P
Pallor of the Face, Nails, or Conjunctiva
Pallor is almost invariably caused by anemia and is best analyzed with the application of pathophysiology. Anemia may be caused by decreased production of blood, increased destruction of blood, or loss of blood. Decreased production results from poor nutrition particularly, poor absorption or intake of B12 (pernicious anemia), iron (iron deficiency anemia), and folic acid (malabsorption syndrome). It may also result from suppressed bone marrow (aplastic anemia) or infiltrated bone marrow (leukemia or metastatic carcinoma). Increased destruction is caused by hemolysis from intrinsic defects in the red cells (e.g., sickle cell anemia and thalassemia) or extrinsic defects in the circulation (autoimmune hemolytic anemia of many disorders). Blood loss may result from peptic ulcers and carcinomas of the gastrointestinal (GI) tract, excessive menstruation or metrorrhagia from tumors of the uterus, or dysfunctional uterine bleeding. These are the principal causes of anemia, but the reader will be able to think of several more. What is important here is to have a systematic method to recall them.
If anemia is ruled out, the less frequent causes of pallor should be considered. Shock, congestive heart failure (CHF), and arteriosclerosis cause pallor by poor circulation of blood to the skin. Patients who have hypertension may be pale from reflex vasomotor spasms of the arterioles supplying the skin. Aortic regurgitation and stenosis, as well as mitral stenosis, cause pallor for the same reasons, but the malar flush of mitral stenosis may negate this. The reason that tuberculosis, rheumatoid arthritis, carcinomatosis, and glomerulonephritis cause pallor even when their victims are not anemic or hypertensive is not known.
Approach to the Diagnosis
The approach to the diagnosis of pallor is obviously to check for anemia first and then to examine for the other chronic disorders. Chest x-ray, electrocardiogram (ECG), sedimentation rate, and a check for rheumatoid factor are all appropriate in specific cases.
Other Useful Tests
Complete blood count (CBC) (anemia)
Sedimentation rate (chronic infection)
Chemistry panel (anemia of liver and kidney disease)
Serum B12 level (pernicious anemia)
Serum folic acid level (folic acid deficiency)
Serum iron and ferritin levels (iron deficiency anemia)
Stool for occult blood (GI bleeding)
Stool for ova and parasites (anemia due to parasite infestation)
Serum haptoglobins (hemolytic anemia)
Antinuclear antibody (ANA) analysis (collagen disease)
Bone marrow examination (aplastic anemia)
Palpitations
Because anxiety is the common cause of palpitations, there is a tremendous temptation to jump to this conclusion as the cause in an otherwise healthy looking individual. If we use the mnemonic VINDICATE, we may avoid a misdiagnosis in many cases.
V—Vascular causes help to recall aortic aneurysms, arteriovenous fistulas, anemia, postural hypotension, migraine, and cardiac disorders such as aortic regurgitation, aortic stenosis, tricuspid insufficiency, mitral valve prolapse, CHF, and various arrhythmias (see page 85).
I—Inflammation reminds us of fever, pericarditis, subacute bacterial endocarditis, and rheumatic fever.
N—Neoplasms are not usually associated with palpitations.
D—Deficiency of thiamine can lead to beriberi heart disease resulting in palpitations.
I—Intoxication prompts us to recall that alcohol, tobacco, coffee, soft drinks, and tea can cause palpitations. It should also remind us that palpitations are common side effects of many drugs, including digitalis, aminophylline, sympathomimetics, ganglionic blocking agents, nitrates, and other drugs.
C—Congenital disorders that may cause palpitations include patent ductus, ventricular septal defect, and hiatal hernia. Disorders of the conduction system such as Wolff–Parkinson–White syndrome should be considered here.
A—Anxiety is a common cause of palpitations.
T—Trauma causes palpitations by inducing the release of epinephrine, but there is no diagnostic dilemma in these cases.
