pediatric rehab 2001-2009

PEDIATRIC REHABSAE Questions 2001-2009

Sunday, January 17, 2010

2001

Copyright © 2001American Academy of Physical Medicine and RehabilitationChicago, Illinois


9. The most useful clinical criterion to distinguish Becker muscular dystrophy from Duchenne

muscular dystrophy is

(a) creatine kinase values at the time of diagnosis.(b) walking ability during the teen-age years.(c) Gowersʼ sign and calf enlargement.(d) age at onset of diagnosis


9. (b) The most useful clinical criterion to distinguish Becker muscular dystrophy (BMD) from Duchenne

muscular dystrophy (DMD) is the continued ability of the patient to walk into late teen-age years.Persons with BMD will typically remain ambulatory beyond 16 years. Outlier DMD casesgenerally stop ambulating between 13 and 16 years of age. Creatine kinase values cannot be usedto differentiate DMD from BMD. Calf enlargement and the presence of Gowers’ sign are anonspecific findings. Studies have shown significant overlap in the observed age at onset betweenDMD and BMD.


19. The leading cause of childhood disability is(a) traumatic brain injury.(b) spinal muscular atrophy.(c) spina bifida.(d) cerebral palsy.


19. (d) Cerebral palsy is the leading cause of childhood disability. The reported incidence is approximately 2-3 per 1,000 live births.

The incidence of spina bifida is 0.5 per 1,000

spinal muscular atrophy 1 in 25,000.

The annual incidence for traumatic brain injury in children is 1-2 per 1,000. However,the great majority of cases are minor and result in no long-term disability. Approximately 15% ofbrain-injured children have moderate and severe injuries resulting in permanent impairment.


29. The earliest weakness seen in skeletal muscle in Duchenne muscular dystrophy is located in

(a) knee extensors.(b) hip flexors.(c) neck flexors.(d) ankle plantar flexors.


29. (c) Neck flexor weakness occurs during preschool years. Weakness is generalized but is predominantly

proximal early in the disease course.

Pelvic girdle weakness precedes shoulder girdle weakness byseveral years. Weakness progresses steadily. Quantitative strength testing is more sensitive thanmanual muscle testing.


39. The most common complication after amputation in the immature child is

(a) phantom limb pain.(b) diffuse edema.(c) terminal overgrowth.(d) painful neuroma.


39. (c) Terminal overgrowth at the transected end of a long bone is the most common complication after

amputation in the skeletally immature child. It occurs most frequently in the humerus, fibula, tibia,and femur, in that order. The oppositional growth may be so vigorous that the bone pierces theskin. The treatment of choice is surgical revision.


49. In children hospitalized with acute burns, early management should include

(a) avoidance of sedation.(b) positioning for comfort to reduce severe pain.(c) use of a pressure garment over areas of full-thickness burns.(d) administration of narcotics and anesthetic agents.


49. (d) Although opiates should be considered the most important part of acute pain management

nonopiates should be used when possible. As the needs become more chronic, other agents shouldbe instituted to minimize the problems seen with opiates. Behavioral management and relaxationtherapy should also be used when possible.

Typically, the position of comfort for a burned child isthe position that promotes deformity and, therefore, should be avoided.

Garments are fitted later inthe course of treatment.


59. In considering selection of a lower limb prosthesis for a child with a congenital transfemoral amputation, a knee joint should be included

(a) at initial fitting.

(b) between 3 to 5 years of age.

(c) when sports activities are anticipated.

(d) when the child pulls to stand.


59. (b) The lower limb deficient child should be fitted with a prosthesis when he or she is ready to pull up to a standing position, usually between 9 and 12 months.

A knee joint is added between 3 and 5 years.


69. The earliest marker of abnormal central nervous system maturation is

(a) diffuse fasciculations.

(b) gross motor delay.

(c) delay of postural responses.

(d) persistence of primitive reflexes.


69. (d) In neonates and young infants, motor behavior is influenced by primitive reflexes because of the immature central nervous system. These reflexes gradually become suppressed. Concurrently, more sophisticated postural responses emerge.

Obligatory persistent primitive reflexes are the earliest markers of abnormal neurologic maturation.


79. A parent of an 18-month-old child reports that the child babbled as an infant but became much quieter after about 8 months of age. She has no true words, though she will wave bye-bye. She follows no verbal commands but will follow occasional pantomime commands. Her gross and fine motor skills have been normal. The most likely diagnosis is

(a) autism.

(b) mental retardation.

(c) hearing impairment.

(d) oral motor apraxia.


79. (c) A history of delay in communication development raises several diagnostic possibilities, including true language dysfunction or a motor dysfunction or significant hearing loss. Infants with hearing loss start to fall behind after 6-8 months of age, when learning of auditory-dependent vocalization begins. Oral motor dysfunction is often associated with cerebral palsy, most often spastic quadriparesis. Difficulty with drinking from a cup and difficulty with the introduction of solid food are early symptoms of oral motor dysfunction. Autism is a spectrum disorder with qualitative abnormalities in communication and in social and behavioral realms.

Pantomime = conveying a story by bodily movements only


99. The most severe form of mental retardation in cerebral palsy occurs in association with

(a) spastic diplegia.

(b) ataxia.

(c) spastic quadriplegia.

(d) athetosis.


99. (c) Mental retardation is the most common serious associated disability in cerebral palsy. The overall incidence of mental retardation is approximately 30%-50%. Severe mental retardation is present in about one-half of the retarded group. Approximately one-third of cases have mild cognitive deficits. The greatest retardation is seen in rigid, atonic, and severe spastic quadriplegic cerebral palsy.

