Case Report

Optic Nerve Root Enhancement in Gadolinium-Enhanced Magnetic Resonance Imaging

of Rathke’s Cleft Cyst

Kohei Tsujino1, Naokado Ikeda1, Seigo Kimura1, Akira Higashiyama2, Motomasa Furuse1, Naosuke Nonoguchi1,

Ryo Hiramatsu1, Ryokichi Yagi1, Shinji Kawabata1, Keigo Osuga2, Masahiko Wanibuchi1

-BACKGROUND: Although most patients with Rathke’s cleft cysts (RCCs)remain asymptomatic throughout their lives, visual impairment in RCCs usuallyindicates surgical interventions, including endoscopic drainage of the cyst. Wereport a case of RCC with unique findings in the optic nerve root (ONR) bymagnetic resonance imaging (MRI).

-CASE DESCRIPTION: A 58-year-old man admitted to our hospital complainedof progressive left visual impairment. Preoperative computed tomography MRIrevealed a suprasellar cystic lesion that extended anteriorly, and the bilateralONRs were lateralized with the cyst and were partially enhanced with gado-linium administration. The cyst content was drained, and the cyst wall waspartially removed by endoscopic transsphenoidal surgery. Postoperatively, thepatient was administered corticosteroid intravenously for 3 days, and his visualacuity improved dramatically. Postoperative MRI revealed that the volume of thecyst decreased, and the position of the bilateral ONRs normalized. Additionally,enhancement of the ONRs with gadolinium administration was not observed.Histological examination of the surgical specimen revealed a ciliated single-layer columnar epithelium with inflammatory cell infiltration.

-CONCLUSIONS: To our knowledge, we report the first case of a patient withRCC with ONR enhancement with gadolinium administration on MRI. This uniquefinding might prove that inflammation is one of the causes of visual impairmentin RCC as in optic neuritis.

Key words- Gadolinium- Magnetic resonance imaging- Optic neuritis- Rathke’s cleft cyst

Abbreviations and AcronymsMRI: Magnetic resonance imagingONR: Optic nerve rootRCC: Rathke’s cleft cyst

From the Departments of 1Neurosurgery andNeuroendovascular Surgery and 2Diagnostic Radiology,Osaka Medical College, Osaka, Japan

To whom correspondence should be addressed:Naokado Ikeda, M.D., Ph.D.[E-mail: neu098@osaka-med.ac.jp]

Citation: World Neurosurg. (2021) 146:157-161.https://doi.org/10.1016/j.wneu.2020.11.019

Journal homepage: www.journals.elsevier.com/world-neurosurgery

Available online: www.sciencedirect.com

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INTRODUCTION

Although most patients with Rathke’s cleftcysts (RCCs) remain asymptomaticthroughout their lives, occasionally, someexperience symptoms including headache,hypopituitarism, and visual disturbance.1-3

Of these symptoms, visual impairmentusually indicates surgical interventions,including endoscopic drainage of the cyst.Herein we report an operative case of apatient with RCC who complained ofpreoperative progressive visual impair-ment and discuss the unique radiologicfindings in this case.

CASE DESCRIPTION

A 58-year-old man who complained ofmild headache, left-side visual impair-ment, and photophobia for about 1 monthwas admitted to the ophthalmologicclinic. The patient has consented to thesubmission of the case report to thejournal. The patient’s head magneticresonance imaging (MRI) scan revealed a

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WORLD NEUROSURGERY 146: 157-161,

suprasellar cystic lesion, and the patientwas admitted to our institution. Neuro-logic examinations on admission revealedno deficits, except for left-side visualimpairment. Two weeks before admission,the patient’s visual acuity was 1.2 (1.2) inthe right eye and 0.2 (0.8) in the left eye.The visual field test with Goldmann peri-metry revealed a nasal visual field defectand paracentral scotoma in the left eye(Figure 1I). However, on admission, thepatient’s visual acuity of the left eyebecame worse to light perception andcould not be corrected. Computedtomography revealed a hypodensesuprasellar cystic lesion withoutcalcification extending anteriorly. MRIrevealed the cyst content was isointenseon T1-weighted images and hyperintenseon T2-weighted images, and the cyst wallwas partially enhanced with gadolinium

