Misc. MSK Disorders Flashcards
(108 cards)
1
Q
Essentials of Osteomyelitis Dx:
A
- Fever associated with bone pain and tenderness.
- Microbiologic dx often made from blood cx.
- Elevated ESR and CRP common.
- Early radiographs are typically negative.
2
Q
causes of osteomyelitis
classification
A
- Hematogenous spread
- Spread from a contiguous site of infection / open wound
- Secondary infection in the setting of vascular insufficiency or concomitant neuropathy
3
Q
duration of osteomyelitis
classification
A
- acute
- chronic
4
Q
osteomyelitis: Hematogenous Spread Typically results from ?
A
bacteremia and begins in the medullary canal
5
Q
osteomyelitis hematogenous spread - MC in what demographic?
A
- MC children
-
Male
- Infection is seen in the metaphysis of long bones
- Hemoglobinopathies, such as sickle cell increase the risk
6
Q
RF for Hematogenous Spread in osteomyelitis
A
Complicated delivery, maternal infection at delivery, prematurity, indwelling catheters, urinary tract anomalies, sickle cell, immunodeficiency disorders
7
Q
MC organism in children to cause osteomyelitis
A
S aureus (MC), Salmonellae (sickle cell), groups A and B Streptococcus, Strep. pneumo, E. coli, and Kingella kingae (other countries)
8
Q
In adults, osteomyelitis often manifests in ?
risk increases with?
A
- vertebral column (LS>TS>CS)
- Risk increases with age and IVDU
9
Q
RF for hematogenous spread of osteomyelitis in adults
A
Diabetes, IVs, and indwelling urinary catheters
10
Q
MC organisms in adults for osteomyelitis
A
- S aureus (MC)
- Pseudomonas (IV drug use)
- gram - organisms (elderly)
11
Q
Infection travels from a soft tissue site
what type of spread in osteomyelitis
A
Contiguous Spread
12
Q
causes fo contiguous spread of osteomyelitis
A
- open fractures/trauma, prosthetic devices, neurosurgery, septic arthritis
- Symptoms often begin 1 month after inoculation
- Infection inoculates the bony cortex and migrates towards the medullary canal
13
Q
contiguous spread is MC in who?
osteomyelitis
A
adults
14
Q
MC organisms for contiguous spread in osteomyelitis
A
S aureus, Staph. epidermidis, Streptococcus
Polymicrobial infections MC for contiguous spread
15
Q
- Often results from a chronic, progressively soft tissue infection of the foot or ankle
- Hip and sacrum can be involved (pressure injury)
- Most often related to diabetes/diabetic ulcers and vascular insufficiency
A
Secondary Infection due to Comorbid Conditions
16
Q
MC pathogens for Secondary Infection due to Comorbid Conditions in osteomyelitis
A
- Polymicrobial infections common
- S. aureus and 𝛃-hemolytic strep MC
17
Q
-
Gradual onset of sx over several days - wks
- Dull pain at the involved site - +/- worse with movement
- Fever and rigors - Tenderness, warmth, erythema and swelling on exam
presentation of what dx
A
Osteomyelitis
18
Q
what type of condition may lees to less severe subjective complaints for osteomyelitis
A
DM
19
Q
osteomyelitis: _____ should be performed when ulcer is present
A
Probing for bone
20
Q
vertebral involvement presentation of osteomyelitis
A
- Slower progression → 3 weeks - 3 months
-
Localized pain and tenderness of involved vertebrae
- Often more than one vertebrae is involved including the intervertebral disks
- Pain is increased with percussion over affected area - (+) fever in ½ of patients
- +/- neurologic sx (present ⅓ of pts)
- Results from extension of infection leading to a spinal epidural abscess
21
Q
other presentations of osteomyelitis
A
- Nonverbal patients/pediatrics - decreased use/movement, fussiness
- Hip, pelvis, vertebral involvement - predominantly pain with few other sx
22
Q
lab dx osteomyelitis
A
-
Organism isolation → blood, bone, or contiguous focus
- Blood cx (+) in 60% of cases
- Cx from overlying wounds, ulcers are NOT reliable - CBC - Elevated WBC - left shift in acute infection