E—Endocrine disorders that cause palpitations include thyrotoxicosis, pheochromocytoma, menopausal syndrome, and hypoglycemia.
Approach to the Diagnosis
Valvular heart disease, anemia, and febrile disorders will usually be revealed on physical examination. It is important to inquire about drug, alcohol, and tobacco use. Caffeine is a frequent offender. It is helpful to eliminate any suspicious medications if possible. A drug screen may be useful in many cases. The initial diagnostic workup should include a CBC, chemistry profile, thyroid profile, sedimentation rate, antistreptolysin O (ASO) titer, ECG, and chest x-ray. If these have normal findings, 24- to 48-hour Holter monitoring or continuous loop event recording of the ECG should be undertaken. Be aware of the long QT syndrome when evaluating the ECG.
Other Useful Tests
24-hour urine catecholamine or vanillylmandelic acid (pheochromocytoma)
Arm-to-tongue circulation time (CHF)
Echocardiography (CHF, valvular heart disease)
Exercise tolerance test (coronary insufficiency)
Upper GI series and esophagram (hiatal hernia)
24-hour blood pressure monitoring (pheochromocytoma)
Psychometric testing (hysteria)
Serum estradiol, follicle-stimulating hormone (FSH), and luteinizing hormone (LH) (menopause)
Case Presentation #71
A 62-year-old physician complained of frequently awakening at night with palpitations. It would take him at least an hour to go back to sleep. He also had to urinate at least twice at night but denied daytime frequency of urination. He denied the use of alcohol, tobacco, or drugs but usually has a cup of coffee in the morning and a coke at lunch.
Question #1. Utilizing your knowledge of physiology and the mnemonic VINDICATE, what is your differential diagnosis?
Physical examination was unremarkable. His blood pressure was 110/70 mm Hg, and his pulse was 66 bpm. Results of laboratory studies and an exercise tolerance test were normal.
Question #2. What is your diagnosis now?
(See Appendix B for the answers.)
Papilledema
No anatomic analysis of this condition is necessary because most cases of papilledema are caused by intracranial pathology. Three notable extracranial conditions are optic neuritis, hypertension, and pseudotumor cerebri. The polycythemia and right heart failure of chronic pulmonary emphysema may combine to produce papilledema, but this is uncommon. Analysis of the intracranial causes of papilledema is performed using the mnemonic VINDICATE.
V—Vascular lesions are aneurysms and arteriovenous malformations that cause subarachnoid hemorrhages. Severe hypertension may lead to an intracerebral hemorrhage or hypertensive encephalopathy, thus causing papilledema. Cerebral thrombosis and emboli rarely lead to papilledema.
I—Infection is not a common cause of papilledema unless a space-occupying lesion is produced or the condition persists. Thus, a brain abscess is often associated with papilledema, whereas acute bacterial meningitis is not. Chronic cryptococcal meningitis, syphilitic meningitis, and tuberculous meningitis, in contrast, are often associated with some degree of papilledema. Viral encephalitis may occasionally be associated with papilledema. Cavernous sinus thrombosis and septic thrombosis of the other venous sinuses may produce papilledema.
N—Neoplasms, primary and metastatic, are the most common cause of papilledema.
D—Degenerative diseases are rarely the cause.
I—Intoxication brings to mind lead encephalopathy, but other toxins and drugs rarely cause papilledema.
C—Congenital malformations that cause papilledema include the aneurysms and arteriovenous malformations already mentioned plus the various types of hydrocephalus, skull deformities (oxycephaly), hemophilia (because of intracranial hemorrhages), and, occasionally, Schilder disease and other congenital encephalopathies.
A—Autoimmune disorders recall lupus cerebritis and periarteritis nodosa (when associated with severe hypertension).
T—Trauma does not usually produce papilledema in the early stages of concussions or epidural or subdural hematomas, but in chronic subdural hematomas, it is the rule.
