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Congenital Cystic Neck Mass in a 2-Month-Old Infant

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 2-month-old, full-term female presented with a left upper neck mass that was first noted at birth. Her parents reported no fevers, constitutional symptoms, or enlargement of the mass. On physical examination, there was a nontender 4-cm mass in the left submandibular region with no overlying skin changes. Ultrasonography results showed an avascular cystic structure with internal debris and a single thin septation. Contrast-enhanced computed tomography (CT) of the neck soft tissue revealed a 3-cm low attenuation, nonenhancing, cystic-appearing mass that elevated the submandibular gland to the level of the parapharyngeal fat space with medial extension of the lesion into the tongue musculature. The walls were thin except for mildly thickened capsule along the posterior aspect of the lesion (Figure, A and B). Fluid that aspirated from the cyst was serous, and cytology showed mature and anucleate squamous cells with acute inflammation. The mass returned after 3 weeks. Further analysis 8 months after the initial CT via contrast-enhanced magnetic resonance imaging (MRI) showed a large, multiseptated cystic structure (Figure, C); the capsule of the wall was mildly thickened and was contrast enhancing. At age 12 months, the mass was excised via neck dissection. A lateral component at the level of the sternocleidomastoid muscle was completely excised. An intraoperative rupture of the thin capsule caused some spillage of cystic contents that were milky white in some areas and mucinous in others.

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A 2-month-old, full-term female presented with a left upper neck mass that was first noted at birth. Her parents reported no fevers, constitutional symptoms, or enlargement of the mass. On physical examination, there was a nontender 4-cm mass in the left submandibular region with no overlying skin changes. Ultrasonography results showed an avascular cystic structure with internal debris and a single thin septation. Contrast-enhanced computed tomography (CT) of the neck soft tissue revealed a 3-cm low attenuation, nonenhancing, cystic-appearing mass that elevated the submandibular gland to the level of the parapharyngeal fat space with medial extension of the lesion into the tongue musculature. The walls were thin except for mildly thickened capsule along the posterior aspect of the lesion (Figure, A and B). Fluid that aspirated from the cyst was serous, and cytology showed mature and anucleate squamous cells with acute inflammation. The mass returned after 3 weeks. Further analysis 8 months after the initial CT via contrast-enhanced magnetic resonance imaging (MRI) showed a large, multiseptated cystic structure (Figure, C); the capsule of the wall was mildly thickened and was contrast enhancing. At age 12 months, the mass was excised via neck dissection. A lateral component at the level of the sternocleidomastoid muscle was completely excised. An intraoperative rupture of the thin capsule caused some spillage of cystic contents that were milky white in some areas and mucinous in others.

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Article Information

Corresponding Author: Alexandra Espinel, MD, Department of Otolaryngology–Head & Neck Surgery, Children’s National Hospital, 111 Michigan Ave NW, Washington, DC 20011 (AEspinel@childrensnational.org).

Published Online: June 9, 2022. doi:10.1001/jamaoto.2022.1279

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s mother for granting permission to publish this information.

References
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Quintanilla-Dieck  L , Penn  EB  Jr .  Congenital neck masses.   Clin Perinatol. 2018;45(4):769-785. doi:10.1016/j.clp.2018.07.012PubMedGoogle ScholarCrossref
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Kieran  SM , Robson  CD , Nosé  V , Rahbar  R .  Foregut duplication cysts in the head and neck: presentation, diagnosis, and management.   Arch Otolaryngol Head Neck Surg. 2010;136(8):778-782. doi:10.1001/archoto.2010.127PubMedGoogle ScholarCrossref
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Edwards  RM , Chapman  T , Horn  DL , Paladin  AM , Iyer  RS .  Imaging of pediatric floor of mouth lesions.   Pediatr Radiol. 2013;43(5):523-535. doi:10.1007/s00247-013-2620-6PubMedGoogle ScholarCrossref
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Gantwerker  EA , Hughes  AL , Silvera  VM , Vargas  SO , Rahbar  R .  Management of a large antenatally recognized foregut duplication cyst of the tongue causing respiratory distress at birth.   JAMA Otolaryngol Head Neck Surg. 2014;140(11):1065-1069. doi:10.1001/jamaoto.2014.2331PubMedGoogle ScholarCrossref
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Haber  MA , Jaimes  C , Lee  EY , Juliano  AF .  Pediatric tongue lesions: an often-overlooked but important collection of diagnoses.   AJR Am J Roentgenol. 2020;214(5):1008-1018. doi:10.2214/AJR.19.22121PubMedGoogle ScholarCrossref
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Dremmen  MH , Tekes  A , Mueller  S , Seyfert  D , Tunkel  DE , Huisman  TA .  Lumps and bumps of the neck in children-neuroimaging of congenital and acquired lesions.   J Neuroimaging. 2016;26(6):562-580. doi:10.1111/jon.12376PubMedGoogle ScholarCrossref
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Zenga  J , Lin  DT , Deschler  DG . Neck masses. In: Lalwani  AK , ed.  Current Diagnosis & Treatment Otolaryngology–Head and Neck Surgery. 4th edition. McGraw Hill; 2020. Accessed May 09, 2022. https://accessmedicine.mhmedical.com/content.aspx?bookid=2744&sectionid=229673422
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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