-
ESR and CRP - elevated
- Helpful to monitor throughout treatment course - BMP
- Assess renal and liver function before starting pharmacotherapeutics
23
Q
imaging dx for osteomyelitis
A
X-ray - Abnormal findings may not be present early in course
- Children - 5-7 d+
- Adults - 10-14 d+
- Early changes → possible soft tissue swelling, loss of tissue planes, periarticular demineralization of bone
-
Later XR changes (>2 weeks after sx)
- Periosteal thickening or elevation
- Bone cortex irregularity - Osteolysis, endosteal scalloping, regional osteopenia - Chronic infection - New bone formation, sclerosis
CT/MRI - highly sensitive
1. Avoid with indwelling metal devices
2. MRI preferred for foot infections
nuclear studies
1. If MRI is CI
2. High sensitivity but a low specificity
24
Q
indications for bone bx for osteomyelitis
A
- All pts with radiologic evidence of osteomyelitis without (+) blood cx
- Osteomyelitis by hematogenous spread doesn’t require bone bx - Do not delay biopsy due to abx use
25
indications for CT/MRI in osteomyelitis
1. Onset < 2 weeks at presentation
1. X-ray is negative in a clinical presentation consistent with infection
1. (+) neurologic findings on exam
26
technique for bone bx for osteomyelitis
1. Open (preferred due to higher sensitivity) → can be collected during debridement
1. _Percutaneous bx_ → often image (CT) guided
- Needed for vertebral osteomyelitis
- Must be collected through **uninfected** soft tissue
1. Assess bx specimen for **Gram Stain, C&S, and Histology**
27
histology shows necrotic bone with extensive resorption adjacent to an inflammatory exudate
dx?
osteomyelitis
28
management osteomyelitis
1. Consult ID and Ortho
2. Empiric Antibiotics
- Must cover _MRSA & gram -_: **vanc + 3rd or 4th gen ceph** (ceftriaxone/ceftazidime/cefepime)
3. **Tailor ABX therapy to cx**
4. Is there hardware present and is going to influence tx?
29
indications for hardware removal for osteomyelitis management
if no longer needed for bone stability or location of the hardware affects debridement
30
osteomyelitis - IV therapy is preferred during ?
the **acute phase** of infection, esp if patient has signs of systemic toxicity
31
IV abx options for osteomyelitis
Tx for staphylococcal osteomyelitis x 4 wks
1. _MSSA_ → IV cefazolin, nafcillin, or oxacillin
1. _MRSA (or severe PCN allergy)_ → vancomycin with goal trough level of 15-20 mcg/mL
32
If S aureus isolates show susceptibility to oral agents, ____ can be effective if given for 4-6 weeks _following_ 2 weeks of administration of appropriate IV agents: ___
combo therapy
1. **Levofloxacin/ciprofloxacin + rifampin**
2. Trimethoprim-sulfamethoxazole, doxycycline, or clindamycin could also be considered
33
Removal of **necrotic material** and culture (if not previously done) of involved tissue and bone
Debridement
34
indications for Debridement in osteomyelitis
1. Infection related to **open fx or surgical hardware**
2. **Extensive disease** involving multiple bony and soft tissue layers
- Vertebral osteomyelitis, subperiosteal collection, abscess, or necrotic bone present
1. The presence of **concomitant joint infection**
1. **Recurrent/persistent** infection despite standard medical therapy
35
monitoring management for osteomyelitis
1. **monitoring needed** for prolonged therapy - IV therapy
- CBC and CMP weekly
- ESR and CRP at the beginning and end of IV therapy and at any time sx worsen
--- _If ESR/CRP remains elevated 2 wks after completion of abx therapy consider persistence of osteomyelitis_
- PO therapy - CBC, Cr and ALT at 2, 4, 8,12 weeks and every 6-12 months after initiation of PO therapy (as long as abx is continued)
1. Serial exams until complete resolution is noted
36
why is serial radiographic imaging not recommended for management?
