Outcome of thyroglossal duct cyst excision isindependent of presenting age or symptomatology§

Rupali Shah a, Kenneth Gow b, Steven E. Sobol a,*

International Journal of Pediatric Otorhinolaryngology (2007) 71, 1731—1735

www.elsevier.com/locate/ijporl

aDepartment of Otolaryngology-Head and Neck Surgery, Emory University School of Medicine,Atlanta, GA, United StatesbDepartment of Surgery, Emory University School of Medicine, Atlanta, GA, United States

Received 8 July 2007; accepted 16 July 2007Available online 31 August 2007

KEYWORDSThyroglossal duct cyst;Neck mass;Sistrunk;Symptoms

Summary

Background: Thyroglossal duct cysts (TGDCs) are the most common form of con-genital neck cyst, accounting for up to 70% of such lesions. There has been noconsensus on which factors predict outcome of thyroglossal duct cyst excision.The objective of the current study is to evaluate the relevance of symptomatologyand age at presentation with outcome of TGDCs.Methods: Retrospective review of patients with TGDC at a tertiary care children’shospital. Data collected included patient’s age, gender, clinical presentation, pre-sence or absence of preoperative infection, imaging modality, type of procedureperformed, size and location of the lesion, postoperative infection, complications,and recurrence of disease.Results: Twenty-nine patients were identified (59% female, 41% male). Age of pre-sentation was bimodal and ranged from 18 months to 14 years. The most commonpresenting symptom was the presence of an asymptomatic midline neck mass (76%). Ahistory of preoperative TGDC infection was present in 22% of patients �2 years of ageand in 43% of patients �5 years of age. Recurrence rate after the Sistrunk procedurewas 3.4%.Conclusions: The finding of a midline neck mass is the most common presentation ofTGDCs in toddlers, whereas infection is the most common presenting symptoms inschool-aged children. The incidence of preoperative infection was 41% in our series,much higher than previously reported. Independent of presenting age and sympto-matology, recurrence of TGDC remains low when the Sistrunk procedure is employed.# 2007 Elsevier Ireland Ltd. All rights reserved.

§ Presented at the American Society of Pediatric Otolaryngology Annual Meeting, San Diego, CA, May 2007.* Corresponding author at: Emory Children’s Center, 2015 Uppergate Drive, NE, Atlanta, GA 30322, United States.

Tel.: +1 404 727 1368; fax: +1 404 727 7996.E-mail address: ssobol@emory.edu (S.E. Sobol).

0165-5876/$ — see front matter # 2007 Elsevier Ireland Ltd. All rights reserved.doi:10.1016/j.ijporl.2007.07.010

1732 R. Shah et al.

1. Introduction

Thyroglossal duct cysts (TGDCs) are the most com-mon form of congenital neck cyst, accounting for upto 70% of such lesions. They commonly present inchildren or adolescents, yet up to one-third ofpatients are 20 years or older. Males and femalesare equally affected [1]. In most cases, a meticulousclinical history and physical exam are sufficient tomake the diagnosis. The true incidence is unknown,and, in fact, many are never detected clinically. Ellisand van Nostrand found a 7% incidence of TGDCs inpostmortem study of 200 adults [2]. Despite therelative frequency of TGDCs, familial occurrencesare rare. The inheritance pattern is usually auto-somal dominant with incomplete penetrance, and afemale preponderance has been noted [3].

1.1. Embryology

The thyroid analage develops from an epithelialproliferation that originates at the junction of theanterior two-thirds and posterior-third of the ton-gue, the future site of the foramen cecum. At 4weeks gestation, a ventral diverticulum forms anddescends in the midline of the neck as the thyro-glossal tract [4,5]. Maintaining its attachment to thebase of the tongue, the tract travels anterior to thehyoid bone and then in front of the thyroid cartilage,cricothyroid membrane, and cricoid cartilage [2].The thyroid assumes its final position anterior to thetracheal rings, where the lobes separate by 7 weeksgestation. Normally, the tract atrophies and disap-pears by 10 weeks gestation [4,5]. Portions of thetract and thyroid tissue remnants may persist at anypoint along the migratory path. Occasionally, thereis no migration, and the entire thyroid is arrested atthe base of the tongue resulting in a lingual thyroid[6]. TGDC result from expansion of a remnant of thethyroglossal tract. The cause of expansion isunknown; however, inflammation is the most likelyetiology. Adjacent lymphoid tissue reacting to infec-tions may stimulate the epithelial remnants toundergo cystic changes [1].

