INTRODUCTION
Esophageal atresia
Esophageal atresia (EA) relatively common congenital malformation of unknown etiology incidence 1 in 3,500 live births PURE EA WITHOUT TEF uncommon variant 8 % of all incidence of 1 in 40,000 live births
First measurement of gap between upper & lower esophageal segment by using radiopaque bougies at 3 weeks of age. gap ~ 5 vertebral bodies long.
Consensus among most pediatric surgeons : conservation of the native esophagus associated with the best postoperative results Last 3 decades : DPA recognised as the ideal procedure in the majority of cases for esophageal reconstruction in LGEA
Data extraction
Unblinded , standardized full-text assessment of relevant articles independently performed by both authors (F.F. and P.P.). Data was extracted into electronic datasheet
PATIENT CHARACTERISTICS
Outcome analysis
primary outcome : complication rates.
COMPLICATIONS:
Anastomotic leaks / strictures symptomatic gastroesophageal reflux (GER) dysphagia, esophagitis recurrent fistula / aspiration pneumonia growth retardation & Barretts metaplasia
Statistical analysis
no. of patients with that complication DIVIDED BY the total number of patients in the cohorts that presented data on that specific complication
Cumulative metaanalysis
RESULTS
13 studies :mean of 3.6 cm (range 1.97.0) or 4.5 vertebral bodies (range 1.58.0) 7 studies : mean of 1.3 cm ( range 0.5-3.
TIME UNTIL DPA:
reported in 14 studies.
FOLLOW-UP TIME
observations
RELATIVE RISK FOR ANASTOMOTIC STRICTURES significantly higher in pts with previous anastomotic leaks (RR 2.4, 95 % CI 1.93.0; p<0.0001) or symptomatic GER (RR 3.3, 95 % CI 2.44.4; p<0.0001).
HIGHER RISK FOR ESOPHAGITIS in pts with symptomatic GER (RR 21.6, 95 % CI 1.3337.9; p = 0.0283). HIGHER RISK FOR DYSPHAGIA in pts with symptomatic GER (RR 30.0, 95 % CI 1.8493.3; p = 0.0174) or with anastomotic strictures (RR 25.6, 95 % CI 1.6418.4;p = 0.0228)
DISCUSSION
Overview of complications and long-term outcome in newborns with LGEA managed by DPA of the available patient cohorts published so far. Based on this data, it is recommended to perform a DPA when the patient is 34 months old. At this age, the distance between the two ends usually is <2.0 cm
Anastomotic leaks
Generally pts kept in the hospital until DPA could be performed In most of the studies, early complications after DPA : anastomotic leaks Mostly minor, subsided spontaneously on TPN
Esophageal stricture
Presence of a previous anastomotic leak one of the most important risk factors in stricture formation. Persistent esophageal strictures mainly in association with symptomatic GER. Most of the strictures responded to periodic dilatations
Symptomatic GER
Symptomatic GER present after DPA: requires aggressive approach ~ 30 % of patients required a fundoplication in 1st year after surgical repair of their LGEA due to symptomatic GER or persistent strictures. Risk for esophagitis higher in patients with symptomatic GER. Severe esophagitis caused by symptomatic GER rarely after DPA : required fundoplication
Other complications
INCIDENCE OF DYSPHAGIA AFTER DPA : low Patients with dysphagia usually had symptomatic GER or associated strictures on contrast studies. RECURRENT ASPIRATION PNEUMONIA uncommon after DPA NEED FOR ESOPHAGEAL REPLACEMENT FOR UNSATISFACTORY RESULTS AFTER DPA : relatively rare and only necessary in a few patients having no lower esophageal segment or only a nub of a lower esophageal segment
SURVIVAL RATE after DPA ~ 90 % Long-term follow-up studies showed: majority having NORMAL GROWTH AND DEVELOPMENT Continued long-term follow-up with regular endoscopic surveillance protocols: potential RISK OF BARRETTS METAPLASIA
Disadvantages
Prolonged hospital stay Constant threat of aspiration pneumonia These factors must be balanced against reduced long-term morbidity in a child who should have a normal life expectancy & against the disadvantages of esophageal replacement.
CONCLUSIONS
DPA provides good long-term functional results. High incidence of GER and associated strictures requires early intervention to prevent feeding problems due to stricture & esophagitis Long-term follow-up is recommended because of the potential risk of Barretts metaplasia
REFERENCES
The surgical approach to esophageal atresia repair and the management of long-gap atresia: results of a survey.
Ron O, De Coppi P, Pierro A. Semin Pediatr Surg. 2009 Feb;18(1):44-9
Most surgeons repair < or =2 LGEA/ year. LGEA should be managed by a limited number of surgeons at each center. Little consensus on the definition of or the optimum technique for repair of long-gap OA.
Long gap esophageal atresia and esophageal replacement: moving toward a separation?
Bagolan P, Iacobelli Bd Bd, De Angelis P, di Abriola GF, Laviani R, Trucchi A, Orzalesi M J Pediatr Surg. 2004 Jul;39(7):1084-90
19cases classified as long gap (> or =3 cm), all underwent primary or shortly delayed repair CONCLUSIONS: (1) long gap EA could be treated successfully with primary repair and anastomosis (2) Strictures & GER represent the most frequent postoperative problem, but additional procedures required seem "acceptable" to maintain the patient's own esophagus and avoid replacement (3) esophageal substitution in long gap EA should be reserved for cases in which a previous attempt of esophageal reconstruction failed
103 pts with EA , 17 (16%) of them were defined as LGEA, with mean gap of 5 cm (SD, 1cm). CONCLUSIONS: LGEA a surgical challenge. Mortality high secondary to associated anomalies. no consensus among APS regarding defn of LGEA general consensus of APS: the preservation of the patient's own esophagus should be attempted before considering the use of an esophageal replacement.
Staged esophageal lengthening with internal and subsequent external traction sutures leads to primary repair of an ultralong gap esophageal atresia with upper pouch TEF
Till H, Muensterer OJ, Rolle U, Foker J. J Pediatr Surg. 2008 Jun;43(6):E33-5
J. Foker described a technique of lengthening the pouches with traction sutures and making primary repair possible. Initially, gastrostomy, replogle suction of the upper pouch, repair of tracheoesophageal fistula & upper pouch brought from the neck into the thoracic inlet. At the same time thoracotomy performed, lower esophageal segment mobilized & fixed to the prevertebral fascia under moderate tension After 4 weeks, both pouches mobilized further traction sutures reconfigured & brought out externally through the skin above and below the incision. Daily increases in tension resulted in the ends virtually touching within 10 days
Repair of long-gap esophageal atresia: gastric conduits may improve outcomea 20-year single center experience
Catherine J. Hunter, Mikael Petrosyan, Meghan E. Connelly,Nam X. Nguyen Pediatr Surg Int. 2009 December; 25(12): 10871091.
Surgeons expertise and patients anatomy should be considered when selecting appropriate operation for LEA. Although native esophagus generally preferred, associated with a high rate of stricture. Although study limited by numbers, pts with gastric conduits found to have lower complication rates and no conduit ischemia. gastric transposition may be favored as an initial reconstructive option.
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