VACTERL anomalies in patients with esophageal atresia: an updated delineation of the spectrum and review of the literature

Abstract

The VACTERL complex refers to anomalies of the bony spinal column (V), atresias in the gastrointestinal tract (A), congenital heart lesions (C), tracheoesophageal defects (TE), renal and distal urinary tract anomalies (R) and limb lesions (L). The incidence of each of these components has not been precisely quantified in the recent literature and the full array of anomalies within each systemic class of the VACTERL complex has not been well described. Therefore, we reviewed our most recent 20-year experience of patients born with esophageal atresia to comprehensively delineate and accurately describe the type and incidence of associated lesions. A retrospective review was then conducted on all patients diagnosed with esophageal atresia between 1985 and 2005. Patient demographics recorded included gestational age, weight and gender. The specific types of lesions were carefully cataloged. The outcome measure recorded was survival. One hundred and twelve patients were diagnosed with esophageal atresia were identified during the study period. The gestational age range was 28-41 weeks with an average of 36.5 weeks. Average birth weight was 2,557 g (range 1,107-3,890). A male predominance was seen with 62 males and 50 females. The overall survival was 92.9%. The categorical breakdown of anomalies were vertebral (24.1%), atresia (14.3%), cardiac (32.1%), tracheoesophageal fistula (95.5%), urinary (17.0%), skeletal (16.1%) and other (10.8%). VACTERL anomalies are common in patients with esophageal atresia, however, they appear to have little impact on overall survival.

VACTERL associations in children undergoing surgery for esophageal atresia and anorectal malformations: Implications for pediatric surgeons

“…For children with EA/TEF, the frequency of associated anomalies identified in the current study was similar to those reported by Quan and Smith in their initial description, by Keckler et al in a review of 112 patients, as well as Brown et al who contacted 579 families of children with EA/TEF from two support groups (Table 6) [2,7,24]. Results are also similar to those reported by Pedersen et al in an analysis of a large European birth defects registry [25].…”

“…Modern descriptions of the frequency of VACTERL associations in children with EA/TEF and ARM have largely come from single institutional reports with differing criteria, making comparisons difficult [5,7,22,23]. For children with EA/TEF, the frequency of associated anomalies identified in the current study was similar to those reported by Quan and Smith in their initial description, by Keckler et al in a review of 112 patients, as well as Brown et al who contacted 579 families of children with EA/TEF from two support groups (Table 6) [2,7,24].…”

“…The "vertebral" category was broadened to include both bony vertebral anomalies and spinal dysraphisms [7,20]. The "anorectal malformations" category was limited to atresia of the rectum and not expanded to include other intestinal atresias as has been suggested by some [7].…”

Clinical profile of neonates with respiratory distress

“…They may develop respiratory distress due to the following: Diagnosis -A stiff red rubber catheter cannot pass into stomach and it may get arrested at a distance of 8 to 10 cm from the mouth. 7 The diagnosis is confirmed by x-ray studies showing the catheter coiled in the upper oesophageal pouch. 8 It occurs mostly among term and near term babies and is due to delayed clearance of lung fluids.…”

Polymer Surgical Clip Migration Into the Trachea of an Infant

“…1 Minimally invasive repair of these fistulas has gained popularity in the past decade as the treatment for cases of EA and TEF. 2 During the repair, the distal TEF is identified and is either ligated with sutures or closed with titanium or polymer locking clips before division.…”