Skip to main content
Log in

Persistent craniopharyngeal canal, bilateral microphthalmia with colobomatous cysts, ectopic adenohypophysis with Rathke cleft cyst, and ectopic neurohypophysis: case report and review of the literature

  • Case Report
  • Published:
Child's Nervous System Aims and scope Submit manuscript

ABSTRACT

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Arey LB (1950) The craniopharyngeal canal reviewed and interpreted. AnatRec 106(1):1–16

    CAS  Google Scholar 

  2. Shields JA, Shields CL (2004) Orbitalcysts of childhood—classification, clinical features and management. Surv Ophthalmol 49(3):281–299. https://doi.org/10.1016/j.survophthal.2004.02.001

    Article  PubMed  Google Scholar 

  3. Abele TA, Salzman KL, Harnsberger HR, Glastonbury CM (2014) Craniopharyngeal canal and its spectrum of pathology. AJNR Am J Neuroradiol 35(4):772–777. https://doi.org/10.3174/ajnr.A3745

    Article  PubMed  CAS  Google Scholar 

  4. Currarino G, Maravilla KR, Salyer KE (1985) Transsphenoidal canal (large craniopharyngealcanal) and its pathologic implications. Am J Neuroradiol 6(1):39–43

    PubMed  CAS  Google Scholar 

  5. Kaushik C, Ramakrishnaiah R, Angtuaco EJ (2010) Ectopic pituitary adenoma in persistent craniopharyngeal canal: case report and literature review. J Comput Assist Tomogr 34(4):612–614. https://doi.org/10.1097/RCT.0b013e3181dbe5d1

    Article  PubMed  Google 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

    Article  PubMed  Google Scholar 

  7. Kizilkilic O, Yalcin O, Yildirim T, Sener L, Parmaksiz G, Erdogan B (2005) Hypothalamic hamartoma associated with a craniopharyngeal canal. AJNR Am J Neuroradiol 26(11):65–67

    PubMed  Google Scholar 

  8. Chen CJ (2001) Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and a review of literature. Neuroradiology 43(9):760–762. https://doi.org/10.1007/s002340100581

    Article  PubMed  CAS  Google Scholar 

  9. Han D, Kim SH, Kim JY, Lee J, Bae KE (2016) Persistent craniopharyngeal canal with posterior pituitary ectopia: a case report. J Korean Soc Radiol 74(3):210–213. https://doi.org/10.3348/jksr.2016.74.3.210

    Article  Google Scholar 

  10. Bonner J, Ide CH (1974) Astrocytoma of the optic nerve and chiasm associated with microphthalmos and orbital cyst. Br J Ophthalmol 58(9):828–831. https://doi.org/10.1136/bjo.58.9.828

    Article  PubMed  PubMed Central  CAS  Google 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

    Article  CAS  Google Scholar 

  12. Fisher YL (1978) Microphthalmos with ocular communicating orbital cyst-ultrasonic diagnosis. Ophthalmology 85(11):1208–1211. https://doi.org/10.1016/S0161-6420(78)35565-2

    Article  PubMed  CAS  Google Scholar 

  13. Guterman C, Abboud E, Mets MB (1990) Microphthalmos with cyst and Edwards syndrome. Am J Ophthalmol 109(2):228–230. https://doi.org/10.1016/S0002-9394(14)75995-1

    Article  PubMed  CAS  Google Scholar 

  14. Kaiser P (1984) Pericentric inversions. Problems and significance for clinical genetics. Hum Genet 68(1):1–47. https://doi.org/10.1007/BF00293869

    Article  PubMed  CAS  Google Scholar 

  15. Jeong SY, Kim BY, Yu JE (2010) De novo pericentric inversion of chromosome 9 in congenital anomaly. Yonsei Med J 51(5):775–780. https://doi.org/10.3349/ymj.2010.51.5.775

    Article  PubMed  PubMed Central  Google 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

    Article  CAS  Google Scholar 

  17. Rao BV, Kerketta L, Korgaonkar S, Ghosh K (2006) Pericentric inversion of chromosome 9[inv(9)(p12q13)]: its association with genetic diseases. Indian J Hum Gent 12(3):129–132

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Nazlı Gülsüm Akyel.

Ethics declarations

Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Akyel, N.G., Alımlı, A.G., Demirkan, T.H. et al. 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 34, 1407–1410 (2018). https://doi.org/10.1007/s00381-018-3747-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-018-3747-4

Keywords

Navigation