Intended for healthcare professionals

Endgames Case Review

Headaches and hormones: a potentially lethal combination

BMJ 2016; 352 doi: https://doi.org/10.1136/bmj.h6752 (Published 26 January 2016) Cite this as: BMJ 2016;352:h6752
  1. Ramdeep Bajwa, foundation year 2 doctor1,
  2. Paven Preet Kaur, foundation year 2 doctor2,
  3. Alessandro Paluzzi, consultant neurosurgeon3
  1. 1Deapartment of Medicine, City Hospital Birmingham, Birmingham, UK
  2. 2Department of General Medicine/Surgery, Townsville Hospital, Townsville, Qld, Australia
  3. 3Department of Neurosurgery, Queen Elizabeth Hospital, Birmingham, UK
  1. Correspondence to: R Bajwa, Department of Emergency Medicine, Gold Coast University Hospital, Qld, Australia rameybajwa{at}gmail.com

An 85 year old man presented to the emergency department with a five day history of headache, vomiting, and progressive visual loss in both eyes. He had no history of weight loss, seizures, or limb or facial weakness. He had hypertension, abdominal aortic aneurysm (under surveillance), hypothyroidism, and benign prostatic hypertrophy.

His blood pressure was 110/75 mm Hg, pulse 80 beats/min and regular, Glasgow coma score (GCS) 15. Peripheral neurological examination was normal. Cranial nerve examination showed bitemporal hemianopia with normal fundoscopy.

Initial investigations showed deranged biochemistry: sodium 126 mmol/L (reference range 135-145), potassium 3.6 mmol/L (3.5-5), urea 5.6 mmol/L (2.5-6.7), creatinine 101 µmol/L (70-150), C reactive protein 66 mg/L (<10). Full blood count and liver function tests were normal.

He was admitted to the acute medical unit after computed tomography of the head showed a mass arising from the pituitary fossa. Magnetic resonance imaging (MRI) showed a sellar mass compressing the optic chiasm and signal changes suggestive of haemorrhagic regions within the mass (fig 1).

Figure1

Coronal T2 weighted magnetic resonance imaging of the head without contrast

Questions

  • 1. What are the differential diagnoses for patients presenting with bitemporal hemianopia?

  • 2. Given the biochemical and radiological abnormalities, how would you immediately management this patient?

  • 3. Will he need pituitary surgery?

  • 4. What long term follow-up would you arrange?

Answers

1. What are the differential diagnoses for patients presenting with bitemporal hemianopia?

Short answer

Pituitary adenomas, pituitary apoplexy, craniopharyngiomas, meningiomas, gliomas, and rarely intracranial aneurysms.

Discussion

The optic chiasm is the anatomical territory where the nasal fibres of the optic nerve decussate. The retinal fibres that receive stimuli from the superior visual field take an inferior course through the optic chiasm, whereas fibres that receive stimuli from the inferior visual field take a superior course.

Bitemporal hemianopia is classically caused by compression of the central optic chiasm as a result of suprasellar extension of, most commonly, a pituitary adenoma. However, various patterns of visual field defects have been described in patients with pituitary adenomas, because the precise type of defect depends on the anatomical relation between the tumour and the optic chiasm. A recent retrospective study conducted in a neurosurgical centre showed that visual loss was the most common presenting feature in patients with pituitary adenoma (39%), followed by endocrine abnormalities (21%) and headache (15%). Bitemporal defects were the most prevalent pattern, followed by homonymous defects.1 Interestingly, visual acuity is often preserved even when visual field loss (which is most accurately assessed by perimetry) is severe.1 2 The onset of the visual deficit is often insidious, with many patients not recognising the deficit for months or even years.3

Craniopharyngiomas are rare sellar or suprasellar tumours derived from remnants of pituitary embryonic tissue, which have bimodal prevalence in paediatric patients and those aged 55-65 years. They often arise from the pituitary stalk (infundibulum), which connects the hypothalamus to the posterior pituitary gland. As the tumour progresses, the mass can extend towards the optic chiasm and eventually lead to bitemporal hemianopia.4

Other intracranial neoplasms, such as meningiomas and gliomas, can also lead to bitemporal hemianopia. Giant aneurysms have also been reported to cause several visual field defects including bitemporal hemianopia.5

2. Given the biochemical and radiological abnormalities, how would you immediately manage this patient?

Short answer

These abnormalities suggest pituitary tumour apoplexy. This medical emergency causes hypopituitarism, particularly cortisol insufficiency, which requires steroid replacement with careful fluid and electrolyte balance.

