Download PDF
J Neurol Surg B Skull Base 2022; 83(03): 223-227
DOI: 10.1055/s-0040-1722230
Original Article

Skull Base Anatomy in Patients with Bilateral Choanal Atresia: A Radiographic Study

Christopher Pool 
1   Department of Otolaryngology – Head and Neck Surgery, Penn State Hershey Medical Center, Hershey, Pennsylvania, United States
,
Einat Slonimsky
2   Department of Radiology, Penn State Hershey Medical Center, Hershey, Pennsylvania, United States
,
Roshan Nayak
3   Ichan School of Medicine at Mount Sinai, New York, New York, United States
,
Lisa Engle
4   Department of Public Health Sciences, Division of Biostatistics and Bioinformatics, Penn State Hershey Medical Center, Hershey, Pennsylvania, United States
,
Junjia Zhu
4   Department of Public Health Sciences, Division of Biostatistics and Bioinformatics, Penn State Hershey Medical Center, Hershey, Pennsylvania, United States
,
Meghan Wilson
1   Department of Otolaryngology – Head and Neck Surgery, Penn State Hershey Medical Center, Hershey, Pennsylvania, United States
› Author Affiliations Funding There are no financial disclosures for any author.
› Further Information
    Permissions and Reprints

Abstract

Introduction The risk of skull base injury during choanal atresia repair can be mitigated via thorough understanding of skull base anatomy. There is a paucity of data describing differences in skull base anatomy between patients with coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear abnormalities (CHARGE) syndrome and those without.

Objectives The aim of this study was to measure nasal and skull base anatomy in patients with isolated bilateral choanal atresia (BCA), CHARGE syndrome, and other syndromic congenital anomalies.

Methods Retrospective chart review of patients with bilateral choanal atresia and computed tomography of the face between 2001 and 2019 were evaluated. Choanal width, height, mid-nasal height, and skull base slope were measured radiographically. Differences in anatomy between healthy patients, those with CHARGE syndrome, and those with other congenital anomalies were compared.

Results Twenty-one patients with BCA and relevant imaging were identified: 7 with isolated BCA, 6 with CHARGE syndrome, and 8 with other congenital anomalies. A t-test indicated insignificant difference in skull base slope, choanal height, choanal width, or mid-nasal skull base height between isolate BCA cases and patients with any congenital anomaly. When comparing CHARGE to isolated BCA cases, mid-nasal height was shorter in CHARGE patients (p = 0.03). There were no differences in measurements between patients with congenital anomalies excluding CHARGE (p > 0.05). Two patients in the congenital anomaly group were found to have bony skull base defects preoperatively.

Conclusion This study represents the largest description of skull base and nasal anatomy in patients with CHARGE syndrome and BCA. Surgeons should be aware of the lower skull base in CHARGE patients to avoid inadvertent skull base injury.

Keywords

skull base anatomy - CHARGE - choanal atresia

Note

This paper was presented as a poster presentation at the North American Skull Base Society Meeting on February 8, 2020 in San Antonio, Texas.




