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Case Report
Revised

Case Report: Severe acute respiratory distress by tracheal obstruction due to a congenital thyroid teratoma.

[version 3; peer review: 2 approved]
Previously titled: Case Report: Severe acute respiratory distress by tracheal obstruction due to a congenital thyroid teratoma: a case report and literature review.
PUBLISHED 22 Oct 2015
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Abstract

Congenital teratoma is a rare condition and is a germ cell tumor composed of elements from one or more of the embryonic germ layers and contain tissues usually foreign to the anatomic site of origin. We report a case of a neck tumor diagnosed during pregnancy, initially thought to be a goiter. After birth the neck mass kept growing until it compressed the trachea and produced respiratory failure. The infant had a difficult tracheal intubation because of the compressing mass. The staff decided to surgically remove the neck mass. After that, the infant became eupneic. The histological analysis showed a mature teratoma with no atypias.

Keywords

thyroid teratoma, pediatric neck mass, pediatric respiratory distress, hypothyroidism, children case report

Revised Amendments from Version 2

In this new version, we discuss more properly the management of the unexpected difficult pediatric airway, the need for a pediatric protocol for the difficult airway and a backup plan (extracorporeal life support) when everything fails. Two new references concerning the management of the pediatric difficult airway were also included.

See the authors' detailed response to the review by Francisco Eulógio Martinez and Walusa Assad Gonçalves-Ferri

Introduction

Congenital thyroid teratoma is a rare condition1,2. We report a case of an infant with a neck mass diagnosed by ultrasound during pregnancy which was initially supposed to be a congenital goiter. Two doses of levothyroxine were administered into the amniotic fluid. The goiter kept growing after birth until it caused severe respiratory distress by compressing the trachea, requiring immediate tracheal intubation. The tumor was surgically resected and the patient went eupneic for the first time in his life. The histological analysis demonstrated a mature teratoma with no atypias. Thyroid hormone substitute therapy was started and the infant is thriving well.

Case report

A 2-months-and-20-days-old Brazilian white male infant weighing 4.2 kg was admitted to the pediatric intensive care unit of our hospital (Santa Catarina Hospital, São Paulo, Brazil) in acute respiratory distress and was immediately intubated and placed in mechanical ventilation.

From a routine ultrasound during pregnancy, the fetus had been diagnosed with a cervical mass, considered initially to be a goiter (Figure 1) by doctors at another institution. Family history of the mother uncovered a cousin with hypothyroidism. The mother was previously healthy, but after diagnosis of the cervical mass of the fetus, she was tested for thyroid hormones and had hypothyroidism diagnosed during pregnancy (TSH: 5.0 mUI/mL – normal: 0.2 to 3.0 mUI/mL; free T4: 0.7 ng/dL – normal: 0. To 1.3 ng/dL; antithyroglobulin antibodies: 65 U/mL – normal: inferior to 60 U/mL and thyroid antiperoxidase antibodies: 166 UI/mL – normal: inferior to 9 UI/mL). Two single doses of 200µg of levothyroxine were administered into the amniotic fluid, one during the 28th and one during the 31st week of pregnancy, in order to treat the supposed fetal thyroid hormone deficiency. Chorioamnionitis appeared after the second levothyroxine administration which triggered a premature cesarean birth which was undertaken in the other hospital. The premature newborn had sepsis due to maternal infection (chorioamnionitis) and remained in mechanical ventilation for 10 days. After tracheal extubation, he remained in nasal continuous positive airway pressure (CPAPn) for 7 more days, and after that was kept on oxygen therapy for 10 days. He was discharged from the hospital 50 days after birth, still presenting with a laryngeal stridor that was attributed to tracheal malacia by the doctors that initially treated the patient.

b719d76c-2099-42b9-9cf9-f604d9df96ae_figure1.gif

Figure 1. Prenatal ultrasound showing the neck mass.

After hospital discharge, he was observed by a pediatric endocrinologist who started research on thyroid disorders. Meanwhile, the infant maintained a euthyroid state, receiving no treatment, waiting for more investigation on the cause of the neck mass. However, the cervical mass kept visibly growing, was palpable and the infant presented a laryngeal stridor that was still attributed, by the pediatrician who followed the infant, to laryngomalacia. In the few days preceding hospitalization at our institution, the infant became increasingly dispneic each day, as related by his mother. One day, after choking and vomiting during breastfeeding he became hypotonic and went into acute respiratory distress.

