Anaesth Crit Care Pain Med 34 (2015) 61–63
Letter to the editor Early diagnosis of Sheehan’s syndrome
A R T I C L E I N F O
Keywords: Sheehan’s syndrome Pituitary MRI Hyponatremia
Sheehan’s syndrome, originally described by Sheehan in 1937, occurs as a result of acute ischemic pituitary necrosis due to severe postpartum haemorrhage and shock. The incidence of Sheehan’s syndrome has been gradually declining through improved management of haemodynamic complications. The diagnosis of Sheehan’s syndrome is often delayed after the resolution of the acute process of pituitary necrosis (from 6 to 30 years with a mean of 14.0 6 years) [1], resulting in a lack of data regarding immediate endocrine and imaging abnormalities. We report the case of a 28-year-old Asiatic pregnant woman with no significant medical history who was admitted to the Intensive Care Unit in haemorrhagic shock after a caesarean delivery complicated by disseminated intravascular coagulopathy. Packed red blood cells and fresh frozen plasma were transfused in addition to crystalloid and colloid fluids. Due to continued haemodynamic instability, an abdominal tomodensitometry was performed showing intra-abdominal extravasation of blood. The patient underwent an exploratory laparotomy. An extensive 3-litre haemoperitoneum was observed, but no active bleeding was still present. Two days after treatment of the hypovolemic shock, moderate hyponatremia (133 mEq/L) was observed. The patient began complaining of asthenia associated with obnubilation and confusion seven days after delivery. She also complained of not being able to breastfeed. On examination, she was normovolemic and had normal findings on neurologic examination. Her blood pressure, pulse, respiratory rate, and temperature were normal. There were no signs of diabetes insipidus. At readmission in the intensive care unit, investigations revealed a very low sodium level of 110 mEq/L. The serum osmolality was low at 218 mOsm/kg and her urine osmolality was 509 mOsm/kg with a high urine sodium of 174 mmol/L. Hormonal tests showed anterior pituitary failure (Table 1). The dynamic test (Synachten 250 mg, i.v.) showed a normal stimulation of the corticotrope axis. Based on these results, the diagnosis of acute pituitary failure (severe deficiency of the somatotrope, thyreotrope, lactotrope and gonadotrope axes and partial deficiency of the corticotrope axis) caused by ischemia of the pituitary gland (Sheehan’s syndrome) was made. The MRI performed at day 10 after delivery revealed
an enlarged hypophysis (15 mm in diameter), presenting an homogenous T1-weighted hypo-signal and T2-weighted highsignal. No contrast enhancement of the enlarged pituitary gland was noted, except for a distinctive peripheral rim (Fig. 1a and b). The left side extended in the suprasellar cistern close to the optic chiasm with a normal Goldman perimetry. The neurohypophysis was normal. The young woman was discharged from the hospital on hormone replacement therapy with hydrocortisone (20 mg/dl) combined with levothyroxine sodium (50 mg/dl). The results of the patient’s clinical examination and laboratory tests at 3 months of follow-up showed a normal corticotrope axis (a cortisol level of 8.1 mg/dl, hydrocortisone being stopped 1 week before evaluation) and the persistence of the other hormonal deficiencies. At three months, only hydrocortisone was stopped and estrogenprogestative replacement was started. The MRI scan showed at that time atrophy of the adenohypophysis with a partially empty sella. The neurohypophysis was normal and there were no modifications of the pituitary stalk (Fig. 2). Diagnosis of Sheehan’s syndrome can be difficult, especially in the acute phase. In this case, the diagnosis was suspected because of the recent history of haemorrhagic shock after a caesarean delivery and lack of milk production after childbirth. Uncommonly, Sheehan’s syndrome can present acutely with severe hyponatremia, circulatory collapse, diabetes insipidus, hypoglycemia, normochromic anaemia, pancytopenia, congestive cardiac failure or psychosis [2]. Our patient had a severe symptomatic hyponatremia (110 mEq/L) 10 days after delivery. Hyponatremia was rapidly corrected by restricting fluids. This hyponatremia was attributed to combined inappropriate secretion of antidiuretic hormone (SIADH) and a dilution effect of the large amounts of crystalloid and colloid fluids given in the intensive care unit. There are several possible mechanisms by which hypopituitarism can cause SIADH-related hyponatremia [3]. Acute hypothyroidism decreases free-water clearance in response to vasopressin. Table 1 Endocrine parameters 10 days after the traumatic delivery. Hormones