Publication History

Received: 08 April 2020

Accepted: 05 October 2020

Article published online:
04 February 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Harris J, Robert E, Källén B. Epidemiology of choanal atresia with special reference to the CHARGE association. Pediatrics 1997; 99 (03) 363-367
    CrossrefPubMedGoogle Scholar
  • 2 Ramsden JD, Campisi P, Forte V. Choanal atresia and choanal stenosis. Otolaryngol Clin North Am 2009; 42 (02) 339-352
    CrossrefPubMedGoogle Scholar
  • 3 Moreddu E, Rizzi M, Adil E. et al. International Pediatric Otolaryngology Group (IPOG) consensus recommendations: diagnosis, pre-operative, operative and post-operative pediatric choanal atresia care. Int J Pediatr Otorhinolaryngol 2019; 123: 151-155
    CrossrefPubMedGoogle Scholar
  • 4 Leclerc JE, Fearon B. Choanal atresia and associated anomalies. Int J Pediatr Otorhinolaryngol 1987; 13 (03) 265-272
    CrossrefPubMedGoogle Scholar
  • 5 Meleca JB, Anne S, Hopkins B. Reducing the need for general anesthesia in the repair of choanal atresia with steroid-eluting stents: a case series. Int J Pediatr Otorhinolaryngol 2019; 118: 185-187
    CrossrefPubMedGoogle Scholar
  • 6 Van Den Abbeele T, François M, Narcy P. Transnasal endoscopic treatment of choanal atresia without prolonged stenting. Arch Otolaryngol Head Neck Surg 2002; 128 (08) 936-940
    CrossrefPubMedGoogle Scholar
  • 7 Teissier N, Kaguelidou F, Couloigner V, François M, Van Den Abbeele T. Predictive factors for success after transnasal endoscopic treatment of choanal atresia. Arch Otolaryngol Head Neck Surg 2008; 134 (01) 57-61
    CrossrefPubMedGoogle Scholar
  • 8 Fearon B, Dickson J. Bilateral choanal atresia in the newborn: plan of action. Laryngoscope 1968; 78 (09) 1487-1499
    CrossrefPubMedGoogle Scholar
  • 9 Muzumdar D, Ventureyra EC. Inadvertent intracranial insertion of a soft rubber tube in a patient with Treacher-Collins syndrome: case report and review of literature. Childs Nerv Syst 2008; 24 (05) 609-613
    CrossrefPubMedGoogle Scholar
  • 10 Nathoo N, Nadvi SS. Intracranial malposition of a nasogastric tube following repair of choanal atresia. Br J Neurosurg 1999; 13 (04) 409-410
    CrossrefPubMedGoogle Scholar
  • 11 Mahdi ES, Whitehead MT. Clival malformations in CHARGE syndrome. AJNR Am J Neuroradiol 2018; 39 (06) 1153-1156
    CrossrefPubMedGoogle Scholar
  • 12 Natung T, Goyal A, Handique A, Kapoor M. Symmetrical chorioretinal colobomata with craniovertebral junction anomalies in CHARGE syndrome - a case report with review of literature. J Clin Imaging Sci 2014; 4: 5
    CrossrefPubMedGoogle Scholar
  • 13 Manica D, Schweiger C, Netto CC, Kuhl G. Retrospective study of a series of choanal atresia patients. Int Arch Otorhinolaryngol 2014; 18 (01) 2-5
    PubMedGoogle Scholar
  • 14 Amir R, Dunham ME. Bilateral choanal atresia associated with nasal dermoid cyst and sinus: a case report and review of the literature. Int J Pediatr Otorhinolaryngol 2001; 58 (01) 81-85
    CrossrefPubMedGoogle Scholar
  • 15 Dunham ME, Miller RP. Bilateral choanal atresia associated with malformation of the anterior skull base: embryogenesis and clinical implications. Ann Otol Rhinol Laryngol 1992; 101 (11) 916-919
    CrossrefPubMedGoogle Scholar
  • 16 Westendorff C, Dammann F, Reinert S, Hoffmann J. Computer-aided surgical treatment of bilateral choanal atresia. J Craniofac Surg 2007; 18 (03) 654-660
    CrossrefPubMedGoogle Scholar
  • 17 Schweinfurth JM. Image guidance--assisted repair of bilateral choanal atresia. Laryngoscope 2002; 112 (11) 2096-2098
    CrossrefPubMedGoogle Scholar
  • 18 Sung JY, Cho KS, Bae YC, Bae SH. Image-guided navigation surgery for bilateral choanal atresia with a Tessier number 3 facial cleft in an adult. Arch Craniofac Surg 2020; 21 (01) 64-68
    CrossrefPubMedGoogle Scholar
  • 19 Aslan S, Yilmazer C, Yildirim T, Akkuzu B, Yilmaz I. Comparison of nasal region dimensions in bilateral choanal atresia patients and normal controls: a computed tomographic analysis with clinical implications. Int J Pediatr Otorhinolaryngol 2009; 73 (02) 329-335
    CrossrefPubMedGoogle Scholar
  • 20 Coniglio JU, Manzione JV, Hengerer AS. Anatomic findings and management of choanal atresia and the CHARGE association. Ann Otol Rhinol Laryngol 1988; 97 (5 Pt 1): 448-453
    CrossrefPubMedGoogle Scholar
  • 21 Fitzpatrick NS, Bartley AC, Bekhit E, Berkowitz RG. Skull base anatomy and surgical safety in isolated and CHARGE-associated bilateral choanal atresia. Int J Pediatr Otorhinolaryngol 2018; 115: 61-64
    CrossrefPubMedGoogle Scholar
  • 22 Gump WC. Endoscopic endonasal repair of congenital defects of the anterior skull base: developmental considerations and surgical outcomes. J Neurol Surg B Skull Base 2015; 76 (04) 291-295
    Article in Thieme ConnectPubMedGoogle Scholar
  • 23 Schulz Y, Wehner P, Opitz L. et al. CHD7, the gene mutated in CHARGE syndrome, regulates genes involved in neural crest cell guidance. Hum Genet 2014; 133 (08) 997-1009
    CrossrefPubMedGoogle Scholar
  • 24 Cheung R, Prince M. Comparison of craniofacial skeletal characteristics of infants with bilateral choanal atresia and an age-matched normative population: computed tomography analysis. J Otolaryngol 2001; 30 (03) 173-178
    CrossrefPubMedGoogle Scholar