E—Endocrine disorders bring to mind the papilledema of malignant pheochromocytomas (with hypertension) and the fact that pseudotumor cerebri occurs in obese, amenorrheic, and emotionally disturbed women.
Approach to the Diagnosis
The approach to the diagnosis of papilledema in someone without hypertension or hypertensive retinopathy must include a thorough neurologic examination and a computed tomography (CT) scan. If focal signs are present or the CT scan shows positive findings, referral to a neurosurgeon is indicated. He or she can decide if a magnetic resonance imaging (MRI) is indicated. A spinal tap is contraindicated. If there are no focal signs, it may be worthwhile to differentiate papilledema from optic neuritis by having an ophthalmologist perform a visual field examination. This may also be helpful in differentiating pseudotumor cerebri because there may be bilateral visual defects in the inferior nasal quadrants. Papilledema from increased intracranial pressure will show only an enlarged blind spot (unless there is a tumor of the optic tracts, radiations, or occipital cortex), whereas optic neuritis will show scotomata peripheral to the blind spot (disk). Appendix A will be useful for confirming the diagnosis of a specific disease.
Other Useful Tests
CBC (polycythemia)
Sedimentation rate (cerebral abscess, infection)
Urinalysis (renal disease associated with hypertension)
ANA analysis (collagen disease)
Blood lead level
Visual evoked potentials (optic neuritis)
Pulmonary function tests (emphysema)
Blood volume (polycythemia vera)
24-hour blood pressure monitoring (hypertension)
Spinal tap when imaging study is negative (pseudotumor cerebri)
Paresthesias, Dysesthesias, and Numbness
Anatomically, tingling and numbness or other abnormal sensations in the extremities result from involvement of the peripheral nerve, the nerve plexus (brachial or sciatic), the nerve root, the spinal cord, or the brain. When each of these is cross-indexed with the etiologies suggested by the mnemonic VINDICATE, most of the causes can be developed (Table 49). Only the most important conditions are mentioned in this discussion.
Table 49 Paresthesias, Dysesthesias, and Numbness | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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Peripheral nerve: Peripheral neuropathies from alcohol, diabetes, and other causes are important in this category, but one should not forget vascular diseases that may cause paresthesias, such as peripheral arteriosclerosis, Raynaud syndrome, and Buerger disease. In addition, metabolic disorders such as tetany and uremia should be considered. Chronic acute inflammatory demyelinating polyneuropathy (Guillain–Barré syndrome) is brought to mind here. Excessive intake of vitamin B6 (Pyridoxine) may cause a peripheral neuropathy. Finally, nerve entrapments such as carpal tunnel syndrome need to be checked. Tingling of the third and fourth toes would suggest Morton neuroma.
Nerve plexus: The brachial plexus may be involved by the scalenus anticus syndrome, a cervical rib, or Pancoast tumor. The sciatic plexus may be compressed by pelvic tumors.
Nerve root: Herniated disks, spondylosis, tabes dorsalis, and infiltration of the spine by tuberculosis, metastatic tumor, and multiple myeloma need to be remembered here. Do not forget Polio and postpolio syndrome.
Spinal cord: Spinal cord tumors, pernicious anemia, and tabes dorsalis are the most important conditions to recall here. Be alert to a myelopathy associated with acute onset of numbness around the waist and lower extremities that may occur in scuba divers.
Brain: Transient ischemic attacks, emboli, and migraines are vascular diseases to remember in addition to the diseases that affect the spinal cord. The aura of epilepsy is also important. One would not want to miss brain tumors, abscesses, and toxic encephalopathy because these are potentially treatable.
Approach to the Diagnosis
This would be the same as the workup of weakness in one or more extremities. If the condition is in the hand, one would check for Tinel and Adson signs and x-ray the cervical spine for a cervical rib or disk degeneration. The next steps are nerve conduction studies and electromyogram (EMG). Objective signs of radiculopathy are a clear indication for an MRI or cervical myelography, preferably combined with a CT scan. MRI may reveal tiny disk herniations. With associated pain in certain roots, diagnostic nerve blocks may be indicated. If there is coldness in the hand, a stellate ganglion block may be helpful.
