Patient 2: The patient was delivered through vaginal delivery. His Apgar scores were 9, 10, and 10 at 1, 5, and 10 min, respectively. Mother had chronic hepatitis B (HBeAg), and the prenatal ultrasound demonstrated polyhydramnios and the double bubble sign. The initial esophageal radiography suggested EA, and abdominal X-rays showed no vertebral anomalies. However, a distended stomach with no distal bowel gas was observed, suggesting duodenal obstruction [Figure 1C]. The failure of passing a NGT confirmed the presence of EA and TEF. His anus was patent, and the renal ultrasound was within the normal limits. Postnatal echo showed a PDA with atrial septal defect (central type) and a right-sided aortic arch. At 24 h after birth, the patient received an emergency operation, in which an operation repairing the EA/TEF and duodenoduodenostomy repairing DA were synchronously performed. The intra-operative pathologic anatomy was shown in Figure 2B. The surgical procedure was the same as the first case, followed by a right thoracotomy with ligation of the TEF, and “diamond-shape” anastomosis for primary repair of EA [Figure 2E–2H]. The chest operation lasted for 1.6 h and chest tube was not placed in the right chest. The abdominal exploration operation was then immediately started during the same anesthesia. On the eighth day, a swallow test showed no esophageal or duodenal leakage. On the 10th day, enteral feedings were initiated, and the infant received full volume oral feedings by the16th day. At the 3-month follow-up, the patient showed adequate growth, without signs of reflux or respiratory problems. A repeat upper gastrointestinal imaging confirmed the presence of esophageal and duodenal anastomosis patency well [Figure 1G]. The patient was in good health at 16-month follow-up.
Patient 3: The patient was born via vaginal delivery. His Apgar scores were 8, 9 and 9 at 1, 5 and 10 min, respectively. The prenatal ultrasound demonstrated polyhydramnios. He was pink and vigorous on room air. An NGT could not be passed successfully. The chest and abdominal X-ray demonstrated that the NGT was in the upper esophageal pouch [Figure 1D], and mediastinal shift, vertebral anomalies and a large gastric bubble with no distal bowel gas were observed, suggesting it had TEF/EA complicated with duodenal obstruction and right lung hypoplasia. The duodenal obstruction was caused by AP. An emergency surgery was performed on the next day after birth. An operation repairing the EA/TEF and duodenoduodenostomy repairing AP were synchronously performed. The intra-operative pathologic anatomy was shown in Figure 2C. The operation procedure was the same as the first case [Figure 2E–2H]. On the ninth day, enteral feedings initiated, and the infant received full volume oral feedings by the 14th day. The NGT was extubated on the eighth day and oral feedings were started on the ninth day after the swallow test, which revealed no esophageal or duodenal leakage. A repeat upper gastrointestinal imaging confirmed the presence of esophageal and duodenal anastomosis patency well [Figure 1H]. The patient was in good health at 10-month follow-up.
Patient 4: The patient was delivered due to fetal distress. Prenatal ultrasound demonstrated polyhydramnios and duodenum dysplasia sign. Her Apgar score was 7 at 1 min, 8 at 5 min and 8 at 10 min. Her anus was patent, and renal ultrasound was within normal limits. Attempts to pass a NGT were unsuccessful. Chest films demonstrated that the NGT was in the upper esophagus, suggesting the presence of EA but without any vertebral anomalies [Figure 1E]. A cardiac echo revealed a patent ductusarteriosus (PDA) and acleistocardia. An NGT could not be passed successfully. A combined chest and abdominal X-ray demonstrated the NGT was in the upper esophageal pouch [Figure 1E]. Vertebral anomalies and a large gastric bubble with no distal bowel gas were observed, suggesting TEF/EA complicated with duodenal obstruction. An emergency surgery was performed and AP caused duodenal obstruction was confirmed on the next day after birth. An operation repairing the EA/TEF and duodenoduodenostomy repairing AP were performed at same time. The intra-operative pathologic anatomy was shown in Figure 2D. The operation procedure was the same as the second and third cases [Figure 2E–2H]. The patient started to accept enteral feedings via a NGT on the 11th day. As oral feeding increased, feedings via the NGT was reduced. The patient reached full volume oral feeds on the 20th day. At the 3-month follow-up, the patient weighed 6100 g. A repeat upper gastrointestinal imaging confirmed the presence of esophageal and duodenal anastomosis patency well [Figure 1I]. At the 6-month follow-up, the patient was in good health condition.