d/t persistent inflammatory changes that can be mistaken for persistent infection
37
complications with osteomyelitis
1. Bone destruction = **pathological fractures**
1. **Chronic osteomyelitis**
1. **Impaired bone growth** in children
- Increased risk if growth plate is affected
38
A long-standing bone infection over months or years results in the development of sequestrum with or without a sinus tract
Chronic Osteomyelitis
39
bone changes of chronic osteomyelitis
1. Increased intramedullary pressure leads to rupture of periosteum = **cloaca** or sinus tract
1. Periosteal blood supply interruptions = **necrosis**
1. This dead bone can lead to a radiographic findings = **sequestrum**
1. New bone will then begin to form in areas where the periosteum was damaged = **involucrum**
40
Chronic Osteomyelitis MC in what type of infections
sternal, mandibular, or foot infections
41
s/s chronic osteomyelitis
1. **Difficulty with weight-bearing** and loss of normal function
- Pain, erythema, or swelling may be present
- +/- draining **sinus tract**
- Fever is usually not present
42
w/u for chronic osteomyelitis
same as acute osteomyelitis
Leukocytosis is uncommon
+/- elevate of ESR/CRP
43
management for chronic osteomyelitis
1. Surgical debridement
1. Obliteration of dead space (to stabilize the bone)
1. Long-term antibiotic therapy
44
complications with chronic osteomyelitis
1. Osteolysis and pathologic fractures
1. Rarely, a chronic osteomyelitis sinus will undergo metaplasia and develop squamous cell carcinoma (*Marjolin Ulcer*)
45
occurs when increased pressure within a limited space compromises the circulation and function of the muscles and nerves within that space.
Compartment Syndrome
46
Compartment Syndrome MC happens where
1. **lower leg compartments**
- 4 compartments: anterior, lateral, superficial posterior, and deep posterior
2. Other compartments: Thigh, upper arm, forearm, hand, foot
47
Normal compartment pressure is ?
**10 mm/Hg**
Pressures up to 20 mmHg can be tolerated without damage
48
compartment syndrome: ___ and ___ are most at risk for cell death from ischemia
muscles and nerves
49
Ischemia >? hours leads to neuropathy and >? hours leads to myocyte death and chronic muscle contractures
8
12
50
Compartment Syndrome: Clinical Presentation
**5 P's**
1. **Pain _out of proportion_ to the injury / PE findings** - _early and sensitive sign_
- Burning, deep and aching
- Worse with passive stretching of involved muscle
2. **Paresthesias** within 30 - 120 minutes of onset
3. Muscle compartment is **tense to palpation**
4. **Muscle weakness** within 2-4 hours of onset
5. **Decreased sensation**
- 2-point discrimination is the most reliable early test
5. **Paralysis** (late finding)
6. **Pallor** (uncommon)
7. **Weak pulse** (only in severe vascular compression)
51
dx for compartment syndrome
1. **Measuring compartment pressure**
1. Laboratory testing is not needed to make the dx but may be needed to evaluate the underlying condition
52
how to measure compartment pressure
1. **Avoid in compartments of hands and feet** d/t small size of these compartments
1. **Two separate pressure readings** should be obtained within 5 cm of the site
1. Pressure readings **>45 mmHg** **require decompression**
- Alt → A difference between DBP and compartment pressure < 30 mm Hg = **inadequate perfusion, decompression needed**
--- Helpful in hypotensive patients
53
management compartment syndrome
1. **Remove casts/dressings** if applicable
1. **Elevated affected limb**
1. Consult for **surgical fasciotomy**
- initially left open to allow for swelling to subside
- After 48-72 hrs consider debridement and closure
- Can delayed closure at 7-10 days if needed
1. Consult plastics for skin grafting if needed prior to closure
54
Contraindications for surgical fasciotomy
missed timely diagnosis (elevated tissue pressures >24-48 hours)
55
disposition compartment syndrome
1. **Admit all patients**
1. Those **who do not meet indication for fasciotomy = serial exams** to monitor for worsening symptoms
56
prognosis of compartment syndrome
1. Depends on time of diagnosis and intervention
- **Within 6 hours** → will have complete recovery
- **Within 12 hours** → a little over ½ of patients will regain normal limb function
