Your feelings about Infertility Pituitary Disease Factfile series: for General Practitioners series: for General › The Pituitary Foundation Information Booklets Working to support pituitary patients, their carers & families 2 Pituitary Disease Factfile Contents and Introduction Introduction 3 cross-referenced. Resources are provided on A Guide to Pituitary Apoplexy 5 each Factsheet and an additional separate list, Non-functioning pituitary tumours 6 including organisations, is also included. Acromegaly 9 Abbreviations Cushing’s disease 13 ACTH Adrenocorticotrophic hormone Hyperprolactinaemia 16 AVP Vasopressin (ADH) Hypopituitarism 20 ADH Antidiuretic hormone CSF Craniopharyngioma 24 Cerebrospinal fluid CT Computed tomography Diabetes insipidus 27 DDAVP Desmopressin Traumatic brain injury 29 DI Diabetes insipidus Pituitary surgery 30 FSH Follicle-stimulating hormone Radiotherapy 33 GH Growth hormone LH Luteinising hormone Hormone replacement therapy 37 GnRH Gonadotrophin-releasing hormone Hypogonadism 40 PRL Prolactin Psychological Issues 44 MRI Magnetic resonance imaging Referrals – Who and When to refer 46 TSH Thyroid-stimulating hormone Resource list 49 Introduction - about this factfile Helpline: These Factsheets were written in response to for general patient support enquiries requests by General Practitioners and their 0117 370 1320 pituitary patients for more information on Monday to Friday 10:00am to 4:00pm pituitary disease. The Pituitary Foundation has already produced an excellent set of patient Endocrine Nurse Helpline: booklets explaining how pituitary disease can 0117 370 1317 Mondays 6:00pm to affect patients’ lives, the kind of treatments 9:00pm and Thursdays 9:00am to 1:00pm available and general advice on how to cope with particular problems. This Factfile includes [email protected] more detailed information, specifically written www.pituitary.org.uk for the GP. Each Factsheet gives background information on the condition, how it is investigated and possible treatments. Since many of the investigations and treatments may be used for several different syndromes, the information on the sheets is extensively Pituitary Disease Factfile 3 Acknowledgements Disclaimer: We would like to thank the many specialists in Every care has been taken in the compilation the world of endocrinology who have written of this Factfile to ensure it reflects current these sheets including Stephanie Baldeweg, practice (as at publishing date). Knowledge and Peter Bayliss, Claire Blessing, Peter Clayton, clinical practice continually evolve. The reader Jurgen Honneger, Trevor Howlett, Stafford is therefore advised to check with a specialist Lightman, John Monson, John Newell-Price, endocrinologist or with a product manufacturer Peter Trainer and John Wass. They, alongside if they have any concerns. The publishers and patients and GPs, read and made valued authors are not responsible for any errors or contributions to achieve this updated Factfile. omissions or for any consequences from the © 1999, 2006, 2011 and 2014 to be reviewed application of the information presented in this June 2016. Factfile. 4 Pituitary Disease Factfile Pituitary Foundation fact sheet 1 Pituitary apoplexy A guide to A Guide to Pituitary Apoplexy Bleeding or reduced blood flow in the The bleeding or pituitary infarction may not pituitary gland can result in pituitary show on a Computerised Tomography (CT) damage. This is called pituitary apoplexy. scan. Blood tests are also important to check if If there is only reduced blood flow with the hormones produced by the pituitary gland no bleeding, this condition is also called are adequate. pituitary infarction. It usually happens in In the event of pituitary apoplexy urgent people with pre-existing pituitary tumours. treatment is required. This consists of intravenous The Greek word ‘apoplexy’ literally means fluids, corticosteroids and close monitoring. sudden brain dysfunction. Surgery may be required to relieve the pressure The pituitary gland is situated in a bony and swelling around the pituitary gland. This hollow and is surrounded by important usually can be done by making a small incision structures, such as the nerves responsible for inside the nostril (transsphenoidal surgery). With vision. In apoplexy the gland swells suddenly treatment, the majority of patients recover and if and this can cause pressure in the surrounding the vision was affected it often gradually improves. brain structures. The swelling can also cause the If the vision was severely affected at the beginning, pituitary gland to stop producing one or more it may not fully recover. If the pituitary gland does of the pituitary hormones. not function properly after recovery patients may Up to 5% of patients with pituitary