Vol.2, No.8, 490-493 (2013) Case Reports in Clinical Medicine
http://dx.doi.org/10.4236/crcm.2013.28128
A decade without diagnosis: Sheehan’s syndrome
Fábio A. Nascimento1*, Juliane Nery1, Gustavo Lenci Marques2, Felipe Dunin dos Santos2,
Mauricio de Carvalho2
1Faculty of Medicine, Federal University of Paraná, Curitiba, Brazil; *Corresponding Author: nascimento.fabio.a@gmail.com
2Department of Internal Medicine, Federal University of Paraná, Curitiba, Brazil
Received 26 September 2013; revised 25 October 2013; accepted 5 November 2013
Copyright © 2013 Fábio A. Nascimento et al. This is an open access article distributed under the Creative Commons Attribution Li-
cense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Sheehan’s Syndrome (SS) occurs as a result of
ischemic pituitary necrosis due to severe post-
partum hemorrhage. We report a 41-year-old
woman with a history of severe postpartum
hemorrhage 18 years prior to presentation.
During this period of time, she experienced se-
vere fatigue. Three months prior to being ad-
mitted to our hospital, the patient started to no-
tice alopecia, generalized edema and cognitive
impairment. After proper investigation, labora-
tory tests and clinical findings indicated pan-
hypopituitarism. In addition to neuroimaging
and past medical history, SS was diagnosed and
treatment started. We emphasize the importance
of thorough investigation, further diagnosis and
management of this condition (especially in
third world countries), since SS is a neurological
and endocrinological emergency and potentially
life threatening.
Keywords: Sheehan’s Syndrome; P i t u i t a r y
Insufficiency; Empty Sella
1. INTRODUCTION
Sheehan’s Syndrome (SS) is a result of ischemic pi-
tuitary necrosis due to severe postpartum hemorrhage.
SS is characterized by varying degrees of anterior pitui-
tary dysfunction. Some degrees of hypopituitarism oc-
curs in nearly one third of patients with severe obstetric
hemorrhage. Its epidemiology is discrepant depending on
the location. The main reason for the different incidence
relates to the quality of medical care a patient receives in
her local hospital.
Although this condition has been first described sev-
enty-five years ago [1], it still is not promptly diagnosed
by physicians. In fact, it is under-diagnosed in third
world countries, given that in these nations the propor-
tion of home baby deliveries is high [2]. On the other
hand, the inciden ce of Sh eeh an’s Syndrome in first wor ld
countries shows to have declined significantly.
Regarding the etiology, the most frequent causes of
postpartum hemorrhage are uterine atony, trauma, re-
tained placenta and coagulopathy [2,3].
The aim of this presented transcript is to report a case
of a patient with a history of obstetric hemorrhage until
the diagnosis of panhypopituitarism. This was due to
ischemic necrosis. We will review the main topics of
how the diagnosis was made, the main complementary
tests and reviewing the disease in general. We intend to
alert clinicians on how important it is to remember that
SS can occur in any patient with a history of postpartum
bleeding, and may be preceded with a clinical presenta-
tion of panhy p opi tuitarism.
2. CASE PRESENTATION
A 41-year-old caucasian female was admitted to our
hospital on October 1st, 2012. Her main complaint was
weakness and edema (Figure 1). She presented with hair
loss, bleeding gums and cognitive impairment. After
further inquiry we found that she had been on amenor-
rhea for 6 years and that she had been on iron supple-
mentation for anemia, which did not resolve despite the
treatment.
Regarding her past medical history, she suffered from
severe postpartum hemorrhage when she delivered her
only child, 18 years ago. At this occasion, she needed to
be admitted to the ICU and received blood transfusions.
Furthermore, she was unable to breastfeed her child.
There was no similar case in her family history. Physical
examination showed generalized non-pitting edema,
psychomotor lentification, altered level of consciousness
and myxedema facies. Her blood pressure was 100 × 75
mmHg, heart rate of 80 beats per minute, respiratory rate
of 16 respirations per minute, dry and coarse hair, alope-
cia and thinning of the outer third of her eyebrows.
