Disorders Involving a Persistent Craniopharyngeal Canal: A Case Series

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

Note

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

Publikationsverlauf

Eingereicht: 08. November 2018

Angenommen: 11. Mai 2019

Artikel online veröffentlicht:
21. Juni 2019

• 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