|Year : 2018 | Volume
| Issue : 2 | Page : 43-48
Ocular motility disorders: unusual presentation of pituitary adenoma
Manal A Kasem1, Mohamed Kassem2, Ahmed N Taha2, Al-Sharawy Kamal3
1 Oththalmology Department, Mansoura Ophthalmic Center, Faculty of Medicine, Mansoura University, Mansoura, Egypt
2 Neurosurgery Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt
3 ENT Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt
|Date of Submission||06-Apr-2018|
|Date of Acceptance||16-May-2018|
|Date of Web Publication||30-Aug-2018|
Manal A Kasem
Dakahlyia, Gomhoria Street, Mansoura City 35511
Source of Support: None, Conflict of Interest: None
Background The aim of this study was to describe ocular palsies as a rare presentation of pituitary adenomas and their relation to the size, type, and extension of tumor.
Patients and methods Patients who presented with acquired onset of diplopia or ptosis during the period from February 2014 to January 2017 were included. Patients were referred to the neurology unit for transphenoidal removal of the gland. Full ophthalmologic and neurologic examinations were performed preoperatively and postoperatively.
Results This study included 15 (12 men and three women) patients. Nine (60%) patients had isolated third nerve palsy; four (26.6%) patients had isolated sixth nerve affection; and two (13.3%) patients had total external ophthalmoplegia. Ocular motor disorders occurred more in nonfunctioning macroadenomas (73.3%) with grade 3 paraseller extension (60%). Apoplexy occurred in 46.7% of the cases. After surgical removal of the tumor, ocular motor improvement started within 3 weeks postoperatively. Full recovery occurred in seven (46.6%) patients within 6 months; five (33.3%) patients showed partial recovery; and three (20%) patients showed no improvement.
Conclusions Acquired ocular motility disorders should raise the possibility of pituitary adenoma. The third nerve is the most affected, followed by the sixth nerve. The majority of the cases occurred in nonfunctioning macroadenoma. Transphenoidal tumor excision has a favorable outcome regarding the improvement of ocular motility.
Keywords: ocular palsies, pituitary adenoma, third nerve palsy
|How to cite this article:|
Kasem MA, Kassem M, Taha AN, Kamal AS. Ocular motility disorders: unusual presentation of pituitary adenoma. J Egypt Ophthalmol Soc 2018;111:43-8
|How to cite this URL:|
Kasem MA, Kassem M, Taha AN, Kamal AS. Ocular motility disorders: unusual presentation of pituitary adenoma. J Egypt Ophthalmol Soc [serial online] 2018 [cited 2018 Sep 21];111:43-8. Available from: http://www.jeos.eg.net/text.asp?2018/111/2/43/240123
| Introduction|| |
Pituitary adenoma is an eminent pathology comprising a heterogeneous group of tumors with an overall incidence of 20–25% in the general population. Pituitary adenoma deems to entail a multidisciplinary management with experienced specialists . Neuro-ophthalmological manifestations are the debut symptoms in 10% of the cases owing to the intimate anatomical proximity of the pituitary gland and the optic chiasm as well as the cranial nerves in the cavernous sinus .
Visual manifestations of pituitary adenomas diverge from lack of presenting symptoms to advanced deterioration of visual acuity up to blindness. The classic presentation is the visual field defects which are typically distinctive, with bitemporal hemianopia being the most familiar .
Ocular motility disorders in pituitary adenoma are very rare presentations. Only few reports addressed ocular palsies as a presentation ranging from isolated single-nerve palsy to total ophthalmoplegia ,. The mechanisms of ocular motor nerve palsy in pituitary tumors are diverse and can occur in adenomas of different sizes, types, and extensions ,.
This study included a series of patients presented with ocular palsies as a rare manifestation of pituitary adenoma who underwent trans-sphenoidal surgery. The purpose of this study is to highlight this rare pattern of ocular presentation with its relation to the size, type, and extension of the tumor. Furthermore, the study aimed to assess ocular motor outcomes after surgery.
| Patients and methods|| |
Study design and patients’ enrollment
A retrospective descriptive case series study included 15 patients presented with acute onset of diplopia and ptosis as a presenting symptom for pituitary adenoma based on neurological and radiological assessment. Patients were recruited from the Mansoura Ophthalmic Center; during the period from February 2014 to January 2017. The patients were referred to the neurosurgery department for excision of their pituitary adenomas. Patients with previous intracranial surgeries or radiation therapy for other causes were excluded from the study.
