Practice of symptomatic treatment in the era of evidence-based medicine: report of 2 cases of diagnosis of Sheehan’s syndrome delayed till eighth decade
- Abdul Majid Wani1,
- Waleed Mohd Hussain1,
- Mousa Ali Al Mejally1,
- Abdulhakeem Amroon Banjar1,
- Khaled Shawkat Ali1,
- Amer Mohd Khoujah2,
- Sadeya Hanif Raja1,
- Mazen G Bafaraj2,
- Wail Al Miamini1,
- Mubeena Akhtar1
- 1Hera General Hospital, Makkah, 21955, Saudi Arabia
- 2Umm Al-Qura University, Makkah, 21955, Saudi Arabia
- Correspondence to Abdul Majid Wani,
- Published 6 May 2010
Sheehan’s syndrome, first described in 1937, is characterised by postpartum haemorrhage, pituitary necrosis, lactational failure and hypopitutarism. Presentation is variable and late presentations are not unusual due to partial ischaemic injury of the pituitary and gradual loss of endocrine function. A history of postpartum haemorrhage is usual but in some cases it is not elicited. Presentations such as malaise, fatigue, hypoglycaemia, decline in cognition, hyponatraemia, pancytopoenia, osteoporosis, secondary infertility, confusion and coma have all been reported. Two interesting cases of Sheehan’s syndrome are presented that were diagnosed in the eighth decade; one due to atypical presentation of recurrent hyponatraemia and confusion, another from hypoglycaemic coma and symptoms of malaise and lethargy.
We decided to publish these case reports to highlight the fact that symptoms and signs attributed to normal ageing could be features of a treatable illness. Also, we wish to emphasise the importance of careful evaluation rather than treating symptoms and signs.
A 77-year-old woman has been attending our hospital for the last 8 years for diabetes mellitus and has been admitted more than 10 times for severe hyponatraemia. Each time the patient was discharged after treatment with hypertonic saline. Presently, the patient was admitted in an acute confusional state and examination revealed a pale, thin, older woman, confused with scanty hair. Blood pressure was 100/60 mm Hg in supine posture and skin was cold, coarse but pale and thin. There was mild puffiness of the face. Deep tendon reflexes showed delayed relaxation. Systemic examination was normal. Complete blood counts were normal and blood sugar was 9.1 mmol/litre. Serum sodium level corrected for blood glucose was 112 mmol/litre (normal 136–145 mmol/litre). Blood urea nitrogen was 8 mg/dl (normal up to18–40). An x-ray of the chest was normal. Panhypopituitarism was suspected and an obstetric history requested. The patient had had eight normal vaginal deliveries, and all the children were breast fed except the last one due to failure of lactation. The patient’s older daughter revealed that her mother had had no menstrual cycles after that. A history of postpartum haemorrhage was not clear as the patient was confused and relatives were not sure. Sheehan’s syndrome was suspected and a complete hormonal profile requested: serum cortisol was74 nmol/litre (normal 138–635), follicle stimulating hormone (FSH) 4.56 mIU/ml (normal 1.00–10.00), luteinising hormone (LH) 2.31 mIU/ml (normal 1.68–15.00), free thyroxin (FT4) 8.0 pmol/litre (normal 12.0–22.0), thyroid stimulating hormone (TSH) 1.000 μIU/ml (normal 0.270–4.200), prolactin (PRL) 7 mIU/litre (normal 16–68), adrenocorticotrophic hormone (ACTH) undetectable and growth hormone was <2 pmol/litre (normal >5). In spite of her menopausal age, the FSH and LH levels were low as was TSH in spite of low FT4. Thus, a diagnosis of Sheehan’s syndrome was made after MRI of the brain was normal. The patient was given hydrocortisone followed by thyroxin and oral prednisolone. Within 3 days the patient’s serum sodium became normal, the patient became alert and was discharged ambulatory. The patient is on regular follow-up and is doing well.
