Pediatric Musculoskeletal disorder

Question 1

List some fractures peculiar in the immature skeletons of children.

Answer 1

See the attached image for details:
1. Typical Buckle or Torus #: bowing of the cortex under axial force.

2. Greenstick #
3. Plastic bowing #: on opposite side of greenstick #
4. Salter-Harris #: growth plate #’s.

Question 2

Greenstick fractures can be m/m with ______; f/up X-rays are indicated at _____ days.

Answer 2

m/m with reduction with casting;

f/up X-ray at 10-14 days.

Question 3

Typical Buckle (aka Torus) #’s involve _____, and usually develop upon application of ____ force on the bone.

Answer 3

involve buckling/bowing of the cortex upon application of axial force (see attached image).

Question 4

Torus # usually occur in _____ bones typically after a fall.

Answer 4

distal radius or ulna.

Question 5

Torus # are managed with _____.

Answer 5

cast immobilization for 3-5 weeks.

Question 6

______ is the most commonly fractured long bone in children.

Answer 6

clavicle

Question 7

Clavicular bone fractures during birth may be a/w _____ complication (s).

Answer 7

brachial plexus palsies or subclavian artery injury (angiogram may be done to confirm).

Question 8

_____ is the most common location of the clavicular fracture, in which case the proximal segment is displaced superiorly d/t pull of the ______ muscle.

Answer 8

middle third; the proximal segment is displaced superiorly d/t pull of the sternocleidomastoid muscle.

Question 9

What is the m/m in the middle third fractures of the clavicle?

Answer 9

Rest, ice, and sling.

Question 10

Management of distal end clavicular fractures includes ______ t/t.

Answer 10

Open reduction and internal fixation.

Question 11

A 3-year-old boy is brought with pain in the right elbow with an inability to bend the elbow, and forearm in the pronated position after he was lifted by the hand. What is the most likely cause of his symptoms?

Answer 11

Subluxation of the radial head (Nursemaid’s elbow) upon being pulled or lifted by hands.

*managed with manual reduction by gentle forearm hyper-pronation, or supination of the forearm at 90 degree of flexion.

Question 12

______ is the most common pediatric elbow fracture, that typically occurs in children aged ___ years, after a fall on the outstretched hand.

Answer 12

supra-condylar # of the humerus (see attached image for details); in children aged 5-8 years.

Image Source: https://radiopaedia.org/articles/supracondylar-humeral-fracture-2

Question 13

Supra-condylar fracture of the humerus may result in ___ complication (s).

Answer 13

-injury to the nerves: most commonly the anterior interosseous nerve (AIN; br. of the median nerve), f/by radial nerve, and then the ulnar nerve.

-injury to the brachial artery (check radial pulse d/t risk of Volkmann contracture with cast immobilization).

*Most AIN, radial, and ulnar nerve injuries resolve spontaneously without any intervention (Vaquero-Picado A, González-Morán G, Moraleda L. Management of Supracondylar Fractures of the Humerus in Children. EFORT Open Reviews. 2018;3(10):526-40. doi:10.1302/2058-5241.3.170049 - Pubmed).

Question 14

______ is characterized by overuse apophysitis of the tibial tubercle (esp. quadriceps contractions), seen in adolescent athletes.

Answer 14

Osgood-Schlatter disease.

Question 15

Osgood-Schlatter disease can be m/m with ______ strategies.

Answer 15

-decreased activity for 2-3 months
-braces for symptom relief

Question 16

Fractures of the growth plate (aka Salter-Harris #) can be classified into ___ types.

Answer 16

MN: SALTeR based on # pattern

Types (see attached image)
I: Physis (Straight across).
II: Metaphysis & Physis (Above)
III: Epiphysis & Physis (Lower)
IV: Epiphysis, Metaphysis & Physis (Through).
V: CRush injury of the physis.

Question 17

Becker muscular dystrophy is a milder form of ______ with ____ inheritance pattern.

Answer 17

Duchenne muscular dystrophy (DMD);

X-linked recessive inheritance.

Question 18

DMD and BMD care caused d/t _____.

Answer 18

dystrophin gene (cytoskeletal protein) mutation

Question 19

True/False?

Even though DMD and BMD are considered to be part of the same clinical entity, the causative mutations in the cytoskeletal “dystrophin” gene are often different in both conditions.

Answer 19

true;

DMD is d/t out-of-frame mutations of the dystrophin open reading frame, -> lack of dystrophin expression.

BMD is d/t in-frame mutations in the dystrophin gene -> dystrophin deficiency or dysfunction -> broader phenotypic presentation.

Question 20

Individuals with BMD have ___ % of the normal dystrophin amount or have a partially functional form of the subsarcolemmal protein.

Answer 20

10% to 40% of the normal dystrophin amount or have a partially functional form of the subsarcolemmal protein.

Question 21

DMD is characterized by ____ muscle weakness.

Answer 21

axial and proximal muscle weakness.

Question 22

Peudo-hypertrophy of the ______ muscle is a characteristic sign of DMD (and BMD).

Answer 22

gastrocnemius (calf)

Question 23

The onset of s/s in DMD is around ___ age, whereas the onset of illness in BMD is around ____ age.