Remember: cerebral palsy is the number one cause of childhood disability


Also:

Spastic diplegic cerebral palsy occurs most commonly in premature infants who have had an intraventricular hemorrhage during the neonatal period.

Intrauterine stroke causes hemiplegia.

Neonatal hyperbilirubinemia most commonly causes athetosis.

Birth asphyxia is more commonly associated with spastic quadriplegic cerebral palsy.


139. Following severe traumatic brain injury, neuroendocrine dysfunction can result in diabetes insipidus, SIADH (syndrome of inappropriate antidiuretic hormone), and cerebral salt wasting.

Cerebral salt wasting is manifested by

(a) hypernatremia.

(b) low serum osmolality.

(c) high urine output.

(d) dehydration.


139. (d)

Diabetes insipidus is characterized by excessive FREE water loss, and therefore affected patients experience hypernatremia, dehydration, polyuria, and polydipsia.

SIADH is associated with decreased urine output, hyponatremia (from dilution), and a decreased serum osmolarity.

Cerebral salt wasting is a result of a neural effect on the renal tubules, causing loss of sodium>water and resulting in hyponatremia (not as much as SIADH) and dehydration.


149. In traumatic brain injury in children, outcome is primarily related to

(a) severity of original injury.

(b) location of injury.

(c) age at time of injury.

(d) associated injuries.


149. (a) Although there is considerable variability from case to case, outcome is primarily related to the severity of the original injury.


END OF 2001



2002



9. Which reflex is typically NOT seen in a normal 4-month-old infant?

(a) Extremities extend on the face side as the head is turned to the side.(b) Fingers flex when the palm is touched.(c) Extremities extend to the direction of displacement when center of gravity is displaced.(d) Shoulder abduction, and shoulder, elbow, and finger extension occur when the neck issuddenly extended.


9. (c) These options all describe reflexes. (a) asymmetric tonic neck reflex(b) palmar grasp(d) Moro are seen until a baby is about 6 months old. Protective extension or parachute reaction (c) does not appear until after 6 months.


29. Which statement is true regarding spinal cord injury without obvious radiologic abnormality in children?

(a) It most commonly occurs in lumbar rather than cervical injuries.(b) There is a lower incidence in younger children.(c) It is associated with larger head size and relatively weak neck muscles.(d) Neurologic impairment, if it occurs, is usually apparent within 2 to 4 hours post-injury.


29. (c) Spinal cord injury without obvious radiologic abnormality (SCIWORA) usually occurs in young

children, is thought to be due to the relatively large head size and weak neck muscles, and motorabnormalities may not be apparent for up to several days. SCIWORA most commonly occurs inthe cervical region.


39. A 3-year-old child has a high thoracic spinal cord injury. When he reaches the age 10 years, which complication is the child most likely to have?

(a) Isolated lumbar lordosis(b) Thoracolumbar scoliosis(c) Deep venous thrombosis(d) Heterotopic ossification


39. (b) Scoliosis requiring surgery is a common complication seen in children who have had an spinal cord injury (SCI) at a young age. Increased lordosis in the absence of scoliosis is rarely seen. Deep venous thrombosis rarely occurs in young children and when it does occur it usually occurs soon after the SCI.

Heterotopic ossification tends to occur soon after the SCI


49. Acquired subluxation or dislocation of the hips in spastic cerebral palsy is usually due to muscular imbalance and pull of the

(a) hip flexors and tensor fascia lata.(b) hip flexors and hip adductors.(c) rectus femoris and hip abductors.(d) tensor fascia lata and hip extensors.


49. (b) Strong hip flexor and adductor muscles can overpower weak extensors and abductors.

Acquired hip dislocation can be prevented in some cases by release of spastic hip flexors and adductors.


59. Your 15-year-old patient with Duchenne muscular dystrophy complains of new onset morning

headaches. What is the most likely cause?

(a) Neck extensor tightness(b) Hypercarbia(c) Migraines(d) Vision changes


59. (b) Migraines do not typically occur only in the morning. Neck extensor tightness usually occurs

before the loss of ambulation in boys with Duchenne muscular dystrophy, which usually occursbefore the age of 15 years. Vision changes usually do not cause morning headaches.

Hypercarbia results from hypoventilation during sleep and is an early sign of impending respiratory failure.

Note: You could treat the hypercarbia by getting patient a CPAP


69. Which insult is the most likely cause of spastic diplegic cerebral palsy?

(a) Intrauterine stroke(b) Hyperbilirubinemia in the neonatal period(c) Postnatal intraventricular hemorrhage(d) Perinatal asphyxia


69. (c) Spastic diplegic cerebral palsy occurs most commonly in premature infants who have had an intraventricular hemorrhage during the neonatal period.

Intrauterine stroke causes hemiplegia.

Neonatal hyperbilirubinemia most commonly causes athetosis.

Birth asphyxia is more commonly associated with spastic quadriplegic cerebral palsy.


79. The best predictor of community ambulation beyond childhood in patients with myelomeningocele is

(a) body mass index.

(b) quadriceps strength.

(c) early surgical closure of the meningocele.

(d) bowel and bladder continence.


79. (b) There are many studies about longterm outcomes of ambulation in children and adults with myelomeningocele. While many factors influence outcome, including intelligence, medical problems, and obesity, the best predictor of ambulation into adulthood is strong quadriceps function.

Bowel and bladder continence has no relationship to ambulation.


