02/03/2013  CLINICAL PRESENTATION                                                      A 41 year old man with sudden onset...
02/03/2013                                                                          Laboratory investigations reveal a hem...
02/03/2013  Coronal gadolinium-enhanced T1 images  revealed that the mass had a heterogeneous                      These f...
02/03/2013 The most common symptom of pituitary tumor                           The increase in intrasellar pressure resul...
02/03/2013                                                                          Pituitary tumor apoplexy following chi...
02/03/2013One study showed that maintenance steroid,thyroid hormone, and testosterone replacement                        A...
02/03/2013Your colleagues responded:- Coronary artery bypass graft              1%- Head trauma                           ...
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Clinical presentation feb 10, 13


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Clinical presentation feb 10, 13

  1. 1. 02/03/2013 CLINICAL PRESENTATION A 41 year old man with sudden onset headache and diplopia. Background: Dr. Juan Carlos Díaz Torre A 41-year-old man presents to the ED with an acute DR. JCDT DR. JCDT severe headache, associated diplopia, photophobia, Pediatra Neonatólogo nausea, vomiting and fever. The patient also has mild dr_diaz_torre@hotmail.com gynecomastia. (779) 100 . 40 . 26 HINT: The neurologic findings on this patients physical examination aid in establishing the etiology of his 1 headache. 2 He denies experiencing any associated seizures,A 41-year-old man presents to the emergency focal weaknesses, previous similar episodes,department (ED) complaining of a severe frontal frequent headaches, or previous visual disturbances.headache that began suddenly and awakened him fromsleep. The headache is associated with nausea, He does not have any prior significant medical DR. JCDT DR. JCDTvomiting, and subjective fevers. problems, and his only medication is occasional sildenafil.He also complains of new-onset diplopia andphotophobia, but denies any decrease in visual acuity. He drinks socially, does not smoke, and denies recreational drug use. 3 4On physical examination, the patient is ill-appearingbut alert and in no apparent distress. His vital signsreveal a temperature of 103.1°F (39.5°C), a bloodpressure of 155/95 mm Hg, and a pulse of 110 bpm.The ocular examination demonstrates ptosis of the righteye (see Figure 1), which is deviated inferolaterally and DR. JCDT DR. JCDThas a dilated and unreactive pupil (see Figure 2).The visual field examination demonstrates bitemporalhemianopsia. Funduscopic examination shows normalvenous pulsation and mild bilateral temporal disc pallor. 5 6 1
  2. 2. 02/03/2013 Laboratory investigations reveal a hemoglobinThe cranial nerves are otherwise without deficit. The concentration of 13 g/dL (130 g/L); a white blood cellneck is supple and without meningismus. Examination (WBC) count of 16.0 × 103/µL (16.0 × 109/L), with 75%of the chest reveals mild bilateral gynecomastia, without neutrophils; and a platelet count of 340 × 103/µL (340 ×nipple discharge. The lungs are clear to auscultation. 109/L). The electrolyte, blood urea nitrogen (BUN),Cardiac auscultation reveals a normal S1 and S2 and no creatinine, and glucose examinations are all withinmurmurs, rubs, or gallops. DR. JCDT DR. JCDT normal limits.The abdomen is soft and nontender, and no Cerebrospinal fluid (CSF) specimens show 420,000 redorganomegaly is detected. Bilateral upper and lower blood ceels (RBC)/μL, 20,000 WBC/μL, a normal glucoseextremity strength is 5/5, with normal deep tendon and of 85 mg/dL (4.72 mmol/L), and an elevated proteinplantar reflexes. The patients sensation is intact to light concentration of 230 mg/dL (2.3 g/L). The CSF Gramtouch and pinprick throughout, and the gait is normal. 7 stain is negative for bacteria. 8 A computed tomography (CT) scan of the What is the most likely diagnosis? brain is performed, followed immediately by magnetic resonance imaging (MRI); (see - Subarachnoid hemorrhage Figure 3). - Cerebellar infarction DR. JCDT DR. JCDT - Pituitary tumor apoplexy - Cavernous sinus thrombosis 9 10 DiscussionYour Colleagues Responded: The noncontrast CT scan of the brain showed a 2-cm- Subarachnoid hemorrhage 13% sellar mass, with suprasellar extension. There was impingement on the optic chiasm and the- Cerebellar infarction 3% hypothalamus, with upward displacement. There was increased density on the right side of the mass, which DR. JCDT DR. JCDT- Pituitary tumor apoplexy **** 61% was suggestive of hemorrhage.- Cavernous sinus thrombosis 22% The sagittal and coronal T1 and T2 MRI scans demonstrated a large soft-tissue mass in the pituitary fossa, with areas of intermediate- and high-intensity 11 signal suggestive of hemorrhage (see Figure 3). 12 2