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administration (Figure 1AeH). Thepituitary stalk was compressed backward,and the bilateral optic nerve roots(ONRs) were lateralized by the lesion. Inaddition, the lateralized bilateral ONRswere mildly enhanced with gadoliniumadministration (Figure 1A, B, D, and E).The size of the cyst did not changecompared with that shown on the MRIperformed 2 weeks before admission.The patient had never complained ofpolydipsia and polyuria. Not onlyhematological and biochemicalparameters but also hormonal profile,including hormone loading test withluteinizing hormone-releasing hormone,growth hormone-releasing peptide-2,corticotropin-releasing hormone, andglucagon, were within normal limits,indicating normal hypothalamo-pituitaryfunction.

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Figure 1. Preoperative magnetic resonance imaging showing thesuprasellar cystic lesion that extended anteriorly—the cyst wall waspartially enhanced with gadolinium administration (AeH), the optic nerveroots (arrows) were compressed by the cyst and were partially enhancedwith gadolinium administration (B and E). (AeF) Coronal images; (G and H)

sagittal images; (A, D, and G) T1-weighted images; (B, E, and H)T1-weighted images with gadolinium administration. Preoperative visualfield test with Goldmann perimetry revealed a nasal visual field defect andparacentral scotoma in the left eye (I).

CASE REPORT

KOHEI TSUJINO ET AL. ONR ENHANCEMENT IN MRI OF RCC

Three days after admission, an extendedendoscopic transsphenoidal surgery wasperformed. The cyst wall and pituitarygland were exposed, and the cyst wall wasincised (Figure 2A). The mild viscouscontent of the cyst (Figure 2B) wasdrained, followed by careful dissection ofthe cyst wall from the surroundingnormal structures, and finally the cystwas removed. Because the intraoperativepathological diagnosis was RCC, the cystwall that adhered to the pituitary stalk,anterior communicating artery complex,and cortical branches of the anteriorcerebellar arteries (Figures 2C and D)were partially left. The bilateral opticnerves were lateralized but were notthinned by the lesion (Figure 2D). Thesellae floor and planum sphenoidalewere reconstructed with abdominal fat,

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bony nasal septum, and vascularizednasal septal mucosal flap.MRI, 2 days after the operation,

revealed that the volume of the cystdecreased, and the position of the bilateralONRs normalized. Additionally, enhance-ment of the ONRs with gadoliniumadministration was not observed (Figure3A-H). The patient’s visual acuityimproved dramatically and immediatelyafter the operation, and the postoperativecomplications, including meningitis, forintraoperative leakage of the cyst’scontents did not occur thanks to theadministration of 2 mg/day of cefazolin-sodium for 3 days. The visual testing per-formed 1 week after the surgery revealedthat the patient’s visual acuity of the lefteye improved to 1.5 (1.5) and the visualfield defect and paracentral scotoma

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disappeared (Figure 3I). Histologicalexamination of the surgical specimenrevealed a ciliated single-layer columnarepithelium with inflammatory cell infil-tration (Figure 4), and the final permanentpathological diagnosis was RCC. Thelesion and the patient’s visualimpairment have not recurred for 3months postoperatively.

DISCUSSION

The unique finding on MRI in the pre-sented case was that the bilateral ONRscompressed by the RCC were enhancedwith gadolinium administration. To thebest of our knowledge, the presented caseis the first reported case of RCC with ONRenhancement on MRI. ONR enhancementwith gadolinium administration as in the

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Figure 2. Intraoperative photograms—the suprasellar cyst (asterisk) and pituitary gland (PG) wereexposed (A); the cyst wall (arrow) was incised, and the mild viscous content (sharp) was drained (B);the cyst wall was partially adhered to the pituitary stalk (C), cortical branches of the anterior cerebralarteries, and anterior communicating artery complex (harpoon); the bilateral optic nerves (arrowhead)were lateralized but were not thinned by the lesion (D).

