Skip to main content

Advertisement

Log in

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

  • Clinical Case Report
  • Published:
Documenta Ophthalmologica Aims and scope Submit manuscript

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.

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
Fig. 4
Fig. 5
Fig. 6
Fig. 7

Similar content being viewed by others

References

  1. Teramoto A, Hirakawa K, Sanno N, Osamura Y (1994) Incidental pituitary lesions in 1,000 unselected autopsy specimens. Radiology 193(1):161–164

    Article  CAS  PubMed  Google Scholar 

  2. Trifanescu R, Ansorge O, Wass JA, Grossman AB, Karavitaki N (2012) Rathke’s cleft cysts. Clin Endocrinol 76(2):151–160

    Article  Google Scholar 

  3. Odom JV, Bach M, Holder GE, McCulloch DL, Tormene AP (2016) ISCEV standard for clinical visual evoked potentials (2016 update). Doc Ophthalmol 133(1):1–9

    Article  PubMed  Google Scholar 

  4. Isono M, Kamida T, Kobayashi H, Shimomura T, Matsuyama J (2001) Clinical features of symptomatic Rathke’s cleft cyst. Clin Neurol Neurosurg 103(2):96–100

    Article  CAS  PubMed  Google Scholar 

  5. Kleinschmidt-DeMasters BK, Lillehei KO, Stears JC (1995) The pathologic, surgical, and MR spectrum of Rathke cleft cysts. Surg Neurol 44(1):19–26 discussion 26-7

    Article  CAS  PubMed  Google Scholar 

  6. Jung JE, Jin J, Jung MK, Kwon A, Chae HW, Kim DH, Kim HS (2017) Clinical manifestations of Rathke’s cleft cysts and their natural progression during 2 years in children and adolescents. Ann Pediatr Endocrinol Metab 22(3):164–169

    Article  PubMed  PubMed Central  Google Scholar 

  7. Kim E (2012) Symptomatic Rathke cleft cyst: clinical features and surgical outcomes. World Neurosurg 78(5):527–534

    Article  PubMed  Google Scholar 

  8. Voelker JL, Campbell RL, Muller J (1991) Clinical, radiographic, and pathological features of symptomatic Rathke’s cleft cysts. J Neurosurg 74(4):535–544

    Article  CAS  PubMed  Google Scholar 

  9. Han SJ, Rolston JD, Jahangiri A, Aghi MK (2014) Rathke’s cleft cysts: review of natural history and surgical outcomes. J Neurooncol 117(2):197–203

    Article  PubMed  Google Scholar 

  10. Bohnstedt BN, Patel NB, Hagen MC, Fulkerson DH (2012) Acute, monocular vision loss from Rathke’s cleft cyst. J Clin Neurosci 19(6):904–906

    Article  PubMed  Google Scholar 

  11. Yum HR, Jang J, Shin SY, Park SH (2015) Rathke cleft cyst presenting as unilateral progressive oculomotor nerve palsy. Can J Ophthalmol 50(2):e31–e33

    Article  PubMed  Google Scholar 

  12. Park BH, Choi YC, Kim WJ, Han SH (2004) Transient isolated trochlear nerve palsy associated with Rathke’s cleft cyst. Yonsei Med J 45(1):166–168

    Article  PubMed  Google Scholar 

  13. Miyajima Y, Oka H, Utsuki S, Kondo K, Sato K, Fujii K (2007) Symptomatic Rathke’s cleft cyst with cavernous sinus syndrome. Neurol Med Chir (Tokyo) 47(12):576–578

    Article  Google Scholar 

  14. Grover V, Hamrahian AH, Prayson RA, Weil RJ (2011) Rathke’s cleft cyst presenting as bilateral abducens nerve palsy. Pituitary 14(4):395–399

    Article  PubMed  Google Scholar 

  15. Pawar SJ, Sharma RR, Lad SD, Dev E, Devadas RV (2002) Rathke’s cleft cyst presenting as pituitary apoplexy. J Clin Neurosci 9(1):76–79

    Article  PubMed  Google Scholar 

  16. Kobayashi N, Oshitari T, Kobayashi K, Onoda T, Ikeda H, Adachi-Usami E (2014) Optic neuritis caused by Rathke’s cleft cyst in young adult. Case Rep Ophthalmol Med 2014:204762

    PubMed  PubMed Central  Google Scholar 

  17. Roncaroli F, Bacci A, Frank G, Calbucci F (1998) Granulomatous hypophysitis caused by a ruptured intrasellar Rathke’s cleft cyst: report of a case and review of the literature. Neurosurgery 43(1):146–149

