Use of Mitomycin C for Refractory Esophageal Stricture following Tracheoesophageal Fistula Repair
Keywords: mitomycin C, esophageal stricture, tracheoesophageal fistula
Esophageal stricture is a well-described complication following tracheoesophageal fistula repair. Herein, we report two patients who had persistent esophageal strictures after several months of repeat balloon dilatations. Each patient was treated with a single application of topical mitomycin C in addition to esophageal dilatation, which resulted in complete resolution of the stricture.
Tracheoesophageal fistulas (TEFs) are treated with operative ligation of the distal fistula and anastomosis of the esophageal limbs. The development of an esophageal stricture at the anastomosis is a well-described complication after repair, occurring in approximately one-third of patients. Endoscopic balloon dilatation is currently the preferred method of treatment for both initial and recurrent esophageal strictures. Recently, the use of topical mitomycin C (MMC) for the treatment of esophageal strictures after surgical repair and caustic injury has been reported. In these studies, MMC treatment was largely successful in the resolution of esophageal strictures with a minimal complication profile. However, the vast majority of the reported cases of MMC use in esophageal strictures have been secondary to caustic injury, with a paucity of reported cases after newborn TEF repair. Herein, we report the successful use of topical MMC in combination with balloon dilatation for the treatment of refractory esophageal strictures following TEF repair in two neonates with complete resolution of the stricture without complications.
(a) Esophagram of a tight stricture (white arrow) at the anastomosis in a 39-week-old infant girl, born with VACTERL syndrome. (b) Esophagram showing resolution (white arrow) of the esophageal stricture after balloon dilatation and treatment with mitomycin C.
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A 39-week infant girl, born with VACTERL syndrome, was initially diagnosed with a long-gap pure esophageal atresia due to inability to pass a nasogastric tube and a gasless abdomen on abdominal radiograph. Shortly after birth, the patient underwent a gastrostomy tube placement and end colostomy for imperforate anus. Two months later, she underwent a right-sided thoracotomy, where a Type C TEF was encountered, as opposed to a pure esophageal atresia. She underwent ligation of the distal fistula, resection of a nonpatent fibrous cord of the distal esophageal limb with subsequent anastomosis of the esophageal limbs under tension. A postoperative esophagram revealed an anastomotic leak, which eventually resolved. Four months postoperatively, she underwent a follow-up esophagram which revealed a tight stricture at the anastomosis. She underwent endoscopic balloon dilatation with fluoroscopic guidance every month for four consecutive months with persistence of the stricture at the anastomosis and no improvement.
At 10 months of age, the patient underwent a fourth endoscopic and fluoroscopic guided balloon dilatation of the stricture as before. After adequately dilating the stricture and visualizing circumferential, superficial linear disruption of the strictured ring at the anastomosis, we soaked 1/2-inch × 1/2-inch cottonoids in MMC solution (0.4 mg/mL). A rigid esophagoscope was utilized to apply the MMC-soaked cottonoid onto the left side of the stricture for a 1-minute time period followed by the placement of another MMC-soaked cottonoid on the right side of the stricture for 1 minute (fig-2). The cottonoids were removed, and the esophagus appeared intact. Follow-up surveillance endoscopy was performed after MMC application, which showed no evidence of an anastomotic stricture (fig-1b). No additional balloon dilatation was required thereafter, and no recurrence of her stricture has since occurred. Now at 3 years of age, she remains asymptomatic, eats regular food after gastrostomy tube removal, and has not required further treatment.
A 31-week twin infant girl underwent repair of a Type C TEF. Before thoracotomy, the patient’s clinical condition deteriorated with marked distention of her abdomen. An open gastrostomy was first performed and placed to water seal, and a thoracotomy with ligation of a distal TEF and esophagus-oesophagostomy was performed under moderate tension. One month later, she developed symptoms of feeding intolerance, reflux, and tracheal aspiration. A repeat esophagram demonstrated a near-obstructing stricture in the mid esophagus. The stricture was short, circumferential, and located at the site of the anastomosis. She was taken to the operating room for endoscopic balloon dilatation with fluoroscopic guidance, and subsequently underwent three more monthly balloon dilatations; however, there was no interval improvement in the stricture.
At 5 months of age, MMC was applied during the fifth esophageal dilatation in the same manner as described above. The patient underwent a follow-up surveillance endoscopy, which demonstrated no evidence of residual stricture. Two and a half years after the sole MMC application and dilatation, she remains asymptomatic.
This is a rare condition to be found in young Children but can be found
There are three types of esophageal diverticula, based on where they form: Zenker’s diverticula, the most common type, are in the upper esophagus, mid-thoracic diverticula in the mid-chest, and epiphanic diverticula just above the diaphragm. The diverticulum grows over time, so symptoms can gradually develop or worsen. One of the popular theories is that Zenker diverticulum may be caused by reflux.
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However, there is absolutely no evidence to support this theory. Some patients with Zenker diverticulum who have been studied have evidence of reflux whereas others do not, and some have heartburn whereas others do not; there is no link between reflux disease and Zenker diverticulum even though I hear this misconception quite often. This supposed relationship is likely an extension of the concept that acid reflux can cause throat symptoms so that it is not just cough and laryngitis but also the sensation of fullness of the throat, Zenker diverticulum and other related symptoms.
Zenker diverticulum to Cricopharyngeal bar, a prominence of the upper esophageal sphincter seen on a radiograph, is a related condition that is often mentioned in radiology reports. Most of the time, it does not cause any symptoms and if a patient does not have dysphagia or a diverticulum, the condition can be ignored. The cricopharyngeal bar is occasionally seen in patients who complain of dysphagia. In this scenario, the same abnormalities of decreased opening described above in regard to Zenker diverticulum appear to occur in patients with cricopharyngeal bar. However, I cannot say that the two conditions are necessarily related, as there are no major or frequent reports of patients developing cricopharyngeal bar and then a diverticulum. Nevertheless, both conditions do appear to be in the same area and appear to share the same abnormality. Zenker diverticulum has been found, on barium swallow studies administered for indications other than dysphagia. However, the true prevalence of asymptomatic diverticula is unknown.
Interesting read with photos