Recurrent thyroglossal duct cysts: a 15-year review of presentation, management and outcomes from a tertiary paediatric institution

Introduction

Thyroglossal duct cysts (TGDC) arise from the incomplete closure of the thyroglossal tract, an integral structure in the embryological development of the thyroid gland. Thyroglossal tract remnants are reported to be present in 7% of the population, however not all develop a cyst (1,2). TGDC are the most common congenital midline neck swelling in the paediatric population, with over half of all cases presenting before 20 years of age (3,4).

The management of TGDC has evolved through a greater understanding of the embryological and histological basis of the disease. Gold standard management is the Sistrunk procedure, which involves en bloc resection of the cyst and the thyroglossal tract, with the superior excision encompassing the central aspect of the hyoid bone and continuing up to the foramen caecum, without breaching the oral mucosa (5). The recurrence rate after Sistrunk is between 3% and 10% (6-8), which is a vast improvement of the recurrence rate of more than 50% after performing an incision and drainage (I&D) (9). Recurrence is attributed to an inadequate initial resection along and/or variability in histological anatomy of the thyroglossal tract, with small ductules extending from the primary thyroglossal duct.

Typically, patients who recur present in a similar fashion to their primary presentation, however, there can be an increased incidence of cutaneous involvement (9). The majority of patients recur within the first postoperative year (10). The operative goal of recurrence surgery is to excise the remaining thyroglossal duct and associated ductules.

In this paper, we present a review of the presentation and management of recurrent TGDC over a 15-year period at a tertiary paediatric hospital to assess trends and outcomes.

Methods

Institutional board review was obtained. A retrospective chart review was performed to include all patients who presented to a tertiary paediatric hospital with a TGDC over a 15-year period. Patients were screened for recurrences of their TGDC through additional admissions to the tertiary hospital, clinic notes, primary care practitioner referrals and requests for admission sent from private consultant rooms.

Electronic and paper records were reviewed to obtain: age, sex, recurrence, primary presentation, primary management, presentation of recurrence/s, length of follow up, postoperative complications and further management. If patients were noted to have a recurrence they were sent a questionnaire with a single question, ‘Have you/your child had another recurrence of a thyroglossal duct cyst or sinus since the last operation performed at The Children’s Hospital at Westmead?’. If patients did not respond in 2 months, they were phoned.

Results

Two hundred and twenty-seven operations were performed for suspected TGDC or recurrent TGDC in paediatric patients over a 15-year period. Histopathology and operation report review identified 191 (84%) cases of TGDC, 16 (7%) cases of recurrent TGDC, 13 (6%) cases of dermoid cysts and 1 (0.4%) each for epidermal cyst, neck abscess and lymph nodes. Patient notes were unable to be located for 4 subjects.

Recurrent TGDC

Demographics

Sixteen patients had a recurrence after initial management of their TGDC, of which 6 (38%) were male and 10 (62%) female. The patients had a mean age of 5.3±3.1 years. Table 1 outlines patient information.

Initial presentation and management

The initial TGDC presentation included non-infected cysts (9 cases, 56%), infected cyst (2, 12%), infected cyst and sinus (1, 6%), infected sinus (1, 6%), sinus (1, 6%) and the presentation was not documented in 2 cases (12%). The first surgical procedure of these patients included a Sistrunk procedure (11 cases, 68.75%), cystectomy (4 cases, 25%) and Schlange procedure (1 case, 6.25%). Four patients had an incision and drainage of an infected cyst, prior to initial cyst excision.

Presentation of recurrence

The mean time to recurrence was 20.7±26.9 months. Patients presented with a non-infected cyst in 7 cases (44%), draining sinus in 7 cases (44%), cyst and sinus in 1 case (6%) and an infected cyst in 1 case (6%). Two patients had a third recurrence after operative intervention, 1 presented with a non-infected cyst and the other a draining sinus. Eight patients (50%) presented with a sinus at recurrence, of these, 3 (19%) had cutaneous involvement at initial presentation.

Management of recurrence

The management of recurrences was dependent on the primary operative intervention. Two operations were required for cure in 88% of patients. Eleven patients recurred after primary Sistrunk and of these, 9 were managed with revision Sistrunk and 2 were managed conservatively. The revision Sistrunk comprised of further dissection along the primary resection, with an extended excision of the remaining hyoid. Two patients (22%) had a further recurrence following revision Sistrunk, 1 was managed with a further revision Sistrunk, however recurred again, while the other had a cystectomy and also recurred. To our knowledge, they were both subsequently managed conservatively. Four patients managed with a cystectomy recurred and in all cases, management with Sistrunk procedure provided cure. One patient recurred following Schlange procedure, which was successfully managed with Sistrunk procedure.

