J Neurol Surg B
DOI: 10.1055/s-0039-1692638
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Disorders Involving a Persistent Craniopharyngeal Canal: A Case Series

Seerat K. Poonia
1  Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
2  Otorhinolaryngology Unit, Department of Neurosciences, University of Padua, Padua, Italy
,
Adam C. Kaufman
1  Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
Michael A. Kohanski
1  Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
3  Department of Otolaryngology - Head and Neck Surgery, University of California, Irvine School of Medicine, Orange, California, United States
,
Charles C. L. Tong
1  Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
Roy D. Carlson
1  Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
Daniele Borsetto
2  Otorhinolaryngology Unit, Department of Neurosciences, University of Padua, Padua, Italy
,
Enzo Emanuelli
2  Otorhinolaryngology Unit, Department of Neurosciences, University of Padua, Padua, Italy
,
James N. Palmer
1  Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
,
Nithin D. Adappa
1  Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
› Author Affiliations
Funding There are no sources of financial support to disclose.
Further Information

Publication History

08 November 2018

11 May 2019

Publication Date:
21 June 2019 (online)

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.

Note

This article was presented as a poster presentation at the 2018 North American Skull Base Society Annual Meeting.