Apoplexy in pituitary macroadenoma presenting with total ophthalmoplegia and preserved vision: A rare entity

Samir J Yelwatkar; Vishakha V Jain; Pankaj Banote

doi:10.4103/0971-9903.117797

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CASE REPORT

Year : 2013 | Volume : 18 | Issue : 2 | Page : 140-143

Apoplexy in pituitary macroadenoma presenting with total ophthalmoplegia and preserved vision: A rare entity

Samir J Yelwatkar¹, Vishakha V Jain¹, Pankaj Banote²
¹ Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Wardha, Maharashtra, India
² Department of Radiology, Jawaharlal Nehru Medical College, Sawangi, Wardha, Maharashtra, India

Date of Web Publication

6-Sep-2013

Correspondence Address:
Vishakha V Jain
Assistant professor, Department of Medicine, Mahatma Gandhi Institute of medical sciences, Sevagram, Wardha, Maharashtra - 442102
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/0971-9903.117797

Abstract

Pituitary macroadenoma is a rare tumor with varied presentation. It can present acutely due to apoplexy. Here, we present a rare presentation of total ophthalmoplegia with normal vision in a case of pituitary macroadenoma with apoplexy.

Keywords: Apoplexy, normal vision, pituitary macroadenoma, total ophthalmoplegia

How to cite this article:
Yelwatkar SJ, Jain VV, Banote P. Apoplexy in pituitary macroadenoma presenting with total ophthalmoplegia and preserved vision: A rare entity. J Mahatma Gandhi Inst Med Sci 2013;18:140-3

How to cite this URL:
Yelwatkar SJ, Jain VV, Banote P. Apoplexy in pituitary macroadenoma presenting with total ophthalmoplegia and preserved vision: A rare entity. J Mahatma Gandhi Inst Med Sci [serial online] 2013 [cited 2023 Jun 7];18:140-3. Available from: https://www.jmgims.co.in/text.asp?2013/18/2/140/117797

Introduction

Pituitary tumors are rare with an incidence of only 14.4% in around 3500 autopsied patients and 22.5% among 200 patients with imaging studies (overall 16.7%). Among these, macroadenomas are seen only 1 in 600 patients. ^[1] Many a times, they present as "incidentelomas" on imaging. Pituitary tumor apoplexy is a rare neuroendocrine syndrome resulting from hemorrhage or infarction of a pre-existing pituitary adenoma. Apoplexy in pituitary tumor is uncommon and underdiagnosed entity. The calculated prevalence from epidemiological surveys of operated patients suggests prevalence of 0.6-12.8. ^[1],[2] Usually, apoplexy presents as neurological and endocrinological signs and symptoms. Headache due to raised intracranial pressure is the commonest complaint (63-100% cases), followed by visual deficits (40-100%), cranial nerve palsies, and vomiting. ^[1] Pituitary tumor with apoplexy involving III cranial nerve or two of the ocular nerves have been documented in literature. But, external ophthalmoplegia with normal vision is the rare presentation. Here, we report a rare case of pituitary macroadenoma with apoplexy presenting as total ophthalmoplegia with normal vision.

Case Report

A 52-year-old farmer presented to us with a history of acute onset drooping of left eyelid and retro-orbital headache since 4 days. There was no history of fever, vomiting, weakness in any limb, facial weakness, seizures, neck stiffness, blurring of vision or ataxia. He was hypertensive since 5 years on anti-hypertensive medications and a non-diabetic. On examination, he was conscious, oriented, with blood pressure of 140/90 mm of Hg and pulse of 84/min. Neurologically, he had complete unilateral ptosis of left eyelid, left III, IV, VI cranial nerve palsy with sluggishly reacting pupils. His visual acuity was preserved; visual field examination and fundus examination was normal. Other cranial nerve examination and rest of the neurological examination were essentially normal. Other systemic examination was unremarkable. Clinically, a possibility of cerebral sinus venous thrombosis (CSVT) or a cerebrovascular accident (CVA) was kept. His random blood sugar was 110 mg/dl, hemoglobin of 12 gm/dl; total leukocyte counts were 4000 c/mm; serum sodium was 134 mEq/l; and serum potassium was 3.9 mEq/l. X-ray skull showed an enlarged sella turcica [Figure 1]. His magnetic resonance imaging (MRI) brain with venous sequences was done. It showed solid mass lesion in the region of pituitary fossa with hyperintense signal on T2-weighted images, with suprasellar extension and left cavernous sinus invasion, with pituitary apoplexy (infarct) likely to be pituitary macroadenoma invading into cavernous sinus [Figure 2] and [Figure 3].