    Article  CAS  PubMed  Google Scholar 

  18. Nishikawa T, Takahashi JA, Shimatsu A, Hashimoto N (2007) Hypophysitis caused by Rathke’s cleft cyst. Case report. Neurol Med Chir (Tokyo) 47(3):136–139

    Article  Google Scholar 

  19. Komatsu F, Tsugu H, Komatsu M, Sakamoto S, Oshiro S, Fukushima T, Nabeshima K, Inoue T (2010) Clinicopathological characteristics in patients presenting with acute onset of symptoms caused by Rathke’s cleft cysts. Acta Neurochir (Wien) 152(10):1673–1678

    Article  Google Scholar 

  20. Go JL, Rajamohan AG (2017) Imaging of the sella and parasellar region. Radiol Clin North Am 55(1):83–101

    Article  PubMed  Google Scholar 

  21. Shin JL, Asa SL, Woodhouse LJ, Smyth HS, Ezzat S (1999) Cystic lesions of the pituitary: clinicopathological features distinguishing craniopharyngioma, Rathke’s cleft cyst, and arachnoid cyst. J Clin Endocrinol Metab 84(11):3972–3982

    CAS  PubMed  Google Scholar 

  22. Midha R, Jay V, Smyth HS (1991) Transsphenoidal management of Rathke’s cleft cysts A clinicopathological review of 10 cases. Surg Neurol 35(6):446–454

    Article  CAS  PubMed  Google Scholar 

  23. Nishioka H, Haraoka J, Izawa H, Ikeda Y (2006) Magnetic resonance imaging, clinical manifestations, and management of Rathke’s cleft cyst. Clin Endocrinol (Oxf) 64(2):184–188

    Article  Google Scholar 

  24. Chotai S, Liu Y, Pan J, Qi S (2015) Characteristics of Rathke’s cleft cyst based on cyst location with a primary focus on recurrence after resection. J Neurosurg 122(6):1380–1389

    Article  PubMed  Google Scholar 

  25. Karavitaki N, Scheithauer BW, Watt J, Ansorge O, Moschopoulos M, Llaguno AV, Wass JA (2008) Collision lesions of the sella: co-existence of craniopharyngioma with gonadotroph adenoma and of Rathke’s cleft cyst with corticotroph adenoma. Pituitary 11(3):317–323

    Article  CAS  PubMed  Google Scholar 

  26. Hofmann BM, Kreutzer J, Saeger W, Buchfelder M, Blumcke I, Fahlbusch R, Buslei R (2006) Nuclear beta-catenin accumulation as reliable marker for the differentiation between cystic craniopharyngiomas and rathke cleft cysts: a clinico-pathologic approach. Am J Surg Pathol 30(12):1595–1603

    Article  PubMed  Google Scholar 

  27. Trifanescu R, Stavrinides V, Plaha P, Cudlip S, Byrne JV, Ansorge O, Wass JA, Karavitaki N (2011) Outcome in surgically treated Rathke’s cleft cysts: long-term monitoring needed. Eur J Endocrinol 165(1):33–37

    Article  CAS  PubMed  Google Scholar 

  28. Megdiche-Bazarbacha H, Ben Hammouda K, Aicha AB, Sebai R, Belghith L, Khaldi M, Touibi S (2006) Intrasphenoidal rathke cleft cyst. AJNR Am J Neuroradiol 27(5):1098–1100

    CAS  PubMed  Google Scholar 

  29. Zada G (2011) Rathke cleft cysts: a review of clinical and surgical management. Neurosurg Focus 31(1):E1

    Article  PubMed  Google Scholar 

  30. Aho CJ, Liu C, Zelman V, Couldwell WT, Weiss MH (2005) Surgical outcomes in 118 patients with Rathke cleft cysts. J Neurosurg 102(2):189–193

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Chiung Fang Chang.

Ethics declarations

Conflict of interest

The authors declare that they have no conflicts of interest.

Informed consent

Written informed consent was obtained from the patient for publication of this case report, including accompanying images.

Statement of human rights and ethical approval

All procedures involving human participants were conducted in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Statement on the welfare of animals

All applicable international, national, and/or institutional guidelines for the care and use of animals were followed.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Chang, C.F., Chen, Y.M. Rathke’s cleft cyst presenting as incomplete cavernous sinus syndrome and disc edema: a case report with literature review. Doc Ophthalmol 140, 55–65 (2020). https://doi.org/10.1007/s10633-019-09713-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10633-019-09713-7

Keywords

Navigation