Intern Report Case Discussion 2.11

intern-report

Presented by Ayse Avcioglu, MD

CC: “I have a headache”.

HPI:  Patient is a 33 year old female who presents to the ED complaining of 8/10 bifrontal headache described as a steady dull ache.  She has had the headache for over a year on and off but for the last two days it has progressively become worse.  Tylenol  makes the pain slightly better, but the pain seems to be more severe early on in the morning on awakening.  She denies any vomiting.  She also has been having trouble driving as she sometimes has difficulty seeing out from the side mirrors.  She has been healthy otherwise except for the inability to conceive as she and her husband have been trying to get pregnant for the past year.  There has also been some white discharge from both breasts.   Otherwise, she denies any fever, neck pain, or any motor weakness.

ROS: all negative except per HPI.
PMH: none.
PSH: none.
Allergies:  NKDA
Medications:  none.
FH: denies.
SH: denies tobacco or illicit drug use.  Occasional alcohol use.
LMP:  irregular, cannot recall last date.

Physical Exam:
Vital signs:  BP:  132/78  P:  107  R:  20   T:  36.8
General:  Pleasant appearing female in no acute distress.
HEENT:  Head is NC/AT.  Pupils 3 mm bilaterally, equal and reactive to light and accommodation.  EOMI no nystagmus noted.  Fundus: clear disc margins, venous pulsations present, no hemorrhages or disc hyperemia.
Neck:  FROM/Supple
Heart:  Normal S1/S2.  No murmurs, rubs, gallops.
Lungs:  Clear bilaterally.
Breast: no discharge visualized.
Abdomen:  Soft, nontender, nondistended.  + bowel sounds.  No organomegaly.
Musculoskeletal:  Normal AROM.  Normal muscular bulk and tone.
Skin: no rashes or striae.
Neurological:  Alert, oriented x 3.  Speech clear.  Diminished peripheral visual fields on confrontation. CN III-XII intact.  + 2 biceps/triceps/knee/achilles equal and symmetrical.  No Babinski.  Sensation: light touch, pain, vibration normal and symmetrical.  Motor: proximal/distal muscle strength 5/5 equal and symmetric.  Coordination/gait:  no extended arm drift, finger to nose/heel to shin intact and symmetric.  Rapid alternating hand and finger movements intact.  Rhomberg negative.  Gait normal, able to walk on toes and heels.

Questions

1.  Based on the case scenario and the image below, the most likely diagnosis is:
a.  metastatic cancer
b.  meningioma
c.  prolactinoma
d.  abscess
e.  tuberculoma

2.  On one busy ER shift you are called to resuscitation bay for a medical code.  EMS brings a 35 year old male with complaints of sudden severe headache described as retro-orbital  with associated vomiting, generalized weakness, and diplopia .  Vital signs:  BP: 80/52, P: 122 ,  RR: 16,   T: 36.8.

The images below correspond to the next two questions.


What is the most likely cause of this patients clinical presentation?
a.  bacterial meningitis
b.  subarachnoid hemorrhage
c.  cerebral vascular accident (CVA)
d.  pituitary apoplexy
e.  CNS tumor

3.  What should be the initial step in management for this patient?
a.  call neurosurgery for STAT decompression
b.  administer 4 units FFP
c.  lumbar puncture
d.  give 100 mg hydrocortisone IV
e.  check coagulation panel

Answers

1.  C  (prolactinoma)
2.  D  (pituitary apoplexy)
3.  D  (give 100 mg hydrocortisone IV)

Discussion

Pituitary adenomas are benign neoplasms that arise from one of the anterior pituitary cell types.  They are the most common cause of sellar masses after the third decade, and account for 10% of intracranial neoplasms.  Adenomas are classified by their size and cell of origin.  Lesions < 1 cm are classified as microadenomas while those > 1 cm as macroadenomas.  Pituitary adenomas are usually discovered incidentally during imaging studies done for some other reason.  There are several types of pituitary tumors, and each secretes different hormones.
The patient in the case presentation had  a pituitary mass measuring 2 x 1.8 x 2.5 cm with involvement of the right cavernous sinus, sphenoid sinus and clivus.  Her prolactin level was 6298.0 (normal 1.2 -29.9).  Given her clinical manifestation and laboratory findings, prolactinoma was diagnosed.

