1. “INTERESTING CASES OF OESOPHAGEAL LEIOMYOMA ASSOCIATED WITH OESOPHAGEAL DIVERTICULUM”
C. Spyratou*, K. Farheed, I. Sdralis, SS. Kadirkamanathan, B. Lorenzi, A. Charalabopoulos
Department of Upper Gastrointestinal Surgery
Mid Essex Hospital Services NHS Trust
Case 1
A 35 year old female presented with dysphagia and post-prandial bloating. Her initial investigations (OGD and USS) revealed a paraoesophageal hernia with Barrett’s oesophagus, but the CT demonstrated an epipherinic diverticulum.
She proceeded to a planed laparoscopic excision of the epiphrenic diverticulum with oesophageal myotomy. Intraoperative findings confirmed an oepsophageal diverticulum near the gastro-oesophageal junction. Histological examination showed an oesophageal diverticulum with a nodular lesion within it being positive for Desmin and SMA consisting with leiomyoma.
One year later the patient redeveloped reflux symptoms. A CT chest-abdomen-pelvis and Barium swallow confirmed a large distal oesophageal diverticulum end extensive reflux. She underwent laparoscopic robotic assisted excision of the oesophageal diverticulum, oesophageal myotomy and hiatal closure with crural repair. Histology revealed a diverticulum containing smooth muscle tumor positive for SMA and Desmin and negative for CD34, CD117, DOG1 and S100 protein in keeping with leiomyoma.
Three years later she had a laparoscopic fundoplication to control her reflux symptoms.
Case 2
A 72 years old lady presented with a 20 year history of dysphagia. She had a barium swallow, which revealed corkscrew oesophagus. She underwent PH and manometry studies, which showed grossly abnormal body motility with very hypertonic contractions in the mid and distal oesophageal body and concluded on diffuse oesophageal spasm. She had 300 units of Botox in 25 unit aliquots applied between 30-35cm. Her symptoms did not settle and she underwent laparoscopic oesophagogastrectomy for end-stage corkscrew oesophagus. Histology revealed an oesophageal diverticulum, which contained a small 9mm nodule positive for SMA and Desmin and negative for CD34 and CD117 identified as a benign leiomyoma.
Post operatively she progressively developed symptoms of delayed gastric emptying. She was diagnosed with pylorospasm post oesophagectomy and underwent endoscopic pyloric balloon dilation to improve drainage, which relieved her symptoms to date.
Discussion
Oesophageal leiomyomas are the most common benign tumors of the oesophagus. It is more common in men and usually arises from the inner circular muscle layer in the distal oesophagus. Large lesions can cause obstructive symptoms. They appear as well circumscribed, solitary masses of 2-5cm and bulge into the lumen. They may be polypoid and appear pink, grey or white in colour. They are similar to classic endometrial leiomyoma. They stain positive for Desmin , SMA (alpha smooth muscle actin) and high molecular weight caldesmon and negative for CD34, CD117, S100, DOG1/ANO1.
Both our patients were initially found to have oesophageal diverticulum before histologically diagnosed with benign leiomyomas.
Oesophageal diverticulum is a rare condition and is predominantly surgically managed1. An oesophageal diverticulum is defined as the outpouching or sac of the epithelial lined tissue of the oesophagus. True diverticulum involve all oesophageal layers, whereas false diverticuli are limited to involving the mucosal or submucosal layers protruding through circular and longitudinal oesophageal muscle layers2. They can be classified by location of occurrence, be it proximally at the pharyngo-oesophageal area, the mid oesophagus or more distal ‘epiphrenic’ diverticula3.
 They are typically treated with endoscopic enucleation of small tumors and oesophagectomy for large tumors.
The association of these two diseases in a singular patient is rare. A case report from Germany was the first to demonstrate a simultaneous thoracoscopic enucleation of a leiomyoma and resection of diverticulum for a 66 year old patient18. Untreated this can be dangerous as leiomyomas giving rise to intrathoracic diverticulum can cause spontaneous oesophageal rupture19.
  Mid oesophageal and epiphrefinc diverticula can be subdivided into traction (due to scars and viscous conditions following chronic inflammation such as TB) and pulsion diverticula (due to movement disorders of the oesophagus such as achalasia)4. Patients may complain of a spectrum of symptoms such as dysphagia, regurgitation, odynophagia and heartburn. They may present to a clinician with signs of aspiration pneumonia and gastrointestinal bleeding2.
Results
Both our patients had oesophageal diverticulum associated with benign leiomyoma treated surgically
Conclusions
The main tools for diagnosis of oesophageal diverticulum are Barium Swallow, Endoscopy and Manometry which is crucial in assessing oesophageal diverticulum secondary to motility disorders and pH monitoring is useful in treatment planning for patient groups complaining of gastro-oesophageal reflux disease3.
The combination of oesophageal diverticulum with oesophageal leiomyoma in a single patient is a rare. By describing those two cases we hope to contribute to the general knowledge for the better treatment of these patients.
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