If the condition is in the lower extremity, a careful examination of the arterial pulses, particularly the femoral, is performed. If these are abnormal, ultrasonography, a flow study, or femoral angiography may be indicated. X-rays of the spine to rule out a herniated disk or tumor of the spine are done routinely. A positive Tinel’s at the ankle or inflated blood pressure cuff test may confirm a tarsal tunnel syndrome. One must not forget a pelvic examination in a female. If other neurologic signs are present, an MRI or CT scan may be necessary. When a disk herniation is still likely, myelography should be ordered. EMG has the same usefulness here as in the upper extremity. When a cerebral lesion is suspected, a CT scan, MRI, and four-vessel angiography should be considered.
Other Useful Tests
CBC (anemia)
Chemistry panel (hypoparathyroidism, electrolyte disturbance, uremia)
Fluorescent treponemal antibody absorption (FTA-ABS) test (neurosyphilis)
Serum B12, thiamine, B2, B3, B6, and folic acid levels (pernicious anemia, nutritional neuropathy)
Schilling test (pernicious anemia)
Blood lead level (lead neuropathy)
ANA analysis (collagen disease)
Glucose tolerance test or HbA1C (diabetic neuropathy)
Urine porphobilinogen (porphyria)
Hair analysis for arsenic
Somatosensory evoked potentials (multiple sclerosis)
Spinal tap (neurosyphilis, multiple sclerosis, demyelinating neuropathy, hypothyroidism, diabetic neuropathy)
Anticentromere antibody (scleroderma)
Muscle biopsy (periarteritis nodosa)
Case Presentation #72
A 25-year-old white male intern complained of intermittent numbness and tingling for several months of the lower extremities and, to a lesser extent, the upper extremities. He had occasional weakness in his left arm and hand but was told on an insurance examination that that was due to a scalenus anticus syndrome. He denies alcohol or substance abuse.
Question #1. Utilizing your knowledge of neuroanatomy, what is your differential diagnosis?
Further history reveals that he had an episode of optic neuritis at age 17. His neurologic examination reveals hyperactive reflexes of the left upper and lower extremities but is otherwise unremarkable.
Question #2. What is your diagnosis now?
(See Appendix B for the answers.)
Pelvic Mass
A mass in the pelvis is usually (but not always) a neoplasm. Is there a quick way to recall all the various causes while examining the pelvis? Anatomy is the key. Apply the mnemonic MINT to develop a list of the many possibilities (Table 50).
Anatomically, there are three major groups of structures: the urinary tract, the female genital tract, and the lower intestinal tract. Breaking these down into their components, there are the bladder and ureters; the vagina, cervix, uterus, fallopian tubes, and ovaries; and the rectum and sigmoid colon. In addition to these structures, the diseases of the aorta and iliac vessels, spine, and surrounding muscles and fascia must be considered. Other structures fill the pelvis from above. The small intestines, the omentum, and the appendix may be felt; even the kidney may drop into the pelvis.
Bladder: Prominent conditions that must be considered here are stones, diverticula, Hunner ulcer, and carcinomas. A distended bladder is deceptive.
Urethra: A cystocele and urethrocele are felt easily during a pelvic examination, but if they are not, have the patient strain or stand up.
Ureters: A ureteral calculus or ureterocele may be felt.
Vagina: Vaginal carcinomas, prolapsed cervix or procidentia, rectocele, and Bartholin cysts may be felt. A foreign body (e.g., a pessary) should be considered.
Cervix: Carcinoma or polyps are the main considerations here, because an inflamed cervix does not usually cause a mass.
Uterus: Fibroids are the most likely tumor to be felt, but pregnancy, chronic endometritis, choriocarcinoma, and endometrial carcinomas all present as a mass. A retroverted uterus may masquerade as a mass in the cul-de-sac.