This study was registered and reviewed by the ethics committee of the local Institutional Review Board (R/17.06.138) with adherence to the principles of the Declaration of Helsinki. Written informed consent was obtained from each patient before surgery after clarifying the potential hazards of operation.
All cases were operated through endoscopic trans-sphenoid approach by a team of neurosurgeons and otorhinolaryngologists. The approach was either uninostril or binostril (four hands technique) that adds more exposure, better visualization, and easy manipulation of the adenomas in the parasellar region.
Preoperative and postoperative evaluation
Adequate and full ophthalmological history was obtained from all the patients including age, sex, ocular, endocrinal, and systemic manifestations related to their pituitary adenomas. Full ophthalmological examination was performed both preoperatively and postoperatively. It included Snellen’s chart monocular and binocular visual acuity measurement (unaided and aided) converted to decimal notation for statistical analysis, cycloplegic refraction, full ocular motility assessment (duction, version, and vergence movements), measurement of the angle of strabismus by prism bar and cover test, the degree of ptosis, Bell’s phenomenon, corneal sensation, pupillary reflexes, anterior segment evaluation by slit-lamp, fundus examination by indirect ophthalmoscope, visual field assessment (central and peripheral Humphery perimetry), and the Hess screen test. Full hormonal profiles were done before the surgery to assess the type of adenoma, whether functioning or nonfunctioning. Contrast MRI study of the brain and the sella was done preoperatively and postoperatively to assess the tumor size (vertical dimension), pattern of extension, and presence of apoplexy. The Knosp grading system for parasellar extension was used . MRI was performed immediately and at 3 months postoperatively. Ophthalmologic assessment was performed at 1 week, 1, 3, and 6 months postoperatively to assess the improvement of ocular motility.
The patients’ demographics data were presented as mean±SD for continuous variables and number (%) for categorical variables.
| Results|| |
Fifteen patients were included in this study with a median age of 44.06 years (range: 25–53). Male predominance was observed in 80% of the patients. Headache was the most common systemic symptom occurring in 80% of the cases. Patients’ demographic data and basic systemic manifestations are outlined in [Table 1]. [Table 2] gives detailed ophthalmologic signs. The majority of patients presented with isolated third nerve palsy (nine cases, 60%); two (13.3%) cases had total third nerve palsy (exotropia with limited adduction, hypotropia with limited elevation and depression, severe ptosis, and dilated fixed pupil), and seven (46.6%) cases had partial third nerve palsy (exotropia with some limitation of adduction, no limitation of elevation or depression, mild to moderate ptosis and sluggish pupil in four cases); one of those patients had bilateral partial third nerve palsy ([Figure 1]). Four (26.7%) patients had isolated sixth nerve palsy (esotropia with limited abduction). Total ophthalmoplegia (third, fourth, and sixth nerve palsies) was found in two (13.3%) patients who were orthotropic in the primary position with limitation of ocular motility in all directions, severe ptosis and dilated fixed pupil. None of the cases presented with isolated fourth nerve palsy. No papilledema was found in any of the patients. Pupillary light reflexes were normal in seven cases (four with sixth nerve palsy and three cases of partial third nerve palsy); four cases had sluggish pupillary reaction and four cases had dilated fixed pupil. Anisocoria (unequal pupillary sizes) was found in eight cases (two cases of total ophthalmoplegia, two cases with total third nerve palsy, and four cases with partial third nerve palsy). Visual field examination has shown peripheral bitemporal hemianopia in nine patients, seven of them showed apoplexy on MRI. [Table 3] shows the relation of ocular nerve palsies to the characters of adenomas. Ocular motor disorders occurred more in nonfunctioning macroadenomas (73.3%) with grade 3 paraseller extension (60%). Apoplexy occurred in 46.7% of the cases. [Table 4] gives details about the surgical plan and postoperative outcome of all cases at sixth month. Twelve (80%) patients showed improvement of their preoperative ocular motility disorder. Improvement started to occur within 3 weeks; full recovery occurred in seven (46.7%) patients within 3 months ([Figure 2]), while five patients developed partial recovery. Three (20%) patients did not show improvement of their ocular symptoms. Visual field improved in77.8% (seven out of nine affected) patients. Postoperative ocular complications were few and transient. Surgery-related ocular and systemic morbidities are given in [Table 4].
