Secondary Aortoesophageal Fistula as a Lethal Complication of Continuous Nasogastric Sondage in a Child with Vater Syndrome

Abstract

The first case of VATER syndrome was described by Quan and Smith in 1973. 1 The syndrome represents a complex anomaly of skeletal structures and internal organs, an association of vertebral defects with anal atresia, tracheoesophageal fistula (TEF), and radial limb dysplasia.2 Generally, TEF may occur with or without esophageal atresia. In 85% of the VATER syndrome cases, TEF results from a fistulation of the distal esophagus to the trachea by an atretic proximal blind end of the esophagus. Polyhydramnion and excessive salivation are early clinical manifestations of TEF, followed by choking, coughing and cyanosis after the first feeding. These symptoms can even appear before the first feeding due to a saliva and gastric content aspiration.3 Therefore, surgical repair of this anomaly within the first 24 hours after delivery presents the treatment of choice and may be of vital importance. 4Our case report deals with a rare secondary aortoesophageal fistula (AEF) which developed after continuous nasogastric intubation of a child with VATER syndrome, who was operated on for congenital esophageal atresia associated with TEF. Case ReportA premature male infant was born at 1730 g in the 35th week of gestation by Cesarean section, which was indicated due to a placental abruption and early amniotic fluid escape. The delivery was complicated by severe asphyxia, cyanosis and bradycardia with the Apgar score of 1/6/8. After complete resuscitation normal vital parameters were restored.A series of clinical, laboratory (including genetic) examinations were done and a complex polymalformation was proved. It consisted of hypoplastic left thumb, 3rd and 5th thoracic hemivertebral malformation, presence of thirteen pairs of ribs, stenosis of penile urethra, proximal esophageal atresia with fistulation of the distal esophageal part to the trachea (type IIIb sec Vogt), 4 and stenosis of the penile urethra. To prevent possible stomach content pulmonary aspiration and to open a feeding passage, it was vital to operate on the newborn on the second day of his life. Dorsal mediastinum was reached surgically via the right-side thoracotomy. In addition to atresia, the situation before the reconstruction was complicated by a pathological position of the proximal esophageal part to the left, and the descending aorta to the right side. The division of fistula and reconstruction of the esophagus by means of end-to-end anastomosis on a nasogastric tube were performed. The postoperative location of these structures was crossbreeding, with an intimate neighboring of their walls.After the operation, the newborn was mechanically ventilated, and on the 2nd postoperative day, a punction epicystotomy was done for a bilateral ureterohydro-nephrosis. The postoperative course was further complicated by a persisting thrombocytopenia, as well as the development of bacterial pneumonia. Pneumonia was treated successfully with an appropriate antibiotic therapy which commenced after sensitivity had been micro-biologically verified.On the 48th day of the infant...

Aortoesophageal fistula in a child

“…[2] Continuous nasogastric tube feeding, especially in children with neurological diseases or other reasons, may lead to the formation of AEF. [6] Continuous nasogastric tube insertion in children with known vascular ring has also been reported as a cause. [5] Two children with double aortic arches with prolonged tracheal intubation and/or nasogastric intubation, who subsequently developed AEF have been reported.…”