Summary
We present the case of a 26-year-old woman who underwent a successful laparoscopic resection of an esophageal duplication cyst without any other congenital abnormalities. Although computed tomography (CT) scan and endoscopic ultrasonography (EUS) both assisted in determining the correct preoperative diagnosis, the definitive diagnosis was made following pathological examination of the resected lesion.
Introduction
Of all mediastinal tumors, 15.2% are congenital cysts of the esophageal wall.1 Congenital cysts originate in the foregut, and occur with relatively low incidence. According to Arbona et al.,2 congenital esophageal cysts are classified into four categories; duplication, bronchogenic, gastric and inclusion cysts. Esophageal duplication cysts result from abnormal development of the esophagus in the embryonic foregut at 5–8 weeks gestation. Esophageal duplication accounts for 10–15% of all digestive-tract duplications.2
This study presents the successful outcome of laparoscopic, transhiatal excision of a duplication cyst in the distal esophagus of a 26-year-old female patient.
Case Report
The patient was a 26-year-old woman with a history of internal haemorrhoids. She visited our hospital for treatment of anal bleeding in April 1999. Ultrasound revealed a cystic mass near the diaphragmatic–esophageal hiatus. She was admitted on May 15, 1999. Upon admission her physical examination was unremarkable. She was a normally developed patient in no apparent distress and weighed 47 kg. Superficial lymphadenopathy was not observed. Hematological and biochemical parameters were within normal limits. Roentgenography of the chest did not show any abnormal growth. Barium swallow revealed marked compression of the lower esophagus due to a right-sided mass. Esophageal surface mucosa was smooth and showed no sign of ulceration.
Computed tomography (CT) scans of the chest revealed a well-differentiated, unilocular cystic mass, 4 cm × 3 cm in size. The wall of the cystic mass was thin and no calcification was observed. The cyst contents were homogeneously low in density (Fig. 1). The contrast was not enhanced using ioversol (Optiray). Sagittal magnetic resonance imaging sections revealed high-signal intensity on T2-weighted images, which showed that the mass was uniformly fluid-filled.
Abdominal computed tomography (CT) scan: a cystic mass (white arrow) locates behind the left lobe of the liver
Endoscopy revealed a narrowing of the lumen due to the expansion of the right anterior distal esophagus, and the existence of a hiatal sliding hernia. No ulceration of the esophageal or gastric mucosa was observed.
Endoscopic ultrasonography (EUS) demonstrated a cystic mass, with low-echo density and a maximum diameter of approximately 4 cm, in the right anterior side of the distal esophageal wall. The echo also showed disruption of the third layer of the esophageal wall (Fig. 2).
Endoscopic ultrasonogram: the wall of a large hypoechoic cystic mass (white arrow) has continuity to the muscularis propria of the esophagus
The patient was diagnosed as having a duplication cyst in the abdominal portion of the esophagus based on these findings. She underwent diagnostic and therapeutic laparoscopic surgery.
Surgery revealed a soft, rubbery, 4-cm diameter mass in the right anterior wall of the distal esophagus, which involved both the anterior wall and muscular layer of the esophagus. We dissected a portion of the muscular layer to remove the mass completely. We wrapped the defective muscular layer using the wall of the gastric fornix, and performed Nissen’s procedure. Removed specimens showed a 4 cm × 3 cm × 3 cm cystic tumor (Fig. 3) filled with a yellow, viscous fluid.
Macroscopic finding of laparoscopically removed cystic mass (4 cm × 3 cm × 3 cm)
Histopathologic examination revealed that the cyst wall contained thick, irregular, two-layered muscularis propriae and that the surface of the lumen was covered by a lining of ciliated, pseudostratified, columnar epithelium (Fig. 4A and B). Mucus glands, cartilage, gastric mucosa and gastric glands were not observed. Pathological diagnosis was an esophageal duplication cyst.
Microscopic findings of cystic wall: two-layered muscle bundles (A) and pseudo-stratified ciliated columnar epithelium (B) were observed histopathologically. However, neither mucus glands, cartilage, gastric mucosa nor gastric glands were observed
Post-operative recovery was uneventful and the patient was discharged 8 days after surgery. She remains symptom-free at 3 years postoperatively.
Discussion
Esophageal duplication cysts are rare congenital anomalies of foregut origin, which can present symptoms in adult life.3 Classification of foregut cysts is based on embryologic origin, microscopic appearance and anatomic location.4 Cysts have a mucosal lining that may or may not resemble the mucosa of the organ to which the cyst is attached and may occur anywhere in the alimentary tract.5,6 To be considered an esophageal duplication cyst, a lesion must: (1) be in the esophageal wall; (2) be covered with a thick muscularis propriae, generally of two layers; and (3) have an epithelial lining consistent with that of a 4-week embryo, which may be columnar or pseudostratified columnar, and may be ciliated.2 Bronchogenic cysts are similar, except that their walls contain cartilage.2 Esophageal duplications may occur in any portion of the esophagus, but are most common in the lower third.2
Pre-operative diagnosis of an esophageal duplication cyst is hampered by the similar appearance on endoscopic examination to other esophageal submucosal tumors with normal overlying esophageal mucosa.7 Barium swallow only demonstrates compression of the esophagus by a smooth mass.8 CT scanning is not very reliable in differentiating esophageal duplication cysts from other mediastinal cysts.8,9 However, EUS can facilitate accurate preoperative diagnosis of an esophageal duplication cyst, differentiating it from other mediastinal cysts.9,10 In most patients the cyst lumen appears to be filled with a relatively echogenic material. The diagnosis of our case was based on observed ‘continuity’ between the muscularis propriae of the cyst and the muscularis propriae of the esophagus.9
Duplication cysts are usually found incidentally in asymptomatic patients. However, they become symptomatic when complications such as infection, displacement of adjacent organs, hemorrhage, rupture and neoplasia develop.11,12 Surgical excision is usually recommended immediately upon cyst discovery, whether symptoms are present or not.2,6,13–15 Prophylactic excision of the cyst eliminates the likelihood of any complications arising and excludes the possibility of malignancy.16
In conclusion, our patient displayed an asymptomatic esophageal duplication cyst. CT scan and EUS allowed a preoperative diagnosis of esophageal duplication cyst. Laparoscopic excision was successfully performed without rupture, hemorrhage or any other complications. Postoperative course was satisfactory, and the patient was discharged at 8 days postoperatively. At 3 years postoperative, she remains symptom-free.
