Long-term results of esophageal atresia: Helsinki experience and review of literature

Abstract

Esophageal atresia (EA) affects one in 2,840 newborns, and over half have associated anomalies that typically affect the midline. After EA repair in infancy, gastroesophageal reflux (GER) and esophageal dysmotility and respiratory problems are common. Significant esophageal morbidity associated with EA extends into adulthood. Surgical complications, increasing age, and impaired esophageal motility predict the development of epithelial metaplasia after repair of EA. To date, worldwide, six cases of esophageal cancer have been reported in young adults treated for EA. According to our data, the statistical risk for esophageal cancer is not higher than 500-fold that of the general population. However, the overall cancer incidence among adults with repaired EA does not differ from that of the general population. Adults with repaired EA have had significantly more respiratory symptoms and infections, as well as more asthma and allergies than does the general population. Nearly half the patients have bronchial hyperresponsiveness. Thoracotomy-induced rib fusion and gastroesophageal reflux-associated columnar epithelial metaplasia are the most significant risk factors for the restrictive ventilatory defect that occurs in over half the patients. Over half the patients with repaired EA are likely to develop scoliosis. Risk for scoliosis is 13-fold after repair of EA in relation to that of the general population. Nearly half of the patients have had vertebral anomalies predominating in the cervical spine, and of these, most were vertebral fusions. The natural history of spinal deformities seems, however, rather benign, with spinal surgery rarely indicated.

Long-term complications of congenital esophageal atresia–single institution experience

“…Shoulder asymmetry, as a long term complication of surgery, is seen in as many as 80% of cases [10], and rib blocks at the site of thoracotomy are present in 30% [11]. These abnormalities are most probably connected with thoracotomy and congenital vertebral and rib anomalies [10][11][12][13][14].…”

“…These abnormalities are most probably connected with thoracotomy and congenital vertebral and rib anomalies [10][11][12][13][14]. All of our patients were operated via posterolateral thoracotomy.…”

“…As a result of denervation, flattening of the muscles occurred, due to decrease in their volume, as well as limitation of chest movements. Asymmetry and limited range of movement of the shoulder and sticking out shoulders are connected with denervation of latissumus dorsi, while chest deformations are connected with weakening of serratus muscle, rib adhesions and scoliosis [10,14].…”

“…The presence [12,15]. This deformation is usually mild, not requiring operative treatment [10,11]. Risk factors for scoliosis include: narrowing of intervertebral spaces, ribs blocks and congenital vertebral anomalies [10][11][12][13].…”

“…This deformation is usually mild, not requiring operative treatment [10,11]. Risk factors for scoliosis include: narrowing of intervertebral spaces, ribs blocks and congenital vertebral anomalies [10][11][12][13]. It has been demonstrated that in 41.5% of patients with congenital vertebral deformations scoliosis develops [13].…”