Basal
Stimulation
Normal range
Cortisol ACTH Prolactin Free T4 Free T3 TSH LH FSH Progesterone Estradiol GH IGF 1
9.9 mg/dl 16 pg/ml 1.6 mg/l 5.5 pmol/l 2.6 pmol/l 1.04 mU/l < 0.2 U/l < 0.2 U/l 0.1 mg/l 68 ng/l < 0.07 ng/ml No data
30.4 mg/dl – – – – – – – – – –
5.0–25.0 5.0–49 2.0–23.0 7.8–18 3.4–6.2 0.2–3.5 4–12 1–24 0.2–25 20–500 –
http://dx.doi.org/10.1016/j.accpm.2014.07.001 2352-5568/ß 2015 Socie´te´ franc¸aise d’anesthe´sie et de re´animation (Sfar). Published by Elsevier Masson SAS. All rights reserved.
62
Letter to the editor / Anaesth Crit Care Pain Med 34 (2015) 61–63
Fig. 1. a: sagittal cerebral MRI T1, 10 days after traumatic delivery; b: coronal cerebral MRI T1 gadolinium, 10 days after traumatic delivery.
compromised by haemorrhage or shock. More than 75% of the gland must be destroyed before clinical manifestations are evident. The adenohypophysis is more vulnerable because of its relatively smaller blood supply, with diabetes insipidus occurring in only 5% of the cases [4]. In our patient, the MRI showed a normal neurohypophysis and there was no sign of diabetes insipidus. In agreement with a few reported cases in the literature, a typical MRI description of the acute-onset of Sheehan’s syndrome may be given as an enlarged non haemorrhagic hypophysis with central hypointensity on T1 and hyperintensity on T2 weighted imaging along with peripheral enhancement. The presumed infarcted areas are interspersed with perfused tissue, explaining the irregular enhancement. Within several weeks, the gland becomes confined to the pituitary fossa, followed by further pituitary gland atrophy for several months with a final image of an empty sella (75% of patients) or partially empty sella. There is no correlation between the severity of hypopituitarism and the degree of empty sella, nor between the degree of postpartum pituitary necrosis and the severity of clinical expression [5]. Author contribution
Fig. 2. Sagittal cerebral MRI T1 gadolinium performed three months after the traumatic delivery.
Glucocorticoid deficiency seems to be an important non-osmotic stimulus of vasopressin secretion, since hypersecretion of ADH relative to plasma osmolality can be corrected by steroid treatment. In addition, hypocortisolism has been shown to exert direct, vasopressin-independent renal tubular effects by decreasing free water clearance. The potassium level in these situations is normal, because adrenal production of aldosterone is chronically not dependent on the pituitary. However, our patient presented normal basal and ACTH-stimulated cortisol levels, suggesting the absence of severe corticotroph deficiency. Nonetheless, in the acute setting, ACTH stimulation is not the optimal test to evaluate the hypothalamic-pituitary-adrenal axis and the low basal cortisol concentration in spite of the critical condition of the patient raises the possibility of initial partial ACTH deficiency, contributing to hyponatremia. During pregnancy, the pituitary gland enlarges to twice its normal size, becoming liable to ischemic injury if blood flow is
R.F, P.O wrote and edited the manuscript. P.O, D.M, R.F reviewed the manuscript. All authors contributed to the discussion. All authors contributed to the data collection. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Feinberg EC, Molitch ME, Endres LK, Peaceman AM. The incidence of Sheehan’s syndrome after obstetric hemorrhage. Fertil Steril 2005;84:975–9. [2] Shivaprasad C. Sheehan’s syndrome: new advances. Indian J Endocrinol Metab 2011;15(Suppl. 3):S203–7. [3] Putterman C, Almog Y, Caraco Y, Gross DJ, Ben-Chetrit E. Inappropriate secretion of antidiuretic hormone in Sheehan’s syndrome: a rare cause of postpartum hyponatremia. Am J Obstet Gynecol 1991;165:1330–3. [4] Wainer P, Ben Israel J, Plavnick I. Sheehen’s Syndrome with diabetes insipidus. Israel J Med Sci 1979;15:431–3. [5] Dash RJ, Gupta V, Suri S. Sheehan’s syndrome: clinical profile, pituitary hormone responses and computed sellar tomography. Aust N Z J Med 1993; 23:26–31.