He was admitted to our pediatric intensive care unit 25 days after he had been discharged from the other hospital, and was immediately intubated. An X-ray showed a small amount of interstitial infiltrate, compatible with aspiration pneumonia. However, the respiratory distress was attributed mainly to an upper airway obstruction. It was difficult to tracheally intubate the infant; only an uncuffed 2.5 mm endotracheal tube (ETT) was able to be inserted into the trachea and it was difficult to place this in the right position. The X-ray after intubation showed the ETT in a high position and the trachea displaced to the right (Figure 2). Magnetic resonance imaging (MRI) revealed the extent of the cervical mass and its compression on the trachea, and the latter’s subsequent displacement (Figure 3a and 3b).

b719d76c-2099-42b9-9cf9-f604d9df96ae_figure2.gif

Figure 2. Endotracheal tube displaced to the right position.

b719d76c-2099-42b9-9cf9-f604d9df96ae_figure3.gif

Figure 3.

a. Sagittal MRI (T2) of the neck showing the teratoma. b. Axial MRI (T2) of the neck showing the teratoma and the tracheal displacement.

Meanwhile, we started investigation into the cause of the neck mass and performed blood tests on the infant: thyroid hormones were in the normal range (free thyroxine (T4): 1.3 ng/dL and thyroid-stimulating hormone (TSH): 4.7 ng/dL). Calcitonin levels, for investigations into potential malignance, were normal (calcitonin: 21 pg/mL), as was the alpha-fetoprotein: 505 µg/L.

We decided to remove the cervical mass, since it was causing the tracheal obstruction. The surgery lasted 35 minutes and was uneventful. The mass was well circumscribed and could be easily dissected, weighed 20 grams and measured 33×61×45 mm Figure 4. The infant returned from surgery in good condition. A bronchoscopy was performed the next day after surgery, during tracheal extubation, which revealed no malacia or any other disorders on the trachea or the upper respiratory tract. The patient has been eupneic since then. The histological analysis revealed a mature teratoma with no atypias or signs of malignancy.

b719d76c-2099-42b9-9cf9-f604d9df96ae_figure4.gif

Figure 4. The resected benign teratoma.

Levothyroxine was started (25µg, once a day) as thyroid hormone substitute therapy and the infant is thriving well according to the pediatric endocrinologist that continues following the patient.

Discussion

Teratomas originate from multipotent primitive germ cells and result in different tissues, diverging from the anatomical site of origin2,3. They are most common during early childhood and the most common location is the sacrococcygeal region in children and the gonadal region in adults24. The frequency of these embryonic tumours is about 1:20,000–40,000 live births. However, only 1.5% to 5.5% of all pediatric teratomas are placed in the neck region. These tumours are usually solitary, with no other associated congenital malformations or chromosomal abnormalities4. Although 95% of all teratomas are benign, the cervical teratomas if not properly treated, lead to death in 80% of the cases due to obstructive respiratory distress3,4,7.

In this case, the acute clinical presentation of the neck mass with severe respiratory distress, requiring ready intervention and immediate tracheal intubation should alert all pediatricians to the risk of these neck masses, and consider it as a potentially fatal case. Successful management of the airway is the first priority when caring for children with respiratory distress and potential difficult airway. While routine airway management in healthy pediatric patients is normally easy in experienced hands, the management of an unexpected difficult airway requires a protocol, since the development of rapid hypoxemia and respiratory acidosis requires time critical, clear concepts and strategies to avoid the worst outcome. There are some proposed protocols for pediatric practice published5 derived from the Difficult Airway Society (DAS) algorithms for adults6. In this case, the cause of the difficult airway is the upper airway obstruction, which is most often a medical emergency requiring rapid evaluation with simultaneous therapy to ensure adequate ventilation and oxygenation. And in most cases there is not enough time for x-rays or laboratory tests, then the diagnosis is almost always clinical. Many different conditions lead to a difficult airway due to obstruction like croupe, epigottites, retropharyngeal or perytonsilar abcess, foreign body, laryngeal diphtheria, angioneurotic edema, laryngomalacia, choanal atresia, subglottic hemangioma and subglottic stenosis. Once initial assessment is over, the most skilled and experienced personnel available are gathered to stabilize the airway. Bag-mask ventilation may be of particular value in stenting open the airway until direct laryngoscopy and endotraqueal intubation are performed. If it is available, a video laryngoscopy device should be used. If there is a failure in intubation, a bedside thaqueostomy or cricothyroidotomy or even a neddle thyroidotomy shoud be performed. In a worst case scenario, when none of the alternatives to ensure a patent airway in the patient is succesful, the use of extracorporeal life support could be life saving. In the presented case, the cause of the difficult airway was the mechanical obstruction caused by the teratoma and the intubation was performed by an experienced member of the staff who was succesful on the first attempt.

The surgical removal of the neck mass was both diagnostic and therapeutic.

Conclusion

Thyroid teratoma is rare in infants, it is usually benign, and can cause airway compression depending on the site and size of the mass. The likelihood of a malignant thyroid teratoma is low in infants, however it could be fatal by causing upper airway obstruction. The use of a protocol for the management of difficult pediatric airway is desirable and prevents the outcome. In this case, surgical resection was required both for diagnosis and treatment. If the surgical removal is a success, the long-term outcome and quality of life should be good7.