A 71-year-old woman had visited the hospital for fatigue, aches and pains and irritability previously on a number of occasions. The patient was given symptomatic treatment and some anxiolytics. The patient had also been admitted in a hypoglycaemic coma twice previously and discharged after intravenous dextrose. Presently, the patient came to the emergency department with altered sensorium for the last 2 days. The patient was disoriented, pale and thin with cold clammy extremities. Blood pressure was 90/70 mm Hg and random sugar 3.0 mmol/litre. The patient was given normal saline and received 50% dextrose. Complete blood counts were normal except total leucocyte count of 13.1 cells/mm3 (normal 4.0–11.0). Serum sodium was 119 mmol/litre and serum potassium 4.9 mmol/litre. Addisonian crisis was suspected and hydrocortisone 100 mg 8 hourly was given in addition to antibiotics. The patient improved and was admitted to a ward. A hormonal profile was requested and the results were: cortisol 50.67 nmol/litre, FSH 5.28 mIU/ml, LH 1.40 mIU/ml, FT4 4.99 pmol/litre and TSH 0.162 μIU/ml. Growth hormone, prolactin and ACTH were undetectable. Panhypopituitarism was confirmed and an MRI of the brain was normal. A medical history was requested and revealed the patient had had five children, with all the children breast fed. The patient had noticed early breast atrophy and had no menstrual cycles after the last delivery. Sheehan’s syndrome was suspected, although a history of postpartum haemorrhage was denied. Hydrocortisone was stopped and replaced with prednisolone 7.5 mg/day and thyroxin 50 μg/day added. The patient improved and is doing well on replacement therapy.
Normal age related symptoms, hypopitutarism, dyselectrolytaemia, hypoglycaemia, septic shock and malnutrition.
Sheehan’s syndrome occurs as a result of ischaemic pituitary necrosis due to severe postpartum haemorrhage. It may rarely be seen without massive bleeding or after normal delivery. Improvements in obstetric care and availability of rapid blood transfusion coincided with a remarkable reduction in the frequency of Sheehan’s syndrome, particularly in developed countries. However, the number of cases from developed countries has recently increased. Due to primitive obstetrics care, it is one of the most common causes of hypopituitarism in underdeveloped and developing countries.1,2 Postpartum haemorrhage leading to pituitary necrosis is one of the explanations for hypopituitarism of Sheehan’s syndrome. Some sources have reported autoimmunity to a partially damaged pituitary gland.3 The clinical presentation can be acute if the pituitary damage is massive and failure of lactation and amenorrhoea are the commonest presentations. Adrenal failure, hypothyroidism and hypogonadism are also well known.4 The diagnosis is easy in adult women of reproductive age because of early presentation, concern about body structure, breast size, hair loss and secondary infertility. Although pregnancy has been reported with Sheehan’s syndrome, these are mostly case reports.5
In older patients presentation is variable as malaise, fatigue, lethargy, loss of bone mass, skin changes, cessation of menses and so on are all attributed to old age.6 However, awareness about the skin changes of growth hormone deficiency such as loss of skin mass, decreased sebum production and increased facial puffiness should always raise the suspicion of Sheehan’s syndrome.7 Our first case had all these features of growth hormone deficiency in addition to hypothyroid features. The presence of recurrent hyponatraemia made our suspicion stronger as did the history of lactational failure. Hyponatraemia and hypoglycaemia have been reported as presentations of hypopitutarism8,9 in older patients that, if ignored or not diagnosed in time, can prove fatal. Hypoglycaemia is another fatal presentation of hypopitutarism and Sheehan’s syndrome and was reported in 44% cases in one study.10 Our second case presented in hypoglycaemic coma and shock, the latter being due to adrenal failure. Absence of a history of diabetes or any hypoglycaemic drug intake made our suspicion stronger, as did the hyponatraemia. This has been the patient’s third episode of hypoglycaemia and coma, which improved with symptomatic treatment. This approach can have disastrous consequences and emphasis must be made on finding the underlying cause irrespective of age. Lethargy, cognitive decline, falls and skin changes are features of old age, but it is worth ruling out hypopitutarism in a suitable clinical setting to provide the appropriate replacement therapy and reverse these pseudoageing symptoms and signs.
Careful evaluation is needed in older patients presenting with coma, confusion and dyselectrolytaemia.
Attention must be given to finding the cause of an illness and the practice of symptomatic treatment should be avoided.
Caution is needed in older patients whose symptoms and signs are attributed to normal ageing, as these could be presentations of a treatable illness.
Sheehan’s syndrome can have atypical presentation and emphasis must be put on obstetric history to avoid delay in diagnosis.
We acknowledge the support of our director of quality control and patient safety advocacy, Ms Fadawa A R Bawazir.
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.