  3. 3. 02/03/2013 Coronal gadolinium-enhanced T1 images revealed that the mass had a heterogeneous These findings are consistent with pituitary pattern of faint peripheral enhancement apoplexy as a result of hemorrhage with or (Figure 4). without infarction, likely into a pituitary adenoma. There was evidence of mass effect on the right Tests for evaluating the hormonal status of the DR. JCDT DR. JCDT cavernous sinus, which was most evident in patient revealed panhypopituitarism. Prior to the the coronal T1- and T2 images. acute apoplectic episode, the patient had findings suggestive of central hypogonadism, probably as a component of his hypopituitarism caused by pituitary macroadenoma (diminished libido and 13 bilateral gynecomastia). 14 His neurologic finding (right-sided ptosis with a fixed and dilated pupil pointing downward and outward) was consistent with a right-sided 3rd The word "apoplexy" stems from a Greek term nerve palsy caused by extension of hemorrhage meaning to "have a stroke". into the right cavernous sinus. Neurologic symptoms and signs are secondary to DR. JCDT DR. JCDT Pituitary tumor apoplexy is defined as displacement of the optic nerve and impingement hemorrhage or infarction of a pituitary gland of the 3rd, 4th, and 6th cranial nerves. associated with the presence of a preexisting pituitary adenoma. It manifests as a sudden, Hormonal dysfunction results from destruction of severe headache, and it is sometimes associated the anterior pituitary gland. with neurologic and hormonal dysfunction. 15 16Pituitary tumor apoplexy is a rare disorder with an Pituitary tumor apoplexy is only rarely associatedannual incidence of about 1.2 per million. Men are with a healthy gland; however, approximately 50%affected twice as often as women, and all age of patients who present with pituitary tumor DR. JCDT DR. JCDTgroups can be affected, with the majority of apoplexy are not diagnosed with a pituitary lesionpatients in the 5th or 6th decades of life. It is prior to their presentation. All types of pituitaryestimated to occur in 1.5-27.7% of cases of tumors carry the same risk for apoplexy.pituitary adenoma. 17 18 3
  4. 4. 02/03/2013 The most common symptom of pituitary tumor The increase in intrasellar pressure results in many apoplexy is headache. Almost all patients of the symptoms and signs of pituitary tumor describe a sudden, severe retro-orbital or apoplexy. bifrontal headache, which is associated with vomiting in two-thirds of cases. Laterally, the increased pressure causes compression of the structures in the cavernous DR. JCDT DR. JCDT The headache and vomiting result from the sinus, namely the 3rd, 4th, and 6th cranial nerves, sudden increase in intrasellar pressure either with the 3rd being most commonly affected as a caused by the hemorrhage or secondary to result of its vulnerable position (parallel to the meningeal irritation from blood or tumor lateral wall of the pituitary gland). The 6th cranial products that leak into the CSF. nerve is the least commonly involved because of its most lateral location within the sinus. 19 20 Ophthalmoplegia (caused by 3rd, 4th, and 6th nerve palsies or any combination thereof) is present in around 80% of patients presenting with Blood leaking into the subarachnoid space may pituitary tumor apoplexy. result in chemical meningitis with fever, meningismus, and photophobia. Fever in patients Also located within the cavernous sinus is the with apoplexy may also be explained by alteration in trigeminal nerve; its involvement may cause facial thermal regulation caused by hypothalamic DR. JCDT DR. JCDT pain or sensory loss. Carotid siphon compression involvement by the hemorrhage or by adrenal may present as hemiplegia. Superiorly, the insufficiency associated with hypopituitarism. increased pressure compresses the optic chiasm, optic tract, or optic nerve, leading to decreased Hemorrhage may extend into the brain parenchyma visual acuity or visual field defects (classically, causing cortical irritation and provoking seizures. bitemporal hemianopsia). 21 22The elevated intrasellar pressure also accounts forthe endocrine abnormalities found in cases of Although not common, patients with pituitary tumorpituitary tumor apoplexy. This pressure increase apoplexy may have diabetes insipidus at presentation.results in compression of the pituitary tissue, The true etiology of diabetes insipidus in this settingcompromising its vascular supply and leading to is unknown, but it may result from the increased DR. JCDT DR. JCDThypopituitarism. pressure on the pituitary infundibulum, which impedes the antidiuretic hormone from passing fromAdrenal insufficiency is the most clinically significant the hypothalamus to the posterior lobe of theresult of hypopituitarism, contributing significantly pituitary.to the mortality of patients with pituitary tumorapoplexy if not promptly recognized and treated. 23 24 4
