International Journal of Pediatric Otorhinolaryngology Extra 5 (2010) 138–140

Case report

The thyroglossal duct cyst and respiratory failure in neonates: Case report andreview of an unusual entity

Matthew Barton a,*, Andrew Bozeman b, Mark Herndon b, Rogelio dela Cruz c

a Center for Sensory Biology, Department of Neuroscience, Johns Hopkins Medical Institute, Baltimore, MD, USAb Department of Surgery, Medical Center of Central Georgia, Mercer University School of Medicine, Macon, GA, USAc Department of Pediatrics, Critical Care Division, Medical Center of Central Georgia, Mercer University School of Medicine, Macon, GA, USA

A R T I C L E I N F O

Article history:

Received 26 September 2008

Received in revised form 23 July 2009

Accepted 29 July 2009

Available online 20 October 2009

Keywords:

Thyroglossal duct cyst

Neonate

Group B streptococcus

Respiratory failure

A B S T R A C T

Thyroglossal duct cysts are unusual neck lesions in neonates. Most cysts are first noticed in preschool-

aged children as a small midline neck swelling, and can become infected causing inflammation,

erythema, and external drainage. In this patient population respiratory symptoms are frequently part of

the initial presentation, and respiratory failure due to cyst mass effect is often fatal in newborns. The case

presented here is unusual in terms of age at presentation (4 days), type of infecting bacteria (GBS), rapid

cyst enlargement, and prominent respiratory symptoms (previously unreported in cysts inferior to hyoid

bone). Although rare, TGDCs should be included in the differential diagnosis of congenital neck masses or

unexplained respiratory compromise in neonates, especially when the presentation includes positional,

intermittent, or progressively worsening obstructive respiratory symptoms. As this case illustrates,

infection of these cysts is common but does not always manifest with visible neck inflammation and

swelling. With rapid diagnosis the potentially fatal complications of TGDCs can be avoided in neonatal

patients.

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1. Case presentation

A 4-day-old African-American female infant presented to anoutside hospital emergency department (ED) with a 3-day historyof irritability and fever. Initial evaluation revealed a rectaltemperature of 100.9 8F, and during examination an episode ofoxygen desaturation below 80% (patient on room air) wasobserved. After initial stabilization measures and presumedneonatal sepsis, the patient was transferred to a pediatric tertiarycare facility for further evaluation and treatment.

History taken from the patient’s mother included an uncompli-cated full term pregnancy and vaginal delivery. She reported regularpre-natal care but was unsure of her GBS status. Both the mother andpatient were discharged after standard 24-h postpartum care.However, soon after arriving home, the patient noticeably developedincreasing irritability manifested by constant crying. On patient’sthird day of life, her mother reported that she developed fever with atemperature of 101.7 8F rectally not associated with any changes inappetite, bowel movement or urine output.

The patient’s vital signs upon arrival were as follows:temperature of 99 8F, heart rate of 135 BPM, blood pressure of75/31 mmHg, respiratory rate of 35 CPM, and oxygen saturation of

* Corresponding author.

E-mail address: mbarton786@gmail.com (M. Barton).

1871-4048/$ – see front matter � 2009 Elsevier Ireland Ltd. All rights reserved.

doi:10.1016/j.pedex.2009.07.006

100% on room air. Physical examination on admission revealed anawake, alert, and active neonate in no acute distress. There were nodysmorphic features noted. Neck was supple with no visibleabnormality. Auscultation of the lungs revealed no rales orwheezing. Cardiac and abdominal examinations were withinnormal limits. Strong and equal pulses were noted in allextremities, with no extremity deformities noted.