99. A child with C5 ASIA A spinal cord injury should eventually become independent in which activity?

(a) Intermittent catheterization

(b) Transfer to level surfaces

(c) Feeding

(d) Bathing


99. (c) A child with C5 ASIA A spinal cord injury should eventually become independent in feeding, and in upper extremity dressing with assistive devices, in driving a power wheelchair, and in propelling a manual wheelchair short distances on level surfaces


109. A 6-month-old child presents in your office for rehabilitation assessment. She was born at full term. There was mild transient respiratory distress at birth. The patient was noted to be diffusely hypotonic at birth except for normal cranial nerves. There were no feeding issues once the respiratory distress resolved within 24 hours. The baby has remained relatively hypotonic since birth. However, she has become very socially alert and aware and attempts to use her arms to reach for toys and pick up lightweight objects. She doesnʼt roll. She cannot sit except very briefly when propped and bearing weight through both arms with elbows extended. On examination, head circumference is normal, length is normal, as is weight. There is a pronounced head lag. Arms, while in the supine position, maintain a “jug-handle” posture. Reflexes are present but diminished. There is no spasticity. The cranial nerves are normal except for fine fasciculations of the tongue.

The most likely diagnosis is(a) myotonic muscular dystrophy.(b) cerebral palsy.(c) infantile botulism.(d) spinal muscular atrophy.


109. (d) Spinal muscular atrophy (SMA) is a term used to describe a group of inherited disorders characterized by weakness and muscle wasting due to degeneration of anterior horn cells of the spinal cord and brainstem motor nuclei. Three subtypes of autosomal recessive predominantly proximal SMA have been linked to chromosome 5q. The majority of cases of SMA type I present within the first 2 months of life with generalized hypotonia and symmetric weakness. Childrentypically sit only with support. Tongue fasciculations have been reported in 56%-61% of patients. Proximal muscles are weaker than distal.

Type I SMA: • aka Werdnig-Hoffman Disease and Infantile spinal muscular atrophy• the most lethal form


129. Secondary injury in pediatric brain trauma is caused by

(a) hypotension, hypoxia, and hydrocephalus.

(b) growing skull fractures.

(c) coup and contrecoup cerebral contusions.

(d) diffuse axonal injuries and punctate hemorrhages.


129. (a) Any disorder that interferes with cerebral perfusion or oxygenation can cause further damage following traumatic brain injury.

This includes • Hypotension• Hypoxia• Increased intracranial pressure because of cerebral edema, acute hydrocephalus, or space-occupying lesions.

Midline shift or herniation may lead to infarction because of pressure or traction on cerebral vessels. Therefore, efforts are made to control intracranial pressure through fluid and electrolyte management, hyperventilation, and maintenance of normal blood pressure and oxygenation.

Growing skull fractures result from the arachnoid protruding through a dural tear, producing a cyst that can contribute to a widening skull deficit, which usually requires operative repair. This is a complication of traumatic brain injury but not a secondary injury.

Coup and contrecoup cerebral contusions and diffuse axonal injuries are examples of primary injury.


139. To prevent contractures, which position is the correct placement for children with major burns?

(a) Shoulder in external rotation

(b) Wrist in extension

(c) Hip in flexion

(d) Metacarpophalangeal joints in hyperextension


139. (b)

Children with major burn injuries should be placed in positions that tend to prevent contractures.

These include:

•neck extension (no pillows)•shoulders at 90° abduction and neutral rotation with •elbows, wrists, hips, and knees extended•feet at neutral dorsiflexion, •metacarpophalangeal joints at 70° to 90° flexion•finger interphalangeal joints in full extension.


149. Which finding would indicate a poor long-term outcome in a 9-year-old child with a severe traumatic brain injury?

(a) Bladder and bowel incontinence

(b) Agitation

(c) Dysphagia

(d) Hypertension and hyperpyrexia


149. (d) Most children with severe traumatic brain injury have dysphagia, incontinence, and agitation at some time during the recovery period.

Central autonomic dysfunction (hypertension, hyperpyrexia, sweating, tachypnea, and rigidity) is associated with worse cognitive and motor outcomes a year or more after injury


END OF 2002



2003



9. In children with spastic cerebral palsy, which intervention strengthens weak muscles?

(a) Ankle-foot orthotics

(b) Tendon transfer surgery

(c) Intrathecal baclofen

(d) Functional training program


9. (d) Children with cerebral palsy often have weakness as part of their disorder. Treatments such as bracing, tendon lengthening or transfers, and medications such as botulinum toxin or intrathecal baclofen add to this weakness.

Strengthening programs or functional training programs can help to strengthen weak muscles

Note: tendon transfer surgery restores motion/function, but does NOT “strengthen weak muscles”


19. One of your 4-year-old patients exhibits the following characteristics: distress over minor changes in environment, echolalia, lack of awareness of the existence of feelings in others, nonparticipation in simple games. The most likely diagnosis is

(a) autism.

(b) cerebral palsy.

(c) hearing impairment.

(d) mental retardation


19. (a) Autism is characterized by echolalia, inability to play reciprocally, and abnormal relationships with people. While children with mental retardation, cerebral palsy, and hearing impairment may have some of these features, they do not have all of them in the absence of autism.

Echolalia = automatic repetition of vocalizations made by another person


29. Which finding is normal in newborn infants?

(a) Extensor tone predominates

(b) Hands are kept fisted

(c) Spine is straight when held in sitting position

(d) Unable to turn head to side in prone position


29. (b) In normal newborn infants flexor tone predominates and hands are kept fisted. In prone position a normal newborn is able to turn the head to either side. The newborn has a rounded spine when placed in supported sitting


39. The family of your 10-year-old patient who had a severe traumatic brain injury 6 weeks ago asks you if they may feed their son. You observe that he is agitated at times, has a hoarse voice, and drools. You try to feed him applesauce and notice that he seems to swallow part of it and does not cough. The most likely finding on the videofluoroscopic feeding study will be

(a) Silent aspiration.

