Persistent craniopharyngeal canal, bilateral microphthalmia with colobomatous cysts, ectopic adenohypophysis with Rathke cleft cyst, and ectopic neurohypophysis: case report and review of the literature
Persistent craniopharyngeal canal (PCC) is a rare anomaly of the base of the skull. PCC is defined as a well-corticated osseous canal, extending from the roof of the nasopharynx to the base of the sella over the course of the sphenoid corpus. We reported a PCC case with unique associations. The magnetic resonance imaging findings are discussed.
We report a case of a 2-year-old boy with persistent craniopharyngeal canal, bilateral microphtalmia with large colobomatous cyst, and ectopic adenohypophysis with Rathke cleft cyst. He also has ectopic neurohypophysis and optic atrophy.
The presence of orbital and optic tract malformations, craniofacial and intracranial anomalies, and tumors can accompany craniopharyngeal canals (CCs). MRI is helpful in the evaluation of PCC and associated anomalies in these patients.
KeywordsCraniopharyngeal canal Colobomatous cyst Ectopic neurohypophysis Rathke cleft Cyst Microphtalmos with cyst
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Conflict of interest
On behalf of all authors, the corresponding author states that there is no conflict of interest.
- 1.Arey LB (1950) The craniopharyngeal canal reviewed and interpreted. AnatRec 106(1):1–16Google Scholar
- 6.Ekinci G, Kılıc T, Baltacıoğlu F, Elmacı I, Altun E, Pamir MN, Erzen C (2003) Transsphenoidal (large craniopharyngeal) canal associated with a normally functioning pituitary gland and nasopharyngeal extension, hyperprolactinemia, and hypothalamic hamartoma. AJR 180(1):76–77. https://doi.org/10.2214/ajr.180.1.1800076 CrossRefPubMedGoogle Scholar
- 11.Bakir M, Sarialioglu F, Bilgic S, Akhan O (1992) Microphthalmos with bilateral colobomatous orbital cyst accompanied by polycystic kidney disease and vacuolization of myeloid progenitorcells. Acta Pediatr 81(12):1054–1057. https://doi.org/10.1111/j.1651-2227.1992.tb12176.x CrossRefGoogle Scholar
- 16.Beltz JL, Behairy AS, Rabionet P, Tirtorahardjo B, Moore MW, Cotter PD (2005) Acquired inv(9): what is its significance? Cancer Genet Cytogenet 160(1):76–78. https://doi.org/10.1016/j.cancergencyto.2004.12.002 CrossRefGoogle Scholar