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KOHEI TSUJINO ET AL. ONR ENHANCEMENT IN MRI OF RCC

presented case is well recognized and acommon finding in optic neuritis. Severalreports speculated that gadoliniumenhancement of the optic nerves in opticneuritis is a sign of bloodebrain barrierbreakdown and indicates acute inflam-mation of the optic nerve.4-7 It is wellknown that inflammation owing to theinfiltration of the contents of an RCC ormicrobleeding of the pituitary glandaround an RCC resulted in hypopituita-rism.8-12 Also, it was reported that the cystcontent of iso-to hyperintensity on the T1-weighted MRI of the cyst contents, as inthe presented case, is usually mucus ofvarying viscosity.3,13,14 Because mucus is astrong stimulator of tissue, mucousmaterial within RCC can cause a foreign-body inflammatory reaction. Thus RCCsare occasionally accompanied by chronicinflammation around the cyst. RCC withiso-to hyperintensity on the T1-weightedimage causes less intense clinical symp-toms than cysts of low intensity.2,15

Inflammation could spread to the normal

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structures around an RCC, including notonly the pituitary gland but also theONRs and optic chiasm. Therefore ONRenhancement after gadoliniumadministration in the present casesuggested that inflammation of the opticnerve was one of the causes of visualimpairment in RCC, as in acute opticneuritis. Unfortunately, we did notperform a microbiological examination ofthe cyst’s contents. However, because thepostoperative clinical course of thepatient was uneventful with minimaldosages of antibiotics for the preventionof postoperative infection, it waspredicted that aseptic inflammation ofthe RCC influenced the ONRs. Visualimpairment in RCC was generallythought to result from directcompression of the optic nerve by thecyst or local ischemia of the opticnerve.16,17 In the presented case, the cystwas large enough to compress the ONRs,which, of course, caused the visualimpairment. However, at least the

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inflammation of the ONRs was probablyinvolved in the cause of the rapidprogression of the visual impairmentbecause the size of the cyst wasunchanged, although the visualimpairment progressed from 0.2 to lightperception in 2 weeks.RCC presents differently depending on

the degree of compression and directionover the surrounding structures.18 In thepresent case, the cyst mainly extendedanteriorly, and the pituitary gland andpituitary stalk were compressed slightlydownward and posteriorly. Thisuncommon extension direction of thecyst might result in the inflammation ofthe optic nerves only, but not thepituitary gland. Accordingly, there werefewer affected pituitary gland tissues inthis case than in RCCs with usualextension.In a case series of patients with acute

optic neuritis, Berg et al.4 reported thatgadolinium enhancement of the opticnerve is detected in those with a moreacute onset of the disease and a higherpotential to prompt progression of visualimpairment. Additionally, they describedthat gadolinium enhancement of thecanalicular and intracranial ONRs inRCC was associated with better visualimprovement after appropriate therapiescompared with intraorbital lesions.According to their results, althoughfurther studies should be needed,gadolinium enhancement of the ONRs inRCC might also be a good predictivefactor of visual improvement immediatelyafter appropriate surgery, even ifpreoperative visual impairment is severeand progressive, as in the presented case.

CONCLUSIONS

We reported the first case, to our knowl-edge, of a patient with RCC with ONRenhancement with gadolinium adminis-tration on MRI. This unique finding mightprove that inflammation is one of thecauses of visual impairment in RCC, as inoptic neuritis.

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Figure 3. Postoperative magnetic resonance imaging showing that thevolume of the cyst decreased, and position of the bilateral optic nerve rootsnormalized (AeH); enhancement of the optic nerve roots (arrows) withgadolinium administration was not observed (A, B, D, and E). (AeF) Coronal

images; (G and H) sagittal images; (A, D, and G) T1-weighted images; (B, E,and H) T1-weighted images with gadolinium administration. Postoperativevisual field test with Goldmann perimetry revealed a visual field defect, andthe paracentral scotoma disappeared in the left eye (I).

Figure 4. Histological examination of the surgical specimen revealed a ciliated single-layer columnarepithelium (A) with inflammatory cell infiltration and hemosiderin deposit (B) (hematoxylin and eosinstaining, original magnification x400).

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CASE REPORT

KOHEI TSUJINO ET AL. ONR ENHANCEMENT IN MRI OF RCC

REFERENCES

1. Komatsu F, Tsugu H, Komatsu M, et al. Clinico-pathological characteristics in patients presentingwith acute onset of symptoms caused by Rathke’scleft cysts. Acta Neurochir (Wien). 2010;152:1673-1678.

