Congenital Nasal Masses and Anomalies

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Congenital Nasal Masses and Anomalies

Pediatric Otolaryngology
Author Credentials 

William O. Collins, MD
Associate Professor
Department of Otolaryngology
University of Florida College of Medicine
Gainesville, FL

Module Summary

Although relatively rare, disruptions in embryologic development can result in a variety of congenital nasal lesions. These primarily result in airway obstruction, either at the front of the nose (pyriform aperture stenosis), the posterior nasal cavity (choanal atresia), or sometimes within the nasal cavity (gliomas or encephaloceles). External nasal lesions are also possible (nasal dermoids). An understanding of the embryologic development of the nose and potential disruptions is important in understanding the surgical options to treat these patients, as medical options are limited. Preoperative recognition of possible intracranial extension is critical to ensure that necessary neurosurgical support is available if needed.

Module Learning Objectives 
  1. Review the etiology of congenital masses.
  2. Describe the anatomic variations present in different types of congenital nasal masses.
  3. Explain surgical treatment options and their associated strategies as they relate to the etiology and anatomic findings of the congenital nasal mass.

 

Embryology

Learning Objectives 

To understand the normal embryologic development of the nose and nasal cavities, and potential perturbations leading to development of:

  1. Pyriform aperture stenosis
  2. Choanal atresia
  3. Midline nasal masses:
    1. Dermoid cysts/sinuses
    2. Glioma
    3. Encephalocele/Meningocele/Meningomylocele

 

References 
  1. Hengerer AS, Wein RO. Congenital Malformations of the Nose and the Paranasal Sinuses. In Pediatric Otolaryngology,fourth edition. Bluestone CD, Stool SE, Alper CM, et al. Saunders. Philadelphia, PA; 2009:979-994.
  2. Brown OE, Myer CMD, Manning SC. Congenital nasal pyriform aperture stenosis. Laryngoscope. 1989;99:86-91.
  3. Cotton RT, Myer CM. Practical pediatric otolaryngology. Philadelphia: Lippincott-Raven; 1999:1011.

Anatomy

Learning Objectives 

To describe the anatomic findings of congenital nasal masses and anomalies:

  1. Pyriform aperture stenosis
  2. Choanal atresia
  3. Midline nasal masses:
    1. Dermoid cysts/sinuses
    2. Glioma
    3. Encephalocele/Meningocele/Meningomylocele

 

References 
  1. Hengerer AS, Wein RO. Congenital Malformations of the Nose and the Paranasal Sinuses. In Pediatric Otolaryngology, fourth edition. Bluestone CD, Stool SE, Alper CM, et al. Saunders. Philadelphia, PA; 2003:979-994.
  2. Brown OE, Pownell P, Manning SC. Choanal atresia: a new anatomic classification and clinical management applications. Laryngoscope. 1996;106:97-101.
  3. Cotton RT, Myer CM. Practical pediatric otolaryngology. Philadelphia: Lippincott-Raven; 1999:1011.

Incidence

Learning Objectives 

To relate the relative incidences of congenital nasal masses.

References 
  1. Hengerer AS, Wein RO. Congenital Malformations of the Nose and the Paranasal Sinuses. In Pediatric Otolaryngology, fourth edition. Bluestone CD, Stool SE, Alper CM, et al. Saunders. Philadelphia, PA; 2003:979-994.

Genetics

Learning Objectives 

To identify any relevant genetic syndromes associated with congenital nasal masses.

References 
  1. Schraff SA, Vijayasekaran S, Meinzen-Derr J, Myer CM. Management of choanal atresia in CHARGE association patients: A retrospective review. Int J Pediatr Otolaryngol. 2006;70:1291-1297.

Patient Evaluation

Learning Objectives 

To be able to perform a thorough history and physical examination in pediatric patient with congenital nasal anomalies.

 

References 
  1. Cotton RT, Myer CM. Practical pediatric otolaryngology. Philadelphia: Lippincott-Raven; 1999. p. xix, 1011.
  2. Hengerer AS, Wein RO. Congenital Malformations of the Nose and the Paranasal Sinuses. In Pediatric Otolaryngology, fourth edition.  Bluestone CD, Stool SE, Alper CM, et al. Saunders. Philadelphia, PA. 2003. 979-994.
  3. Adil E, Robson C, Perez-Atayde A, Heffernan C, Moritz E, Goumnerova L, Rahbar R. Congenital Nasal Neuroglial Heterotopia and Encephaloceles: An Update on Current Evaluation and Management.  Laryngoscope. 2016;126(9):2161-7.
  4. Van Den Abbeele T, Triglia JM, Francois M, Narcy P. Congenital Nasal Pyriform Aperture Stenosis: Diagnosis and Management of 20 Cases. Ann Otol Rhinol Laryngol. 2001;110:70-75.

Imaging

Learning Objectives 

To describe the best imaging techniques and indications for each, depending on suspected etiology of the nasal lesion.

  1. CT scanning
  2. MRI imaging

 

References 
  1. Saettele M, Alexander A, Markovich B, Morelli J, Lowe LH. Congenital midline nasofrontal masses. Pediatr Radiol. 2012;42:1119-1125.
  2. Hedllund G. Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatr Radiol. 2006;36:647-662.

