Rheumatic Fever

Definition:
Rheumatic fever is a multisystem inflammatory disease that occurs as a sequel to
pharyngeal infection with the bacterium, Group A Streptococcus pyogenes. Major
features are arthritis and carditis.

Clinical Features:
These are varied. They include fever, pain (with or without swelling) in one or more
joints, endocarditis, pericarditis, myocarditis, pleurisy, subcutaneous nodules, a
characteristic skin lesion (erythema marginatum) and an even more characteristic
disturbance of central nervous system function, Sydenham’s chorea. Carditis is
more frequent in the youngest age groups, and the majority of rheumatic fever
attacks occurring in adults are manifested primarily by arthritis.

1. Arthritis usually affects larger joints, particularly wrists, elbows, knees and ankles.
   Hips are less often affected, small joints of hands and feet rarely, and spine almost
   never. Characteristically, fleeting arthritis occurs but more commonly, only arthralgia alone occurs. Untreated, the joint pains settle within 1-4 weeks. The
   arthritis of rheumatic fever does not lead to permanent damage to the joints
   affected.
2. Carditis is the most important clinical manifestation of rheumatic fever, being the
   one with permanent effects. Endocarditis is detected clinically by new or changing murmurs, and pericarditis by a friction rub. Heart failure is the most serious presentation,
   occurring in younger patients.
3. Chorea is now rare. The latent period is 1 to 6 months. It features jerky, purposeless
   movements, exaggerated by tension but disappearing in sleep. It occurs
   predominantly in females.
4. Other less common manifestations include erythema marginatum, an evanescent
   macular eruption and subcutaneous nodules, which may
   appear as painless lumps in people with long-standing carditis.

Recurrence:

An attack of rheumatic fever greatly increases the chances that a subsequent
streptococcal throat infection will be followed by another attack of rheumatic
fever. The risk of recurrence is greater in children, in patients with pre-existing
rheumatic heart disease, and in those experiencing symptomatic throat infection.
The risk declines with advancing age, but nevertheless rheumatic patients remain at
increased risk well into adult life.

Diagnosis:

Revised Jones' criteria - evidence of preceding strep infection (positive throat culture or rapid streptococcal antigen test, increased or rising anti-strep Ab, recent scarlet fever) plus 2 major or 1 major and 2 minor criteria.

Major criteria

1. Polyarthritis
2. Carditis
3. Chorea
4. Subcutaneous nodules
5. Erythema marginata

Minor criteria

1. History of acute rheumatic fever
2. Rheumatic heart disease
3. Fever
4. Arthralgias
5. Increased WBC, increased ESR, increased C-reactive protein
6. Prolonged PR interval on ECG

Complications:

The major complication of rheumatic fever is valvular disease of the heart giving rise to pansystolic blowing mitral murmur, less commonly diastolic aortic murmur at left sternal border.

Treatment:

• Treat streptococcal infection with antibiotics : penicillin G 600,000-1.2 million units as 1 injection or PO for 10 days
• Erythromycin if allergic to penicillin
• Anti-inflammatory treatment - aspirin is treatment of choice for ARF
• Sedating drugs for severe chorea - phenobarbital, chlorpromazine, haloperidol

Developmental Dysplasia of the Hip (DDH)

Causes:

• Intrauterine malpositioning.
• Genetic factors
• Maternal hormones.
• Post-natal postural influence (hence rare in India)

Complications:

• Recurrence of dislocation
• Incomplete reduction
• Avascular necrosis of femoral head
• Residual deformity of hip joint
• Late onset of premature osteoarthritis

Clinical Features:

• Clunks are sensations felt when femoral head dislocates with Barlow maneuver or relocates with Ortolani maneuver

Diagnosis:

X-ray findings

1. Shenton's line should be continuous.
2. Acetabular index more vertical (closer to 90).

Treatment:

Depends on age.

1. Newborn - 3 months

• Pavlick harness
• manipulative reduction
• hip spica cast

2. 3-6 months

• Pavlick harness
• Manipulative reduction
• Hip spica cast
• Ooccasionally open reduction necessary.

3. 6-12 months

• pre-reduction traction, open or closed reduction, hip spica cast

Osteogenesis Imperfecta

Description:

Also called Brittle bone disease it is a congenital connective tissue disorder.