  5. 5. 02/03/2013 Pituitary tumor apoplexy following childbirthA precipitating factor is identified in 50% of cases of associated with significant postpartum hemorrhagepituitary tumor apoplexy. Predisposing factors include in nontumorous glands is termed "Sheehandopamine agonist treatment, head trauma, pituitary syndrome".irradiation, pregnancy, coronary artery bypassgrafting, surgical operations, and anticoagulation. The hypertrophy of the pituitary gland that occurs in normal pregnancy combined with the arterial spasm DR. JCDT DR. JCDTEndocrine stimulation tests are also associated with of the pituitarys blood supply (caused by bleedingpituitary tumor apoplexy. It is postulated that and hypotension) both contribute to thehormones used in these tests may increase blood flow development of Sheehan syndrome; however thein pituitary adenomas, provoking bleeding in friable clinical presentation of pituitary apoplexy in thesevessels. cases is usually less dramatic, with a more gradual 25 development of signs and symptoms of 26 hypopituitarism. Once recognized, effective treatment of pituitary tumor apoplexy requires prompt administration ofThe diagnosis of pituitary tumor apoplexy is best high-dose corticosteroids. Steroids should beestablished by MRI; however, this is usually preceded administered in supraphysiologic doses to not onlyby a rapid diagnostic CT scan to screen for intracranial replace endogenous hormone deficiency during ahemorrhage. MRI is superior to CT scanning for stressful condition, but also to take advantage of itsevaluating the pituitary gland and possibly visualizing DR. JCDT DR. JCDT anti-inflammatory effect by decreasing swelling onhemorrhage not seen by CT. parasellar structures.In one study, the detection rate of pituitary tumor The definitive treatment for pituitary tumorapoplexy by CT scanning was 21%, whereas the apoplexy is emergent surgical decompression.detection rate was 100% with MRI. Transsphenoidal resection is the most common 27 approach in this situation. 28In cases where there is significant extension of With prompt recognition, timely surgery, and properhemorrhage into the brain parenchyma beyond the medical management, the majority of patients withdiaphragma sella, an intracranial approach may be pituitary tumor apoplexy improve. Ophthalmoplegiapreferred. In a minority of cases, conservative medical is usually the first symptom to resolve. Less readilytherapy is an acceptable alternative; examples of this restored is the optic nerve defect resulting ininclude patients who are poor surgical candidates and decreased visual acuity and restricted visual fields. DR. JCDT DR. JCDTselected patients who present with isolatedmeningismus or ophthalmoplegia and show More than half of patients, however, will havesignificant improvement with steroid administration. permanent hormone deficiencies resulting from pituitary injury and will require hormoneMedical management includes monitoring of replacement.endocrine, neurologic, and ophthalmologic function 29 30combined with hormone replacement. . 5
  6. 6. 02/03/2013One study showed that maintenance steroid,thyroid hormone, and testosterone replacement An endocrinology evaluation was completed, andwas essential postoperatively in 82%, 89%, and 64% the patient was confirmed to have hypopituitarism.of patients, respectively. Two weeks following surgery, his ophthalmoplegia and visual field deficits had completely resolved.Following immediate administration of high dose DR. JCDT DR. JCDTcorticosteroids, the patient in this case underwent Pharmacologic management of the patientsan emergent transsphenoidal resection. An infarcted hypopituitarism included replacement therapy withadenoma was identified, with extensive areas of corticosteroids, levothyroxine, and testosterone.hemorrhage and necrosis consistent with apoplexy.Resolution of the headache and improvement ofvisual and extraocular function were noted 24 hours 31 32after surgery.A patient presents to the ED with a rapid onset ofheadache accompanied by diplopia, photophobia,and decreased visual acuity, raising concern for anumber of potentially serious conditions. Which of Your colleagues responded:the following diagnostic tests is most likely to assistin confirming pituitary tumor apoplexy as the - Lumbar puncture 1%diagnosis? DR. JCDT DR. JCDT - MRI of the brain **** 89 % - CT of the brain 10 %- Lumbar puncture - Carotid ultrasonography 0%- MRI of the brain - Dilated retinal examination 0%- CT of the brain- Carotid ultrasonography- Dilated retinal examination 33 34 The patient you are examining is confirmed to have pituitary tumor apoplexy. Which of the following The diagnosis of pituitary tumor apoplexy is best choices, if they were part of this patients history, is a established by MRI. Although this may be potentially responsible precipitating factor for this preceded by a CT scan to screen for intracranial occurrence? hemorrhage. DR. JCDT DR. JCDT - Coronary artery bypass graft Lumbar puncture may reveal xanthochromia - Head trauma and/or red blood cells, but this may also be seen in - Endocrine stimulation tests subarachnoid hemorrhage resulting from a - Dopamine agonist treatment ruptured aneurysm. - All of the above 35 36 6
  7. 7. 02/03/2013Your colleagues responded:- Coronary artery bypass graft 1%- Head trauma 5% Gracias por su atención- Endocrine stimulation tests 7%- Dopamine agonist treatment 4% DR. JCDT DR. JCDT- All of the above **** 83 % Dr. Juan Carlos Díaz Torre Pediatra NeonatólogoPredisposing factors for pituitary apoplexy includebromocriptine treatment, head trauma, pituitary dr_diaz_torre@hotmail.comirradiation, pregnancy, coronary artery bypass (779) 100 . 40 . 26grafting, surgical operations, anticoagulation, and 37 38endocrine stimulation tests. 7