Prolactinoma is the most common type of pituitary tumor.  Patients typically come to medical attention due to headache, visual impairment, or hormonal abnormalities. Typical symptoms include amenorrhea, galactorrhea, and infertility in females, with hypogonadism, decreased libido, gynecomastia, and impotence in males.  Upward extension of the tumor with associated compression of the decussating nasal fibers of the optic chiasm can lead to bitemporal hemianopsia.   If the mass invades laterally, there may be impingement on the cavernous sinus which may compress its neural contents, leading to cranial nerve III, IV, and VI palsies.  There may also be effects on the ophthalmic and maxillary branches of the fifth cranial nerve.  Patients may present with diplopia, ptosis, ophthalmoplegia, and decreased facial sensation.  Extension into the sphenoid sinus indicates that the pituitary mass has eroded through the sellar floor.

Suspicion of a pituitary adenoma is based on the presenting signs and symptoms, and in addition to a thorough history and physical, initial workup should include a prolactin level, pregnancy test, and thyroid function tests.  Other laboratory evaluation could include serum FSH, LH, GH, and insulin levels.  MRI is the imaging modality of choice.
Emergency department management for this patient included consultation with neurosurgery who subsequently recommended starting her on parlodel (bromocriptine) which is a dopamine agonist that works to shrink the tumor.  Transpenoidal surgical resection would likely be indicated given that the optic nerve was being compressed and causing peripheral visual-field loss.  Other indications include recurrent tumors, or tumors that exhibit invasive properties.

Pituitary Mass

MRI with coronal view of the pituitary gland and surrounding structures.

Pituitary apoplexy, an acute massive infarction of the pituitary gland, is a rare but life-threatening disorder that often requires prompt diagnosis and rapid medical intervention to avoid significant morbidity and mortality.  T1 images in the question demonstrate a heterogeneous mass in the sella, displacing but not effacing the chiasm, and extending into the right cavernous sinus, but not encasing the carotid.

Most commonly occurring in preexisting pituitary adenomas, infarction of a seemingly normal gland has also been reported.  Several studies have shown that hemorrhagic degeneration occurs in 9% to 15% for these tumors.  The reason for the tendency for hemorrhage is not clearly understood, but a rapidly growing adenoma may outstrip its blood supply producing ischemic necrosis followed by hemorrhage.    Clinical manifestations may be acute and dramatic with rapidly developing headache, opthalmoplegia, neurological deficits, coma, and death.  Infarction of the tumor with secondary hemorrhage and edema causes rapid expansion of the lesion, with compression of adjacent structures.  With continued swelling, other neighboring structures may be compressed, producing visual field deficits, and  hemiparesis (from entrapment of internal carotid artery).  The hemorrhagic expansion also may disrupt the dural covering of the pituitary, thereby allowing blood to enter into the subarachnoid space and produce meningeal irritation sometimes mimicking a subarachnoid hemorrhage.

Acute adrenal crisis caused by loss of adrenocorticotropic hormone is the most life-threatening endocrine disturbance. Patients may present in profound shock.  Immediate administration of intravenous hydrocortisone and then every 6 to 8 hours is mandatory until surgery.  Electrolytes and hydration status should be monitored closely for evidence of diabetes insipidus.  Neurosurgical decompression is the definitive therapy.  Obtundation or rapidly deteriorating course are absolute indications for surgery.  Severe visual loss is a relative indication for surgery and depending on the duration of symptoms, complete restoration of sight may be possible with decompression.

Clinical Pearls
1.  Pituitary apoplexy is a potentially life-threatening condition,  and should be considered in the differential diagnosis of any patient with abrupt neurological and ophthalmologic deterioration.
2.  Response to analgesics does not rule out life threatening causes of secondary headache.
3.  Many pituitary infarctions and hemorrhages will not be easily visualized on CT.  MRI is the diagnostic modality of choice.

References

Emergency Department Presentation of Pituitary Apoplexy. American Journal of Emergency Medicine.
Volume 18, Issue 3 (May 2000).

Kahn, Ali Nawaz. (2009, March 12). Pituitary Adenoma. http://emedicine.medscape.com.

Melmed Shlomo, Jameson J. L, . Chapter 333. “Disorders of the Anterior Pituitary and Hypothalamus.” Harrison’s Principles of Internal Medicine, 17e.

Peter J. Snyder. ( 2009, January 27).  Causes of Hypopituitarism.  http://www.utdol.com/online.

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