Fallopian tubes: Tubo-ovarian abscesses and endometriosis of these structures account for most cases. Ectopic pregnancy is always possible.
Ovary: Ovarian cysts and carcinomas must be considered as well as endometriosis.
Rectum: Carcinoma, abscesses, diverticula, and prolapse are good possibilities here. Feces may masquerade as a mass.
Sigmoid colon: Again, the disorders mentioned in the section on the rectum (see page 372) must be considered. Granulomatous or ulcerative colitis may present as a mass.
Arteries: It is unusual for an aortic or iliac aneurysm to be felt here, but they should be kept in mind.
Spine: Deformities of the spine (e.g., lordosis), tuberculosis (Pott disease), and metastatic or primary malignancies of the spine (e.g., myeloma) may present as a pelvic mass.
Miscellaneous: A pelvic kidney may be felt. An inflamed segment of ileum (regional ileitis) or the appendix should be considered, as should omental cysts and adhesions.
Approach to the Diagnosis
The association with other symptoms is the key to the clinical diagnosis. A painless mass is likely to be a neoplasm, whereas a tender mass with fever suggests pelvic inflammatory disease (PID) or a diverticular abscess. Obviously, an ectopic pregnancy should be associated with tender breasts, frequency of urination, and morning sickness. The next logical step is ultrasonography and a gynecologic consult. Ultrasonography should be performed on obese patients or whenever the clinician is unable to do a thorough pelvic examination for whatever reason.
Laboratory tests include urinalysis and culture, pregnancy test, stool for blood and parasites, and vaginal cultures. A proctoscopy and barium enema may be useful. Colonoscopy, culdoscopy, peritoneoscopy, and cystoscopy may all need to be done before an exploratory laparotomy is performed.
Other Useful Tests
Sedimentation rate (PID)
Tuberculin test (tuberculosis of the fallopian tubes)
Catheterization for residual urine
Culdocentesis (ruptured ectopic pregnancy)
Laparoscopy (ectopic pregnancy, neoplasm)
CT scan of the pelvis (neoplasm, stone, diverticulum, abscess)
Aortogram (aortic aneurysm)
Exploratory laparotomy
Urology consult
Gynecology consult
CA 125 (ovarian carcinoma)
Therapeutic trial of oral contraceptives (ovarian cysts)
Urinary hCG (embryonal cell carcinoma)
Pelvic Pain
Visualizing the anatomy of the pelvic area is the key to forming a list of the causes of pelvic pain. Starting at the skin and working inward, we have the muscles and fascia, bladder, peritoneum, uterus, ovaries, fallopian tubes, intestines, rectum, and spine. The skin helps to recall herpes zoster, the muscle and fascia suggest contusion and hernia, and the peritoneum would remind one of peritonitis and endometriosis. The uterus, ovary, and tubes would prompt consideration of PID, dysmenorrhea, pelvic congestion, and ectopic pregnancy. Ovarian tumors can also cause pelvic pain by twisting on their pedicle. A pedunculated uterine fibroid can also twist on its pedicle causing severe pain. If the pelvic pain is related to the menstrual cycle, one should recall mittelschmerz. Considering the intestines, one should recall appendicitis and diverticulitis. Considering the rectum should prompt recall of hemorrhoids, fissures, and rectal abscess. Finally, thinking of the spine should suggest rheumatoid spondylitis, osteomyelitis, herniated disk, and other conditions.
Approach to the Diagnosis
A good pelvic and rectal examination is essential. These will often disclose a mass or other pathology to explain the pain. If there is a vaginal discharge, a smear and culture for gonococcus and Chlamydia need to be done. A pregnancy test will help rule out an ectopic pregnancy, but ultrasonography is most useful.
A gynecology consult should be obtained when there is any doubt. In acute cases, the gynecologist may proceed with an exploratory laparotomy immediately.