- **After 12 hours** → < 10% will regain function
57
1. Associated with **crush injuries** to muscle, immobility, drug toxicities, and hypothermia.
1. Serum **elevations in muscle enzymes (CK)** and marked **electrolyte abnormalities** characteristic.
1. Release of **myoglobin** leads to direct **renal toxicity**.
rhabdomyolysis
58
A syndrome of acute skeletal muscle cell death leading to the release of intracellular contents
Rhabdomyolysis
59
renal conditions involving Rhabdomyolysis
**Acute tubular necrosis (ATN)** resulting in **acute kidney injury (AKI)**, occurs in 30-40% of patients
60
ATN results from hypovolemia and a combination of the following:
| Rhabdomyolysis
1. Precipitation of **myoglobin and uric acid crystals** within renal tubules
- Uric acid is a metabolite of purine which is released from injured muscles
2. Decreased **glomerular perfusion**
- Hypovolemia results from "third-spacing" due to the influx of extracellular fluid into injured muscles
3. Nephrotoxic effect of **ferrihemate** (a metabolite of myoglobin)
61
common causes of rhabdo
1. **Muscle Injury** – Crush trauma, deep burns, bite wounds, necrotizing myopathy
1. **Muscle Ischemia** – Shock, localized compression (casts/splints), immobilization, compartment syndrome
1. **Drugs or Toxins** – ETOH, Statins, Fibrates, Antipsychotics, SSRIs, Benzos, recreational drugs, salicylates, thrombolytics, chemo agents, and more
1. **Exertional** – Intense physical exercise, Seizures
1. **Infection** - Coxsackie virus, influenza A and B, Epstein-Barr virus, HIV
1. **Inflammation** - Autoimmune muscle damage
1. **Body temp** - Hyperthermia/heat illness, Hypothermia
1. **Metabolism disorders and blood supply issues** - thrombosis, embolism, clamping of artery in surgery
62
1. **Dark “tea colored” urine**
1. **Myalgias** and **weakness** (present in only ½ of patients)
1. Malaise, low-grade fever
1. N/V, abdominal pain, and tachycardia in severe rhabdomyolysis
1. Swelling and tenderness of the involved muscle groups may occur
- Swelling may not become apparent until after rehydration with IV fluids
1. Mental status changes if severe renal failure due to urea-induced encephalopathy
rhabdo
63
dx rhabdomyolysis
1. **CK - most sensitive indicator**
1. UA
- **(+) reddish-brown color** when urine myoglobin is > 100 mg/dL
- **(+) blood on dip, (-) RBC on microscopic** suggests myoglobinuria
1. Urine myoglobin
1. CMP
- K+; BUN,Cr; AST/ALT = elevated
- hypocalcemia
1. Phosphorus - elevated
1. Uric acid - elevated
1. EKG - Cardiac dysrhythmias related to hyperkalemia or hypocalcemia may occur
1. CBC and coags - Look for signs DIC (complication)
1. Imaging - Indicated on a case by case basis if underlying etiology requires imaging for dx
64
tx rhabdo
1. **IV fluids - Early and aggressive for up to 72 h**
- 0.9% NS @ 1-2L / hr and titrate to UO goal
- CAUTION: Monitor for fluid overload in heart/renal pts
2. **I&O** - Foley to get accurate output measurements
- **Goal of 200-300 mL/h UO**
3. Urine alkalinization
4. Cardiac monitoring and serial EKG’s
5. Electrolytes
6. Lab monitoring
- CK q6h - peak CK levels > 6000 IU/L increase risk of AKI
- K+ 1-2 hours after initiation of treatment
- Glucose every hour if treating hyperkalemia with insulin/glucose
65
indications for urine alkalinization
CK levels > 5000 IU/L, acidemia, dehydration, or underlying renal disease
Regimen: 150 mEq of sodium bicarbonate added to 1 L of 5 percent dextrose or water, administered at 200 mL/h with goal urine pH >6.5
66
electrolyte tx in rhabdo
1. Treatment of hyperkalemia may be needed (options)
- IV insulin (with IV glucose)
- IV calcium (only if severe hyperkalemia in the setting of hypocalcemia)
2. Hypocalcemia is only treated if in the presence of severe hyperkalemia
3. Phosphate and uric acid rarely need treatment
67
management for rhabdo
1. Consult nephrology if…
- Oliguric renal failure, persistent electrolyte abnormalities/acidosis, signs of fluid overload