Clinical features:

• Fragility of bone
• Osteopenia
• Variable degrees of short stature
• Progressive skeletal deformities
• HEENT - blue sclerae (translucent thin connective tissue over choroid)
• 50% hearing loss (maturity-onset deafness)
• Dentinogenesis imperfecta

Osteopetrosis

Definition:
Osteopetrosis also known as marble bone disease and Albers-Schonberg disease is an extremely rare inherited disorder whereby the bones harden, becoming denser, in contrast to the more prevalent osteomalacia, in which the bones soften.

Variants:

1. Autosomal recessive (malignant type)
2. Autosomal dominant (benign type)
3. Carbonic anhydrase II deficiency (Type 3 Renal tubular acidosis)

Pathogenesis:


Pathologic alteration of osteoclastic bone resorption leads to thickening of cortical and lamellar bone.

Clinical Features:

• Brittle bones
• May have blindness, deafness and cranial nerve involvement if skull base involvement
• Osteopetrosis tarda
• Fractures related to brittle osteopetrotic bones or osteomyelitis
• Osteopetrosis congenita
• Failure to thrive
• Growth retardation
• Sshort stature
• Intracranial calcifications
• Sensorineural hearing loss
• Psychomotor retardation

Lab Investigations:

• X-rays

• Blood tests: anemia, elevated acid phosphatase level in some cases.
• Imaging studies: markedly increased density of skeleton, osteopetrosis tarda often detected as incidental finding, typical dense deformed sclerotic bones.

Differential Diagnosis:

• Hypervitaminosis D
• Hypoparathyroidism
• Paget's disease
• Diffuse bone metastasis of breast or prostate cancer .
• Intoxication with fluoride, lead or beryllium.
• Hematological disorders such as myelofibrosis, sickle cell disease and leukemia.

Complications:

• Multiple fractures
• Anemia (decreased marrow space)
• Hepatosplenomegaly
• Hypersplenism

Treatment:

1. Surgery

• Surgery useful if significant alterations of facial profile, recurrent fractures with subsequent deformity or severe related degenerative joint disease.
• Bone marrow transplant is only curative treatment for osteopetrosis congenita.
• Placental blood transplant (umbilical cord blood) as useful source of allogeneic hematopoietic stem cells for bone marrow reconstitution.

2. Medical

• Interferon gamma-1b
• 1,25-dihydroxyvitamin D

Prognosis:

• Good for osteopetrosis tarda, poor for other types.
• Osteopetrosis congenita usually results in death by 2 years from severe anemia, bleeding or infection.

Chronic Sinusitis

Clinical features:
HEENT examination elicits tenderness and edema. Mild mucopurulent rhinorrhoea, nasal obstruction and facial pain/frontal headache, with or without upper toothache.
Diagnosis:
2 major criteria or 1 major and 2 minor criteria
Major criteria

• Purulent nasal discharge
• Purulent pharyngeal drainage
• Cough

Minor criteria

• Periorbital edema
• Headache
• Facial pain
• Tooth pain
• Earache
• Sore throat
• Foul breath
• Increased wheeze
• Fever

Treatment:

Medical

• Corticosteroids
• Administering nasal steroids with head inverted may be more effective in cases of sinusitis refractory to traditionally applied nasal steroids.
• Antibiotics may provide short-term relief but not long-term benefit.
• Saline nasal irrigation
• Intranasal hypertonic saline washes

Surgery

• Surgical drainage if medical treatment fails
• Functional Endoscopic Sinus Surgery (FESS)
• Adenoidectomy is an alternative

Complications:

• Suppurative CNS complications
• Pott's puffy tumor
• Frontal bone osteomyelitis and subperiosteal abscess.

Mooren's Ulcer (Chronic Serpiginous Ulcer)

Definition:
Mooren's ulcer is strictly a peripheral ulcerative keratitis (PUK) with no associated scleritis.

Clinical Features:

1. It commences as one or more grey infiltrates which break down to form small
   spreading ulcers which eventually coalesce. The ulcer undermines the epithelium
   and superficial lamellae at the advancing border, forming a characteristic
   overhanging edge, the base becoming vascularized.
2. Erosion of the epithelium is accompanied by pain and watering of the eye.
3. Perforation is rare but intermittent progression over several months is usual until a
   thin nebula forms over the whole cornea with diminution of sight.
4. In about 25% of cases both corneae are affected but not always simultaneously.

Exact aetiology is unknown. It is suspected to be a degenerative condition.

Treatment:

1. Topical steroids
2. Conjunctival resection
3. Systemic immunosuppressives
4. Additional surgical procedure
5. Rehabilitation

Abdominal Aortic Aneurysm (AAA)

An unruptured AAA

Causes:
95% cases due to atherosclerosis in United States. Mycotic abdominal aortic aneurysm is caused by bacterial infection, most commonly Salmonella

Pathogenesis:
Intimal dissection causes aortic dilatation and creation of false lumen, into which blood collects.

