|Year : 2014 | Volume
| Issue : 4 | Page : 550-553
Sheehan's syndrome with acute renal failure and severe preeclamptic toxemia
Bahaa El-Din Ewees, Hatem S Abdel Hamid
Department of Anesthesia and ICU, Faculty of Medicine, Ain Shams University, Cairo, Egypt
|Date of Submission||01-Sep-2014|
|Date of Acceptance||17-Sep-2014|
|Date of Web Publication||28-Nov-2014|
Hatem S Abdel Hamid
Department of Anesthesia and ICU, Faculty of Medicine, Ain Shams University, Cairo, 11566
Source of Support: None, Conflict of Interest: None
Sheehan's syndrome is a well-known cause of panhypopituitarism secondary to pituitary apoplexy, which generally occurs after an intrapartum or postpartum bleeding episode characterized by severe hypertension or hemorrhagic shock. The diagnosis can be difficult and is often formulated after some years from the syndrome occurrence. We report the case of a woman with an early diagnosis of early onset Sheehan's syndrome associated with severe preeclamptic toxemia complicated by acute renal failure.
Keywords: acute renal failure, obstetric ICU, panhypopituitarism, severe preeclamptic toxemia, Sheehan′s syndrome
|How to cite this article:|
Ewees BE, Abdel Hamid HS. Sheehan's syndrome with acute renal failure and severe preeclamptic toxemia. Ain-Shams J Anaesthesiol 2014;7:550-3
|How to cite this URL:|
Ewees BE, Abdel Hamid HS. Sheehan's syndrome with acute renal failure and severe preeclamptic toxemia. Ain-Shams J Anaesthesiol [serial online] 2014 [cited 2020 Feb 21];7:550-3. Available from: http://www.asja.eg.net/text.asp?2014/7/4/550/145720
| Introduction|| |
Sheehan's syndrome is a rare but potentially serious postpartum complication. It was first described in 1937 by Sheehan ; it is the ischemic necrosis of the pituitary gland secondary to a brutal and extended shock due to obstetric hemorrhage.
This syndrome generally occurs after an intrapartum or postpartum bleeding episode characterized by severe hypotension or hemorrhagic shock. Vasospasm, thrombosis, and vascular compression of the hypophyseal arteries have also been described as possible causes of the syndrome. Some authors believe that Sheehan's syndrome may occur even in the absence of any detectable postpartum hemorrhage, but this seems to be a very rare occurrence .
Hypertrophy and hyperplasia of lactotrophs during pregnancy results in the enlargement of the anterior pituitary, without a corresponding increase in blood supply.
Second, the anterior pituitary is supplied by a low-pressure portal venous system .
These vulnerabilities, when affected by major hemorrhage or hypotension during the peripartum period, can result in ischemia of the affected pituitary regions leading to necrosis.
The posterior pituitary is usually not affected because of its direct arterial supply.
Most common initial symptoms of Sheehan's syndrome are agalactorrhea (absence of lactation) and/or difficulties with lactation. Many women also report amenorrhea or oligomenorrhea after delivery. In some cases, a woman with Sheehan syndrome might be relatively asymptomatic, and the diagnosis is not made until years later, with features of hypopituitarism. Such features include secondary hypothyroidism with tiredness, intolerance to cold, constipation, weight gain, hair loss, and slowed thinking, as well as a slowed heart rate and low blood pressure (BP). Another such feature is secondary adrenal insufficiency, which, in the rather chronic case, is similar to Addison's disease with symptoms including fatigue, weight loss, hypoglycemia (low blood sugar levels), anemia, and hyponatremia (low sodium levels). Such a woman may, however, become acutely exacerbated when her body is stressed by, for example, a severe infection or surgery years after her delivery, a condition equivalent with an Addisonian crisis. Gonadotropin deficiency will often cause amenorrhea, oligomenorrhea, hot flushes, or decreased libido. Growth hormone deficiency causes many vague symptoms including fatigue and decreased muscle mass .
Uncommonly, Sheehan syndrome may also appear acutely after delivery, mainly by hyponatremia. There are several possible mechanisms by which hypopituitarism can result in hyponatremia, including decreased free-water clearance by hypothyroidism, direct syndrome of inappropriate antidiuretic hormone (ADH) hypersecretion, and decreased free-water clearance by glucocorticoid deficiency (independent of ADH). The potassium level in these situations is normal, because adrenal production of aldosterone is not dependent on the pituitary. There have also been cases with acute hypoglycemia .
The order of frequency of hormone loss has generally been found to be growth hormone, gonadotropins [follicle-stimulating hormone (FSH), luteinizing hormone (LH)], adrenocorticotropin (ACTH), and thyroid-stimulating hormone (TSH) .
