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 (D)</p> Signup and view all the answers

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

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

What is the estimated incidence rate of congenital diaphragmatic hernia?

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

Congenital diaphragmatic hernia is more commonly observed in which gender?

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

Which anomaly is NOT typically associated with congenital diaphragmatic hernia?

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

What parameter indicates evidence of good tissue perfusion after stabilization?

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

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

<p>Laparotomy (D)</p> Signup and view all the answers

What occurs during inspiration in a normally functioning diaphragm?

<p>The diaphragm contracts downward. (D)</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 (B)</p> Signup and view all the answers

What is the primary cause of congenital diaphragmatic eventration?

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

Which symptom is commonly associated with Morgagni hernia in children?

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

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

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

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

<p>Prosthetic mesh (B)</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. (B)</p> Signup and view all the answers

Which imaging technique is primarily used to confirm diaphragmatic eventration?

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

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

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

What is the treatment method for Morgagni hernia?

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

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

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

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

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

What is a common feature of acquired diaphragmatic eventration?

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

What result can occur from large congenital diaphragmatic eventration?

<p>Lung development interference. (C)</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 (D)</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 (A)</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 (D)</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 (A)</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 (A)</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 (D)</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 (B)</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 (D)</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 (B)</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 (D)</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 (D)</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 (D)</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 (B)</p> Signup and view all the answers

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

<p>50-67% (C)</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 (D)</p> Signup and view all the answers

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

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

What is a primary cause of congenital diaphragmatic hernia?

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

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

<p>Signs of respiratory distress (C)</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 (A)</p> Signup and view all the answers

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

<p>Scaphoid abdomen (A)</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 (B)</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 (B)</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 (C)</p> Signup and view all the answers

Flashcards

Congenital Diaphragmatic Hernia (CDH)

A condition where abdominal organs protrude into the chest cavity due to a birth defect in the diaphragm, the muscle separating the chest and abdomen.

Bochdalek CDH

The most common type of CDH, where the defect is located in the back portion of the diaphragm, near the spine.

Morgagni CDH

A less common type of CDH, where the defect is located in the front portion of the diaphragm, near the breastbone.

Embryological Development of the Diaphragm

The diaphragm begins to form during the fourth week of pregnancy through the fusion of four embryonic structures.

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Septum Transversum

The most anterior structure that contributes to the diaphragm's development, located above the liver and below the heart.

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Esophageal Mesentery

The posterior structure that contributes to the diaphragm's formation, connecting to the esophagus.

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Pleuroperitoneal Folds

Lateral structures that contribute to the diaphragm's development, located along the sides of the esophagus.

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Diaphragm Completion and Maturation

The diaphragm's development is completed by the 7-8th week of pregnancy, involving the closure of the pleuroperitoneal canals and muscular ingrowth from the body wall.

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Blood Gas Analysis

A blood test that measures the pH, oxygen levels (PaO2), carbon dioxide levels (PaCO2) in the blood, and acid-base balance.

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Blood Gas Analysis in CDH

In CDH, blood gas analysis reveals low blood oxygen levels (hypoxia), high carbon dioxide levels (hypercapnia), and respiratory acidosis.

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Chest X-ray

A radiographic imaging test that uses X-rays to create detailed images of the chest.

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Chest X-ray Findings in CDH

A chest X-ray in CDH shows bowel loops in the chest, mediastinal shift, minimal bowel gas in the abdomen, and displaced nasogastric tube.

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Echocardiography

A non-invasive ultrasound imaging technique used to visualize the heart's structure and function.

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Echocardiography in CDH

Echocardiography in CDH assesses heart function, pulmonary hypertension, blood flow shunting, and potential cardiac defects.

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Renal Ultrasonography

A non-invasive ultrasound imaging technique used to assess kidney structure and function.

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What causes Congenital Diaphragmatic Hernia?

The failure of the pleuroperitoneal fold to completely close the pleuroperitoneal canal during fetal development, leading to a diaphragm defect.

