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 Jul;34(7):1407-1410. doi: 10.1007/s00381-018-3747-4. Epub 2018 Feb 14.


Introduction: 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.

Case presentation: 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.

Conclusion: 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.

Keywords: Colobomatous cyst; Craniopharyngeal canal; Cyst; Ectopic neurohypophysis; Microphtalmos with cyst; Rathke cleft.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Central Nervous System Cysts / complications*
  • Child, Preschool
  • Choristoma / pathology
  • Coloboma / complications*
  • Humans
  • Male
  • Microphthalmos / complications*
  • Osteoarthropathy, Primary Hypertrophic / complications*
  • Pituitary Gland, Anterior / pathology*
  • Pituitary Neoplasms / complications*

Supplementary concepts

  • Microphthalmia, Isolated, with Coloboma 4