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Normal Anatomy of Diaphragm

  • Composed of a central tendinous portion and a peripheral muscular portion
  • Muscular portion consists of sternal, costal, and lumbar components
  • Three major openings: aortic (aorta, azygos vein, thoracic duct), esophageal (esophagus, vagus nerves), caval (IVC)
  • Right and left phrenic arteries arise from the abdominal aorta
  • Additional arterial supply from pericardiophrenic and musculophrenic arteries
  • Venous drainage is via right and left phrenic veins to the IVC; some drainage to the left renal vein as well
  • Right and left phrenic nerves supply both sensory and motor innervation

Eventration of the Diaphragm

Congenital Diaphragmatic Hernias

Bochdalek's Hernia

  • Occurs posterolateral in the area of the 10th and 11th ribs
  • 90% occur on the left
  • 2:1 male to female incidence
  • Usually isolated and not associated with other congenital defects
  • Typically manifests as acute respiratory distress
  • CXR demonstrates intestine in the thorax and shift of mediastinal contents to the right
  • Initial treatment includes NG decompression, positive-pressure ventilatory support, and surgical correction
  • Approach left-sided defect through the abdomen in order to explore for malrotation and obstruction
  • Right-sided defects are repaired through a thoracotomy
  • Postoperative mortality can be as high as 50%, mostly attributed to increased pulmonary vascular resistance
  • ECMO is useful to reduce pulmonary vascular resistance and help resolve persistant fetal circulation

Morgagni's Hernia

  • Defect occurs in a subcostosternal location
  • Uncommon (less than 3% of diaphragmatic hernias) and usually asymptomatic
  • Well defined hernia sac becomes symptomatic typically after age 40, when obesity, pregnancy, or trauma increases intraabdominal pressure
  • The transverse colon is the most common organ to herniate, and can present as an acute colonic obstruction
  • Repair is usually performed through a upper midline incision

Esophageal Hiatal Hernia

  • Congenital defects causing these hernias are uncommon in adults, but some neonates and infants may have reflux associated with an esophageal hiatal hernia
  • Typical symptoms are vomiting, respiratory complications, anemia, and failure to thrive
  • Diagnosis rests on esophagography, fluoroscopy, and pH monitoring
  • Treatment is primarily medical; surgery is indicated for medical failure

Tumors of the Diaphragm

Primary

  • Rare tumors; cysts are more common than inflammatory masses, which are more common than neoplasms
  • Equal male:female incidence; left-sided tumors are slightly more common than right-sided tumors
  • Symptoms include pain, cough, dyspnea, and GI symptoms
  • CXR and CT scan will localize the tumor
  • The majority of neoplasms are benign (60%), which are usually cysts
  • Up to 40% are malignant, usually sarcomas
  • Treatment includes excision and closure of the diaphragmatic defect

Metastatic

  • Most neoplastic involvement of the diaphragm occurs from contiguous extension of nearby tumors
  • The most common lesions arise from lung, esophagus, stomach, liver, and the retroperitoneum
  • Treatment is based on the primary tumor

Traumatic Perforation

  • Penetrating perforation should be suspected with any thoracic injury below the level of the nipples (5th ICS)
  • Most blunt hernias are caused by automobile accidents, and about 90% occur in the left hemidiaphragm
  • Blunt trauma defects are large, usually about 10-15 cm, and typically located in the posterior left hemidiaphragm
  • Stomach is the most commonly herniated organ, followed by spleen, colon, small bowel, and liver
  • Respiratory insufficiency is common early, while intestinal obstruction predominates later
  • CXR and CT scan will diagnose most; barium contrast is contraindicated, as it can produce a total obstruction in this setting
  • Missed injury and delayed diagnosis commonly leads to bowel incarceration and obstruction
  • Mortality is relatively high (15-40%) due to high incidence of associated injuries
  • Repair should be undertaken promptly with full exploration for other injuries
  • Left-sided perforation should be repaired through the abdomen to allow correction of associated injuries
  • Right-sided perforations may require thoracotomy

Pacing

Indications

  • Sarnoff (1940's) and Glenn (1950's) were the primary developers of diaphragmatic pacers
  • Pacing is indicated in patients who have chronic ventilatory insufficiency with normal nerves, lungs and diaphragm
  • This includes some quadriplegic patients and central alveolar hypoventilation
  • Contraindications to pacing are lower motor neuron dysfunction, muscular dystrophy, and extensive lung disease

Mechanism

  • There are four components to a diaphragmatic pacer:
  • Transmitter: sets respiratory rate and length of inspiration
  • Antennae: transfers signal across intact skin to the receiver
  • Receiver: obtains signal and energy from external portion by inductive coupling
  • Electrode: stimulates the phrenic nerve
  • The electrode portion is usually implanted on the phrenic nerve through the 2nd ICS anteriorly
  • The receiver is placed in a subcutaneous pocket

Central Alveolar Hypoventilation

  • Features of CAH include: hypoxemia and hypercapnia increasing with sleep, hypoventilation or apnea during sleep, and clinical findings of cyanosis, polycythemia, and cor pulmonale
  • These patients have near-normal ventilatory capacity tests, but have a reduced response to induced hypoxemia and hypercapnia
  • Absence of upper airway obstruction or persistence after relief must also be demonstrated
  • These patients should begin pacing within 3 weeks of operation

Quadriplegia

  • Patients with high cervical lesions (C1 or C2) are suitable candidates; injury to C3-C5 may injure the motor component of the phrenic nerves, preventing adequate pacing
  • Delay surgery for several months to allow for potential recovery after spinal cord injury
  • Pacing should be gradually introduced to avoid diaphragmatic fatigue and permanent damage
  • Patients should be selected who are good candidates for long-term rehabilitation


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