2. Consult ortho/surgery if concern about compartment syndrome
68
disposition of rhabdo - Discharge criteria (all must be met) after rehydration
1. Normal renal function
1. Normal electrolytes
1. Alkaline urine
1. An isolated cause of the muscle injury
1. No uncontrolled comorbidities
69
complications of rhabdo
1. **Acute Kidney Injury** - Risk increases with dehydration, sepsis, and acidosis
1. **Compartment Syndrome** - A potential complication of severe rhabdomyolysis that may develop after fluid resuscitation, with worsening edema of the limb and muscle
1. **Disseminated Intravascular Coagulation** - Occurs in severe Rhabdomyolysis due to the release of thromboplastin and other prothrombotic substances from the damaged muscle
70
A chronic condition characterized by **widespread musculoskeletal pain** with **multiple tender points** but no _clear objective findings_
Fibromyalgia
71
fibromyalgia is MC in who
women aged 20–55
72
cause of Fibromyalgia
* Hypothesis: the presence of a central sensitization to pain and deficits in endogenous pain inhibitory mechanisms
* Sleep disorders, depression, and viral infections may have some influence
73
1. **Chronic fatigue and generalized aching pain**
- Prominence of _neck, shoulders, low back, and hips_
- Most days for ≥ 3 months
- Worsened by minor exertion
2. Associated sx/conditions
- Depression, anxiety, sleep disorders (unrefreshed sleep), cognitive dysfunction, subjective numbness, fatigue, chronic headaches, and IBS
3. Exam is normal, except for widespread soft tissue tenderness
- It is no longer recommended to assess “trigger points”
4. Joints are unaffected
fibromyalgia
74
ACR New Diagnostic Criteria for Fibromyalgia
1. Widespread pain index (**WPI**) >7 and symptom severity (**SS**) scale >5 OR WPI 3-6 and SS scale >9
1. Symptoms have been present for **at least 3 months**
1. There is **no other disorder** that would explain the patient's symptoms
75
dx fibromyalgia
**diagnosis of exclusion**
You must consider and rule out other ddx
76
generalized management fibroyalgia
1. _multidisciplinary approach_ most effective
1. **Reassurance** the condition is diagnosable and treatable
1. **Goals** → improved function and quality of life rather than elimination of pain
1. **Pt ed is essential**
- Course of disease is non-progressive
- tx available but not curable
- Compliance and expectations regarding treatment are key to success
1. Non-pharmacologic
- **Cognitive behavioral therapy**: focus on sleep hygiene and mood disorders
- **Exercise**: low aerobic activity with slow progression (aqua aerobics) to strength training
- **Weight loss** if overweight
77
med management fibromyalgia
1. **Muscle relaxant**: cyclobenzaprine (Flexeril)
1. **Antidepressants**: amitriptyline (Elavil), duloxetine* (Cymbalta), milnacipran (Savella)*
1. **Anticonvulsants**: gabapentin (Neurontin)*, pregabalin (Lyrica)*
1. **Analgesic**: tramadol (Ultram)
1. Avoid opiates and corticosteroids
78
fibromyalgia - Regimen is often based on type and severity of patient sx:
1. Most: initial treatment with cyclobenzaprine or amitriptyline at bedtime
1. Severe fatigue: start with SNRI (Cymbalta/Savella)
1. Severe sleep disturbance: start with Neurontin or Lyrica
79
A condition characterized by a progressive destruction of bone and soft tissues at weight bearing joints
Leads to joint dislocations, pathologic fractures, and debilitating deformities
Neurogenic Arthropathy
80
The hallmark deformity associated with this condition is midfoot collapse, described as a “rocker-bottom” foot
Neurogenic Arthropathy
81
pathophys Neurogenic Arthropathy
unknown but likely multifactorial:
- Neuro-traumatic
- Neurovascular
82
conditions associated with Neurogenic Arthropathy
Any condition that causes sensory or autonomic neuropathy:
- **DM - MC**
- Cerebral palsy
- Alcoholic neuropathy
- Spinal cord injury
- Syphilis
83
where does neurogenic arthropathy MC affect?