Answer 23

DMD: onset ~ 3-5 years of age;

BMD: onset ~ 5-15 years or beyond.

Question 24

Patients with DMD are wheelchair-dependent by ____ age, while individuals with BMD may remain ambulatory even after age _____.

Answer 24

DMD: wheelchair-dependent before age 13 years;

BMD patients may remain ambulatory even after age 16 years.

Question 25

The average life expectancy in DMD is around ___ age, whereas in BMD is around ____ age.

Answer 25

DMD: ~ teens -20/30 years of age

BMD: ~ 40-50 years

Question 26

Intellectual disabilities are common in ____ (? DMD vs BMD)

Answer 26

DMD

Question 27

Serum creatine kinase levels may be ___ times higher in DMD versus BMD.

Answer 27

DMD: 10-20 times of normal levels;

BMD: 5 times of normal levels.

Question 28

True/False?

Gowers maneuver and a waddling gait or toe walking may be early signs of DMD in a toddler.

Answer 28

True

Question 29

Confirmation of DMD is made with ___ tests.

Answer 29

genetic testing

Question 30

When is muscle biopsy indicated in the diagnosis of DMD?

Answer 30

When genetic testing is inconclusive;

*muscle biopsy reveals replacement of muscle tissue with fat and fibrotic tissue.

Question 31

EMG study in DMD may reveal __ findings.

Answer 31

polyphasic potentials and increased recruitment.

Question 32

Death in DMD (and BMD) mostly occur d/t ___ complication.

Answer 32

dilated cardiomyopathy induced heart or respiratory failure.

Question 33

Other than dilated cardiomyopathy, ____ cardiac abnormalities are also common in patients with DMD/BMD.

Answer 33

conduction abnormalities in the form of life-threatening arrhythmias (d/t due to left ventricular wall fibrosis).

Question 34

____ is an autosomal dominant subgroup of myopathy and the most common type of muscular dystrophy that begins in adulthood.

Answer 34

Myotonic dystrophy (DM);

THINK: Long handshake d/t inability of the muscles to relax after contraction.

Question 35

True/False?

Myotonic dystrophy type II (DM2) aka proximal myotonic myopathy is a milder form of myotonic dystrophy type I (DM1) aka Steinert disease.

Answer 35

true

Question 36

DM1 (severe type) is caused by triple repeat expansion of ______ in the 3’-untranslated region of the DM1 protein kinase (DMPK) gene, whereas DM2 results from the expansion of ____ in the intron of the CCHC-type zinc finger nucleic acid-binding protein.

Answer 36

DM1: d/t CTG (triple) repeat expansion in the 3’-untranslated region of the DM1 protein kinase (DMPK);

DM2: d/t CCTG tetranucleotide repeat located in the intron of the CCHC-type zinc finger nucleic acid-binding protein.

Question 37

Symptoms in DM1 include ____ (list all).

Answer 37

-myotonia: distal > proximal (unlike DM2).
-dysphagia
-balding
testicular atrophy
-cataracts
-cardiac conduction abnormalities

MN: CTG triple repeat p/w Cataracts, Toupee, Gonads!

Question 38

What are the three main types of DM1 (severe DM)?

Answer 38

(1) Congenital (fetal) in ~15%
-muscle & CNS involvement.
-neonatal mortality ~18% d/t respiratory involvement.
-Hypotonia (rather than myotonia) is hallmark feature.

(2) Mild: weakness, myotonia, and cataracts that develop between 20 to 70 years; NORMAL life-span.

(3) Classic: onset in 2/3/4 decades with myotonia with characteristic *“warm-up phenomenon”.
-Distal muscle weakness -> impaired fine motor tasks involving hands, and impaired gait d/t foot drop.
-characteristic “myopathic face” or “hatchet face”.
-Cardiac conduction abnormalities.
-reduced lifespan.

(4) Childhood (infantile) DM:
-onset ~ age 10.
-Initial s/s: learning difficulties and psychosocial problems; dysarthria and hand muscle myotonia.
-Cardiac conduction abnormalities.

*(more pronounced after rest and improves with activity).

Question 39

Hearing loss is more frequent in DM type ___.

Answer 39

DM2 (mild DM).

Question 40

DM2 typically manifests in adulthood around a median age of ____ years.

Answer 40

48 years

Question 41

DM2, the milder form of DM with onset typically in adulthood (median age 48 years) p/with ___ s/s.

Answer 41

-variable c/p with the weakness and/or myalgia as the mc initial symptom in ~ 50% of cases.

Weakness: axial & proximal muscle esp. flexors of the neck, long fingers, and hip, and hip extensors.

-Myalgias: abdominal, musculoskeletal/myofascial, and exercise-related pain.

-early-onset cataracts (at < 50 years),
-hearing loss.

Question 42

DM2 presents mostly with ____ muscle weakness in contrast to DM1 which typically manifests as ____ weakness.

Answer 42

DM2: axial and proximal muscle weakness (flexors of neck, long finger flexors, and hip, and hip extensors);

DM1 (severe form): distal muscle weakness.