Figure 1: Right lateral view of X-ray skull showing enlarged sella turcica

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Figure 2: MRI showing solid mass lesion at sella with suprasellar and left lateral extension

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Figure 3: MRI showing solid mass lesion at sella with suprasellar and left lateral extension (Indicated by yellow arrows)

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His sinus venous system on MRI- venous sequences were normal. There was no evidence of sub-archanoid hemorrhage or meningitis. Laboratory investigations revealed normal hormonal profile (thyroid-stimulating hormone-TSH = 4.8 μIU/ml, T3 = 163 ng/dL, T4 = 8 mcg/dL, Follicle stimulating hormone-FSH = 10.2 mIU/ml, Luteinizing hormone-LH = 7.2 mIU/ml, S. Testosterone = 35.7 pg/ml, Basal Cortisol = 450 nmol/ml, Growth hormone (GH = 8 ng/ml) and Insulin-like growth factor-1 (IGF-1 = 213 ng/ml)), suggesting a non-functioning pituitary macroadenoma. He was treated with intravenous fluids and Inj. hydrocortisone 100 mg IV bolus, followed by 50 mg IV 6 hrly. In view of his clinical and imaging findings, patient was referred to a higher center with a neurosurgeon and endocrinologist for better management. On follow-up after 4 weeks of discharge, patient had partial improvement in his ophthalmoplegia and continued to have normal hormonal profile.

Discussion

Pituitary macroadenoma usually present with endocrinological disturbances or due to mass effect causing a pressure on the adjoining structures like optic chaisma leading to visual disturbances. Both of these features are gradual and present a chronic course. ^[3] Acute presentation in a case of pituitary macroadenoma may be due to infarction or hemorrhage in the tumor. Classical pituitary apoplexy refers to a clinical syndrome, characterized by sudden onset of headache, vomiting, visual impairment, and decreased consciousness caused by hemorrhage and/or infarction of the pituitary gland. ^[4],[5],[6] Clinical presentation may be variable either with all clinical signs or minimal signs, and the lesion can be detected on MRI examination. ^[7] Apoplexy usually occurs in patients with pre-existing pituitary adenomas and evolves within hours or days. Precipitating factors (Systemic hypertension, major surgery, dynamic pituitary function tests with GnRH, TRH and CRH, anti-coagulation therapy, coagulopathies etc.) have been identified in up to 40% of cases of pituitary apoplexy. But, hypertension is the commonest (26%) predisposing factor. ^[6] In our patient, the presentation was with classical features of headache and ptosis. Patient was a known case of hypertension, which may have precipitated this apoplexy.

The earliest and most common presentation of pituitary apoplexy is headache (up to 100%), followed by ocular palsies in 70% patients. Headache is usually retro-orbital or bifrontal. Cranial nerve palsies are usually due to lateral compression and most often involve the third cranial nerve (50% cases). Decreased visual acuity and visual field defects, specifically bitemporal hemianopia, are seen in nearly 75% of the patients and are caused by upward enlargement of the intrasellar contents, leading to optic chiasmal compression. ^[7] Extravasation of blood or necrotic tissue in to the sub-arachnoid space can cause meningism resulting in fever, photophobia, and altered consciousness level. Rarely, cerebral ischemia can result from either mechanical compression of the carotid artery against the anterior clinoid or vasospasm secondary to sub-arachnoid hemorrhage. ^[8] Our patient presented with headache and unilateral ophthalmoplegia, ptosis, and normal vision, which was probably due to the lateral growth of tumor on the left side invading into the cavernous sinus.

The immediate management of a case of pituitary apoplexy depends on the hemodynamic stability and the fluid electrolyte status of the patient. Patients with pituitary apoplexies that are hemodynamically unstable or have altered consciousness or have decreased visual acuity and severe visual field defects should be commenced on empirical steroid therapy. In adults, hydrocortisone 100-200 mg as an intravenous bolus is appropriate, followed either by 2-4 mg per hour by continuous intravenous infusion or by 50-100 mg six hourly by intramuscular injection. Also, patients with serum cortisol less than 550 nmol/l are candidates for empirical steroid therapy. ^[6] Though our patient had normal serum cortisol levels, steroid was started due to presence of cranial nerve palsies.

Prognosis of pituitary tumor apoplexy has improved with declining morbidity and mortality. Factors that have contributed to this include improvement in early diagnosis, better therapeutic support, use of glucocorticoids, and refinement of surgical and post-operative techniques. ^[2] The rarity of pituitary apoplexy renders it a difficult subject for audit; hence, there are no evidence-based standards of optimum care for such patients. The main controversy lies in referral of patients for acute neurosurgical intervention. Some author's have suggested that patients presenting with pituitary apoplexy without any neuro-ophthalmic signs or in which visual deficits are mild and stable or improving may be managed conservatively by glucocorticoids replacement as there is no identifiable deleterious effect on visual or endocrine outcome. In patients with reduced visual acuity or defective visual fields, formal assessment of visual fields and acuity should be performed every day until a clear trend of improvement is observed. ^[4],[6]

Indications for surgery are severe neuro-ophthalmic signs such as severely reduced visual acuity, severe and persistent or deteriorating visual field defects, or deteriorating level of consciousness. Ocular paresis because of involvement of III, IV, or VI cranial nerves in the cavernous sinus in the absence of visual field defects or reduced visual acuity is not in itself an indication for immediate surgery. Resolution will typically occur within days or weeks with conservative management. ^[6]

Conclusions

Pituitary apoplexy is an uncommon and underdiagnosed complication of pituitary adenomas. Patients can have varied presentations. Timely diagnosis by imaging studies can help decrease the morbidity and mortality. Conservative management with steroids is given to hemodynamically unstable patients. Surgical intervention is offered to patients with severe neuro-ophthalmic signs.

References

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