1.2. Clinical presentation

Patients with a TGDC typically present with a mid-line upper neck mass that moves with tongue pro-trusion and swallowing. The cyst is generallynontender and may reach a diameter of up to10 cm. While most lies in the midline, they may lesscommonly be located laterally, usually to the left[1]. Patients may report a recent upper respiratorytract infection, which can cause a sudden increasein the size of the mass [7]. The position of the TGDC

can be described as lying in one of the following fourlocations: (1) intralingual, (2) suprahyoid, includingsubmental, (3) thyrohyoid, and (4) suprasternal. Inapproximately 60% of cases, the cyst is found inti-mately associated with the hyoid bone adjacent tothe thyrohyoid membrane [1]. Although mostTGDCs are asymptomatic, those found intralin-gually may cause choking sensations, dysphagia,and dysphonia [1,8]. A cutaneous or oral fistula isa secondary feature of the disease and may resultfrom suppuration, trauma or inadequate surgery ofa thyroglossal cyst [1,5,8]. Adults are more likelythan children to present with a complaint otherthan mass or infection, including pain, sore throat,dysphagia, hoarseness, and fistula formation [9]. Itis unclear if adolescents present differently thaninfants.

The differential diagnosis of a TGDC includesdermoid cysts, sebaceous cysts, branchial cleftcysts, thyroid nodules, lymphadenopathy, and lipo-mas [7,8]. In 1—2% of patients with suspectedTGDCs, the mass is actually an ectopic thyroid gland[10]. Preoperative detection of hypothyroidism orabsence of thyroid tissue in the normal site may aidin making this diagnosis [10,11]. Thyroglossal ductcarcinomas are rare and occur in approximately 1%of TGDCs. They are usually not predicted uponpresentation and are incidentally diagnosed aftersurgery. The vast majority has a papillary thyroidorigin, but there are rare reported cases of squa-mous cell carcinoma [12].

1.3. Diagnosis

The epithelial lining of a TGDC ranges from squa-mous epithelium to pseudostratified ciliated colum-nar epithelium. Salivary gland tissue or thyroidgland tissue may be seen in the wall of the cyst.The cyst fluid can be described as mucoid, gelati-nous, or purulent yellowish-white to dark brownthat may contain cholesterol [1].

Preoperative imaging is important to confirmdiagnosis, identify normal functioning thyroid tis-sue, and to detect malignancy. High-resolutionultrasound remains the ideal initial imaging modal-ity for TGDCs. It is easily accessible, inexpensive,non-radiating, and does not require sedation[11,13]. An uncomplicated cyst will appear anechoicand well circumscribed or pseudosolid from protei-nacious fluid contents. Doppler may identify anysolid component suggestive of malignant disease.Routine radioisotope thyroid scanning is not neces-sary if a normal thyroid gland is demonstrated onpreoperative ultrasound since the cyst is unlikely tocontain the only functioning thyroid tissue [11,13].Other modalities that have been employed for the

Outcome of thyroglossal duct cyst excision 1733

Table 1 Clinical presentation of thyroglossal ductcysts

Symptom Number (%)

Midline neck mass(no infection or drainage)

22 (76)

Moves with tongue protrusionand/or swallowing

9 (31)

Infection 6 (21)Recurrence 2 (7)Tenderness 2 (7)Dysphagia 2 (7)Fistula 2 (7)

Table 2 Preoperative infection distributed by age

Age at operation(no. of patients)

Number withpreoperative

diagnosis of TGDCs include computed tomography(CT) or magnetic resonance imaging (MRI).

1.4. Management

There is no effective medical therapy for the treat-ment of TGDCs; the use of sclerosing solutions andcoagulation are not recommended unless surgery isrefused [1]. The standard surgical procedure,described by Sistrunk in 1920, aims for removal ofall possible thyroglossal tract remnants to preventrecurrence. In this procedure, themidline portion ofthe hyoid bone is resected along with a wide core oftissue belonging to the midline area between thehyoid and foramen cecum [14]. The Sistrunk proce-dure is associated with a very low morbidity. In aretrospective review of 35 patients, Maddalozzoet al. reported no major complications followingsurgery and only minor wound-related complica-tions in 29% of patients [13]. Of most concern afterresection is recurrence of disease due to incompleteremoval of tract remnants. Using Sistrunk’s techni-que on 270 patients, Brown and Judd were able toreduce the recurrence rate to 4% [15]. Simple cystexcision is associated with a recurrence rate of up to40% [8]. The best chance of curative resection is atinitial presentation [5]. Although many havereported preoperative cyst infection as a significantrisk factor for recurrence, [16] there has been noconsensus on which factors predict outcome of thyr-oglossal duct cyst excision.

The objective of the current study is to evaluatethe relevance of symptomatology and age at pre-sentation with outcome of TGDCs.

infection (%)

<2 (3) 0 (0)2 (6) 2 (33)3 (3) 2 (67)4 (3) 2 (67)5 (4) 1 (25)7 (2) 1 (50)8—9 (2) 2 (100)10—11 (2) 1 (50)12 (2) 0 (0)14 (2) 1 (50)

Total patients = 29 Total preoperativeinfections = 12/29 (41)

Table 3 Patient evaluation

2. Materials and methods

After obtaining institutional review board approval,a retrospective analysis of charts from 2000 to 2005was undertaken. Twenty-nine patients met inclusioncriteria for this study. All patients were less than 18years of age and had a postoperative diagnosis of‘‘thyroglossal duct cyst’’. Data collected includedpatient age, gender, clinical presentation, presenceor absence of preoperative infection, imaging mod-ality, type of procedure performed, size and loca-tion of the lesion, postoperative infection,complications, and recurrence of disease.