(b) Reflux.

(c) Coughing and gagging.

(d) Normal swallow.


39. (a) The lack of coughing in a patient with neurologic impairment when he/she is presented with food may mean a normal swallow, but is more likely to mean silent aspiration. A normal videofluoroscopic swallowing study is unlikely in a patient with a traumatic brain injury (TBI) who is drooling and hoarse. Hoarseness may be a sign of reflux, but in a child with a TBI it is more likely to mean vocal cord abnormality


49. Which measure is the first sign of respiratory muscle dysfunction in boys with Duchenne muscular dystrophy?

(a) Vital capacity

(b) Oxygen saturation

(c) Maximal expiratory force

(d) Negative inspiratory force


49. (c) Recent studies by McDonald and by Bach showed that reduction of maximal expiratory force (MEF) to 40%–60% of normal in the 7- to 14-year-old age group was the first sign of respiratory muscle dysfunction in boys with Duchenne muscular dystrophy (DMD). The earlier and more severe decreases of MEF that are greater than the decreases in maximal inspiratory force, correspond to the clinically observed weakness of abdominal muscles, which like coughing are important in forced expiration. Vital capacity was not found to decrease until an average of 15–16 years. Low oxygen saturation is a late manifestation in DMD, developing after hypercapnia.


59. A 10-year-old child with L4-5 myelodysplasia and shunted hydrocephalus develops spasticity in her legs. The most likely cause of this spasticity is

(a) shunt malfunction.

(b) symptomatic Chiari malformation.

(c) growth.

(d) tethered cord.


59. (d)

Tethered cord is the most common cause of new onset spasticity in patients with myelodysplasia. Linear growth does not cause new spasticity.

Symptoms of Chiari malformation include cranial nerve disorders and respiratory problems.

Shunt malfunction may be associated with headaches, vomiting, eye muscle abnormalities, and sometimes abdominal symptoms.


69. A 9-year-old girl with an L1 ASIA class A spinal cord injury that occurred at age 5 years presents inyour office with a 1-day history of a swollen left leg. History is that she woke up with the swollenleg the day before. There is no history of trauma, fever, or shortness of breath. On examination, youfind a prepubertal girl in no distress with normal vital signs. Upper extremities are normal. Lowerextremities have moderate spasticity and no voluntary movement. Skin is normal. The left leg iswarm and swollen from the ankle to the knee. There is no sensation in the legs. Which test is mostlikely to yield the correct diagnosis?

(a) Bone scan(b) Plain radiograph(c) Venous Doppler study(d) White blood cell count with differential


69. (b) Deep venous thromboses (DVTs) which can be diagnosed by Doppler study usually occur in the first 3 months after spinal cord injury (SCI) and are rare in prepubertal children. In lower leg DVTs the foot and leg are usually swollen (NOT warm).

Heterotopic ossification (HO), which can be detected by bonescan, occurs in about 3% of children with SCI and has onset an average of 14 months after injury. Heterotopic ossification most commonly involves the hip.

Cellulitis is usually associated with skin lesions and usually involves a discrete area.

A fracture is the most likely cause of swelling in this case and can be diagnosed by plain radiographs.


79. Which positive effect of ankle-foot orthotics has been proven beneficial in the treatment of children with cerebral palsy?

(a) Improved gait efficiency as measured by gait analysis(b) Prevention of contractures(c) Improved knee extensor strength(d) Decreased plantar flexor posture


79. (a) There are no large, randomized, controlled studies that show the long-term effects of any type of Ankle-Foot Orthosis (AFO) on function or contracture formation. Small studies have shown that both rigid and hinged AFOs improve gait efficiency by preventing plantar flexion.


99. Juvenile rheumatoid arthritis (JRA) differs from adult onset rheumatoid arthritis: in JRA

(a) joint destruction occurs earlier.(b) large joint involvement is less frequent.(c) the cervical spine is involved less frequently.(d) systemic features are more common.


99. (d) Children with juvenile rheumatoid arthritis are more likely to have:• systemic features (fever, rash, swollen lymph nodes)• large joints involved• cervical spine involvement.

Adults with rheumatoid arthritis have joint destruction earlier.


109. Children with which physical disorder tend to have higher verbal skills compared to overall cognitive ability?

(a) Muscular dystrophy(b) Myelodysplasia(c) Cerebral palsy(d) Autism


109. (b) Children with myelodysplasia (spina bifida) have deceptively good verbal facility that creates the impression of higher intellectual functioning than is found on formal testing (“cocktail party syndrome”).

Children with cerebral palsy, autism, and muscular dystrophy do not typically demonstrate this finding.


129. Your 10-year old patient with T6 ASIA class B paraplegia complains of right knee pain. On

examination there is no swelling of the knee or leg. Knee examination is normal. The right legappears shorter when the hips and knees are flexed. What is the most likely cause of these findings?

(a) Knee sprain(b) Right hip subluxation(c) Hip adductor spasticity(d) Dysesthetic pain


129. (b) Hip subluxation is the most likely cause of knee pain in a child with T6 ASIA B SCI. Pain from hip pathology is often referred to the knee in children.

While hip adductor spasticity contributes to subluxation or dislocation (spastic hip adductors and hip flexors), the spasticity itself is not painful.

Dysesthetic pain is usually generalized.

An abnormal knee examination is usually found in a knee injury that causes pain.


149. A 16-year-old girl who had a severe traumatic brain injury 4 weeks ago with left frontal contusion and left basilar skull fracture demonstrates worse auditory than visual attention. Which diagnostic

test would be most likely to explain this finding?