Letter to the editor / Anaesth Crit Care Pain Med 34 (2015) 61–63
Raluca Maria Furnicaa,*, Philippe Gadisseuxb, Claudine Fernandezc, Ste´phane Dechambred, Dominique Maitere, Philippe Oriota,b a Department of Endocrinology, General Hospital of Mouscron, Mouscron, Belgium b Department of Intensive care unit, General Hospital of Mouscron, Mouscron, Belgium c Department of Obstetrics, General Hospital of Mouscron, Mouscron, Belgium
63
d
Department of Radiology, General Hospital of Mouscron, Mouscron, Belgium e Department of Endocrinology, Universite´ Catholique de Louvain, Cliniques Universitaires Saint-Luc, Brussels, Belgium *Corresponding
author at: Centre hospitalier de Mouscron, 49, avenue de Fe´camp, 7700 Mouscron, Belgium. Tel.: +32 56 85 89 74; fax: +32 56 85 81 44 E-mail address: [email protected] (R.M. Furnica) Available online 5 March 2015
Letter to the editor Early diagnosis of Sheehan’s syndrome
A R T I C L E I N F O
Keywords: Sheehan’s syndrome Pituitary MRI Hyponatremia
Sheehan’s syndrome, originally described by Sheehan in 1937, occurs as a result of acute ischemic pituitary necrosis due to severe postpartum haemorrhage and shock. The incidence of Sheehan’s syndrome has been gradually declining through improved management of haemodynamic complications. The diagnosis of Sheehan’s syndrome is often delayed after the resolution of the acute process of pituitary necrosis (from 6 to 30 years with a mean of 14.0 6 years) [1], resulting in a lack of data regarding immediate endocrine and imaging abnormalities. We report the case of a 28-year-old Asiatic pregnant woman with no significant medical history who was admitted to the Intensive Care Unit in haemorrhagic shock after a caesarean delivery complicated by disseminated intravascular coagulopathy. Packed red blood cells and fresh frozen plasma were transfused in addition to crystalloid and colloid fluids. Due to continued haemodynamic instability, an abdominal tomodensitometry was performed showing intra-abdominal extravasation of blood. The patient underwent an exploratory laparotomy. An extensive 3-litre haemoperitoneum was observed, but no active bleeding was still present. Two days after treatment of the hypovolemic shock, moderate hyponatremia (133 mEq/L) was observed. The patient began complaining of asthenia associated with obnubilation and confusion seven days after delivery. She also complained of not being able to breastfeed. On examination, she was normovolemic and had normal findings on neurologic examination. Her blood pressure, pulse, respiratory rate, and temperature were normal. There were no signs of diabetes insipidus. At readmission in the intensive care unit, investigations revealed a very low sodium level of 110 mEq/L. The serum osmolality was low at 218 mOsm/kg and her urine osmolality was 509 mOsm/kg with a high urine sodium of 174 mmol/L. Hormonal tests showed anterior pituitary failure (Table 1). The dynamic test (Synachten 250 mg, i.v.) showed a normal stimulation of the corticotrope axis. Based on these results, the diagnosis of acute pituitary failure (severe deficiency of the somatotrope, thyreotrope, lactotrope and gonadotrope axes and partial deficiency of the corticotrope axis) caused by ischemia of the pituitary gland (Sheehan’s syndrome) was made. The MRI performed at day 10 after delivery revealed
