Disorders Involving a Persistent Craniopharyngeal Canal: A Case Series

Seerat K. Poonia; Diego Cazzador; Adam C. Kaufman; Michael A. Kohanski; Edward C. Kuan; Charles C. L. Tong; Roy D. Carlson; Daniele Borsetto; Enzo Emanuelli; James N. Palmer; Nithin D. Adappa

doi:10.1055/s-0039-1692638

Journal of Neurological Surgery Part B: Skull Base, Table of Contents

J Neurol Surg B Skull Base 2020; 81(05): 562-566
DOI: 10.1055/s-0039-1692638

Original Article

Disorders Involving a Persistent Craniopharyngeal Canal: A Case Series

Authors

Seerat K. Poonia

¹Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
Diego Cazzador

²Otorhinolaryngology Unit, Department of Neurosciences, University of Padua, Padua, Italy
Adam C. Kaufman

¹Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
Michael A. Kohanski

¹Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
Edward C. Kuan

³Department of Otolaryngology - Head and Neck Surgery, University of California, Irvine School of Medicine, Orange, California, United States
Charles C. L. Tong

¹Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
Roy D. Carlson

¹Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
Daniele Borsetto

²Otorhinolaryngology Unit, Department of Neurosciences, University of Padua, Padua, Italy
Enzo Emanuelli

²Otorhinolaryngology Unit, Department of Neurosciences, University of Padua, Padua, Italy
James N. Palmer

¹Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
Nithin D. Adappa

¹Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States

Abstract

Objectives A persistent craniopharyngeal canal (CPC) is a rare embryologic remnant that presents as a well-corticated defect of the midline sphenoid body extending from the sellar floor to the nasopharynx. Our case series aims to describe three unique presentations of this congenital anomaly and their subsequent management.

Design Retrospective review.

Setting Tertiary academic medical center.

Participants Patients who underwent endoscopic transnasal surgical repair of a CPC lesion.

Main Outcome Measures Resolution of symptoms and surgical outcomes.

Results A total of three patients were identified. The clinical presentation varied, however, all cases prompted further imaging which demonstrated a persistent CPC and associated pathologic lesion. The presentation of a persistent CPC with nasal obstruction and subsequent iatrogenic cerebrospinal fluid leak as in Case 1 demonstrates the importance of imaging in this work-up. Cases 2 and 3 in the series were representative of the larger subset of patients in the literature who present with the defect incidentally but still warrant surgical management. Nonetheless, a standard approach to diagnosis with preoperative imaging and subsequent transnasal endoscopic repair of the skull base defect was undertaken.

Conclusion The persistent CPC is a rare congenital anomaly associated with diverse pathology and careful review of preoperative radiology is critical to the management. When warranted, subsequent surgical repair and reconstruction is associated with excellent postoperative outcomes.

Keywords

craniopharyngeal canal - congenital - endoscopic - skull base - surgery - cerebrospinal fluid

Full Text

References

References
1 Abele TA, Salzman KL, Harnsberger HR, Glastonbury CM. Craniopharyngeal canal and its spectrum of pathology. AJNR Am J Neuroradiol 2014; 35 (04) 772-777
2 Arey LB. The craniopharyngeal canal reviewed and reinterpreted. Anat Rec 1950; 106 (01) 1-16
3 Kaushik C, Ramakrishnaiah R, Angtuaco EJ. Ectopic pituitary adenoma in persistent craniopharyngeal canal: case report and literature review. J Comput Assist Tomogr 2010; 34 (04) 612-614
4 Richter E, Pirsig W, Tänzer A. A persistent craniopharyngeal canal combined with a meningoencephalocele (author's transl) [in German]. HNO 1975; 23 (08) 240-245
5 Braun M, Boman F, Hascoet JM, Chastagner P, Brunet A, Simon C. Brain tissue heterotopia in the nasopharynx. Contribution of MRI to assessment of extension. J Neuroradiol 1992; 19 (01) 68-74
6 Akyel NG, Alımlı AG, Demirkan TH, Sivri M. Persistent craniopharyngeal canal, bilateral microphthalmia with colobomatous cysts, ectopic adenohypophysis with Rathke cleft cyst, and ectopic neurohypophysis: case report and review of the literature. Childs Nerv Syst 2018; 34 (07) 1407-1410
7 Currarino G, Maravilla KR, Salyer KE. Transsphenoidal canal (large craniopharyngeal canal) and its pathologic implications. AJNR Am J Neuroradiol 1985; 6 (01) 39-43
8 Kizilkilic O, Yalcin O, Yildirim T, Sener L, Parmaksiz G, Erdogan B. Hypothalamic hamartoma associated with a craniopharyngeal canal. AJNR Am J Neuroradiol 2005; 26 (01) 65-67
9 Ekinci G, Kiliç T, Baltacioğlu F. et al. Transsphenoidal (large craniopharyngeal) canal associated with a normally functioning pituitary gland and nasopharyngeal extension, hyperprolactinemia, and hypothalamic hamartoma. AJR Am J Roentgenol 2003; 180 (01) 76-77
10 Hughes ML, Carty AT, White FE. Persistent hypophyseal (craniopharyngeal) canal. Br J Radiol 1999; 72 (854) 204-206
11 Chen CJ. Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and review of the literature. Neuroradiology 2001; 43 (09) 760-762