(a) Magnetic resonance imaging(b) Electroencephalogram(c) Audiogram(d) Visual evoked response


149. (c) A basilar skull fracture is often associated with a permanent sensorineural hearing loss. Other

traumatic brain injuries may be associated with hearing loss, but this is less common. All patientswith basilar skull fracture should be assumed to have a hearing loss and have audiologicalevaluation as soon as possible.


Note: • includes fractures to the base of the skull, whether posterior AND anterior (temporal bone, occipital bone, sphenoid bone, and/or ethmoid bone)• Can cause tearing of meninges --> drain of CSF into middle ear (and out thru perforated drum), eustachian tube (salty taste) and out nose

Basilar Skull Fractures

Battle sign

Raccoon eyes


159. What condition causes the typical “myopathic gait” seen in a young boy with Duchenne muscular

dystrophy with accentuated lumbar lordosis and toe walking?

(a) Hip and knee extensor weakness(b) Hip flexion and ankle plantar flexion contractures(c) Hip extensor weakness and plantar flexion contracture(d) Hip flexion contracture and knee extensor weakness


159. (a) The typical “myopathic gait” seen in early Duchenne muscular dystrophy is caused by weakness of

the gluteus maximus and quadriceps muscles. In order to maintain upright posture the childassumes the hyperlordotic stance. Contractures of the gastrocsoleus and iliopsoas muscles occurlater in the disorder.


169. In which type of cerebral palsy is a seizure disorder most commonly seen?

(a) Tetraplegia(b) Diplegia(c) Athetosis(d) Hemiplegia


169. (d) Approx. 70% of children with hemiplegia have seizures. About 50% of children with tetraplegic cerebral palsy have seizures. Seizures are rare in children with diplegia or athetosis.

Remember: Cerebral palsies• Intrauterine stroke --> hemiplegic• Intraventricular hemorrhage --> spastic diplegic• Neonatal hyperbilirubinemia --> athetosis• Birth asphyxia --> spastic quadriplegic


179. A 1-year-old child with a midlength transfemoral limb deficiency presents for prosthetic

management. Which component should be in the prosthetic prescription?

(a) Socket with a growth liner(b) Single action knee joint(c) Vertical shock pylon(d) Dynamic foot


179. (a) The 1-year-old child should be fit with a simple prosthesis which suspends securely and allows for growth.

The knee joint should be added between 3 and 5 years.

The SACH foot is most commonly prescribed because of its simple design and durability. SACH = solid ankle cushion heel

All prostheses for growing children should incorporate a removable growth liner in the socket.


END OF 2003



2004



9. A 2-year-old patient with spinal muscular atrophy type 2 (intermediate form) presents with a 25°,

C-shaped scoliosis. What is the best treatment option at this time?

(a) Muscle strengthening(b) Electrical stimulation(c) Spinal fusion(d) Spinal orthosis


9. (d) Muscle strengthening will not reduce the curve or prevent it from progressing and is not easily

accomplished in 2-year-old children. Posterior or anterior spinal fusion is not indicated with acurve of this size and is to be avoided in a young child if at all possible. Spinal orthotics are used inyoung children with spinal muscular atrophy to improve sitting balance and to attempt to halt curveprogression.


19. The most common spinal problem seen with achondroplasia during childhood is

(a) kyphosis.(b) scoliosis.(c) spinal stenosis.(d) low back pain.


19. (a) While scoliosis may occur in children with achondroplasia, it is less common than kyphosis, which begins in infancy.

Spinal stenosis occurs frequently in individuals with achondroplasia, with 38 years being the average age of symptom onset.

Low back pain is extremely frequent in adults with achondroplasia, but rare in children.

Progressive kyphosis that occurs in infants and young children with achondroplasia is treated with a spinal orthosis


39. In which activity should a 16-year-old girl with C5 ASIA class A spinal cord injury be independent with the use of assistive devices?

(a) Self catheterization(b) Transfers to level surfaces(c) Self feeding(d) Bathing


39. (c) While boys with C5 spinal cord injury (SCI) may learn to perform bladder self-catheterization with

assistive devices, girls do not.

Level transfers require active elbow and wrist extension, whichwould not be present in a person with C5 SCI.

Self-feeding with assistive devices such as a palmar band can usually be done by persons with C5 tetraplegia.


49. Your 6-month-old patient had burns to his head and both arms in a house fire. What approximate percent of his total body surface area (TBSA) was burned?

(a) 37(b) 18(c) 27(d) 49


49. (a) An infantʼs head is approximately 19% and each arm constitutes 9% of the total body surface area

(TBSA). In adults and older children the head is approximately 9% of the TBSA.


59. Which joints are most commonly involved in juvenile rheumatoid arthritis?

(a) Shoulder, hip, fingers(b) Atlantoaxial, costomanubrum, hip(c) Sternomanubrum, shoulder, sacroiliac(d) Elbow, hip, temporomandibular


59. (d) The elbow is involved 90% of the time in juvenile rheumatoid arthritis (JRA), the

temporomandibular and hip 50% each. The shoulder is involved about 8% of the time in early JRAand about 33% later.


69. Which of the following is part of neurodevelopmental therapy (NDT)?

(a) Promotion of primitive reflexes(b) Use of taping and icing(c) Strengthening exercises(d) Facilitating automatic reactions


69. (d) Neurodevelopmental therapy, developed by Bobath, emphasizes inhibition of reflex patterns,

normalizing tone, and facilitating automatic reactions. The therapy does not include strengthening exercises.


79. Your 14-year-old patient with spastic diplegic cerebral palsy has increasing problems with

spasticity. He walks with ankle-foot orthoses (AFOs) and crutches and is independent in hisactivities of daily living. Which medication would reduce his spasticity while minimizingundesirable side effects?