an enlarged hypophysis (15 mm in diameter), presenting an homogenous T1-weighted hypo-signal and T2-weighted highsignal. No contrast enhancement of the enlarged pituitary gland was noted, except for a distinctive peripheral rim (Fig. 1a and b). The left side extended in the suprasellar cistern close to the optic chiasm with a normal Goldman perimetry. The neurohypophysis was normal. The young woman was discharged from the hospital on hormone replacement therapy with hydrocortisone (20 mg/dl) combined with levothyroxine sodium (50 mg/dl). The results of the patient’s clinical examination and laboratory tests at 3 months of follow-up showed a normal corticotrope axis (a cortisol level of 8.1 mg/dl, hydrocortisone being stopped 1 week before evaluation) and the persistence of the other hormonal deficiencies. At three months, only hydrocortisone was stopped and estrogenprogestative replacement was started. The MRI scan showed at that time atrophy of the adenohypophysis with a partially empty sella. The neurohypophysis was normal and there were no modifications of the pituitary stalk (Fig. 2). Diagnosis of Sheehan’s syndrome can be difficult, especially in the acute phase. In this case, the diagnosis was suspected because of the recent history of haemorrhagic shock after a caesarean delivery and lack of milk production after childbirth. Uncommonly, Sheehan’s syndrome can present acutely with severe hyponatremia, circulatory collapse, diabetes insipidus, hypoglycemia, normochromic anaemia, pancytopenia, congestive cardiac failure or psychosis [2]. Our patient had a severe symptomatic hyponatremia (110 mEq/L) 10 days after delivery. Hyponatremia was rapidly corrected by restricting fluids. This hyponatremia was attributed to combined inappropriate secretion of antidiuretic hormone (SIADH) and a dilution effect of the large amounts of crystalloid and colloid fluids given in the intensive care unit. There are several possible mechanisms by which hypopituitarism can cause SIADH-related hyponatremia [3]. Acute hypothyroidism decreases free-water clearance in response to vasopressin. Table 1 Endocrine parameters 10 days after the traumatic delivery. Hormones
Basal
Stimulation
Normal range
Cortisol ACTH Prolactin Free T4 Free T3 TSH LH FSH Progesterone Estradiol GH IGF 1
9.9 mg/dl 16 pg/ml 1.6 mg/l 5.5 pmol/l 2.6 pmol/l 1.04 mU/l < 0.2 U/l < 0.2 U/l 0.1 mg/l 68 ng/l < 0.07 ng/ml No data
30.4 mg/dl – – – – – – – – – –
5.0–25.0 5.0–49 2.0–23.0 7.8–18 3.4–6.2 0.2–3.5 4–12 1–24 0.2–25 20–500 –
http://dx.doi.org/10.1016/j.accpm.2014.07.001 2352-5568/ß 2015 Socie´te´ franc¸aise d’anesthe´sie et de re´animation (Sfar). Published by Elsevier Masson SAS. All rights reserved.