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Pulmonary Hypoplasia

The underdeveloped state of the lungs due to compression by herniated abdominal organs during fetal development.

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What are the characteristics of Pulmonary Hypoplasia?

Reduced branching of airways, fewer bronchioles and alveoli, thickened intra-alveolar septations, surfactant deficiency, and abnormally thick pulmonary blood vessels.

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Pulmonary Hypertension in CDH

High blood pressure in the lungs caused by thickened pulmonary blood vessels, leading to a persistent fetal circulation pattern.

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Right-to-Left Shunt in CDH

Deoxygenated blood bypasses the lungs and travels to the body, causing low oxygen levels, high carbon dioxide levels, and acidic blood.

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What are the common clinical signs of CDH?

Breathing difficulties, bluish skin discoloration, a sunken abdomen, and a wider than normal chest shape.

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Presentation of CDH

CDH can present immediately at birth, later with respiratory problems, or even remain undetected.

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Diaphragmatic Eventration

A condition where the diaphragm moves abnormally during breathing, affecting lung function.

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Morgagni Hernia

A condition where abdominal organs protrude through a hole in the diaphragm, typically on the right side.

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Laparotomy

This approach involves making an incision in the abdomen to access the diaphragm.

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Laparoscopy

A minimally invasive surgical procedure that uses a small camera and instruments inserted through small incisions in the abdomen.

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Thoracotomy

A surgical procedure that involves making an incision in the chest cavity to access the diaphragm.

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Thoracoscopy

A minimally invasive surgical procedure that uses a small camera and instruments inserted through small incisions in the chest.

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Prosthetic Mesh

A synthetic mesh used to repair defects in the diaphragm.

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Autologous Tissue Flap

A flap of tissue taken from another part of the body (like the abdomen or chest) to repair the diaphragm.

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Diaphragm Function

A dome-shaped muscle that contracts during inspiration, pulling downward to increase chest cavity volume for lung expansion. It relaxes during expiration, pushing upward to expel air.

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Congenital Diaphragmatic Eventration

Diaphragmatic eventration caused by incomplete development of the diaphragm during fetal development.

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Acquired Diaphragmatic Eventration

Diaphragmatic eventration caused by injury to the phrenic nerve, often due to trauma or surgery.

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Clinical Features of Diaphragmatic Eventration

Difficulty breathing, wheezing, recurrent infections, and feeding problems can be signs of diaphragmatic eventration.

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Fluoroscopy

A radiographic study that uses a series of X-ray images to examine the movement of the diaphragm during breathing.

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Diaphragmatic Plication

A surgical procedure to tighten and immobilize the diaphragm using non-absorbable sutures.

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Observation for Diaphragmatic Eventration

If diaphragmatic eventration is minor and doesn't cause major symptoms, it can be observed without immediate surgery.

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What is Meconium Ileus?

A condition affecting newborns where the meconium in the intestines is abnormally thick and sticky, causing a blockage.

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Non-Operative Management of Meconium Ileus

The most common approach, where a special enema with warm saline and N-acetylcysteine (NAC) is used to soften and loosen the sticky meconium, allowing it to pass naturally.

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N-acetylcysteine (NAC) in Meconium Ileus

A medication that helps to dissolve the thick meconium, used in both enemas and given directly into the stomach via a tube.

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Operative Management of Meconium Ileus

If the non-operative methods fail, this involves a surgery to manually remove the thick meconium from the intestines.

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What is an Ileostomy?

A temporary opening created in the intestines to divert waste outside the body, often used after surgery for meconium ileus.

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What is Complicated Meconium Ileus?

A serious complication where the blocked intestines become damaged or inflamed, often requiring surgery.

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Prognosis of Meconium Ileus

The outcome for babies with meconium ileus has improved significantly, with survival rates much higher than in the past.

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Previous Mortality Rate of Meconium Ileus

The previous mortality rate for babies with meconium ileus was unacceptably high, ranging from 50% to 67%.

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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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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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