(a) Diazepam (Valium)(b) Baclofen (Lioresal)(c) Dantrolene (Dantrium)(d) Oxybutynin (Ditropan)


79. (b) Diazepam has lethargy and sleepiness as major side effects. Dantrolene works at the level of the

muscle and often causes weakness, which can interfere with function. Oxybutynin relaxes themuscles of the bladder, not skeletal muscles.


109. You are asked to evaluate an 8-month-old child with developmental delay. On exam you find low

tone, but brisk deep tendon reflexes at the knees and biceps, full passive range of motion, and poorhead and trunk control. This childʼs diagnosis is likely

(a) myotonic dystrophy.(b) cerebral palsy.(c) spinal muscular atrophy.(d) Hunterʼs syndrome.


109. (b) This patient presents with hypotonia, weakness, and hyperreflexia, a combination most commonly

seen in central nervous system lesions such as cerebral palsy. A child with a neuromusculardisorder would not have hyperreflexia with the hypotonia and weakness.

In severe cerebral palsy it is common to see early hypotonia with brisk reflexes that changes to hypertonia as the child gets older.


129. Which endocrine abnormality is most likely to occur 5 years after severe traumatic brain injury in a 2-year-old girl?

(a) Diabetes insipidus(b) Precocious puberty(c) Hypothyroidism(d) Hyperparathyroidism


129. (b) Precocious puberty occurs in up to 50% of girls who sustain a severe traumatic brain injury (TBI) in

early childhood.

Diabetes insipidus is an early complication of TBI. While other endocrineabnormalities may occur, they are less common.


169. On physical examination an 8-year-old patient stands on his toes and has increased lumbar

lordosis. He has a Trendelenburg gait with circumduction. What else would you expect to find onhis exam?

(a) Decreased sensation in his feet(b) Anterior tibialis weakness(c) Quadriceps weakness(d) Hyperreflexia at the ankle


169. (c) The exam describes typical findings in a boy with myopathy such as Duchenne muscular dystrophy

(DMD). The earliest weakness in DMD is proximally in the gluteus maximus. The boy assumes aposture of lumbar lordosis to place the center of gravity posterior to the hip joint to preventhyperflexion of the hip and thus a fall.

Toe walking is a compensatory adaptation to knee extensor weakness.


189. You are asked to evaluate a child who was born at 25 weeks gestation and had a grade 4 intraventricular hemorrhage. What type of cerebral palsy are you most likely to find?

(a) Athetoid(b) Hemiplegic(c) Diplegic(d) Hypotonic


189. (c) Grade 4 intraventricular hemorrhages in premature infants are most commonly associated with spastic diplegia.

Remember: Cerebral palsies• Intrauterine stroke --> hemiplegic• Intraventricular hemorrhage --> spastic diplegic• Neonatal hyperbilirubinemia --> athetosis• Birth asphyxia --> spastic quadriplegic


199. You are asked to evaluate a child with arthrogryposis and equinovarus. Which treatment strategy would be the best to employ?

(a) Short leg braces attached to orthopedic shoes(b) Botulinum toxin injections, stretching, and plastic ankle-foot orthoses(c) Oral baclofen (Lioresal), range of motion, and extra depth shoes(d) Casting followed by surgical releases


199. (d) Arthrogryposis multiplex congenita (AMC) may be caused by a variety of conditions, but is usually not accompanied by spasticity. Thus botulinum toxin and baclofen are not indicated. Most club

feet (equinovarus) in patients with AMC are resistant to conservative methods and require surgical releases.

arthro = jointsgryposis = twisted

http://www.pediatric-orthopedics.com/Topics/Muscle/Diseases/Arthrogryposis_Multiplex/

arthrogryposis_multiplex.html


http://www.pediatric-orthopedics.com/Topics/Muscle/Diseases/Arthrogryposis_Multiplex/arthrogryposis_multiplex.html






END OF 2004



2005



19. The Gross Motor Functional Measure (GMFM) is designed to measure

(a) motor changes over time.(b) quality of motor performance.(c) achievement of motor milestones.(d) only walking, running, and jumping ability.


19. (a) The Gross Motor Functional Measure (GMFM) evaluates motor changes over time in children with cerebral palsy. It includes activities in prone and supine positions, rolling, sitting, crawling, kneeling, standing, walking, running, and jumping.

It does NOT measure the quality of motor performance.


49. What percentage of American children with myelomeningocele requires a shunt to manage hydrocephalus?

(a) 10–20(b) 25–50(c) 60–70(d) 80–90


49. (d) Seventy-five (75%) percent of lesions in spina bifida cystica (myelomeningocele) affect the lumbosacral spine.

Ninety percent (90%)of children with spina bifida have hydrocephalus that requires a shunt for management.


69. A 9-year-old girl with C5 ASIA A spinal cord injury sustained 2 years ago is evaluated for upper extremity splinting. Which statement regarding this scenario is TRUE?

(a) A resting hand splint should be prescribed for daytime use to preserve function.(b) A wrist extension splint would be contraindicated for daytime use because it would interferewith function.(c) A mobile arm support or balanced forearm orthosis could be prescribed to make self-feeding possible.(d) A short hand splint should be prescribed to strengthen wrist extensor muscles.


69. (c) A mobile arm support or balanced forearm orthosis would enable the child with C5 ASIA A spinal cord injury (SCI) and weak arm muscles to move the arm through useful active range of motion and to position the hand for function. In patients with C5 SCI, these orthoses are typically combined with wrist extension splints or a universal or palmar cuff for feeding.

