International Journal of Pediatric Otorhinolaryngology Extra
Volume 3, Issue 1 , Pages 31-34, January 2008

Recurrent thyroglossal duct cyst presenting as upper airway obstruction: A case presentation and review of the literature

University of Tennessee, Memphis Health Science Center, Department of Otolaryngology, 956 Court Avenue, Suite B224, Memphis, TN 38163, United States

Received 21 August 2007; accepted 14 September 2007. published online 31 October 2007.

Article Outline

Summary 

Thyroglossal duct cysts are the most common congenital midline cervical masses in the pediatric population. Accurate preoperative diagnosis and subsequent adequate surgical excision are essential to prevent recurrence. The Sistrunk procedure is the surgical approach most often advocated. Although rare, treatment failure is most commonly attributed to the failure to perform adequate hyoid bone excision and dissection of the tract to the base of tongue. We report a case of a 7-year-old female who presented with airway obstruction 5 years after having had a Sistrunk procedure and excision of a lateral neck mass. Emergent tracheotomy and marsupialization of the base of tongue cyst were performed. After the diagnosis of recurrent thyroglossal duct cyst was established, core excision of the foramen cecum was performed transcervically when the cyst recurred a third time. The literature on recurrent thyroglossal duct cysts is reviewed and a discussion of its embryology, pathophysiology, epidemiology, presentation, treatment and prognosis is presented in an effort to increase awareness of this situation.

Keywords: Airway obstruction, Recurrent thyroglossal duct cyst, Tongue mass

 

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1. Introduction 

Thyroglossal duct (TGD) cysts are the most common congenital midline cervical masses in the pediatric population. Although cysts may develop anywhere along thyroglossal tract, they are most commonly found in an infrahyoid location and least commonly in an intralingual location [1]. The reported incidence of various cyst locations include 60% infrahyoid, 24% suprahyoid, 13% suprasternal, 1–2% intralingual [2]. Diagnosis may be missed due to the occasional lateral location of the cyst which is seen in approximately 10% of patients [3]. Case identification and adequate surgical resection are essential for preventing recurrence.

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2. Case report 

A 7-year-old Caucasian female with aplastic anemia was referred to our clinic with a tongue mass discovered on routine examination by her hematologist. She had a prior history of a left-sided cystic cervical mass, which had been locally excised at 14 months of age. Pathology was consistent with a TGD cyst. This mass subsequently recurred in the midline 3 months later. A Sistrunk procedure was then performed at 16 months of age. For over 4 years the patient remained asymptomatic. Recently the patient had developed dysphagia, snoring and a muffled voice. Past medical history was significant for aplastic anemia at 5 years of age treated medically without a bone marrow transplant.

On clinical examination, she was in no respiratory distress. Her voice was muffled. Oral cavity and oropharyngeal exam revealed a mucosalized posterior midline tongue mass, which was more visible with tongue protrusion (Fig. 1). The mass was spherical, measured approximately 2cm×3cm, had prominent vascularity and did not significantly obstruct her airway. The remainder of her head and neck examination was unremarkable.

Multiple longitudinal and transverse ultrasound images of the submental region revealed a 2.8cm×2.2cm×2.7cm cystic mass with internal debris. No flow was seen to the walls or into the substance of the mass. An I-123 nuclear thyroid scan revealed normal uptake in both lobes of the thyroid in anatomic position. There were no other foci of uptake to suggest ectopic thyroid. Computed tomography (CT) imaging with contrast consisting of 3mm-thick helical images of the neck demonstrated a 2.7cm×2.2cm×2.4cm mildly enhancing cystic mass at the base of the tongue protruding into the hypopharynx, in the midline (Fig. 2). The mass continued inferiorly to the level of the hyoid bone remnant. The wall of the mass was mildly enhancing. She was electively scheduled for re-excision of mass.

In the interim period, she presented to the emergency room in acute respiratory distress following a viral upper respiratory tract infection. Emergency tracheotomy and transoral marsupialization of the lingual cyst were performed. Pathology revealed squamous mucosa with an edematous submucosa. Three months later the cyst recurred. A repeat CT scan showed 1.3cm×1.1cm residual mass at the base of tongue without obstruction of the airway (Fig. 3). A transcervical wide local excision of the cyst and foramen cecum was performed. Postoperatively the patient was given total parenteral nutrition for 10 days to allow the pharyngotomy to heal. Three months later direct laryngoscopy revealed no recurrence and the patient was decannulated. The patient has not had further recurrence after 5 years.