Response to questionnaire

Eight patients (50%) responded to the questionnaire, with no recurrences reported.

Follow up

Follow up ranged from 2 weeks to 15 years, with a mean of 48.5±68.2 months.

Surgical speciality directing management

All patients who recurred had their initial intervention performed by a paediatric General Surgeon. Fifteen patients (94%) had their recurrence managed by a paediatric General Surgeon, while 1 patient (6%) was managed by a paediatric Otolaryngologist.

Discussion

TGDC recurrences are attributed to the incomplete excision of the thyroglossal tract and its branching ductules. The residual tract is present postoperatively due to variable histology or a limited surgical resection. Histologically, the thyroglossal duct can have multiple ductules extending from the main ventral duct which may not be detected intraoperatively, complicating the initial management and increasing the risk of recurrence due to residual thyroglossal tract. Historically, incision and drainage, cystectomy and Schlange procedure were performed to manage TGDC, however they are associated with high recurrence rates as they do not completely excise the thyroglossal tract, thus are no longer recommended treatment options. I&D prior to Sistrunk procedure has been associated with the need for multiple operations for cure (11). In our series, there were 4 patients who had an I&D prior to Sistrunk procedure. Three patients had no recurrence, however 1 patient has persistent disease despite 3 further operative interventions.

Failed surgical intervention alters the presentation of TGDC, with recurrences having a greater cutaneous involvement. In our series, 19% of initial TGDC presentation had a draining sinus, however with recurrent TGDC, the incidence rose to 50%. A previous review of 28 patients by Mickel et al. (9), found a similar increase in risk of cutaneous involvement following failed initial operative interventions. The majority of patients with a recurrence have been reported to present in the first postoperative year (10), however in our series, the majority presented in the second postoperative year. Younger children have a higher risk of recurrence, which has been suggested to be due to a conservative initial surgical approach, thus increasing the risk of residual thyroglossal tract (12,13).

The gold standard surgical management of TGDC is the Sistrunk procedure, as it offers the best chance of a complete excision of the thyroglossal tract and cyst. In our institution, there were 11 patients who had a recurrence following a primary Sistrunk procedure, 9 of whom underwent a revision Sistrunk, with the other 2 monitored. Seven (78%) of these patients had no further recurrence over a mean follow up of 21.8±29.2 months. Two (22%) patients required further operative interventions without cure. Patients who recurred after being managed with a cystectomy or Schlange procedure (5 cases, 31%), had a subsequent Sistrunk procedure with no further recurrences. Thus, the Sistrunk procedure is a safe and effective operative intervention for patients who have a TGDC recurrence following inadequate initial resection.

The management of recurrent TGDC following Sistrunk procedure in children has been discussed, however papers are limited by patient numbers. The revision Sistrunk procedure, whereby the surgeon removes further tissue along the previous resection, has been well described in the literature. A systematic review by Ibrahim et al. (14) documented that the Sistrunk procedure was performed on 83 cases of recurrent TDGC in children, with 25 (30%) having further recurrences. In our series, 22% of patients managed with a revision Sistrunk had a further recurrence. Variations of an en block neck dissection have also been described in the literature, with an increased width of anterolateral dissection compared to the revision Sistrunk. Papers are limited by patient numbers and documentation of patient details, however the results are promising (9,15,16). A wide anterior neck dissection excising the central compartment between the lateral borders of the sternohyoid muscles has been performed in adults with recurrent TGDC, demonstrating good outcomes with minimal morbidity (17).

The subspecialty fellowship training of the primary operator has been reported to impact on the recurrence rate, with fellowship trained otorhinolaryngologists having a lower recurrence rate than fellowship trained paediatric and plastic surgeons (18). In our review, all recurrences had their initial intervention performed by fellowship trained paediatric general surgeons and in similar fashion to Geller et al. (18), a proportion of these cases had an inadequate primary resection.

Our research is limited by small patient numbers, though this is expected given the low recurrence rate of TGDC after Sistrunk procedure and the relatively low incidence of the disease. Our patient numbers are in keeping with other published articles on the subject. We are also limited by the response of patients to the questionnaire, with half of the patients not contactable or declined participation. Given the long period of time between admission and research for this paper, many patients had changed address and contact details.

