Saccular Cyst Marsupialization and Ventriculotomy

Procedure: Micro direct laryngoscopy and bronchoscopy with marsupialization of left saccular cyst followed by micro direct laryngoscopy and bronchoscopy with extended ventriculotomy for recurrence. Introduction: Laryngeal saccular cysts result from obstruction of the laryngeal saccule and are classified as either anterior or lateral based on their anatomic location in the larynx. Although saccular cysts are exceedingly rare, the possibility of a saccular cyst should not be overlooked in an infant presenting with symptoms such as stridor, difficulty feeding, or a weak cry. Though marsupialization or needle aspiration have conventionally been employed in the management of saccular cysts, evidence suggests that endoscopic ventriculotomy can better prevent recurrence. Materials and Methods: Marsupialization involves excision of the roof of the cyst using a curved microlaryngeal scissor. In the event of recurrence, extended ventriculotomy is performed removing the medial wall of the cyst with microlaryngeal scissors. Patients are monitored in the intensive care unit post-operatively and bedside speech and swallow studies are performed. Results: A 10-month old female with a left-sided saccular cyst underwent marsupialization, followed by endoscopic ventriculotomy for recurrence. Surgery and postoperative care were uncomplicated resulting in a well-healed repair. 4 years after the ventriculotomy, there has been no mass recurrence. Conclusion: Endoscopic extended ventriculotomy can be performed in patients with saccular cysts to prevent recurrence.

Laryngeal saccular cysts are anomalies of the upper airway that can present in the neonatal period or in early infancy. The laryngeal saccule is a pouch of mucous membrane arising between the false vocal cord, the base of the epiglottis, and the inner surface of the thyroid cartilage that expresses secretions to lubricate the true vocal folds.  A saccular cyst can be either congenital or acquired, and is typically the result of obstruction of the laryngeal saccule, either due to developmental failure to maintain its patency or occlusion of the orifice later in life [1]. These cysts are mucus-filled dilatations of the saccule that are distinctly submucosal and covered by a normal mucous membrane, thus saccular cysts do not communicate with the lumen of the larynx [2]. Saccular cysts are classified as either anterior or lateral saccular cysts based on their anatomic location in the larynx. An anterior saccular cyst extends posteromedially from the saccule into the laryngeal lumen while a lateral saccular cyst extends posterosuperiorly from the obstructed saccule into the false vocal cord and aryepiglottic fold [2]. Infants with a saccular cyst typically present with stridor, difficulty with feeding, and a weak cry [3]. Although saccular cysts are an exceedingly rare occurrence, with a reported incidence of just 1.82 cases per 100,000 live births, the possibility of a saccular cyst should not be overlooked in patients presenting with stridor [4]. Due to the high risk of severe airway obstruction, especially in neonates, prompt diagnosis of a saccular cyst is imperative. Direct laryngoscopy is the primary mode of diagnosis for saccular cysts, but preoperative radiographic imaging can provide additional important information [1]. Diagnosis is confirmed via intraoperative excisional biopsy or needle aspiration of the cyst [1]. Historically, management of saccular cysts has consisted of endoscopic measures, such as needle aspiration and marsupialization, but these techniques result in a high rate of recurrence of the cyst, thus necessitating additional surgical interventions [5]. A 2006 case series by Kirse et al suggested the use of endoscopic extended ventriculotomy as an intervention to both excise a saccular cyst and prevent its recurrence [5]. The case presented here adopts this approach as a more definitive treatment for a saccular cyst that recurred in a patient 7 weeks after initial marsupialization.

