BACKGROUND: The VACTERL association (VA) is the non-random co-occurrence of vertebral anomalies, anal atresia, cardiovascular malformations, tracheoesophageal fistula and/or esophageal atresia, renal anomalies, and/or limb anomalies, and is referred to by the first letters of its components. Studies investigating the clinical characteristics of VA patients and probing of the observed current six component types are limited, and none of them is focused on neonates. We investigated the clinical characteristics of our patients diagnosed as having VA in the newborn period. METHODS: We retrospectively reviewed the neonates whose final diagnosis was VACTERL association. Presence of at least three components of previously reported six anomalies was accepted as VACTERL association. Sex, birthweight, gestational age, postnatal age, anomalies of the systems that are included in VA, and the other features were recorded. RESULTS: There was a male predominance (14/11) of 28 patients; and there were three patients with ambiguous genitalia. The most common observed VACTERL component was vertebral anomalies (n= 26), followed by anal atresia (n= 19), tracheoesophageal fistula/esophageal atresia (n= 17), renal anomalies (n= 15), limb anomalies (n= 15) and cardiac anomalies (n= 14). The most frequent combination was VCTL (n= 4). Fifteen (57%) patients had non-VACTERL anomalies and the most frequent of these was ambiguous genitalia (n= 3). CONCLUSION: VA patients may have different clinical characteristics in different populations, and clinicians may miss some component features if the patients are evaluated after the neonatal period.
BACKGROUND: The VACTERL association (VA) is the non-random co-occurrence of vertebral anomalies, anal atresia, cardiovascular malformations, tracheoesophageal fistula and/or esophageal atresia, renal anomalies, and/or limb anomalies, and is referred to by the first letters of its components. Studies investigating the clinical characteristics of VA patients and probing of the observed current six component types are limited, and none of them is focused on neonates. We investigated the clinical characteristics of our patients diagnosed as having VA in the newborn period. METHODS: We retrospectively reviewed the neonates whose final diagnosis was VACTERL association. Presence of at least three components of previously reported six anomalies was accepted as VACTERL association. Sex, birthweight, gestational age, postnatal age, anomalies of the systems that are included in VA, and the other features were recorded. RESULTS: There was a male predominance (14/11) of 28 patients; and there were three patients with ambiguous genitalia. The most common observed VACTERL component was vertebral anomalies (n= 26), followed by anal atresia (n= 19), tracheoesophageal fistula/esophageal atresia (n= 17), renal anomalies (n= 15), limb anomalies (n= 15) and cardiac anomalies (n= 14). The most frequent combination was VCTL (n= 4). Fifteen (57%) patients had non-VACTERL anomalies and the most frequent of these was ambiguous genitalia (n= 3). CONCLUSION: VA patients may have different clinical characteristics in different populations, and clinicians may miss some component features if the patients are evaluated after the neonatal period.
Authors: Benjamin D Solomon; Linda A Baker; Kelly A Bear; Bridget K Cunningham; Philip F Giampietro; Colleen Hadigan; Donald W Hadley; Steven Harrison; Marc A Levitt; Nickie Niforatos; Scott M Paul; Cathleen Raggio; Heiko Reutter; Nicole Warren-Mora Journal: J Pediatr Date: 2013-12-12 Impact factor: 4.406
Authors: Simona La Placa; Mario Giuffrè; Antonella Gangemi; Stefania Di Noto; Federico Matina; Federica Nociforo; Vincenzo Antona; Maria Rita Di Pace; Maria Piccione; Giovanni Corsello Journal: Ital J Pediatr Date: 2013-07-10 Impact factor: 2.638
Authors: Desiree van den Hondel; Charlotte H W Wijers; Yolande van Bever; Annelies de Klein; Carlo L M Marcelis; Ivo de Blaauw; Cornelius E J Sloots; Hanneke IJsselstijn Journal: Eur J Pediatr Date: 2015-10-24 Impact factor: 3.183