Question 43

_______ is the forward slipping of one vertebral body over the other leading to lower back pain with bowel and bladder symptoms, and a palpable “step-off” on PE.

Answer 43

Spondylolisthesis (see attached image).

Image source: Image Source: https://www.google.com/search?q=spondylolisthesis+vs+spondylolysis&rlz=1C5CHFA_enUS1049US1050&oq=spondylolisthesis+&aqs=chrome.4.69i57j0i131i433i512l2j0i433i512j0i512l4j0i131i433i512j0i512.9805j0j7&sourceid=chrome&ie=UTF-8#fpstate=ive&vld=cid:fd99f448,vid:sHdAGSa1Opc,st:0

Question 44

Spondylolisthesis of _____ vertebral body is commonly seen in children, and adolescents esp. gymnasts, and weight-lifters.

Answer 44

L5 slipping over S1.

Question 45

Spondylolisthesis is commonly a/with _____.

Answer 45

-congenital malformation of the lumbosacral joint, or

-spondylolysis (pars inter-articularis defect) (see attached image).

Image Source: https://www.google.com/search?q=spondylolisthesis+vs+spondylolysis&rlz=1C5CHFA_enUS1049US1050&oq=spondylolisthesis+&aqs=chrome.4.69i57j0i131i433i512l2j0i433i512j0i512l4j0i131i433i512j0i512.9805j0j7&sourceid=chrome&ie=UTF-8#fpstate=ive&vld=cid:fd99f448,vid:sHdAGSa1Opc,st:0

Question 46

What is the difference between spondylolysis, spondylolisthesis, spondylitis, and spondylosis?

Answer 46

See attached image.
1. SPONDYLOSIS: degenerative OA of the joints between the vertebral body and foramina neurons -> narrowing of the intervertebral foramen space -> compression of nerve roots (Radiculopathy) or spinal cord (Myelopathy) p/w pain, sensory and motor changes.
-Causes/Risk factors: Ageing, work/lifestyle related RSI (Repetitive Strain Injury), genetics, and smoking.

2. SPONDYLOLYSIS: a stress # in the lumbar pars inter-articularis.
   -Risk Factors: sports requiring excessive/repeated bends (gymnastics, football); genetics.
   -back pain, exacerbated by activity and exercise, and by leaning back.
   -progress to spondylolisthesis.
3. SPONDYLOLISTHESIS: vertebral slippage (L5-S1).
   -congenital or developmental anomaly at birth;
   -less common in older adults with degenerative changes in the spine.
4. Spondylitis: arthritis of vertebral structures.

Question 47

Under what condition, a Metatarsus Adductus (forefoot turned inward) deformity is manageable with physical therapy and support for spontaneous resolution?

Answer 47

if the foot is flexible

*if the foot is not flexible, then surgical correction is required.

Question 48

What is the difference between Metatarsus Adductus versus Clubfoot deformity?

Answer 48

Metatarsus Adductus:
-inward-turned forefoot;
-may/may not be flexible.

Clubfoot aka Talipes Equinovarus:
-inward-turned (adducted) forefoot, PLUS varus deformity of the midfoot, calcaneum, and talus.
-Not flexible.
-requires immediate t/t with serial casting, or
-surgical correction within 3-6 months if serial casting is ineffective.

Question 49

A pediatric patient with an ACUTE, PAINLESS, unilateral limp involving the right hip joint, collapse of the capital femoral epiphysis, and mild widening of the medial joint space likely has ______ disease, an idiopathic, progressive, *noninflammatory osteonecrosis of the hip that occurs most commonly in children ages 4 to 10.

*no fever, ESR and CRP normal.

Answer 49

A pediatric patient with a painless limp, unilateral involvement of the right hip joint, collapse of the capital femoral epiphysis, and mild widening of the medial joint space likely has LCP disease, a progressive, noninflammatory syndrome of idiopathic osteonecrosis of the hip that occurs most commonly in children ages 4 to 10.

Question 50

X-rays obtained early in the course of the LCP disease often show no abnormalities. Confirming the diagnosis often requires advanced imaging such as MRI or bone scan.

Question 51

Once the diagnosis of LCP is established, children need to be nonweight bearing and referred to an orthopedic surgeon. Treatment focuses on containing the femoral head within the acetabulum with a variety of approaches that range from splints to surgery.

Question 52

_____ is an acute bone infection that can cause pain and limp with fever, elevated ESR and CRP, and may have unremarkable x-rays.

Answer 52

osteomyelitis.

Question 53

Slipped capital femoral epiphysis is a disorder of the proximal femoral epiphysis growth plate. It results in slippage of the femoral head and is visible on an x-ray. Functionally, it is a Salter-Harris type I fracture and is the most common hip disorder in adolescents. The cause is poorly understood, but it is more common in obese Black boys who are 11 to 15 years old. This patient’s findings and characteristics suggest another disease.

Question 54

Transient synovitis is an idiopathic inflammation of the hip joint. It is the most common cause of limp in young children. The limp is painful though, which is not seen in this patient. Though idiopathic, transient synovitis usually follows a history of trauma or recent upper respiratory tract infection, which is not seen in this patient. Ultrasound will show an effusion.