2. Nishioka H, Haraoka J, Izawa H, Ikeda Y. Head-aches associated with Rathke’s cleft cyst. Headache.2006;46:1580-1586.

3. Nishioka H, Haraoka J, Izawa H, Ikeda Y. Mag-netic resonance imaging, clinical manifestations,and management of Rathke’s cleft cyst. ClinEndocrinol (Oxf). 2006;64:184-188.

4. Berg S, Kaschka I, Utz KS, et al. Baseline mag-netic resonance imaging of the optic nerve pro-vides limited predictive information on short-term

s://doi.org/10.1016/j.wneu.2020.11.019

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KOHEI TSUJINO ET AL. ONR ENHANCEMENT IN MRI OF RCC

recovery after acute optic neuritis. PLoS One. 2015;10:e0113961.

5. Katz D, Taubenberger JK, Cannella B,McFarlin DE, Raine CS, McFarland HF. Correla-tion between magnetic resonance imaging find-ings and lesion development in chronic, activemultiple sclerosis. Ann Neurol. 1993;34:661-669.

6. Youl BD, Turano G, Miller DH, et al. The patho-physiology of acute optic neuritis. An associationof gadolinium leakage with clinical and electro-physiological deficits. Brain. 1991;114(Pt 6):2437-2450.

7. Deschamps R, Gout O, Fontaine B, et al. [Acuteoptic neuritis: clinical and MRI prognostic factors.Study of fifty patients]. Rev Neurol (Paris). 2002;158:446-452.

8. Hama S, Arita K, Tominaga A, et al. SymptomaticRathke’s cleft cyst coexisting with central diabetesinsipidus and hypophysitis: case report. Endocr J.1999;46:187-192.

9. Nishikawa T, Takahashi JA, Shimatsu A,Hashimoto N. Hypophysitis caused by Rathke’scleft cyst. Case report. Neurol Med Chir (Tokyo).2007;47:136-139.

WORLD NEUROSURGERY 146: 157-161,

10. Murakami M, Nishioka H, Izawa H, Ikeda Y,Haraoka J. Granulomatous hypophysistis associ-ated with Rathke’s cleft cyst: a case report. MinimInvasive Neurosurg. 2008;51:169-172.

11. Roncaroli F, Bacci A, Frank G, Calbucci F. Gran-ulomatous hypophysitis caused by a rupturedintrasellar Rathke’s cleft cyst: report of a case andreview of the literature. Neurosurgery. 1998;43:146-149.

12. Kim E. Symptomatic Rathke cleft cyst: clinicalfeatures and surgical outcomes. World Neurosurg.2012;78:527-534.

13. Ikeda H, Yoshimoto T. Clinicopathological studyof Rathke’s cleft cysts. Clin Neuropathol. 2002;21:82-91.

14. Kim JE, Kim JH, Kim OL, et al. Surgical treatmentof symptomatic Rathke cleft cysts: clinical featuresand results with special attention to recurrence.J Neurosurg. 2004;100:33-40.

15. Laws ER, Kanter AS. Rathke cleft cysts.J Neurosurg. 2004;101:571-572 [discussion: 572].

16. Chang CF, Chen YM. Rathke’s cleft cyst present-ing as incomplete cavernous sinus syndrome and

FEBRUARY 2021 www.journals.el

disc edema: a case report with literature review.Doc Ophthalmol. 2020;140:55-65.

17. Bohnstedt BN, Patel NB, Hagen MC,Fulkerson DH. Acute, monocular vision loss fromRathke’s cleft cyst. J Clin Neurosci. 2012;19:904-906.

18. Pawar SJ, Sharma RR, Lad SD, Dev E,Devadas RV. Rathke’s cleft cyst presenting as pi-tuitary apoplexy. J Clin Neurosci. 2002;9:76-79.

Conflict of interest statement: The authors declare that thearticle content was composed in the absence of anycommercial or financial relationships that could be construedas a potential conflict of interest.

Received 27 May 2020; accepted 3 November 2020

Citation: World Neurosurg. (2021) 146:157-161.https://doi.org/10.1016/j.wneu.2020.11.019

Journal homepage: www.journals.elsevier.com/world-neurosurgery

Available online: www.sciencedirect.com

1878-8750/$ - see front matter ª 2020 Elsevier Inc. Allrights reserved.

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