Esophageal Duplication Cyst With Esophageal Web and Tracheoesophageal Fistula

By Charles L. Snyder, Steven W. Bickler, George K. Gittes, V. Ramachandran, and Keith W. Ashcraft Kansas City, Missouri

0 The authors report the case of lower cervical/upper

thoracic esophageal duplication associated with an obstruct- ing esophageal web. This presented in the newborn period as

an esophageal atresia. Initial resection of the web and closure of the fistula were performed. The duplication was

excised electively at 2 months of age. Persistent symptom- atic tracheomalacia required aortopexy, after which the child

recovered uneventfully. There are only three other reports (four cases) in the literature of esophageal atresia (or vari- ants) associated with esophageal duplication.

Copyright o 1996 by W.B. Saunders Company

INDEX WORDS: Esophageal duplication, esophageal web,

tracheoesophageal fistula, esophageal cyst.

E SOPHAGEAL DUPLICATION cysts are rare entities. From an autopsy series of nearly 50,000

patients, the incidence of esophageal duplication cysts was estimated to be 1 in 8,200 patients.’ Al- though most of these lesions are asymptomatic, com- plications include perforation, bleeding,2 mass ef- fect,3 and malignant deterioration.4,5 There are three reports (involving four patients) of esophageal dupli- cation associated with esophageal atresia (EA) in the literature.3,6,7 Herein we report an unusual variant of proximal esophageal duplication associated with tra- cheoesophageal fistula (TEF) and membranous esophageal obstruction, which presented as EA in the newborn period.

CASE REPORT

A full-term newborn was admitted in transfer because of inability to tolerate feedings. Attempts to pass a nasogastric (NG) tube were unsuccessful. The ventilatory status worsened, and bilateral upper lobe atelectasis developed. A temporizing gastros- tomy was placed. Within 2 days the respiratory status improved markedly, and a right thoracotomy was performed. A large trache- oesophageal fistula was found. This was divided and oversewn. There was muscular continuity of the esophagus. A catheter passed from the oropharynx met an obstruction in the esophagus, slightly above the level of the fistula. The esophagus was opened and the mucosal web excised. The mucosal edges were approximated, and the longitudinal muscular esophageal incision was closed trans- versely. The catheter was advanced into the stomach without

From the Department of Surgery, The Children’s Mercy Hospital,

Kansas City, MO. Address reprint requests to Charles L. Snyder, MD, Department of

Surgery, The Children’s Mercy Hospital, 24th and Gillham Rd, Kansas City, MO 64108.

Copyright o 1996 by W.B. Saunders Company 0022-3468196/3107-0025$03.00/0

968

resistance. A contrast study performed before discharge (Fig 1) showed a Y-shaped duplication of the esophagus in the upper thorax and lower cervical region. No vertebral anomalies were found. The patient was discharged on oral feeding.

At 2 months of age the child returned for elective resection of the proximal esophageal duplication. A Tucker dilator was passed down the esophagus and was left in situ during the operation. Through an incision in the lower right part of the neck (anterior to the sternocleidomastoid), a duplicated esophagus was found (Fig 2) which consisted of a thin cyst beneath the muscular coat of the esophagus. The duplication was followed through the neck incision into the upper thorax, and its mucosa was excised. The junction with the normal esophagus was identified and oversewn, and the muscular layer of the esophagus was closed. An intraoperative contrast study showed no leakage. The patient’s early postopera- tive course was uneventful except for mild stridor, which resolved spontaneously. Life-threatening stridor developed 3 weeks postop- eratively, and the baby was readmitted for evaluation. The results of a barium swallow and a 24-hour pH study were normal. Rigid bronchoscopy confirmed the presence of severe tracheomalacia. Aortopexy was performed, and the patient’s clinical condition improved dramatically; the stridor and retractions resolved, and there were no further respiratory symptoms.

DISCUSSION

There are three previous reports of EA associated with an esophageal duplication cyst.3,6,7 There are many reports of duplication cysts of the esophagus located within the muscularis of the esophageal wall, as in the present case. 8,9 In three of the four reported cases, the EA associated with the duplication cyst was not recognized during the initial operation, and additional procedures were required. In the present case, a preoperative contrast study probably would have been helpful in demonstrating the anatomic abnormality.

In our case, pathological examination of the re- sected specimen showed a cystic duplication lined by squamous epithelium. In other cases, squamous, gas- tric, enteric, or respiratory epithelium has been found.lO Most cystic esophageal duplications have occurred in the lower or middle esophagus (94% in one study).9

There are reports of an increased incidence of reflux esophagitis among adults who have esophageal duplication cysts.9 Our patient has not demonstrated gastroesophageal reflux or esophagitis, although the presence of EA/TEF alone clearly indicates an in- creased risk of reflux and its attendant complications.

A common presentation of esophageal duplication

Journal of Pediatric Surgery, Vol31, No 7 (July), 1996: pp 968-969

ESOPHAGEAL DUPLICATION CYST 969

Duplicated Esophagus(at level of T

Fig 1. Contrast is seen in the normal esophagus as well as in the proximal duplication cyst.

cysts in the newborn period is respiratory embarrass- ment secondary to obstruction.l’ Our patient had no obstructive respiratory symptoms, either as a new- born or during the 2 months before cyst resection.

Although developmental anomalies of the esopha- gus and trachea are commonly encountered by pediat- ric surgeons, the particular combination of anomalies found in this patient had not, to our knowledge, been

Fig 2. Drawing shows the intramural location of the cystic duplica- tion, with a distal entry into the normal esophagus.

reported. Cystic duplications of the esophagus associ- ated with EA/TEF are rare and have a significant potential for complications. Usually they are ame- nable to complete resection, which remains the treat- ment of choice.

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