Full sepsis evaluation was performed with the following results:complete blood count with differential revealed normal hemoglo-bin, hematocrit, and platelets with a WBC of 21.7 including 46%neutrophils, 3% bands, 3% metamyelocytes, 16% lymphocytes, 30%monocytes, 2% eosinophils, and no basophils. Urinalysis and CSFgram stain were normal. Urine, blood, and CSF cultures werenegative after 24 h. Influenza and RSV antigen testing were negative.Chest X-ray was normal. Empiric antibiotic therapy using Ampicillinand Gentamycin was initiated after sepsis work-up was complete.Thirteen hours after transfer into our care, the patient experiencedan episode of desaturation below 80% with respiratory distress andaudible stridor. Initially respiratory improvement was observedwith repositioning of the neck and administration of oxygen by nasalcannula. However, a second desaturation event was observed soonthereafter, with worsening stridor and retractions. Further exam-ination at this time revealed a large, cystic, moveable mass on theanterior aspect of the neck slightly left of midline. Repeat portablechest X-ray indicated significant displacement of the trachea to theright. The patient was transferred to the pediatric intensive care unit

Fig. 1. Coronal view of neck CT scan.

Fig. 3. Intra-op aspiration of purulent fluid.

M. Barton et al. / International Journal of Pediatric Otorhinolaryngology Extra 5 (2010) 138–140 139

and was placed on Heliox. She was later electively intubateddue to recurrent stridor and desaturation. Neck CT revealed4.0 cm� 2.5 cm cystic lesion with an air-fluid level located in theanterior triangle of the neck just left of midline (Figs. 1 and 2).Thyroid function studies were within normal limits. The patient wastaken to the operating room the next day for neck exploration.Intraoperatively, a cystic mass was exposed, and from it more than20 ml of thick, green, foul smelling, purulent fluid was aspirated(Fig. 3). The mass was surgically removed by Sistrunk procedure dueto suspicion of a thyroglossal duct cyst (Fig. 4). Gram stain of theaspirate revealed numerous white blood cells with many Gram-positive and Gram-negative organisms; culture grew Group Bstreptococcus after 24 h. Pathologic evaluation identified the massas a thyroglossal duct cyst.

2. Introduction

TGDCs are well known, oft-encountered lesions in pediatric andotolaryngology practice. Thyroglossal duct cysts are one of themost common causes of pediatric neck masses, second only tolymphadenopathies [1]. The thyroglossal duct typically involutesbetween 5 and 10 weeks of gestation [2], but failure of ductobliteration occurs in approximately 7% of the population [3]. Ofthe TGDCs that do become clinically obvious the large majority doso before the age of 4 years, but rarely in the neonatal period.Despite the relative paucity of neonatal presentation of TGDCs,those cysts that do present at this age are responsible for aconsiderable portion of the morbidity and mortality associatedwith TGDCs. Cysts located in the lingual area are often fatal andshould be recognized early [4]. Six of 15 patients with cysts located

Fig. 2. Sagittal view of neck CT scan.

at the base of the tongue died due to upper airway obstruction.Three of these deaths were initially attributed to sudden infantdeath syndrome, indicating the difficult diagnosis that theseinfrequent cysts represent [5].

3. Clinical manifestations and diagnosis

In infants, respiratory symptoms are the most common causes ofpresentation to a medical facility. Stridor, apnea, dyspnea, respira-tory distress and/or failure are possible on presentation. Diaz et al.recently published a case involving a 27-day-old infant whopresented with apnea, cyanosis, and stridor after extubation.Laryngoscopy revealed compression of the epiglottis by a roundsoft tissue mass, indicated by CT as a thyroglossal duct cyst betweenthe base of the tongue and hyoid bone [5]. In a similar case publishedby Lofgren, the patient presented with chronic cough and wheezingassociated with poor weight gain, vomiting, and feeding difficulty.The infant was initially treated for respiratory infection until anotolaryngologist noted the presence of a midline mass at the base ofthe tongue. Presentation differs substantially in older children andadults, with secondary infection and cosmetic defects as thepredominant reasons for seeking medical attention [4].

Diagnosis can be extremely difficult without any visible orpalpable midline neck mass. In patients with history and physicalexamination highly suggestive of TGDC, ultrasound examination isthe most useful and least invasive tool in defining the consistencyof the mass and analyzing the surrounding anatomy [4]. Soft tissuefilms of the neck and barium swallow studies are also minimally

Fig. 4. Gross appearance of mass after excision.