Preoperative management of patients includes a full history and physical exam including fiberoptic flexible laryngoscopy. If exam is suggestive of a saccular cyst, an MRI or CT Scan is performed. This video demonstrates a saccular cyst managed initially by marsupialization followed by ventriculotomy for recurrence. This patient was a 10-month-old female with a history of noisy breathing for one week who was transferred from an outside hospital for progressive dysphagia and work of breathing. An initial flexible fiberoptic laryngoscopy revealed a left-sided supraglottic mass, and a subsequent CT scan was consistent with a combined internal and external saccular cyst. The patient initially underwent marsupialization of the cyst. One dose of intravenous steroids (decadron 0.5 mg/kg up to 10 mg maximum dose) and antibiotics are given prior to the start of the procedure. First the larynx is exposed by inserting a size 1 miller blade into the vallecula, allowing the visualization of the left supraglottic mass. Bronchoscopy is then performed to visualize the distal airway. Exposure of the larynx is established with a Lindholm laryngoscope (Karl Storz Endoscopy-America, Inc., El Segundo, CA) of appropriate size for the patient. The laryngoscope is inserted into the vallecula and placed in suspension with the aid of a self-retaining laryngoscope holder (Karl Storz Endoscopy-America, Inc., El Segundo, CA) secured to a Mayo stand over the patient. During the procedure, a timer is set to go off after 15 minutes of suspension after which the suspension is released for at least 60 seconds to prevent compression injury to local neurovascular structures. A 17-gauge needle is used to enter and aspirate fluid from the cyst. Using a cup forceps, the roof of the cyst is grasped and is excised with a curved microlaryngeal scissor. External massage of the neck can be used to express the remainder of the cyst contents. The cyst is then copiously irrigated with saline, and hemostasis is achieved using oxymetazoline soaked pledgets. A tongue stitch is placed at the conclusion of the procedure as a fail-safe mechanism to open the patient’s airway should there be post-operative obstruction due to edema. Post-operatively the patient is extubated in the operating room and admitted to the intensive care unit for at least 24 hours for airway observation.  The patient is given intravenous steroids (decadron 0.5 mg/kg up to 10 mg maximum dose) every 8 hrs for 24 hours, humidified air, and antibiotics for 5 days. A clinical swallow evaluation is obtained on postoperative day 1. If this evaluation is normal, the patient may resume oral intake, including thin liquids, and is placed on a soft diet for 1 week. If the clinical swallow evaluation on the first postoperative day demonstrates coughing during feeds, a video swallow study is obtained that same day. This patient was seen 8 days post-operatively, and an in-office flexible fiberoptic laryngoscopy showed a well healed left supraglottic marsupialization site. 7 weeks later, however, the patient presented to the ED with progressively worsening stridor and a bedside flexible fiberoptic laryngoscopy visualized a recurrent cyst. The patient then underwent endoscopic extended ventriculotomy as described by Kirse et al [5]. After induction via inhalational anesthesia, the patient is orotracheally intubated and a Lindholm laryngoscope is inserted into the vallecula and placed in suspension from the mayo stand. The cyst is entered with an incision made in the false vocal fold. Using microlaryngeal scissors, an extended ventriculotomy is performed removing the medial wall of the saccular cyst. Using a cup forceps, the remainder of the saccular cyst is grasped and removed with microlaryngeal scissors. Hemostasis is achieved using oxymetazoline soaked pledgets and a tongue stitch is placed. Post-operative management is as mentioned previously.

This video demonstrates a saccular cyst managed initially with marsupialization followed by ventriculotomy for recurrence. Flexible fiberoptic laryngoscopy is performed one week post operatively to reveal that the surgical site is healing. Follow up one year after the ventriculotomy showed the surgical site to be well healed and, four years later, the patient has had no mass recurrence.

Due to the inherent difficulties of visualizing and accessing the pediatric airway, repair of congenital saccular cysts can be incredibly challenging. Surgeons must take care to avoid a number of important structures in the region, such as the recurrent laryngeal nerve, and should ideally avoid the need for subsequent surgeries. Though traditional management of saccular cysts consists of endoscopic marsupialization, it has been widely recognized that this intervention may not be adequate as a definitive treatment to both remove the cyst and prevent recurrence [5]. In addition to endoscopic marsupialization, other proposed interventions for saccular cysts include a less aggressive needle aspiration and more aggressive transcervical approaches for resection. In addition to the above described case, the aforementioned study by Kirse et al described 4 patients, 3 of which were successfully treated with a single endoscopic ventriculotomy, strengthening the argument in favor of ventriculotomy in the management of saccular cysts [5]. More recently, a case series by Rosas et al used a modification of the technique described in Kirse et al for 2 patients, both of whom were treated successfully with no signs of recurrence on follow up [3]. In addition to lower rates of cyst recurrence, endoscopic ventriculotomy also avoids the need for an external approach which can put the superior laryngeal nerve at risk of injury [5].

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Thank you to Anna Cornelius-Schecter for providing the voice-over material.

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