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Persisting embryonal infundibular recess.

Andrej Steno1, A John Popp, Stefan Wolfsberger

  • 1Department of Neurosurgery, Comenius University School of Medicine, Slovakia.

Journal of Neurosurgery
|October 28, 2008
PubMed
Summary

Persisting embryonal infundibular recess (PEIR) is a rare developmental anomaly. This case suggests PEIR arises from failed obliteration of embryonic brain structures, extending the third ventricle into the sella.

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Area of Science:

  • Neuroscience
  • Developmental Biology
  • Endocrinology

Background:

  • Persisting embryonal infundibular recess (PEIR) is a rare anomaly of the third ventricular floor.
  • Previously described cases were in adults, associated with hydrocephalus and empty sella, suggesting increased intraventricular pressure.
  • The pathogenesis of PEIR remains unclear.

Observation:

  • A 24-year-old man presented with PEIR without hydrocephalus or empty sella, but with childhood-onset pituitary dysfunction.
  • MRI revealed a sellar cyst within an enlarged sella turcica.
  • Surgical exploration confirmed a communication between the third ventricle and the sellar cyst via the pituitary stalk.

Findings:

  • PEIR represents an extension of the third ventricle into the sella turcica.
  • This case suggests PEIR may result from the failure of obliteration of the distal part of the primary embryonal diencephalic evagination.
  • A direct communication between the third ventricle and the sellar cyst through the pituitary stalk was identified.

Implications:

  • Accurate identification of PEIR is crucial for surgical planning of sellar cystic lesions.
  • Transsphenoidal approaches may inadvertently create a communication between the third ventricle and nasal cavity.
  • Watertight reconstruction of the sellar floor is necessary following procedures involving PEIR.