A resting hand splint may be used at night to improve or maintain range of motion, but would interfere with daytime function.

A short hand splint would not strengthen wrist extensor muscles, but may be useful to improve function.


99. A normal 6-month-old infant may demonstrate which reflex?

(a) Rooting(b) Automatic walking(c) Plantar grasp(d) Posterior protective extension


99. (c) Rooting and automatic walking reflexes are present at birth, and are integrated by 4 months of age.

Posterior protective extension does not appear until 7 to 8 months of age. Plantar grasp is present at birth and not integrated until after independent walking occurs at approximately 12 months of age.

Reflex Age of Emergence Age of Suppression (or Integration)Moro Birth 4–6 monthsRooting Birth 4 monthsAsymmetric tonic neckreflex (ATNR) 1–3 months 6–7 monthsPlantar grasp Birth 12–14 months–ie, when walking wellAutomatic walking Birth 3–4 monthsPosterior protectiveExtension 7–8 months


109. Which statement is TRUE about swallowing in infants?

(a) Sucking and swallowing are well-coordinated for oral intake by 34 weeks of gestation.(b) The infantʼs larynx is low, about the level of the sixth cervical vertebra.(c) The infantʼs tongue fills less of the oral cavity than the adultʼs tongue.(d) Oral breathing occurs at birth and may interfere with sucking.


109. (a) Sucking and swallowing are well-coordinated at 34 weeksʼ gestation.

Because the infantʼs tongue is more anterior than the adultʼs, the tongue fills more of the oral cavity than in the adult.

The larynx in the infant is high, about at the C2–3 level.

Newborn infants are obligate nose breathers and oral breathing is not observed until 3 to 4 months of age.


129. You are performing a consult on an 8-year-old child who has sustained a traumatic brain injury.

The child has hyperthermia, hypertension, tachycardia, and rigidity. The best management for thischild would be

(a) Propranolol (Inderal).(b) Baclofen (Lioresal).(c) Nonsteroidal anti-inflammatory drugs (NSAIDs).(d) Amantadine (Symmetrel).


129. (a) Fever in a child with a severe traumatic brain injury should be investigated and infections treated with appropriate antibiotics. In the absence of infection, the fever in central autonomic dysfunction is poorly responsive to nonsteroidal anti-inflammatory drugs. Baclofen may help to control the

spasticity, but propranolol is more effective in controlling the hypertension, tachycardia, and hyperthermia.

Remember: Central autonomic dysfunction (hypertension, hyperpyrexia, sweating, tachypnea, and rigidity) is associated with worse cognitive and motor outcomes a year or more after injury


149. The Education for All Handicapped Children Act (EHA, PL 94-142), passed in 1976, and the Individuals with Disabilities Education Act (IDEA, PL 105-17), passed in 1997, guarantee that children with disabilities have

(a) education in special schools.(b) medical care at school.(c) education in the least restrictive environment.(d) education in regular classrooms.


149. (c) The Education for All Handicapped Children Act and the Individuals with Disabilities Education

Act guarantee children with disabilities education in the least restrictive environment. They alsoguarantee necessary health care be provided in the school environment (eg, intermittentcatheterization) but do NOT require medical care be provided.


169. Once an individual becomes board-certified in the specialty of physical medicine and rehabilitation,

he/she must continue to fulfill certain requirements in order to maintain certification status. Whichaction is NOT a requirement for maintenance of certification?

(a) Continuing medical education credits(b) Maintenance of active medical licensure(c) Completion of a recertification examination every 10 years(d) Publication of at least 1 article in a scientific journal every 10 years


169. (d) Once an individual becomes board certified in the specialty of physical medicine and rehabilitation,

he/she must continue to fulfill certain requirements in order to maintain their certification status.Publication of 1 article in a peer-reviewed journal every 10 years is not a requirement formaintenance of certification. All of the other options listed are required.


189. You have just finished admitting a 60-year-old diabetic man who has recently undergone a right below-knee amputation. The patient's son stops you in the hallway and inquires about his father's health status and prognosis for walking again. You have never met the patient's son before, and before answering the questions, you would first

(a) further review the patient's medical record and determine his cardiac status.(b) perform a literature review of outcomes research in individuals with below-knee amputations.(c) ask the patient for permission to discuss his health status with his son.(d) ask the son if the patient has a living will or a health care power-of-attorney.


189. (c) Maintaining confidentiality of patient information is important even when discussing health

information with family members. Before discussing the patient's health status with his son, theappropriate first step would be to ask the patient for permission. The other options listed would notbe appropriate initial management strategies.


END OF 2005



2006



19. A 10-year-old girl presents with scoliosis 5 years after sustaining a severe traumatic brain injury.

Radiographic studies reveal a 25° levoconvex curve from C8 to T12 with the apex at T4. Afterconsultation with the orthopedic surgeon, you prescribe a spinal orthosis. Which type of orthosisshould be used in this patient?

(a) Cervicothoracolumbosacral orthosis (CTLSO)(b) Thoracolumbosacral orthosis (TLSO)(c) Thermoplastic Minerva body jacket (TMBJ)(d) Sterno-occipital mandibular orthosis (SOMI)


19. (a) A thoracolumbosacral orthosis is used for scoliosis having an apex at T9 or lower. A sternooccipital mandibular orthosis immobilizes the neck. A thermoplastic Minerva body jacket is also used for cervical immobilization. A cervicothoracolumbosacral orthosis such as the Milwaukeebrace extends from the pelvic section to the neck ring and has been shown to correct scoliotic curves throughout that area.