1. MC foot and ankle
1. MC joints
- Tarsometatarsal (TMT) joint
- Cuneonavicular, talonavicular, and calcaneocuboid articulations
84
presentation of neurogenic arthropathy
1. Vary based upon severity
1. **Unilateral warmth, redness, and edema** over joint region (foot/ankle)
1. Often there is a **h/o minor trauma**
1. **Pain** is often present but at a **much lower severity** than what would be expected
1. **Loss of arch, bony protrusions**
1. 40% of patients will have a **concomitant ulceration**
85
dx neurogenic arthropathy
1. Labs will be case based to look for underlying etiologies
1. **X-ray** - weight bearing if possible
- nml or nonspecific in early disease: Soft tissue swelling, loss of joint space, osteopenia
- progressed: **Fractures, subluxation and frank dislocations are seen**
1. **MRI → if x-ray negative or if osteomyelitis Ddx**
- MRI sensitive for early disease showing bone marrow edema with or without microfracture
- Helps r/o osteomyelitis
86
X-ray shows
Progressive decrease of calcaneal inclination
Equinus deformity at the ankle (inability to dorsiflex)
Destruction of the tarsometatarsal joint with the typical rocker-bottom deformity.
what dx
neurogenic arthropathy
87
Neurogenic Arthropathy: Clinical & Radiographic Staging
Stage 0: Early or inflammatory
Stage 1: Development
Stage 2: Coalescence
Stage 3: Remodeling
88
Localized swelling, erythema, and warmth
X-ray: little or no radiological abnormalities
staging of neurogenic arthropathy?
Stage 0: Early or inflammatory
89
Swelling, redness, and warmth persist
X-ray: Bony changes such as fracture, subluxation/dislocation, and bony debris
what staging of neurogenic arthropathy
Stage 1: Development
90
Clinical signs of inflammation decrease
X-ray: fracture healing, resorption of bony debris, and new bone formation are evident
what staging of neurogenic arthropathy
Stage 2: Coalescence
91
No signs of inflammation; bony deformity, which may be stable or unstable, is present.
X-ray: may show mature fracture callus and decreased sclerosis
what staging of neurogenic arthropathy
Stage 3: Remodeling
92
management for neurogenic arthropathy
stage 0-2
1. **Avoid wt bearing → cast**
- Continue until signs of inflammation resolve and improvement in radiologic findings (ranging from 3 to 25 months)
2. nml wt bearing w/ **Charcot Restraint Orthotic Walker (CROW)**
stage 3 and those who fail offloading therapy - Discuss risk/benefit of **surgery**
93
a syndrome of paroxysmal digital ischemia, most commonly caused by an exaggerated response of digital arterioles to cold or emotional stress
Raynaud’s Phenomenon (RP)
94
pathophys of RP
Vasoconstriction leading to well-demarcated digital pallor or cyanosis followed by rapid vasodilation resulting in intense hyperemia and rubor
95
RP MC affects what?