Risk factors:

1. Smoking
2. Clinical vascular disease
3. Male
4. Old age
5. Hypertension
6. Hyperlipidemia
7. Family history of AAA

Signs and Symptoms:
Normal vital signs may be present initially with rupture, but patients can become severely hypotensive rapidly.
Abdomen usually presents as asymptomatic palpable pulsatile nontender mass, with bruits. Abdominal palpation is an important symptom.

Diagnosis:
Abdominal ultrasound or other imaging.

Treatment:

• Surgery recommended for AAA > 5.5 cm or symptomatic AAA of any diameter.

• Surgery for AAA <>
• Intervention not recommended for asymptomatic infrarenal or juxtrarenal AAA <>

Senile Dementia of Alzheimer Type

Clinical Presentation:

• Usually presents with memory deficit or personality changes
• Recent memory loss that affects job performance, difficulty performing familiar tasks, problems with language, disorientation of time and place, poor or decreased judgment, problems with abstract thinking, misplaced things, changes in mood or behavior, changes in personality, loss of initiative.
• May present as new-onset auditory hallucinations in elderly

Histology:

Prognosis:
Median survival is 4-6 years after initial diagnosis of Alzheimer disease

Carpal Tunnel Syndrome

Definition:
Carpal tunnel syndrome (CTS) is a compression neuropathy caused by constriction
of the median nerve where it passes beneath the flexor retinaculum of the wrist. It is
a common condition. The crude incidence rate in the UK of about one per thousand
person-years in hospital-diagnosed patients and twice that in primary care.

Clinical Features:

1. Tingling (paraesthesia), numbness, or discomfort in the thumb, index and
   middle fingers. Distressing tingling is often prominent by night, and the patient
   may need to exercise the fingers or shake the hand in attempt to gain relief.


2. Weakness and/or clumsiness of the hand, particularly in carrying out fine
   movements such as those concerned in writing, typing, sewing and other tasks
   necessitating pinch grip.


3. There is often forearm or elbow pain, aching in nature, poorly localised and
   exacerbated by activity. Some patients have shoulder pain. In advanced cases
   of median nerve entrapment, there is impaired sensation and thenar atrophy.

Aetiology:

Carpal tunnel syndrome usually presents as an isolated phenomenon but it is often
associated with other conditions especially where there is fluid retention or
inflammation. These include, diabetes mellitus (where it may be due to diabetic
mono-neuropathy rather than compression), the prolonged use of oral
contraceptives, pregnancy, Colles' or scaphoid fractures, other direct trauma, electric
shock, tenosynovitis, rheumatoid disease, osteoarthritis and gout. Obesity and short
stature are independent risk factors.

Colles' Fracture

Causes

The fracture is most commonly caused by people falling backward onto a hard surface and breaking their fall with extended outstretched hands.

Displacements

1. Proximal impaction
2. Dorsal displacement
3. Dorsal tilt
4. Ulnar tilt

Treatment

Treatment of depends on the severity of the fracture. An undisplaced fracture may be treated with a cast alone.The cast is applied with the distal fragment in palmar flexion and ulnar deviation. A fracture with mild angulation and displacement may require closed reduction. Significant angulation and deformity may require an open reduction and internal fixation or external fixation.

Radiograph showing a typical Colle's Fracture

Myositis Ossificans

Types:

• Nonhereditary Myositis Ossificans


• Myositis Ossificans Progressiva

Clinical Features:

Myositis ossificans usually presents with pain, tenderness, focal swelling, and joint muscle reduction, in the aftermath of a painful muscle contusion which resolved more slowly than expected, if at all.
The condition rarely is asymptomatic, and may sometimes be diagnosed from radiographs obtained for unrelated problems.
Most (i.e. 80%) ossifications arise in the thigh or arm, and are caused by a premature return to activity after an injury. Other sites include intercostal spaces, erector spinae, pectoralis muscles, glutei, and the chest. Hazy densities are sometimes noted ca. one month after injury, while the denser opacities eventually seen may not be apparent until two months have passed

Treatment:

Treatment is initially conservative, as some patients' calcifications will spontaneously be reabsorbed, and others will have minimal symptoms. In occasional cases, surgical debridement of the abnormal tissue is required, although success of such therapy is limited.

Radiograph of both feet

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