Imaging modality Number of patients

None (clinical exam) 12Ultrasound 12MRI 4 (2� in add to US)CT 2Neck soft tissue 2 (1� in add to US)

3. Results

Of the 29 patients, 17 (59%) were female and 12(41%) were male. Nine patients (31%) were definedas infants (�2 years), while 14 (48%) patients were

defined as school aged (�5 years) at presentationwith the remaining patients falling in between. Theaverage age at operation was 6 years. Presentingsymptoms are displayed in Table 1. Of the ninepatients in the infant age range, two (22%) had apositive history of preoperative infection while 6 ofthe 14 school-aged children (43%) had a positivehistory of preoperative infection (Table 2). Imagingstudies were obtained in many but not all cases(Table 3). Location of the thyroglossal duct cyst inrelation to the hyoid bone is described in Table 4.The majority of patients were found to have theirthyroglossal duct cysts in the typical thyrohyoidlocation (69%).

1734 R. Shah et al.

Table 4 Thyroglossal duct cyst location

Relationship of TGDCto hyoid bone

Number ofpatients (%)

Intralingual 0 (0)Suprahyoid 5 (17)Thyrohyoid 20 (69)Suprasternal 4 (14)

Of the 29 cases, 28 cases were treated surgicallywith the Sistrunk procedure originally described in1920 [14]. One of these patients had a recurrenceandwas successfully treatedwith re-excision. In thiscase, at 2-years follow-up there was no evidence ofrecurrence. One patient was treated by aspirationfor a suspected cystic hygroma; this patient didexperience a recurrence of their TGDC. Two patientspresented to our institution with a history of recur-rent disease and underwent their first Sistrunkoperation at our institution; they experienced nosubsequent recurrences or complications.

The total recurrence rate in this series was 6.9%(3.6% when the Sistrunk procedure was performedinitially). No other complications were reported.

4. Discussion

Thyroglossal duct cysts are the most common mal-formation found in the neck. While the currentliterature reports an equal incidence of thyroglossalduct cysts in males and females, we report a higherincidence in females (59%). There are many studiesin the literature with contradictory results [1]. Alarger population is necessary to determine the trueincidence. It is important to note, however, that ithas been suggested that a true female predomi-nance does exist amongst familial thyroglossal ductcysts [3].

Little data exists as to actual incidence of pre-senting symptoms in patients with TGDC. In ourseries, 76% of patients had a mass at presentationwithout infection or fistula. In 31% of patients, theneck mass would move with tongue protrusion orswallowing. Dysphagia, fistula, tenderness, andrecurrence were identified in two patients eachat a rate of 7%. Of note, Ostlie et al. series showed22% of patients had preoperative infection [17]. Inthis series, a history of preoperative infectionappeared with almost twice the frequency for atotal of 41% of our patients. While only 22% of theinfants (�2 years) in this series had preoperativeinfection, 43% of the school-aged children had pre-operative infections. This suggests that older chil-dren are more likely to become infected. In ourexperience, we could not directly correlate age with

incidence of preoperative infection; however, alarger study population may show this to be truewith statistical significance.

Diagnosis of a TGDC heavily relies on a thoroughhistory and head and neck exam. It is well docu-mented in the literature that MRI, CT, and ultra-sound are all good diagnostic tools for confirming thepresence of thyroglossal duct cysts. If imaging isnecessary, US is the ideal initial imaging technique,because it is easily accessible, inexpensive, non-invasive, and does not involve radiation or sedation,which is particularly important in the pediatricpopulation [11]. In our series, 12 patients did notreceive any imaging. Lack of imaging did not lead toany adverse events in these cases. Imaging is impor-tant for confirmation of diagnosis, identification ofnormal thyroid tissue, and detection of malignancy.In these cases, diagnosis was strongly suspectedbased on history and clinical exam and the masswas not located intralingually, which is more oftenassociated with ectopic thyroid. In accord with theliterature we report a significant majority of TGDCoccurring at the thyrohyoid level (69%) with feweroccurring suprahyoid (17%) or suprasternally (14%).We had no cases occurring intralingually, while theliterature reports 2% occurring at this location [1].

Thyroglossal duct cysts are well known to recurwhen treated with resection or aspiration only [8].Our lone patient who underwent aspiration didrecur, and the two patients who presented withrecurrence had never undergone a Sistrunk opera-tion. Only one of our patients who underwent theSistrunk procedure had recurrence (3.6% of all Sis-trunk procedures).

5. Conclusions

Mass and infection are the most common presentingsymptoms of thyroglossal duct cysts. The incidenceof preoperative infection was 41% in our series,much higher than previously reported [17]. It issuggested that school-aged children are more likelyto have preoperative infections than younger chil-dren. Of all patients who underwent the Sistrunkprocedure, only 1 (3.6%) had evidence of recurrentdisease at follow-up. Independent of symptomatol-ogy and age, outcome of thyroglossal duct cystexcision remains good when using the Sistrunk pro-cedure.

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