Rathke's cleft cyst presenting as incomplete cavernous sinus syndrome and disc edema: a case report with literature review

Doc Ophthalmol. 2020 Feb;140(1):55-65. doi: 10.1007/s10633-019-09713-7. Epub 2019 Sep 6.

Abstract

Purpose: To report a Rathke's cleft cyst (RCC) presenting as unilateral incomplete cavernous sinus syndrome and disc edema METHOD: Case report with literature review RESULTS: We present an 18-year-old girl who has been experiencing intermittent binocular diplopia for 1 month. Upon examination, the best corrected visual acuity was 20/20 (OU). Pronounced limitation of ocular motility with ptosis in her right eye was noted. Her pupil constricted normally. However, a relative afferent pupillary defect in the right eye was observed. Fundus examination showed disc edema in the right eye without peripapillary hemorrhage. Moreover, her thyroid function and acetylcholine receptor antibody levels were normal. Brain magnetic resonance imaging revealed a 1.6-cm mass at the suprasellar fossa and optic nerve sheath of right eye enhancement. She underwent transsphenoidal surgery, and pus-like formation was noted. Pathological findings were consistent with RCCs. After the cyst was removed, her ocular motility remarkably improved and ptosis subsided.

Conclusion: A case of RCC presenting as multiple cranial nerve palsy was reported. RCCs are benign and usually asymptomatic. Symptomatic RCCs often manifest as headache or visual problems, such as reduced visual acuity and visual field defect. Multiple cranial nerve involvement is uncommon and indicates inflammation or compression of the cavernous sinus and optic nerve. Neuropathy may resolve after surgery; therefore, surgery is indicated for symptomatic RCCs. Further, RCC should be included in the differential diagnosis of patients with disc edema.

Keywords: Cavernous sinus syndrome; Disc edema; Rathke’s cleft cyst.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Brain Neoplasms / diagnosis*
  • Brain Neoplasms / surgery
  • Cavernous Sinus / pathology*
  • Central Nervous System Cysts / diagnosis*
  • Central Nervous System Cysts / surgery
  • Diagnosis, Differential
  • Diplopia / diagnosis
  • Electroretinography
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Neurosurgical Procedures
  • Papilledema / diagnosis*
  • Pupil Disorders / diagnosis
  • Visual Acuity / physiology