Copyright © 2013 SciRes. OPEN ACCESS
F. A. Nascimento et al. / Case Rep orts in Clinical Medicine 2 (2013) 490-493 491
Figure 1. Patient at presentation. Note the facial edema and
alopecia.
Laboratory findings indicated normochromic normo-
cytic anemia, free T4 level of 0.4 ng/mL and TSH level
of 1.23 mUI/mL. We suspected central hypothyroidism.
Therefore, the patient was started on 1.5 mcg/kg/day of
levothyro xine and pre dni sone in doses of 5 mg/day.
As the clinical manifestations associated to the patient’s
past medical history suggested Sheehan’s syndrome, re-
lated endocrinological tests were ordered (Table 1) as
well as an MRI of the skull (Figure 2). After all these
tests were performed the diagnosis of SS was made.
3. DISCUSSION
In 1937 Sheehan reported 11 cases of women who
died in the puerperium, all of whom had necrosis of the
anterior pituitary gland-adenohypophysis. Nine of the 11
cases had severe hemorrhage at delivery. The other two
cases had no hemorrhage but were gravely ill prior to
delivery [4]. Postpartum hypopituitarism has been known
as Sheehan’s Syndrome ever since [5].
Regarding its pathogenesis, the basic process is in-
farctation secondary to the arrest of blood flow to the
anterior lobe of th e pituitar y gland (usu ally th ere is blood
loss of at least 1 to 2 liters and consequently hypo-
volemic shock). Whether this process results from vaso-
spasm, thrombosis, or vascular compression is unclear.
The fact that the posterior lobe of the pituitary is less
commonly involved can be explained by how the neuro-
hypophysis’ vascular supply arrangement: it contains an
anastomotic ring of blood vessels, which the adenohy-
pophysis lacks [6,7]. The patient described by us had
these symptoms, consistent to that described in literature,
as well as a history of significant blood loss after delivery,
which resulted in a blood transfusion and admission to
the intensive care unit.
Only a small number of patients with SS develop acute
postpartum hypopituitarism after postpartum hemorrhage.
The most frequent scenario is a woman with amenorrhea
occurring years later, with the diagnosis of SS being
made retrospectively. Ho wever, it is impor tant to empha-
size that SS is a neurological and endocrinological
emergency and is potentially lethal [8]. According to a
Figure 2. Sagittal gadolinium-enhanced T1-weighted MR im-
age shows an empty sella (red circle).
Table 1. Laboratory findings at presentation.
Laboratory findings a t presentation
Patient’s
measurements Reference
Growth hormone
(ng/mL) 0.1 0.06 - 5.00
Prolactin (ng/m L) 0.83 3.46 - 19.40
Follicle-stimulating
hormone (mUI/mL) 0.36 27.72 - 133.4
(menopause)
Luteinizing hormone
(mUI/mL) 0.09 10.39 - 64.57
(menopause)
Plasmatic cortisol at 8 am
(mcg/mL) 0 3.7 - 18.4
24-Hour urinary fr ee
cortisol (mcg/24 h) 0.4 4.3 - 176.0
Hematocrit (%) 28.7 36.7 - 46.3
Hemoglobin (g/dL) 9.9 12.5 - 15.7
MCV (fL) 86.7 80 - 99
MCHC (g/dL) 34.5 32.2 - 36.0
a. MCV: mean corpuscular volume; MCHC: mean corpuscular hemoglobin
concentration.
group of 60 patients with Sheehan’s Syndrome, studied
by Gei-Guardia et al. [9], the period of time between the
postpartum episode of bleeding and the diagnosis of SS
is 13 years. Focusing again on our patient, she was diag-
nosed after 18 years from the obstetric event of bleeding.