M. Barton et al. / International Journal of Pediatric Otorhinolaryngology Extra 5 (2010) 138–140140

invasive, but can be difficult to interpret. Computed tomographyand magnetic resonance imaging provide the best detail of TGDCsin terms of anatomical location, margins, and compression ofsurrounding structures, but both require a cooperative or sedatedpatient. Obviously, these requirements may be difficult to achievein patients with a compromised airway [5]. For suspected lingualcases, direct visualization using nasopharyngeal laryngoscopyshould be performed emergently, especially in infants presentingwith unexplained upper airway obstructive symptoms.

4. Surgical management

It is important to note that for many TGDCs prompt surgicalintervention may not be indicated, as many acutely infectedTGDCs can be managed with antibiotics and needle aspirationwith deferment of Sistrunk until the patient and surgical site aremore stable. Open incision and drainage is to be avoided as thismay rupture the capsule and lead to iatrogenic seeding ofcontiguous sterile ductal epithelium, thereby increasing therecurrence rate. Surgical therapy is reserved for instances ofincreasing cyst size, the risk for cyst infection, recurrentrespiratory compromise due to cyst mass effect, or the presenceof carcinoma [6]. While only 10% of ectopic thyroid tissue isconfined to the neck, it may represent the only thyroid tissue in75% of these patients [7]. Because of the possibility of a medianectopic thyroid within the duct itself, some experts recommendperforming a pre-operative thyroid scintigraphy and baseline TSHlevel to prevent the repercussions of surgical removal of thepatient’s only functioning thyroid tissue [1].

5. Summary

The literature to date describes very few TGDCs presenting withrespiratory distress or failure in neonates. Of those described, allare attributed to TGDCs causing posterior deflection of theepiglottis and subsequent intermittent respiratory compromise.This type of thyroglossal duct cyst is known to represent only 1–2%of all thyroglossal duct lesions, and many such cases are fatal [5].While the case reported here is unique and uncommon in severalrespects, it nevertheless highlights the importance of recalling thepotentially lethal aspects of TGDCs presenting in the neonatalperiod and the corresponding necessity of rapid diagnosis.

References

[1] S.P. Acierno, J.H. Waldhausen, Congenital cervical cysts, sinuses, and fistulae,Otolaryngol. Clin. North Am. 40 (February (1)) (2007) 161–176, vii–viii.

[2] R.H. Allard, The thyroglossal cyst, Head Neck Surg. 5 (November–December (2))(1982) 134–146.

[3] J.J. Nelson, D.A. Hall, G.T. Wolf, M.E. Prince, An unusual thyroglossal duct cystinfection with coccidioidomycosis, J. Otolaryngol. 36 (February (1)) (2007) 69–71.

[4] R. Lofgren, Respiratory distress from congenital lingual cysts, Am. J. Dis. Child. 106(December) (1963) 610–612.

[5] M.C. Diaz, A. Stormorken, N.C. Christopher, A thyroglossal duct cyst causing apneaand cyanosis in a neonate, Pediatr. Emerg. Care 21 (January (1)) (2005) 35–37.

[6] B.W. Warner, Pediatric surgery, in: C.M. Townsend, Jr., R.D. Beauchamp, B.M.Evers, K.L. Mattox (Eds.), Sabiston Textbook of Surgery: The Biological Basis ofModern Surgical Practice, 18th ed., Saunders Elsevier, Philadelphia, 2008 , pp.2053–2054.

[7] R.F. Wetmore, W.P. Potsic, Differential diagnosis of neck masses, in: C.W. Cum-mings, P.W. Flint, L.A. Harker, B.H. Haughey, M.A. Richardson, K.T. Robbins, et al.(Eds.), Otolaryngology—Head & Neck Surgery, 4th ed., Elsevier Mosby, Philadel-phia, 2005, pp. 4213–4214.