Consent

Written informed consent was obtained from parents of the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor of this journal.

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Version 3
VERSION 3 PUBLISHED 22 Jun 2015
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Colleti Junior J, Tannuri U, Monti Lora F et al. Case Report: Severe acute respiratory distress by tracheal obstruction due to a congenital thyroid teratoma. [version 3; peer review: 2 approved]. F1000Research 2015, 4:159 (https://doi.org/10.12688/f1000research.6589.3)
NOTE: If applicable, it is important to ensure the information in square brackets after the title is included in all citations of this article.
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ApprovedThe paper is scientifically sound in its current form and only minor, if any, improvements are suggested
Approved with reservations A number of small changes, sometimes more significant revisions are required to address specific details and improve the papers academic merit.
Not approvedFundamental flaws in the paper seriously undermine the findings and conclusions
Version 3
VERSION 3
PUBLISHED 22 Oct 2015
Revised
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Reviewer Report 10 Nov 2015
Daniel Garros, Pediatric Intensive Care Unit, Stollery Children’s Hospital, Edmonton, AB, Canada 
Approved
VIEWS 7
I confirm that I have read this submission and believe that I have an ... Continue reading
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Garros D. Reviewer Report For: Case Report: Severe acute respiratory distress by tracheal obstruction due to a congenital thyroid teratoma. [version 3; peer review: 2 approved]. F1000Research 2015, 4:159 (https://doi.org/10.5256/f1000research.7787.r11130)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
Version 2
VERSION 2
PUBLISHED 23 Jul 2015
Revised
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Reviewer Report 30 Sep 2015
Daniel Garros, Pediatric Intensive Care Unit, Stollery Children’s Hospital, Edmonton, AB, Canada 
Approved with Reservations
VIEWS 11
The authors describe an interesting case, with a positive outcome despite some delay in diagnosis.
The title and the description of the case are all well written.

However, the discussion and the conclusion require more work. This case brings up an important ... Continue reading
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CITE
HOW TO CITE THIS REPORT
Garros D. Reviewer Report For: Case Report: Severe acute respiratory distress by tracheal obstruction due to a congenital thyroid teratoma. [version 3; peer review: 2 approved]. F1000Research 2015, 4:159 (https://doi.org/10.5256/f1000research.7320.r10593)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
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Reviewer Report 25 Aug 2015
Francisco Eulógio Martinez, Department of Pediatrics, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Ribeirão Preto, Brazil 
Walusa Assad Gonçalves-Ferri, Department of Pediatrics, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Ribeirão Preto, Brazil 
Approved
VIEWS 19
Coletti et al described in a case report of severe acute respiratory distress by tracheal obstruction due to congenital thyroid teratoma. This case report adds knowledge to the literature, not only by tumor description, but also the clinical management of ... Continue reading
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HOW TO CITE THIS REPORT
Eulógio Martinez F and Gonçalves-Ferri WA. Reviewer Report For: Case Report: Severe acute respiratory distress by tracheal obstruction due to a congenital thyroid teratoma. [version 3; peer review: 2 approved]. F1000Research 2015, 4:159 (https://doi.org/10.5256/f1000research.7320.r9516)
NOTE: it is important to ensure the information in square brackets after the title is included in all citations of this article.
  • Author Response 10 Sep 2015
    Jose Colleti Junior, Pediatric Intensive Care Unit, Santa Catarina Hospital, São Paulo, 01310-000, Brazil
    10 Sep 2015
    Author Response
    I would like to thank Dr Martinez and colleague for the remarks and suggestions above.

    Answering the questions regarding this case report:
    1. The patient was born with 31 weeks of gestational age,
    ... Continue reading
COMMENTS ON THIS REPORT
  • Author Response 10 Sep 2015
    Jose Colleti Junior, Pediatric Intensive Care Unit, Santa Catarina Hospital, São Paulo, 01310-000, Brazil
    10 Sep 2015
    Author Response
    I would like to thank Dr Martinez and colleague for the remarks and suggestions above.

    Answering the questions regarding this case report:
    1. The patient was born with 31 weeks of gestational age,
    ... Continue reading

Comments on this article Comments (0)

Version 3
VERSION 3 PUBLISHED 22 Jun 2015
Comment
Alongside their report, reviewers assign a status to the article:
Approved - the paper is scientifically sound in its current form and only minor, if any, improvements are suggested
Approved with reservations - A number of small changes, sometimes more significant revisions are required to address specific details and improve the papers academic merit.
Not approved - fundamental flaws in the paper seriously undermine the findings and conclusions
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