In Sheehan’s syndrome, inability to lactate after deliv-
ery due to prolactin deficiency and the development of
amenorrhea from gonadotrophin deficiency classically
occurs. In addition, these patients become infertile, there
is failure to regrow shaved pubic hair and signs of hypo-
thyroidism and hypoadrenalism occur [10,11]. Our pa-
tient, in agreement to documented literature, informed us
that she could not lactate after delivery and that she
noted failure to regrow her pubic and axillary hair.
Copyright © 2013 SciRes. OPEN ACCESS
F. A. Nascimento et al. / Case Rep orts in Clinical Medicine 2 (2013) 490-493
492
Regarding laboratory exams, in order to diagnose and
confirm SS, literature recommends dosing the following
substances: prolactin, free thyroxine (fT4), thyroid-
stimulating hormone (TSH), cortisol, luteinizing hor-
mone (LH), estradiol and insulin-like growth factor 1
(IGF-1) [1,12,13]. Our patient reports showed low levels
of all these hormones, except for adrenocorticotropic
hormone (ACTH), which was not measured (Table 1).
Similar to what occurred to our patient, there have
been reports of individuals that were submitted to long-
standing clinical treatment for chronic anemia, which
never resolved, before actually being diagnosed with
Sheehan’s syndrome [14-17]. Gokalp et al., in 2009,
showed that 80% of the studied population with this
syndrome suffered from anemia. Leucopenia, iron defi-
ciency and thrombocytopenia were also commonly found
on these patients [18]. These hematologic findings, and
their refractoriness to standard clinical therapy, showed
to be useful for ultimately diagnosing Sheehan’s syn-
drome.
The neuroimaging characteristics of SS are distinctive.
In pregnancy, there is enlargement of the pituitary from
diffuse nodular hyperplasia of prolactin secreting cells.
The MRI shows the normal pituitary gland is largest in
the immediate postpartum period, measuring up to 11.8
mm in height and convex in appearance. The anterior
pituitary is usually hyperintense on T1-weighted images
in pregnant and postpartum women when compared to
controls. After delivery, the size of the pituitary gland
rapidly returns to normal beyond the first week postpar-
tum. In a chronic phase of the disease, neuroimaging
classically shows atrophy of the pituitary and empty sella
[19,20]. Our patient’s imaging evidently showed the last
two findings in an MRI performed 18 years after the de-
livery. Unfortunately, the patient did not undergo a pre-
vious MRI. Therefore, the only MRI available is the one
showed in the photo (Figure 2).
Sheehan’s syndrome treatment aims is to replace the
missing hormones and restore endocrine homoeostasis.
The hormones ACTH and TSH may be replaced in addi-
tion to glucocorticoids and thyroxine respectively. Re-
placing mineral corticoids, on the other hand, is not nec-
essary in most cases. It is important to replace a patient's
sexual hormones as part of the treatment before meno-
pause and replacing GH is useful for lipid-lower therapy
and to enhance th e patient’s quality of life [21,22]. In our
patient, the treatment showed an excellen t result (Figure
3).
In terms of prognosis, it depends on how soon the di-
agnosis is made and the proper treatment is initiated. If
the syndrome is early diagnosed and adequate medical
therapy is provided, the prognosis of Sheehan’s syn-
drome is excellent [23,24]. On the other hand, if there is
delay in identifying and managing these patients, they
may present to medical services (at some point after the
Figure 3. Patient after treatment. Evident facial
edema regression and alopecia correction.
event of postpartum hemorrhage) with severe and multi-
ple clinical abnormalities—such as adrenal crisis, symp-
tomatic hypogly c emia, symptomatic hy ponatremia, among
many others [25-27].
4. CONCLUSION
Evidently, it is important to emphasize the clinical
importance of a careful and meticulous review of a pa-
tient’s medical history, physical exam and complemen-
tary tests, thus reminding clinicians and yielding aware-
ness that Sheehan’s Syndrome does occu r, and it is not as
rare as one may think.
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