**fingers**, but it can affect the toes, nose and ears
96
no vascular structural abnormalities
An exaggeration of normal vasoconstriction to cold exposure
MC onset in **healthy females between 15 and 30 years of age**
(+) FMHx in 30% of patients
what RP classification
primary
97
1. less common
1. An underlying systemic condition leads to RP
1. MC onset in **male over age 40**
1. MC with **rheumatologic conditions**
1. Other RF/precipitating events: frostbite, chronic use of certain tools (i.e. jackhammer)
1. Sx more severe with an increased risk of digital ulceration or gangrene
which RP classification
secondary
98
presentation of RP
1. “Attacks” - **Sudden onset of cold digits** with a demarcation of **skin pallor (white attack) or cyanosis (blue attack)**
- White attacks are more likely to lead to digital ischemia
1. During rewarming, there is vascular reperfusion, resulting in the erythema secondary to rebound blood flow
1. Associated symptoms → aching or throbbing pain, paresthesia, numbness, stiffness, mild swelling may occur after rewarming
1. Early - single finger may be affected; With time multiple fingers of both hands will be affected
1. MC digits - **index, middle, and ring fingers **
99
RP presentation - Look for signs of ____ (3)
sclerodactyly, calcinosis, or digital ulcers
100
RP - Examine the ___ under magnification (ophthalmoscope)
nailfold capillaries - Abnormally large loops, alternating with areas without any capillaries
101
management approach for RP
1. Primary RP (normal PE and nailfold capillary microscopy)
- Patient education
- Follow up regularly to ensure signs and/or symptoms of secondary causes of RP do not emerge - RP is occasionally the 1st manifestation of these disorders
2. Secondary RP
- Order labs to look for underlying causes → individually based upon H&P
3. Treat underlying disease
102
Pt ed for RP
1. Wear mittens and stockings when outside in cold weather - “stay warm”
1. Avoid vasoconstricting medications
- Decongestants, diet pills, sumatriptan (Imitrex), opiates
1. Smoking cessation if applicable
103
meds for RP
1. **Indicated if failure to control sx** with non-pharm therapies or if evidence of digital ulcers
1. **CCB’s - 1st line - Amlodipine**
1. Others - Topics vasodilators (nitroglycerine), PDE5 inhibitors
1. Refer to vascular surgery if resistant to pharmacologic therapy
104
A genetic disorder (autosomal dominant) of the connective tissue characterized by skeletal, ocular, and cardiovascular abnormalities
Marfan Syndrome
104
presentation of Marfan Syndrome
1. Affected patients are typically tall, with long arms, legs, and digits (arachnodactyly)
- There can be wide variability in the clinical presentation
1. Scoliosis
1. Pectus excavatum/carinatum
1. Ectopia lentis (displacement of the eye lens) is present in about ½ of patients
1. Severe myopia (nearsightedness)
1. Retinal detachment can occur
1. MVP seen in about 85% of patients
1. Aortic root dilation is common and leads to aortic regurgitation or dissection with rupture
105
dx marfan syndrome
1. Echocardiogram / abdominal US to assess aorta
1. Slit lamp exam to check lens displacement
1. Genetic testing
- Mutations in the fibrillin gene (FBN1) on chromosome 15
106
Ghent scoring system is used for ?
Marfan Syndrome
Diagnostic Criteria
107
management for marfans
1. **Annual ophthalmologic evaluation**
- Monitor and correct visual acuity and thus prevent amblyopia
2. **Annual orthopedic consultation**
- monitor development of scoliosis at an early enough stage so that bracing might delay progression
3. **Echo annually** (and prn) to monitor aortic diameter and mitral valve function
- Follow with cardiology annually
- Surgical intervention may be necessary to repair mitral valve or aorta
4. **Long-term BB** - slows rate of aortic dilation
- Atenolol or metoprolol
5. **Restriction from vigorous physical exertion** protects from aortic dissection
