Pyriform Sinus Diverticulum: An Unusual Case Presentation

EDUCATIONAL OBJECTIVE: At the conclusion of this presentation, participants should be able to describe the embryology associated with and endoscopic management of pyriform sinus diverticula. OBJECTIVES: To present a case describing the atypical etiology for an adult woman's dysphagia (an unusual presentation of a pyriform sinus diverticulum) and its management. STUDY DESIGN: Case report. METHODS: A single case was reported and relevant literature was reviewed. CONCLUSIONS: 1) Endoscopic management of a pyriform sinus diverticulum, can improve resultant symptoms while limiting morbidity; and 2) Unusual congenital abnormalities should be considered in the differential workup for dysphagia. Case Description A 34 year-old woman was referred by a community Otolaryngologist for evaluation of a patient with 10 years of progressive dysphagia and an unusual laryngoscopic examination. The dysphagia occurred primarily with small, firm solids, such as carrots or peanuts. Soon after swallowing, the patient experienced a “foreign body” sensation that would persist for hours to days. Before presentation, the patient underwent a barium esophagogram, (figure 1) revealing pooling of thin liquids at the junction of the posterior oropharyngeal and the right pyriform sinus at the level of the epiglottis. This collection diminished in size only minimally over the course of the procedure. On flexible laryngoscopy (figure 4), the anterior wall of the patient’s right pyriform sinus had a deeper cavity extending anteriorly, which would expand with valsalva maneuver.. The remainder of the laryngoscopy, including visualization of the aryepiglottic folds and the saccule, was within normal limits. The patient underwent a CT scan revealing a cavity extending from the level of the pyriform sinus to the right true vocal fold (figure 2). Also noted was a hypodensity in the right thyroid lobe. The patient denied dysphonia and dyspnea at any point but her symptoms remained significantly bothersome. The options of continued observation, endoscopic laser ablation and external excision were discussed with the patient. After some Discussion Pyriform sinus anomalies (cysts, sinuses and fistulae) are usually the result of failure of third or fourth pharyngeal pouch obliteration1. These branchial remnants manifest clinically with airway distress, neck mass or with localized or regional infection, oftentimes in the form of acute suppurative thyroiditis2,3,4. Diagnosis can occur via ultrasonography, CT scan and/or barium swallow with confirmation during direct laryngoscopy. Surgical excision via an external approach can be performed to remove the remnant. These malformations are primarily diagnosed in childhood and found on the left side. Our case is unusual in several ways; the anomaly manifested as an adult, though this has been described previously5. Secondly, our patient’s anomaly was on the right side while the vast predominance occur on the left. Lastly, our case differs from most in that it did not present with infection, but rather with dysphagia. Although the initial differential diagnosis contained pathologies ranging from esophageal diverticulum to esophageal dysmotility, the laryngoscopy and radiographic studies quickly focused our attention on a previously undiagnosed congenital malformation. While most branchial pouch remnants causing thyroiditis are managed with complete removal of the epithelial tract including its extension to the thyroid6, because the patient never suffered from thyroiditis, an endoscopic approach was pursued averting potential injury to the vital neural and vascular structures in close proximity. This approach was successful in significantly reducing the patient’s symptoms while minimizing potential risk and avoiding morbidity. References 1. Liberman M, Kay S, Emil S, Flageole H, Nguyen LT, Tewfik TL, Oudjhane K and Laberge JM. Ten years of experience with third and fourth branchial remnants. J Pediatr Surg. 37(5):685-690, 2002 May. 2. Digoy GP. Greenberg M. Magit A. Congenital stridor secondary to an upper airway cyst in a patient with Ellis-van Creveld syndrome. Internat J of Pediatr Otorhinolar. 69(10):1433-5, 2005 Oct. 3. de Buys Roessingh AS. Quintal MC. Dubois J. Bensoussan AL Obstructive neonatal respiratory distress: infected pyriform sinus cyst.. J of Pediatr Surg. 43(5):E5-8, 2008 May. 4. Chin AC. Radhakrishnan J. Slatton D. Geissler G. Congenital cysts of the third and fourth pharyngeal pouches or pyriform sinus cysts. J of Pediatr Surg. 35(8):1252-5, 2000 Aug. 5. Robitschek J, Bothwell N, Takahashi G, Hall D, Sniezek J. Adult presentation of a fourth branchial pouch sinus. Ear Nose Throat J. 88(10):E31-3. 2009 Oct. 6. Hazarika P, Nayak DR, Balakrishnan R, Vishvanathan A, Pujary K. Fourth branchial pouch sinus: a case report. Am J Otolaryngol. 22(6):435-8. 2001 Nov-Dec.