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Clinical Challenge
September 2016

Congenital Neck Mass

Author Affiliations
  • 1Department of Otolaryngology–Head and Neck Surgery, University of Iowa Hospitals and Clinic, Iowa City
JAMA Otolaryngol Head Neck Surg. 2016;142(9):907-908. doi:10.1001/jamaoto.2016.0007

An ex utero intrapartum treatment (EXIT) procedure was performed for a neonate born by cesarean delivery at 37 weeks of gestation with a prenatally diagnosed neck mass. Initial laryngoscopy with a Parsons laryngoscope identified airway deviation secondary to mass effect, with intubation ultimately achieved with assistance of a bronchoscope. Examination revealed a large neck mass extending from the mandible to the clavicles, with mass effect causing neck extension and rotation. The mass was multicystic, with prominent vasculature, and a small area of skin necrosis. Following birth, the mass continued to grow, with inability to palpate the sternal notch by day of life 4 (Figure, A). The patient underwent a magnetic resonance image (MRI) on day of life 3 which showed a 15.7 × 10.0 × 14.4-cm midline neck mass with solid and cystic components. Flow voids were present throughout the mass. The mass was isointense to hypointense on T1, and isointense to hyperintense on T2. The patient also underwent a computed tomographic (CT) scan, which demonstrated calcifications present within the multicystic neck mass. Owing to continued growth and risk of airway compromise, the patient was taken to the operating room on day 5 of life for resection of the mass and tracheostomy. Pathologic results showed a multinodular 19-cm mass, 80% solid and 20% cystic, with multiple tissue types present (Figure, B-D).

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