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Vascular Ring

1.Introduction

Dr. Robert Gross
First used the phrase 'vascular ring'
First successful division of double aortic arch in 1945
Congenital vascular anomalies
Encircle and compress the esophagus and trachea

2.Incidence
Vascular Rings; Childrens' Memorial Hospital (1947-1996)
   double aortic arch
88
   Rt aortic arch/Lt ligamentum
79
   Innominate artery compression syndrome
81
   Pulmonary artery sling
10
   complete tracheal rings
25
   Ring-sling complex
10
   total
293

3.Clinical presentation

  1. Classic symptom; seal bark cough, wheezing, stridor, respiratory distress, aspiration, apnea, dysphagia, recurrent asthma, cyanotic spell, pneumonia, bronchiolitis, swallowing difficulty, choking on food
  2. Respiratorysymptom;worsen with agitation, crying, superimposed respiratory tract infection
4.Diagnostic evaluation
  1. Chest radiography; location of aortic arch, trachea deviation & compression, lung hyperinflation
  2. Barium esophagram; typical compression of esophagus, bilateral & posterior indentation-double aortic arch, anterior indentation - pulmonary artery sling
  3. Bronchoscopy; important diagnostic tool,
    tracheomalacia, stenosis, complete tracheal ring, innominate artery compression syndrome
  4. Echocardiography; pulmonary artery sling, R/O congenital heart dz. with cyanotic spell
5.Surgical intervention

*Indicated in essentially all patients with vascular ring
*Early and appropriate repair helps avoid serious complication such as hypoxic or
  apneic episodes
*Goal of surgical intervention
   -devide the compressive vascular ring
   -relieve tracheobronchial and esophagial compression 1)Double aortic arch(Fig.1)
    -50~60 % of vascular rings
    -Early in life (newborn to six weeks)
    -Symptom; classic barky cough & constant stridor
    -Disgnosis; chest x-ray, barium swallow
    <Surgical theraphy> (Fig.2)
    -Divide the smaller of the two arches
    -Does not compromise the blood flow to the head vessel
    -Before dividing, check right and left radial and carotid pulse
    -Left thoracotomy
2)Right aortic arch

  -Present later on life (3 to 9 months), because the ring is 'looser'
  -Two primary branching patterns
  1. Retro-esophageal left subclavian artery(Fig.3)
    -Formed by the right arch,pulmonary artery, ligmentum
    -Kommerall's divertuculum; aneurysm at the origin of the left subclavian A
  2. Mirror image branching(Fig.4)
    -Ring is not formed
    <Surgical theraphy>
    -Muscle sparing left thoracotomy
    -Dividing the ligamentum arteriosum
3)Innominate artery syndrome
    -Anomalous innominate A. from the aortic arch
    -stridor, cyanosis, apnea, respiratory arrest
    -Diagnosis; bronchoscopy
    anterolateral compression of the trachea, lumen is classic triangular shape
    <Surgical theraphy>(Fig.5)
    -Suspending the innominate artery from the post. aspect of the sternum
    -Suture through adventitia of the innominate A and the posterior table of the sternum
    -Elevates the anterior tracheal wall and enlarges the tracheal lumen
4)Pulmonary artery sling(Fig. 6)
    -Left pulmonary artery originates from the right pulmonary artery, encircles the distal trachea,
    -Pessure on the distal trachea and the right main bronchus
    -50% of patients have complete tracheal rings (ring-sling complex)

    <Surgical theraphy>(Fig.7)
    -median sternotomy->transect lt. pul. a.->anastomosis to the main pulmonary a
5)Complete tracheal ring(Fig.8)
    -Congenital absence of post. membranous trachea
    -Diagnosis; rigid bronchoscope
    -accompanied by pulmonary artery sling (30%), intracardiac defect (15%)

    <Surgical theraphy>
    -Median sternotomy
    -Resection with end-to-end anastomosis
    -Tracheoplasty (Fig.9,10,11); patched autologous pericardium, tracheal autograft
6.Result
    Backer et al - Children's Memorial Hospital (Chicago, Illinois)
    -no operative mortality from an isolated vascular ring or pulmonary a. sling since 1959
    -survival rate of infants with complete tracheal rings, 84%, new tracheal autograft
      technique, 100%
    -92% of the infants who undergo vascular ring repair are free of respiratory Sx.
      1year post-operatively.
7.Conclusion
    -vascular rings are rare congenital abnormalies
    -compression of the trachea and esophagus
    -stridor, barky cough, respiratory distress, cyanosis, apnea, dysphagia
    <Diagnosis>
    *Barium esopharam : double aortic arch , right aortic arch with left ligamentum
    *Bronchoscopy : innominate artery syndrome, complete tracheal rings
    *Echocardiogram : pulmonary a. sling
    <Surgical approach>
    *Left thoracotomy : double aortic arch, right aortic arch with ligamentum
    *Right thoracotomy : innominate artery suspension
    *Median sternotomy : pulmonary artery sling, complete tracheal rings

    -Operation : Indicated in essentially all patients with respiratory symptom
    -Early repair : effective and allows for normal tracheal growth
 
 



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