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3. Discussion 

3.1. Anatomy and pathophysiology of TGD cyst 

The thyroid gland develops from a median diverticulum of the ventral wall of the pharynx around 4th week of gestation. It grows caudally as a tubular duct, which bifurcates and subsequently subdivides into a series of cellular cords, from which the isthmus and lateral lobes of thyroid gland develop. The connection of the median diverticulum with the pharynx is termed the TGD. Its cranial end is represented by foramen cecum of the tongue and caudal end by the pyramidal lobe of thyroid gland [4]. This tract is lined by pseudostratified ciliated columnar epithelium, squamous epithelium, or both [5]. Its continuity is subsequently interrupted and it undergoes degeneration.

TGD cysts develop from persistent epithelial tissue along the course from foramen cecum to the thyroid isthmus. Approximately 7% of the population have TGD remnants [6].

3.2. Epidemiology/presentation 

Thyroglossal duct cysts are generally slow-growing and asymptomatic solitary cystic midline neck masses but may become rapidly apparent after infection. A variability of presentation is very common. Occasionally, this cyst can present lateral to the midline, with infection, as a solid mass rather than cystic and may contain epithelial elements consistent with a dermoid cyst. Also in a recurrent cyst or infected cyst, the ductal epithelium is hard to find on histopathological examination.

Patients with lingual location of the cyst usually present with dysphagia and respiratory distress. The low incidence of lingual TGD cyst may be related to the fact that the duct initially atrophies from the oral side where thyroid decent first begins. This low incidence makes overall experience with these lesions uncommon [7].

The differential diagnosis of TGD cyst at the base of tongue includes ectopic thyroid tissue, hemangioma or dermoid cyst. The most significant but rare complication secondary to upper airway obstruction by base of tongue cyst is sudden death. This has been observed only in infants less than 12 months of age which emphasizes the need for early diagnosis and excision of cyst [8].

3.3. Treatment/prognosis 

Simple excision of thyroglossal duct cysts result in a recurrence rate of 50% which is reduced to 20% by additional excision of the central part of hyoid bone as proposed by Schlange in 1893. The recurrence rate can be further reduced to 3–10% by following Sistrunk's procedure [9].

The Sistrunk procedure consists of excision of the cyst and epithelium-lined tract, running from the cyst to the foramen cecum [10]. Complication rate after Sistrunk procedure is low [11]. Failure after Sistrunk's procedure is attributed to partial hyoid resection [11], arborization of the tract or infrahyoid extension of the tract [12]. The thyroglossal duct above hyoid bone is not a single structure. It may spread out into many ductulii as it approaches foramen cecum [13].

The extent of dissection between the hyoid bone and foramen cecum is debated and it is difficult to determine how much tongue musculature has been actually excised. There is often a conical narrowing of the surgical specimen as dissection approaches the tongue base with a risk of duct avulsion before reaching foramen cecum. Age and inflammation can also affect the pliability of the tissue [14], [15].

Treating patients with recurrence after adequate surgery can be problematic. Causes of recurrence are ductal remnants secondary to incomplete excision and multiple diverticula connected to the tract. In patients requiring revision surgery, the recurrence rate is 30–40% [16]. The optimal conditions to lower the risk of recurrence from TGD cyst remnants in children include age >2 years, absence of prior surgery, absence of previous infection, and cyst without multiple extensions [17].

Solutions proposed include an extension of the Sistrunk procedure [18] or core excision of foramen cecum [19].

There are several case reports of lingual thyroid or thyroglossal duct cysts presenting as airway obstruction in the newborn. However, as in our case airway obstruction in a previously treated cervical TGD cyst is exceedingly rare. Marsupialization of a cyst can relieve airway obstruction in adults [20] but tracheostomy may be necessary to secure the airway in pediatric population.

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4. Conclusion 

This case illustrates several important points regarding thyroglossal duct cyst management in children: (1) cystectomy alone is inadequate; (2) TGD cysts can recur anywhere along the tract from the foramen cecum to the thyroid; (3) marsupialization of lingual cyst may be inadequate; (4) core excision of the foramen cecum is necessary to ensure proximal control of the TGD; (5) airway management may necessitate tracheotomy in certain patients.

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References 

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PII: S1871-4048(07)00075-5

doi:10.1016/j.pedex.2007.09.004

International Journal of Pediatric Otorhinolaryngology Extra
Volume 3, Issue 1 , Pages 31-34, January 2008

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