أسئلة الثالثة جراحة رابعة دمياط (أطفال)

• Congenital diaphragmatic hernia (CDH) is a pediatric surgery concern and is level 4 complexity.

Definition

• CDH is a birth defect characterized by an abnormal opening in the diaphragm.

Incidence

• CDH occurs in approximately 1 in 2000 live births.
• CDH is considered a highly lethal or morbid disease.
• More common on the left side than the right, with a ratio of approximately 4-5 to 1.
• About 20% of cases have associated anomalies.
• Severity depends on the size and timing of the hernia; larger and earlier hernias cause more severe pulmonary hypoplasia.

Normal Embryological Development

• The transverse septum and pleuroperitoneal folds fuse during the 8th week of embryonic development.
• The intestine returns to the abdomen for rotation and fixation at 10th week.

Abnormal Embryological Development of CDH

• Incomplete diaphragmatic formation due to an embryologic fusion defect causes the pleuroperitoneal hiatus (foramen of Bochdalek) to persist.
• This leads to herniation of intra-abdominal contents into the chest, resulting in pulmonary hypoplasia, hypertension, and cardiac dysfunction.
• Intestinal non-rotation of the midgut commonly leads to bowel herniation into the thorax instead of undergoing normal rotation and fixation.

Types of CDH

• Foramen of Morgagni
• Inferior vena caval foramen
• Congenital absence of the diaphragm
• Esophageal hiatus
• Foramen of Bochdalek (hiatus pleuroperitonealis)
• Aortic hiatus

The Foramen of Morgagni

• It accounts for only 2% of CDH cases.
• Occurs at the junction of the septum transversum and the anterior thoracic wall, representing a central diaphragmatic defect.
• Symptoms typically appear later in life.

Bochdalek Hernias

• These are the most common type of CDH.
• The defect is located posteriorly.
• These hernias are posterior-lateral in location.
• Affected children may be asymptomatic, and the defect is discovered incidentally later in life through a chest radiograph taken for unrelated reasons.

Prenatal Diagnosis

• Includes mediastinal shift and viscera herniation (stomach, intestines, liver, kidneys, spleen, and gall bladder).
• Abnormal positioning of certain viscera inside the abdomen and stomach visualization outside of its usual position.
• Indicators are intrauterine growth retardation, polyhydramnios, and fetal hydrops.
• Ultrasound diagnosis can be made as early as the 2nd trimester.

Postnatal Large Diaphragmatic Defects

• Infants are symptomatic upon delivery with tachypnea, grunting respirations, retractions, and cyanosis.

Postnatal Smaller Defects

• Infants might not be symptomatic until several days or months after birth.

Abdomen Findings

• Scaphoid, due to most of the abdominal viscera being in the hemithorax.

Chest Findings

• Duller to percussion on the side of the hernia, indicating bowel sounds.
• If the hernia is on the left, heart sounds may be heard best on the right side of the chest.

Investigations

• A chest X-ray may show minimal gas in the abdomen, a radiopaque hemithorax (if the bowel doesn't contain much gas or if the left lobe of the liver occupies most of the hemithorax), loss of the normal ipsilateral diaphragmatic contour, bowel in the thorax, contralateral mediastinal shift, and a coiled nasogastric tube in the hemithorax.
• CDH is a medical emergency, not a surgical emergency.

Preoperative Treatment

• Utilize an NG tube to decompress the stomach and prevent distention of the herniated bowel, which can further compress the lungs.
• Use mechanical ventilator with caution to "never use an oxygen mask," to manage hypoxemia, hypercapnia, and acidosis both before and after repair.

Surgical Treatment

• A subcostal abdominal incision is typically made, though some surgeons prefer thoracic approaches for right-sided defects.
• The herniated bowel is reduced from the pleural space.
• The defect is closed using nonabsorbable sutures.
• Closed with a synthetic material for large defects.
• If there is bleeding: a chest tube is placed in the pleural space (optional), connected to a water seal, and not to vacuum.

Surgical Notes

• Repair is not a surgical emergency; it is done once the infant is stable with minimal/no pulmonary hypertension.
• Repair is needed in the asymptomatic patient with no pulmonary hypoplasia or hypertension to avoid bowel obstruction.
• The viscera are reduced, and any associated hernia sac is excised.
• The defect is closed by suturing the posterior rim of the diaphragm to the posterior rectus sheath.
• Rarely, prosthetic patch closure is needed.

Eventration of the Diaphragm

• Characterized by an abnormally elevated or attenuated portion of the diaphragm.

Etiology of Eventration of the Diaphragm

• Congenital: Idiopathic, associated with congenital myopathies or intrauterine infections, resulting in variable thinning or absence of diaphragmatic muscle.
• Acquired: Results from phrenic nerve injury during forceps delivery or surgery.

Clinical Presentation of Eventration of the Diaphragm

• Elevated hemidiaphragm.
• Abnormalities of chest wall mechanics.
• Impaired pulmonary function.
• Respiratory distress and pneumonia.

Investigations for Eventration of the Diaphragm

• Chest radiograph.
• Fluoroscopy.
• Ultrasound to demonstrate paradoxical movement of the diaphragm during spontaneous respiration.

Treatment for Eventration of the Diaphragm

• Indicated when there are associated respiratory symptoms.
• Plication of the diaphragm using interrupted nonabsorbable sutures.