Discussion

Apoplexy describes an accumulation of blood or infarction in any tissue or organ.6 Pituitary tumour apoplexy is usually caused by haemorrhage or infarction of a pre-existing pituitary tumour. It has an incidence of 2-7% in pituitary adenomas and is a medical emergency.7 Patients with macroadenomas have a significantly higher risk of developing pituitary tumour than those with microadenomas.8 The precise pathogenesis of this condition is not clear, but the peculiar vascular supply of the pituitary gland through the hypophyseal portal system has been implicated.9 Morphologically, pituitary vessels display poor fenestration and fragmented basal membranes, which may also confer a higher risk of apoplexy developing.10

The diagnosis of pituitary tumour apoplexy can be challenging because the tumour itself has often not been diagnosed at the time of presentation.8 The pituitary gland lies within a rigid wall and the clinical manifestations are caused by a rapid increase in intrasellar pressure leading to:

  • Headache, which may be associated with nausea and vomiting

  • Compression of the normal vascular supply to the pituitary gland, which leads to hypopituitarism, particularly cortisol insufficiency as a result of impaired secretion of adrenocorticotrophin from the anterior pituitary gland

  • Compression of the optic chiasm, which leads to visual field defects and decreased visual acuity

  • Compression of structures in the cavernous sinus, which causes cranial nerve palsies.

Acute secondary cortisol insufficiency is seen in about two thirds of patients with pituitary tumour apoplexy and is the major cause of mortality associated with the condition. Empirical steroid therapy should be started in patients who are haemodynamically unstable, have an altered consciousness level, or have reduced visual acuity or visual field defects. Guidelines suggest a bolus of intravenous hydrocortisone 100-200 mg in adults, followed by a 2-4 mg per hour continuous infusion.7 Routine biochemistry and haematology tests plus serum gonadotrophins, growth hormone, testosterone, oestradiol, thyrotropin, free thyroxine, prolactin, and insulin-like growth factor 1 measurements must be performed before starting steroid replacement. Early specialist endocrine and neurosurgical advice is imperative.

Prompt steroid replacement can be life saving. Reports published before corticosteroid treatment was available indicated a mortality rate of 50%.11

3. Will he need pituitary surgery?

Short answer

Pituitary apoplexy with deteriorating neuro-ophthalmic signs or reduced GCS is a surgical emergency and the tumour is commonly resected. Stable patients can be managed conservatively but surgery may be needed if they do not improve.

Discussion

Decisions about conservative versus surgical management of patients with pituitary apoplexy should be made in a specialist centre using a multi-disciplinary approach, with involvement of neurosurgery, ophthalmology, and endocrinology teams.7 All management plans must be tailored to the individual patient with regular clinical evaluation.

Surgery is indicated in all patients who present with severe neuro-ophthalmic signs, including greatly reduced visual acuity, worsening visual field defects, or decreasing GCS.7

If a previously stable patient shows evidence of new or worsening clinical signs, urgent re-imaging is recommended with a view to considering emergency surgery to relieve pressure on the optic chiasm and cavernous sinus.7 While awaiting further specialist input, patients who are unstable require hourly neurological assessment.

Conservative management is effective in haemodynamically stable patients with no neuro-ophthalmic signs or improving signs. Studies show complete or near complete remission of reduced visual acuity, visual field defects, and ocular paresis in most of these patients.12 13 14

Because of the variable and unstable nature of the condition, patients who are managed conservatively will require close monitoring, with four to six hourly neurological assessments of visual fields and visual acuity, as well as cranial nerve examination. Daily monitoring of renal function and electrolytes is also required. Once an obvious trend of improvement is apparent the frequency of clinical and biochemical assessment can be reduced.

Patients with pituitary apoplexy who do not present with visual defects or reduced consciousness can be safely managed with dexamethasone treatment for a week.15 However, surgery should be considered in the first seven days in patients with disturbed visual field or acuity because early intervention improves recovery.16

4. What long term follow-up would you arrange?

Short answer

Guidelines recommend reviewing all patients four to eight weeks after initial presentation and then annually in an endocrinology clinic. MRI of the head is recommended three to six months after initial presentation, yearly for five years, and then biennially.

Discussion

All patients require an endocrine review four to eight weeks after the initial event, with subsequent annual review. A full biochemical assessment of pituitary function and a cranial nerve assessment with particular attention to visual acuity, ocular movements, and visual fields, must be conducted. Patients will require annual pituitary function tests.7

MRI of the head is recommended three to six months after presentation. Because of the risk of tumour regrowth and recurrent apoplexy, surveillance MRI is recommended yearly for the first five years and then biennially.7

Patient outcome

Our patient underwent successful endoscopic trans-sphenoidal resection of the pituitary tumour and was discharged with regular hydrocortisone. Neuro-ophthalmology outpatient review showed improved visual acuity in the right eye of 6/9, improving to 6/6 with a pinhole, and improved visual fields. He had no perception of light in the left eye and poor colour vision in both eyes.

Notes

Cite this as: BMJ 2015;351:h6752

Footnotes

  • Competing interests: We have read and understood BMJ policy on declaration of interests and declare that we have none.

  • Provenance and peer review: Not commissioned; externally peer reviewed.

  • Patient consent obtained.

References

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