Pathology

Learning Objectives 

To understand the histologic features of each type of congenital nasal mass, particularly as it pertains to their etiology and embryologic development.

References 
  1. Hengerer AS, Wein RO. Congenital Malformations of the Nose and the Paranasal Sinuses. In Pediatric Otolaryngology, fourth edition. Bluestone CD, Stool SE, Alper CM, et al. Saunders. Philadelphia, PA; 2003:979-994.

Treatment

Learning Objectives 

To describe treatment strategies and timing of any interventions.

2015 Pediatric Otolaryngology Review Course

References 
  1. Cotton RT, Myer CM. Practical pediatric otolaryngology. Philadelphia: Lippincott-Raven; 1999:1011.
  2. Hengerer AS, Wein RO. Congenital Malformations of the Nose and the Paranasal Sinuses. In Pediatric Otolaryngology, fourth edition.  Bluestone CD, Stool SE, Alper CM, et al. Saunders. Philadelphia, PA; 2003:979-994.
  3. Schraff SA, Vijayasekaran S, Meinzen-Derr J, Myer CM. Management of choanal atresia in CHARGE association patients: A retrospective review. Int J Pediatr Otolaryngol. 2006;70:1291-1297.
  4. Adil E, Robson C, Perez-Atayde A, Heffernan C, Moritz E, Goumnerova L, Rahbar R. Congenital Nasal Neuroglial Heterotopia and Encephaloceles: An Update on Current Evaluation and Management.  Laryngoscope. 2016;126(9):2161-7
  5. Van Den Abbeele T, Triglia JM, Francois M, Narcy P. Congenital Nasal Pyriform Aperture Stenosis: Diagnosis and Management of 20 Cases. Ann Otol Rhinol Laryngol. 2001;110:70-75.

Surgical Therapies

Learning Objectives 

To describe surgical procedures to treat:

  1. Pyriform aperture stenosis
  2. Choanal atresia
  3. Midline nasal lesions:
    1. Dermoid cysts/sinuses
    2. Gliomas
    3. Encephaloceles

 

References 
  1. Kennedy DW, Papel ID, Holliday M. Transpalatal approach to the skull base. Ear Nose Throat J. 1986;65:125,127-33.
  2. Roger G, Morisseau-Durand MP, Van Den Abbeele T, et al. The CHARGE association: the role of tracheotomy. Arch Otolaryngol Head Neck Surg. 1999;125:33-38.
  3. Stankiewicz JA. The endoscopic repair of choanal atresia. Otolaryngol Head Neck Surg. 1990;103:931-37.
  4. Hengerer AS, Wein RO. Congenital Malformations of the Nose and the Paranasal Sinuses. In Pediatric Otolaryngology, fourth edition. Bluestone CD, Stool SE, Alper CM, et al. Saunders. Philadelphia, PA. 2003. 979-994.
  5. Schraff SA, Vijayasekaran S, Meinzen-Derr J, Myer CM. Management of choanal atresia in CHARGE association patients: A retrospective review. Int J Pediatr Otolaryngol. 2006;70:1291-1297.
  6. Adil E, Robson C, Perez-Atayde A, Heffernan C, Moritz E, Goumnerova L, Rahbar R. Congenital Nasal Neuroglial Heterotopia and Encephaloceles: An Update on Current Evaluation and Management. Laryngoscope. 2016 Sep;126(9):2161-7.
  7. Van Den Abbeele T, Triglia JM, Francois M, Narcy P. Congenital Nasal Pyriform Aperture Stenosis: Diagnosis and Management of 20 Cases. Ann Otol Rhinol Laryngol. 2001;110:70-75.
  8. Teissier N, Kaguelidou F, Couloigner V, Francois M, Van Den Abbeele T. Predictive Factors for Success After Transnasal Endoscopic Treatment of Choanal Atresia.  Arch Otolaryngol Head Neck Surg. 2008;134 (1):57-61.
  9. Bonne NX, et al. Endonasal endoscopic approach for removal of intranasal glial heterotopias. Rhinology. 2012;50 (2):211-217.
  10. Newman JR, et al. Operative management of choanal atresia: a 15-year experience. JAMA Otolaryngol Head Neck Surg. 2013;139(1):71-75.

Case Studies

  1. A 4 year old boy is noted to have a small pit on the midline dorsum of his nose.  His parents noted this when he was born, but it has never caused him any symptoms.  What is the next step of action to investigate the etiology of this finding?  If imaging is desired, what are the relative advantages and disadvantages of CT scanning vs. MRI imaging?  If an intracranial extension is identified on imaging, how would that affect the timing of any surgical repair?
  2. A newborn infant is noted to have significant respiratory distress.  When crying, the child seems more comfortable.  Difficulty was noted in passing a red rubber catheter through the right nasal cavity.  What is the best method to evaluate this child?  If an intranasal mass is identified, what would be the best next step: biopsy vs. imaging?  If a flexible nasopharyngoscope can not be passed, what is the preferred imaging?  How would the above findings contribute to the timing of any surgical repair?

Complications

Learning Objectives 

To describe common complications that may result from either the treatment or observation of congenital nasal lesions.

References 
  1. Asher BF, McGill TJ, Kaplan L, et al. Airway complications in CHARGE association. Arch Otolaryngol Head Neck Surg. 1990;116:594-95.