|Figure 1 A female patient aged 32 years; she presented with bilateral partial third nerve palsy: (a) right exotropia and mild ptosis in the primary position; (b) limitation of adduction in the left eye; (c) limitation of adduction in the right eye (the limitation of adduction is more in the right than in the left eye); (d); and (e) preserved bilateral elevation and depression.|
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|Figure 2 A female patient aged 50 years who presented with right third nerve palsy: (a) ptosis, (b) limited adduction, (c) limited elevation, (d) MRI brain axial view shows pituitary adenoma (Knosp grade 2 parasellar extension), (e) 3 months postoperative with improvement of ptosis (recovery of the third nerve). The red arrow points to invasion of right cavernous sinus and encasement of right internal carotid artery.|
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| Discussion|| |
Ocular motor palsy due to pituitary adenoma was reported to occur in only 5–17% of cases . Several published studies had suggested various mechanisms for the pathophysiology of such palsies, including either indirect compression on the ocular cranial nerves by compressing the cavernous sinus or direct compression through cavernous sinus invasion ,. Adenomas that usually invade the cavernous sinus grow through fragile medial sinus wall with mediolateral expansion resulting in lateral displacement of the internal carotid artery, with third nerve compression in the oculomotor trigone (roof of the cavernous sinus). Thus the most commonly involved by the compression is the third nerve. At the oculomotor trigone there is a whole cistern of cerebrospinal fluid which represents a possible route for tumor invasion ,. Vascular occlusion also has been reported as a mechanism for third nerve palsy, due to compression of its blood supply originating from the internal carotid artery. Yokoyama et al.  demonstrated the natural history for a nonfunctioning adenoma that usually started asymptomatic, then reaching a large size to invade the cavernous sinus, finally compressing the third nerve to be symptomatic. These findings are consistent with the results of our study, the majority of the cases (60%) presented by isolated third nerve palsy; of them, seven cases were nonfunctioning adenoma. Same findings were reported by Bujawansa et al. .
Another proposed mechanism for ocular palsy was the occurrence of sudden increase in the size of the pre-existing adenoma, with rapid deterioration of symptoms . This condition is known as pituitary apoplexy, which represents a widely acceptable theory for the occurrence of total external ophthalmolpegia in more than 70% of cases of apoplexy, or sudden onset of ocular palsy in cases of pituitary adenoma ,,. In our series, two of the cases presented with total ophthalmoplegia were diagnosed with apoplexy.
Isolated sixth nerve palsy as a presentation of pituitary adenoma is very rare . The sixth nerve passes within the cavernous sinus adjacent to the internal carotid artery being less amenable to compression. Lateral rectus palsy occurs when the sixth nerve is compressed by posterior tumor expansion toward the petrous bone where the abducent nerve is tethered to a firm structure at Dorello’s canal. This is commonly observed with aggressive expansion of pituitary neoplasms as reported by many studies ,,. In the current series, isolated sixth nerve palsy was presented in four cases (two cases of adrenocorticotropic hormone-secreting adenoma secreting macroadenoma and two cases of nonfunctioning macroadenoma with apoplexy).
The fourth nerve is protected at its entry into the cavernous sinus by a doubling of the dura mater; so isolated fourth nerve palsy is very rare except in case of massive compression of the cavernous sinus, involving all the ocular motor nerves ,,. None of our cases developed isolated fourth nerve palsy, only involving two cases of total external ophthalmoplegia due to pituitary apoplexy. It was noted that in cases of ocular palsy occurring due to pituitary tumors of all muscles supplied by the third nerve, the levator palpebrae superioris was the most commonly affected, as shown by partial or complete ptosis. It was evident in our cases where 11 (73.33%) cases presented with ptosis of variable degrees. The development of ptosis in the early stages of third nerve palsy suggested an effect on the fibers going to the levator palpebrae muscle which is located superficially in the medial portion of the nerve within the cavernous sinus . The postoperative recovery of cranial nerves, either the optic nerve or the cranial nerves controlling ocular muscles, was achievable if rapid surgical decompression was done ,,. In our study, 12 patients had improved (full improvement in seven patients and partial in five patients).
| Conclusion|| |
Ocular cranial nerve palsy may raise the possibility of pituitary adenomas. The most common presentation was the isolated third nerve palsy followed by sixth nerve palsy. The most common type was the nonfunctioning macroadenoma. Majority of the cases developed pituitary apoplexy. Early tumor excision with cranial nerves decompression has a favorable outcome regarding the improvement of ocular motility.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3], [Table 4]