The diagnosis of Sheehan's syndrome is based on the patient's history and physical examination, on laboratory tests - including hormone levels and hormone stimulation tests - and on computed tomographic (CT) scans or, preferably, MRI scans. Laboratory tests will reveal panhypopituitarism with low thyroxine, estradiol, and cortisol levels and with inadequately low levels of TSH, FSH, LH, and ACTH. Frequently, laboratory tests will also reveal hyponatremia, which occurs in 33-69% of cases and represents the most common electrolytic disorder in Sheehan's syndrome . Hyponatremia has a late onset and can be induced by different causes, such as volume depletion, cortisol deficiency, hypothyroidism, or a syndrome characterized by inappropriate secretion of ADH. In contrast, hyponatremia is rarely observed during the early postpartum period as a direct consequence of panhypopituitarism.
The MRI study of the pituitary gland may reveal different features depending on the stage of the disease. Early scans demonstrate a nonhemorrhagic enlargement of the pituitary gland leading to its subsequent involution, whereas late scans typically show an empty sella. A secondary empty sella is considered a characteristic finding in the classical form of Sheehan's syndrome . However, very few observations of CT or MRI features in the acute phase are available.
| Case history|| |
A 24-year-old woman presented to our ICU Unit in Ain Shams Obstetrics and Gynecology Hospital after emergency lower segment Cesarean section, complicated by accidental hemorrhage with severe preeclamptic toxemia; the patient received her treatment as per ICU protocol for preeclampsia and was transferred to the ward. After 24 h, the patient was readmitted to ICU with anuria over the last 12 h before readmission and elevated renal functions.
Upon ICU admission, the patient was hemodynamically stable, with BP of 115/80, pulse of 85 bpm regular, temperature of 36.8°C (axillary), and SpO 2 of 100% on room air.
Glasgow Coma Scale upon admission was 15; the patient was alert, conscious, and oriented, with no neurological deficit.
Chest and heart examination revealed no detected abnormality; abdominal examination showed abdominal distension, and limbs examination was apparently normal.
Laboratory data upon admission revealed hemoglobin of 9.3 g/dl, white blood cells 17.3 × 10 9 /l, platelets 429 × 10 9 /l, SGOT 98 IU/l, prothrombin time 15.9 s, SGPT 137 IU/l, partial thromboplastin time, 47.3 s, total bilirubin 3.9 mg/dl, international normalized ratio 1.6, direct bilirubin 2.9 mg/dl, blood urea nitrogen 57 mg/dl, albumin 2.5 g/dl, creatinine 3.8 mg/dl, sodium 122 mEq/l, potassium 6.2 mEq/l, calcium 7.9 mg/dl, magnesium 5.8 mg/dl, phosphorus 7.6 mg/dl, lactate dehydrogenase 2701 IU/l, chlorine 95 mEq/l, and random blood sugar 112 mg/dl.
Arterial blood gases on room air revealed: pH 7.32, pO 2 110 mmHg, pCO 2 38.5 mmHg, HCO 3 19.7, SBE -5.9, and Sat 98.4%. Central venous line was inserted upon admission with central venous pressure around 12 cmH 2 O. Chest radiograph was normal. Radiograph abdomen erect and supine was normal (for the abdominal distension).
Nephrology consultation requested abdominal ultrasound and recommended adequate hydration and close monitoring of fluid balance, acid - base status, and serum electrolytes with follow-up of renal profile for the potential need of renal replacement therapy.
Abdominal ultrasound revealed mild perisplenic collection in Morrison's pouch.
Patient received intravenous crystalloids guided by central venous pressure monitoring, in addition to dextrose+insulin infusion.
Furosemide infusion was initiated at a rate of 5 mg/hour; prophylactic antibiotic (ceftriaxone 1 g/24 h intravenously), antistress measures (omeprazole 40 mg/24 h intravenously), and deep venous thrombosis prophylaxis (enoxaparine 20 IU/24 h subcutaneously) were given, and the patient was kept NPO for nonaudible intestinal sounds.
The patient became hypotensive 6 h after admission: BP, 80/40 and heart rate, 60/min; dopamine infusion was initiated at a rate of 10 μg/kg/min.
Cardiology consultation with echocardiography revealed ejection fraction of 62%, normal left ventricle dimensions and contractility, no regional wall motion abnormalities, no pericardial effusion, and trivial tricuspid regurge.
Patient began to suffer from disturbed consciousness and incoherent speech, still anuric. Nephrology recommended to initiate hemodialysis session; patient was placed on noradrenaline (100 ng/kg/min; BP: 120/60 h:80/min).
On the following day, the patient underwent a session of hemodialysis with ultrafiltration of 1 l that passed uneventful.
Noradrenaline was gradually withdrawn; on the following day, the patient was hemodynamically stable and conscious but still incoherent speech, and UOP increased to 800 ml in 2 h.
Thyroid profile revealed decreased TSH, T 3 , and T 4 levels: TSH: 0.066; reference range (0.39-3.5); T 3 : 0.5; reference range (2.1-3.8); and T 4 : 0.23; reference range (0.82-1.63).
CT scan revealed a hyperdense area in the sella turcica (suspected ischemic pituitary apoplexy). Serum prolactin was markedly decreased. Serum prolactin was 12 μg/l; reference range (100-150 μg/l). Serum cortisol and ACTH levels were markedly decreased. Serum cortisol was: morning, 1.9 μg/dl; reference range (5-25 μg/dl) and midnight, 0.6 μg/dl; reference range (2.9-13 μg/dl). ACTH was 2.4 pg/ml; reference range (10-60 pg/ml).