62
Letter to the editor / Anaesth Crit Care Pain Med 34 (2015) 61–63
Fig. 1. a: sagittal cerebral MRI T1, 10 days after traumatic delivery; b: coronal cerebral MRI T1 gadolinium, 10 days after traumatic delivery.
compromised by haemorrhage or shock. More than 75% of the gland must be destroyed before clinical manifestations are evident. The adenohypophysis is more vulnerable because of its relatively smaller blood supply, with diabetes insipidus occurring in only 5% of the cases [4]. In our patient, the MRI showed a normal neurohypophysis and there was no sign of diabetes insipidus. In agreement with a few reported cases in the literature, a typical MRI description of the acute-onset of Sheehan’s syndrome may be given as an enlarged non haemorrhagic hypophysis with central hypointensity on T1 and hyperintensity on T2 weighted imaging along with peripheral enhancement. The presumed infarcted areas are interspersed with perfused tissue, explaining the irregular enhancement. Within several weeks, the gland becomes confined to the pituitary fossa, followed by further pituitary gland atrophy for several months with a final image of an empty sella (75% of patients) or partially empty sella. There is no correlation between the severity of hypopituitarism and the degree of empty sella, nor between the degree of postpartum pituitary necrosis and the severity of clinical expression [5]. Author contribution
Fig. 2. Sagittal cerebral MRI T1 gadolinium performed three months after the traumatic delivery.
Glucocorticoid deficiency seems to be an important non-osmotic stimulus of vasopressin secretion, since hypersecretion of ADH relative to plasma osmolality can be corrected by steroid treatment. In addition, hypocortisolism has been shown to exert direct, vasopressin-independent renal tubular effects by decreasing free water clearance. The potassium level in these situations is normal, because adrenal production of aldosterone is chronically not dependent on the pituitary. However, our patient presented normal basal and ACTH-stimulated cortisol levels, suggesting the absence of severe corticotroph deficiency. Nonetheless, in the acute setting, ACTH stimulation is not the optimal test to evaluate the hypothalamic-pituitary-adrenal axis and the low basal cortisol concentration in spite of the critical condition of the patient raises the possibility of initial partial ACTH deficiency, contributing to hyponatremia. During pregnancy, the pituitary gland enlarges to twice its normal size, becoming liable to ischemic injury if blood flow is
R.F, P.O wrote and edited the manuscript. P.O, D.M, R.F reviewed the manuscript. All authors contributed to the discussion. All authors contributed to the data collection. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Feinberg EC, Molitch ME, Endres LK, Peaceman AM. The incidence of Sheehan’s syndrome after obstetric hemorrhage. Fertil Steril 2005;84:975–9. [2] Shivaprasad C. Sheehan’s syndrome: new advances. Indian J Endocrinol Metab 2011;15(Suppl. 3):S203–7. [3] Putterman C, Almog Y, Caraco Y, Gross DJ, Ben-Chetrit E. Inappropriate secretion of antidiuretic hormone in Sheehan’s syndrome: a rare cause of postpartum hyponatremia. Am J Obstet Gynecol 1991;165:1330–3. [4] Wainer P, Ben Israel J, Plavnick I. Sheehen’s Syndrome with diabetes insipidus. Israel J Med Sci 1979;15:431–3. [5] Dash RJ, Gupta V, Suri S. Sheehan’s syndrome: clinical profile, pituitary hormone responses and computed sellar tomography. Aust N Z J Med 1993; 23:26–31.
Letter to the editor / Anaesth Crit Care Pain Med 34 (2015) 61–63
Raluca Maria Furnicaa,*, Philippe Gadisseuxb, Claudine Fernandezc, Ste´phane Dechambred, Dominique Maitere, Philippe Oriota,b a Department of Endocrinology, General Hospital of Mouscron, Mouscron, Belgium b Department of Intensive care unit, General Hospital of Mouscron, Mouscron, Belgium c Department of Obstetrics, General Hospital of Mouscron, Mouscron, Belgium
63
d
Department of Radiology, General Hospital of Mouscron, Mouscron, Belgium e Department of Endocrinology, Universite´ Catholique de Louvain, Cliniques Universitaires Saint-Luc, Brussels, Belgium *Corresponding
author at: Centre hospitalier de Mouscron, 49, avenue de Fe´camp, 7700 Mouscron, Belgium. Tel.: +32 56 85 89 74; fax: +32 56 85 81 44 E-mail address: [email protected] (R.M. Furnica) Available online 5 March 2015
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