Conclusions

TGDC should be managed with Sistrunk procedure to minimse the risk of recurrence. The average age of patients in our series was 5.3 years old and they typically recurred within the second postoperative year. Patients had a higher risk of cutaneous involvement when a TGDC recurred. Sistrunk procedure should be considered in the management of recurrences after a limited initial surgical excision, as there were no reported recurrences in our series. Management of recurrence following initial Sistrunk is challenging, as revision Sistrunk had a high recurrence rate and 2 (22%) patients weren’t cured despite 2 subsequent excisions. Further research into en bloc anterior neck dissections after failed Sistrunk in the paediatric population is required.

Acknowledgments

Funding: None.

Footnote

Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at http://dx.doi.org/10.21037/ajo.2018.05.02). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was conducted in accordance with the Declaration of Helsinki (as revised in 2013). The study was approved by the Sydney Children’s Hospitals Network Human Research Ethics Committee, approval number LNR/14/SCHN/181. Informed consent was waived due to the retrospective nature of the study.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

References

1. Ellis PD, van Nostrand AW. The applied anatomy of thyroglossal tract remnants. Laryngoscope 1977;87:765-70. [Crossref] [PubMed]
2. Mondin V, Ferlito A, Muzzi E, et al. Thyroglossal duct cyst: personal experience and literature review. Auris Nasus Larynx 2008;35:11-25. [Crossref] [PubMed]
3. Allard RH. The thyroglossal cyst. Head Neck Surg 1982;5:134-46. [Crossref] [PubMed]
4. Maddalozzo J, Venkatesan TK, Gupta P. Complications associated with the Sistrunk procedure. Laryngoscope 2001;111:119-23. [Crossref] [PubMed]
5. Sistrunk WE. Technique of removal of cysts and sinuses of the thyroglossal duct. Surg Gynecol Obstet 1928;46:109-12.
6. Galluzzi F, Pignataro L, Gaini RM, et al. Risk of recurrence in children operated for thyroglossal duct cysts: A systematic review. J Pediatr Surg 2013;48:222-7. [Crossref] [PubMed]
7. Hirshoren N, Neuman T, Udassin R, et al. The imperative of the Sistrunk operation: review of 160 thyroglossal tract remnant operations. Otolaryngol Head Neck Surg 2009;140:338-42. [Crossref] [PubMed]
8. Athow AC, Fagg NL, Drake DP. Management of thyroglossal cysts in children. Br J Surg 1989;76:811-4. [Crossref] [PubMed]
9. Mickel RA, Calcaterra TC. Management of recurrent thyroglossal duct cysts. Arch Otolaryngol 1983;109:34-6. [Crossref] [PubMed]
10. Rohof D, Honings J, Theunisse HJ, et al. Recurrences after thyroglossal duct cyst surgery: Results in 207 consecutive cases and review of the literature. Head Neck 2015;37:1699-704. [Crossref] [PubMed]
11. Perkins JA, Inglis AF, Sie KC, et al. Recurrent thyroglossal duct cysts: a 23-year experience and a new method for management. Ann Otol Rhinol Laryngol 2006;115:850-6. [Crossref] [PubMed]
12. Ducic Y, Chou S, Drkulec J, et al. Recurrent thyroglossal duct cysts: a clinical and pathologic analysis. Int J Pediatr Otorhinolaryngol 1998;44:47-50. [Crossref] [PubMed]
13. Marianowski R, Ait Amer JL, Morisseau-Durand MP, et al. Risk factors for thyroglossal duct remnants after Sistrunk procedure in a pediatric population. Int J Pediatr Otorhinolaryngol. 2003;67:19-23. [Crossref] [PubMed]
14. Ibrahim FF, Alnoury MK, Varma N, et al. Surgical management outcomes of recurrent thyroglossal duct cyst in children--A systematic review. Int J Pediatr Otorhinolaryngol 2015;79:863-7. [Crossref] [PubMed]
15. Howard DJ, Lund VJ. Thyroglossal ducts, cysts and sinuses: a recurrent problem. Ann R Coll Surg Engl 1986;68:137-8. [PubMed]
16. Patel NN, Hartley BE, Howard DJ. Management of thyroglossal tract disease after failed Sistrunk's procedure. J Laryngol Otol 2003;117:710-2. [Crossref] [PubMed]
17. O'Neil LM, Gunaratne DA, Cheng AT, et al. Wide anterior neck dissection for management of recurrent thyroglossal duct cysts in adults. J Laryngol Otol 2016;130:S41-4. [Crossref] [PubMed]
18. Geller KA, Cohen D, Koempel JA. Thyroglossal duct cyst and sinuses: a 20-year Los Angeles experience and lessons learned. Int J Pediatr Otorhinolaryngol 2014;78:264-7. [Crossref] [PubMed]