tumours need hormone replacement therapy. Patients will may develop apoplexy at some point. In need regular check ups at a specialist endocrine fact, studies have shown that bigger tumours clinic to monitor the condition. (macroadenomas) carry a higher risk of apoplexy In 2010, a national working group developed compared with smaller ones (microadenomas). guidelines for the management of pituitary High or low blood pressure, head injury, certain apoplexy, to increase awareness amongst doctors medications such as warfarin, cardiac surgery and and to standardise and improve the treatment of very rarely certain endocrine dynamic tests may this rare, but potentially life-threatening condition. increase the risk of apoplexy. It is important that pituitary apoplexy is Indicative References diagnosed and treated. If it remains untreated, 1. Murad-Kejbou S, Eggenberger E. Pituitary apoplexy may cause seriously ill health and apoplexy: evaluation, management, and even death. The most common symptoms are prognosis. Current Opinion in Ophthalmology headache, nausea or vomiting, changes in 2009; 20:456-61. eye-sight, such as double vision, restriction in eye 2. Nawar R, AbdelMannan D et al. Pituitary movement and drowsiness. These symptoms are Tumor Apoplexy: A Review. Journal of not unique in apoplexy but may occur in other Intensive Care Medicine 2008; 23(2): 75-90. conditions such as meningitis, subarachnoid 3. Randeva HS, Schoebel J et al. Classical haemorrhage or (rarely) migraine, from which pituitary apoplexy: clinical features, pituitary apoplexy has to be distinguished. The management best way to diagnose pituitary apoplexy is a and outcome. Clin Endocrinology (Oxf) 1999; Magnetic Resonance Imaging (MRI) scan. 51:181-188. Pituitary Disease Factfile 5 Pituitary Foundation fact sheet 2 Non-functioning pituitary tumours The most common type of pituitary tumour In the presence of pressure signs/ pituitary tumours Non-functioning is non-functioning (i.e. it does not cause symptoms excessive hormone production). Tumours of Patients will usually need transsphenoidal this type most commonly become apparent surgery (factsheet 10), which may be followed when the patient has visual symptoms or by radiotherapy to prevent recurrence (factsheet headaches due to pressure on the optic nerve 11). If pituitary hormones are deficient, and it is often the optician who refers the pituitary hormone replacement therapy will be patient with abnormal visual fields to the GP given (factsheet 12). (in which case the GP would want to refer to an ophthalmologist who, in turn, is likely to Patient management refer to an endocrinologist). The tumour may Post-operative also damage the adjacent, normal pituitary Most patients will have improved, or at least gland (causing hypopituitarism - factsheet 6) stabilised, visual fields. Removal of the nasal or occasionally compress the pituitary stalk, packing, if this method used, is often the causing hyperprolactinaemia (factsheet 5). only part of the procedure that patients find uncomfortable. Some patients may find that Please see ‘Who and When to Refer’ (factsheet 15). their frequency of headaches changes. Other complications, such as Cerebral Spinal Fluid Presenting symptoms (CSF) leaks can occur, although rarely, and • visual disturbance need to be treated by a further small operative • oligomenorrhoea or amenorrhoea in women procedure (factsheet 10). • tiredness and lack of energy • reduced libido and potency in men Long-term • headache Regular visual field assessments may be needed. MRI scans are usually repeated within the Investigations first post-operative months and follow-up scans An MRI scan will be carried out to determine are initially carried out at increasing intervals the size and site of the tumour. Visual field tests from 6 months to 5 years. are used to determine the degree of functional impairment of the visual pathway. Blood tests Radiotherapy will be needed to assess pituitary function. Pituitary radiotherapy (factsheet 11) may be used after surgery to reduce the risk of regrowth Treatment possibilities of the tumour. Since radiotherapy can cause In the absence of pressure signs/ hypopituitarism at variable times after symptoms treatment, patients should be tested for Patients may not need treatment, but will be pituitary function on a regular basis, probably monitored closely using MRI scans and visual field at 6 months, 1 year, 2 years and then bi- or checks at intervals of 6 or 12 months. Alternatively triennially. surgery or, rarely, radiotherapy may be advised. 6 Pituitary Disease Factfile Watchpoints pituitary tumours Urgent - refer to hospital good if the tumour is treated promptly. Non-functioning • Deterioration of vision Treatment is effective in stopping any • Clear