> Signup and view all the answers

    Administration of which treatment is indicated for patients with extreme prematurity and congenital diaphragmatic hernia?

    <p>Surfactant therapy</p> Signup and view all the answers

    Which of the following indicates the need for operative intervention in simple meconium ileus?

    <p>Persistent abdominal distension after two non-operative attempts</p> Signup and view all the answers

    What is a common postoperative intervention following surgery for either simple or complicated meconium ileus?

    <p>Instillation of N-acetylcysteine through nasogastric tube</p> Signup and view all the answers

    What are the targets for adequate resuscitation in terms of blood gas levels for patients with congenital diaphragmatic hernia?

    <p>PaO2 &gt; 85 mmHg, PaCO2 &lt; 70 mmHg</p> Signup and view all the answers

    What is the recommended initial course of action for managing simple meconium ileus?

    <p>Non-operative management with isotonic water-soluble contrast enema</p> Signup and view all the answers

    What percentage range is reported for the mortality rate of infants with meconium ileus?

    <p>50-67%</p> Signup and view all the answers

    Which surgical procedure is often created after evacuation of tenacious meconium in cases of surgical management?

    <p>Ileostomy</p> Signup and view all the answers

    What complication might necessitate surgical intervention due to non-operative treatment failure?

    <p>Bowel perforation</p> Signup and view all the answers

    What is a primary cause of congenital diaphragmatic hernia?

    <p>Failure of the pleuroperitoneal fold to close</p> Signup and view all the answers

    Which of the following is NOT a characteristic of pulmonary hypoplasia associated with congenital diaphragmatic hernia?

    <p>Increased airway branching</p> Signup and view all the answers

    What clinical feature is commonly observed in newborns with congenital diaphragmatic hernia?

    <p>Signs of respiratory distress</p> Signup and view all the answers

    Which of the following could lead to pulmonary hypertension in congenital diaphragmatic hernia?

    <p>Pulmonary vasoconstriction due to hypoxia</p> Signup and view all the answers

    Which of the following findings on physical examination is indicative of congenital diaphragmatic hernia?

    <p>Scaphoid abdomen</p> Signup and view all the answers

    What is a potential consequence of a right-to-left shunt in congenital diaphragmatic hernia?

    <p>Deoxygenated blood entering systemic circulation</p> Signup and view all the answers

    Which symptom may present beyond the neonatal period in some cases of congenital diaphragmatic hernia?

    <p>Intestinal obstruction</p> Signup and view all the answers

    How might the mediastinum be affected in cases of congenital diaphragmatic hernia?

    <p>Shifted to the contralateral side</p> Signup and view all the answers

    Study Notes

    Congenital Diaphragmatic Hernia (CDH)

    • CDH is a developmental defect in the diaphragm, allowing abdominal organs to enter the chest cavity
    • Bochdalek hernias (90% of cases) are posterolateral defects, often left-sided (80%)
    • Morgagni hernias (10% of cases) are anterior defects
    • Incidence rate is approximately 1/2000 live births
    • More common in males
    • Associated with other anomalies in about 40% of cases (cardiovascular, urogenital, musculoskeletal, neurological, and chromosomal)

    Etiology & Pathophysiology of CDH

    • Diaphragm develops in the 4th week of gestation, involving the septum transversum, esophagus mesentery, and pleuroperitoneal folds
    • Failure in the fusion of these components can lead to a diaphragmatic defect
    • Herniated organs compress the lung, causing pulmonary hypoplasia
    • Results in decreased airway branching and surfactant deficiency, leading to pulmonary hypertension

    Clinical Features of CDH

    • Newborns typically show respiratory distress and cyanosis (some delay is possible)
    • Physical findings include tachypnea, chest wall retraction, grunting, and cyanosis
    • Scaphoid (sunken) abdomen, increased anteroposterior chest diameter (barrel chest), decreased breath sounds bilaterally, and shifted cardiac impulse
    • Intestinal obstruction/volvulus might be complications
    • Symptoms can present beyond the neonatal period with respiratory infections, feeding intolerance

    Investigation of CDH

    • Blood gas analysis to assess PH, PaO2, PaCO2, acid-base status (hypoxemia, hypercapnia, respiratory acidosis)
    • Serum lactate (elevated due to tissue hypoxia)
    • Chest X-ray to show bowel loops in the hemithorax, mediastinal shift, minimal bowel gases, and nasogastric tube in thoracic cavity
    • Echocardiography to assess ventricular contractility, pulmonary hypertension, right-to-left shunting, and associated cardiac defects
    • Renal ultrasound, cranial ultrasound, MRI, chromosomal studies to rule out other associated anomalies

    Management of CDH

    • Resuscitation and stabilization (oxygen, nasogastric tube, IV fluids, arterial and venous access)
    • Surfactant therapy (prematurity)
    • Ventilatory support (hypoxemia/hypercapnia)
    • Endotracheal intubation and conventional ventilation (pressure-controlled mode, maximum safe pressure of 25cm H2O)
    • HFOV or ECMO to correct hypoxemia/hypercapnia if ventilation fails
    • PaO2 > 85 mmHg, PaCO2 < 70 mmHg, pH > 7.2, evidence of good tissue perfusion (absence of cyanosis, good urine output, serum lactate < 3mmol/L)
    • Surgical repair (open or minimally invasive)
    • Reduction of herniated viscera, excision of hernia sac, and closure of the defect

    Morgagni Hernia

    • Arise from failure of fusion of sternocostal diaphragm.
    • Less common (around <2% of CDH).
    • Typically involves the right side.
    • Contains stomach, omentum, small intestine, and colon.
    • Presents in childhood with recurrent respiratory infections, epigastric discomfort, vomiting, and sometimes intestinal obstruction

    Meconium Ileus

    • Ileal obstruction due to extremely thick meconium.
    • Frequently the first symptom of cystic fibrosis (16% of CF patients)
    • Simple or complicated,
    • Simple usually resolves with enema/contrast enema
    • Complicated cases need surgical intervention
    • Common causes of intestinal obstruction in newborns
    • Incidence of 1/2000 live births

    Diaphragmatic Eventration

    • Diaphragmatic eventration: is abnormal paradoxical movement of diaphragm during respiration.
    • Congenital: incomplete diaphragm development
    • Acquired: Injury to phrenic nerve
    • Signs: elevated hemidiaphragm, paradoxical movement noted on fluoroscopy, possibly respiratory distress

    Surgical Management of Meconium Ileus

    • In simple cases, enema treatment sometimes works
    • In complicated cases, surgical intervention
    • Evacuation of meconium, bowel resection/closure
    • Ileostomy is usually created and closed after 4-6 weeks post-surgery
    • N-acetylcysteine can be used to dissolve meconium

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    Congenital Diaphragmatic Hernia - PDF

    Description

    This quiz provides an in-depth overview of Congenital Diaphragmatic Hernia (CDH), including its etiology, pathophysiology, and clinical features. It covers the different types of hernias, their incidence, and associated anomalies in newborns. Test your knowledge and understanding of this critical condition affecting neonates.

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