Endocrinology consultation was performed and the patient was initiated on l-troxin 50 mg/24 h postoperatively and solucortef 100 mg/8 h intravenously.
The patient condition gradually improved within 7 days of ICU admission; hemodynamics was stable (weaned off inotropic and vasoactive support), was conscious, alert, and had adequate UOP around 50-100 ml/h.
Upon ICU discharge, the patient was hemodynamically stable: BP: 140/80, pulse: 70 bpm regular, temperature: 37.1°C (axillary), and SpO 2 : 98% on room air.
Glasgow Coma Scale was 15; patient was alert, conscious, and oriented, with no neurological deficit.
Chest and heart examination revealed no detected abnormality; abdominal examination (tolerating oral intake with positive bowel motion) as well as limbs examination were apparently normal.
Laboratory data upon discharge revealed hemoglobin of 10 gm/dl, white blood cells 11.3 × 10 9 /l, platelets 325 × 10 9 /l, SGOT 56 IU/l, prothrombin time 11 s, SGPT 45 IU/l, partial thromboplastin time 31 s, total bilirubin 2.5 mg/dl, international normalized ratio 0.9, direct bilirubin 1.6 mg/dl, blood urea nitrogen 24 mg/dl, albumin 3.5 g/dl, creatinine 0.8 mg/dl, sodium 140 mEq/l, potassium 3.9 mEq/l, calcium 7.2 mg/dl, magnesium 1.9 mg/dl, phosphorus 2.2 mg/dl, lactate dehydrogenase 450 IU/l, chlorine 97 mEq/l, and random blood sugar 123 mg/dl.
Arterial blood gases on room air revealed: pH 7.42, pO 2 100 mmHg, pCO 2 37.5 mmHg, HCO 3 22.9, SBE −1.2, and Sat 97.4%. Patient was transferred to the ward on day 7 for follow-up by endocrinologist.
| Discussion|| |
Sheehan syndrome, or necrosis of the pituitary gland, is a rare complication of postpartum hemorrhage initially described in 1937 by Sheehan . This case illustrates an example of early Sheehan syndrome during postpartum period in a patient presented with acute renal failure with pre-existing antepartum preeclampsia and accidental hemorrhage. The pituitary gland is physiologically enlarged in pregnancy, and therefore is very sensitive to the decreased blood flow caused by massive hemorrhage and hypovolemic shock. Women with Sheehan syndrome have varying degrees of hypopituitarism, ranging from panhypopituitarism to only selective pituitary deficiencies. The anterior pituitary is more susceptible to damage than the posterior pituitary.
Failure to lactate or difficulties with lactation are common initial symptoms of Sheehan syndrome. Many women also report amenorrhea or oligomenorrhea after delivery. In some cases, the diagnosis is not made until years later, when features of hypopituitarism, such as secondary hypothyroidism or secondary adrenal insufficiency, become evident in a woman who had a postpartum hemorrhage. A woman with undiagnosed hypopituitarism from Sheehan syndrome might be relatively asymptomatic until her body is stressed by a severe infection or surgery years after her delivery, and she goes into an adrenal crisis.
Diagnosis of Sheehan syndrome can be difficult. The diagnosis is based on clinical evidence of hypopituitarism in a woman with a history of a postpartum hemorrhage. Deficiencies of specific anterior pituitary hormones will cause varied symptoms. Corticotropin deficiency can cause weakness, fatigue, hypoglycemia, or dizziness. Gonadotropin deficiency will often cause amenorrhea, oligomenorrhea, hot flushes, or decreased libido. Growth hormone deficiency causes many vague symptoms including fatigue, decreased quality of life, and decreased muscle mass. Secondary hypothyroidism is clinically indistinguishable from primary hypothyroidism, but patients with hypothyroidism caused by hypopituitarism have low T 3 and T 4 levels with normal or even inappropriately low TSH levels. A woman with panhypopituitarism will have low levels of pituitary hormones (LH, corticotropin, and thyrotropin) as well as the target hormones (cortisol and thyroxine). In this case, the diagnosis of Sheehan syndrome was suspected because of her history, hyponatremia, and low baseline cortisol, ACTH, and thyroid hormones, TSH levels, as well as decreased prolactin level in comparison with reference range during the postpartum period. Diagnosis was confirmed with CT brain in this case, although radiologic imaging with either CT or MRI is usually not helpful in the acute phase and has not been used frequently in acute diagnosis. However, several studies have shown an empty sella. Treatment of young women with hypopituitarism usually includes replacement of hydrocortisone and replacement of thyroid hormone and estrogen with or without progesterone.
| Conclusion|| |
Although Sheehan syndrome is uncommon as a result of improved obstetric care, it should be a consideration in any woman who has a history of postpartum hemorrhage and who reports signs or symptoms of pituitary deficiency. Clinical suspicion is important especially in cases complicated with multiple comorbidities, to be confirmed by hormonal profile as well as radiological imaging.
| Acknowledgements|| |
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