DA was well-described association of TEF/EA and the prevalence was approximately 6%, and this combined anomaly was associated with significant morbidity and mortality.[5,6] A study involving 17 patients with combined defects reported an overall survival rate of 88%. Therefore, associated DA in babies with EA is a cause of increased morbidity. The combined occurrence of TEF, EA, and DA leads to several management challenges. On the one hand, the presence of TEF predisposes the patient to respiratory compromise from aspiration. TEF also fills the stomach with air that cannot traverse through the rest of the gastrointestinal tract due to the DA. This gastric distension also cannot be decompressed with a NGT due to the EA. Therefore, it is difficult to determine which condition should be addressed first and whether the defects should be treated together or in a staged manner.
Although the management of EA/TEF and DA as isolated lesions is well defined with an established trend of not performing gastrostomy in most patients, the treatment protocol for combined EA/TEF and DA varies in the published literature. The authors also recommended a staged repair of EA/TEF with mandatory gastrostomy first, followed by duodenoduodenostomy a few days later. In the absence of an associated TEF, a primary duodenoduodenostomy and gastrostomy with a transanastomotic feeding tube is the approach of choice.
In this report, one of the patients underwent a delayed repair of DA. During the first operation, the EA-TEF repair was carried out via a posterolateral thoracotomy using an extrapleural approach. The other 3 newborns with combined EA/TEF and duodenal obstruction caused by DA or AP safely received one surgery to synchronously repair the anomalies. Our experience suggested that an early combined chest and abdomen X-ray examination was helpful for DA diagnosis. Two patients had esophageal secretions due to aspiration pneumonia. Besides this, none of the patients suffered significant pre- or post-operative complications, and none of the associated anomalies compromised the patient's recovery. There was no death. The average hospital stay length was 20 days. The follow-up data (between 7 and 28 months) of this study suggested that all patients eventually outgrew their reflux and respiratory symptoms. There were no patients with anastomotic strictures or severe gastroesophageal reflux disease in this study.
The gastrostomy might provide better decompression of the stomach and hence protect the oesophageal and duodenal anastomoses. However, this argument is disputed. Dave et al concluded that a primary simultaneous repair of these anomalies without a gastrostomy was adequate without notable increase in the morbidity or mortality compared to a staged approach. In addition, they argued that the use of the Kimura diamond anastomosis for DA allowed for early functioning of the duodenal anastomosis and might preclude the use of a gastrostomy in patients with EA and DA. Although with the limited experience, we believed that the omission of a gastrostomy in neonates with combined EA/TEF and DA was justifiable and safe.
Simultaneous correction of combined major anomalies in neonates can be tried due to advances in neonatal surgery, anesthesia and intensive care. In this report, simultaneous repair (EA repair followed by AP repair) in the last three patients was performed. No mortality or significant additional morbidity was observed. None of our patients suffered significant pre-operative complications. At the end of follow-up, all patients were able to tolerate enteral feedings without significant sequelae from their congenital conditions or surgeries. Our results further confirmed the effectiveness of a simultaneous repair. We expected that the experience and lessons of the diagnosis and treatment of the four cases of complicated digestive tract multiple atresia in the newborn can provide some reference values for the peers. Due to the rareness, there was no consensus regarding the optimal treatment strategy for the combined TEF/EA and duodenal obstruction. We believed that in a stable baby, a primary simultaneous repair of the EA and AP should be attempted. An initial thoracotomy with fistula ligation and oesophageal repair through extrapleural separation should be performed. This might abolish the risk of aspiration and allow the laparotomy to proceed under stable ventilatory status. Surgical therapy of EA/TEF is evolving; thoracoscopic ligation is increasingly being performed. In the future, we will try our best to complete the operation treatment of multiple malformations by combining thoracoscope and laparoscope.
In conclusion, we recommended a synchronous operation repairing the EA/TEF and performing duodenoduodenostomy in one surgery without gastrostomy for the treatment of combined EA/TEF and duodenal obstruction. It is of great importance to identify the anomalies in TEF/EA beyond the VACTERL spectrum and to develop new therapies in infants with multiple anomalies.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patients’ parents/guardians have given their consents for the images and other clinical information to be reported in the journal. The patients’ parents/guardians understand that the names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We thank Dr. Rui Yan at Department of Radiology, the Northwest Women's and Children's Hospital for imaging examination. We are very grateful for the support and assistance of the staff of the medical record information management department for this study.
This work was supported by a grant from the National Natural Science Foundation of Shaanxi Province, China (No. 2014D85).
Conflicts of interest
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