Minerva body jacket

Sterno-occipital mandibular


59. Randomized controlled trials examining intrathecal baclofen (ITB) use in children with cerebral palsy show that children who receive ITB have

(a) improved upper extremity function.(b) reduced spasticity in lower extremities.(c) improved walking and transfers.(d) improved knee range of motion.


59. (b) A comprehensive review of published English language studies on intrathecal baclofen (ITB)

showed evidence of statistically significant improvement in upper AND lower extremity tone with ITB use in children with cerebral palsy (CP).

Other reported improvements with ITB in childrenwith CP are either anecdotal or not substantiated by randomized controlled trials.


69. You are called to see your 3-year-old inpatient with a C5 ASIA class A spinal cord injury. She has a headache and complains of not feeling well. Vital signs are pulse 60, respirations 20, blood pressure 120/80. Weight 33 lbs (15kg). Physical examination is unchanged from previously. You order:

(a) Place the patient in the supine position.(b) Administer acetaminophen (Tylenol) orally.(c) Empty the bladder.(d) Obtain computed tomography of the head.


69. (c) The child is experiencing autonomic dysreflexia. The 90th percentile for blood pressure in an average sized 3-year-old girl is 103/62. A child with C5 tetraplegia would be expected to have even lower average blood pressure.

Initial treatment consists of positioning the patient in anupright position and emptying the bladder. If this does not correct the problem, medications should be considered. If medications are needed, either nitropaste 2% or nifedipine may be used. For achild weighing 15kg the correct initial dose is 0.25 to 0.5 mg/kg/dose (3.75–7mg) of nifedipine or1/2 inch of nitropaste.


89. The most common congenital limb deficiency is a

(a) transverse tibial and fibular limb deficiency (below-knee limb deletion).(b) transverse transmetacarpal limb deficiency (partial hand deletion).(c) longitudinal fibular deficiency (fibular hemimelia).(d) transverse radial limb deficiency (below-elbow limb deletion).


89. (d) The left short transradial congenital limb deficiency (below-elbow limb deletion) is the most

common congenital limb deficiency.

It is thought to be caused by a clot which occludes the artery,resulting in resorption of the distal limb, often leaving nubbins of fingers at the end of the stump.


109. Disorders of executive functioning are common in children after severe traumatic brain injury.

Which sign indicates problems of executive function?

(a) Low intelligence quotient(b) Attention and memory problems(c) Aphasia(d) Agitation


109. (b) Problems of executive function include impairments in attention, memory, and abstract reasoning.

While aphasia and low intelligence may be seen following traumatic brain injury (TBI), they are not problems of executive function. Agitation is usually seen early in recovery from TBI, at the Rancho Los Amigos stage 4. The full consequences of a TBI that occurs in a young child may notbe seen until much later, at an age when the child is expected to have that skill. For example,problems in abstract reasoning in a child who had a TBI at age 5 may not be seen until the childreaches 9 or 10 years of age.


129. What is a reasonable long-term rehabilitation goal for a 6-year-old child with a C6 ASIA class A

spinal cord injury?(a) Independent lower extremity dressing(b) Bed mobility(c) Independent bathing(d) Independent feeding


129. (d) A child with C6 ASIA class A spinal cord injury would be expected ultimately to independently

self-feed, but not bathe, do lower extremity dressing, or perform bed mobility.

Note:A C6 pt would have forearm extensors (C6) for tenodesis action to grasp a utensil, and biceps/deltoid fxn to bring the food to the mouth.


149. A 6-month-old infant presents to you with hypotonia. You perform an electrodiagnostic study which

shows normal motor conduction velocity, normal sensory conduction velocity and amplitude, normalmotor units, and occasional fibrillations and positive waves. The most likely cause of these findingsis(a) congenital myotonic dystrophy.(b) spinal muscular atrophy.(c) metachromatic leukodystrophy.(d) infantile botulism.


149. (a) Hypotonia in infants can be caused by many abnormalities, including cerebral lesions, spinal cord

pathology, polyneuropathies, and myopathies. These electrodiagnostic findings are most consistentwith congenital myotonic dystrophy.

Side note:Operations and anaesthetics can be risky. It is very important that any surgeon and anesthesiologist should know a child has congenital myotonic dystrophy before surgery is planned.


169. Which action is NOT required of a certified physiatrist to maintain certification?

(a) Obtain continuing medical education credits.(b) Maintain active medical license.(c) Complete a recertification examination every 10 years.(d) Publish at least 1 article in a scientific journal every 10 years.


169. (d) Once a physician is certified by the American Board of Physical Medicine and Rehabilitation, he or

she must continue to fulfill certain requirements in order to maintain certification status.Publication of an article in a peer-reviewed journal every 10 years is not a requirement formaintenance of certification. All the other options listed are required.


179. As the medical director of an inpatient rehabilitation program, you become concerned because you

have recently noticed an increased number of urinary tract infections in the patients on your service.Which action would NOT be considered a reasonable initial management strategy?

(a) Discuss the issue with the rehabilitation centerʼs Quality Improvement Committee andexamine the rate of urinary tract infections over the past year.(b) Perform a literature review examining the incidence and prevalence of urinary tract infectionsin an inpatient rehabilitation setting.(c) Immediately order that a urine culture be obtained on every patient at the time of admission tothe rehabilitation service.(d) Provide an educational inservice to the nursing staff regarding catheter and bladdermanagement.


179. (c) Continuous quality improvement should be a part of each physician's clinical practice. All the

options listed would be appropriate to consider with the exception of immediately ordering a urineculture on every patient at the time of admission to the rehabilitation service. This would not be anappropriate option without gathering more information and understanding the implications of thisintervention strategy.


189. You have just finished admitting a 60-year-old man with diabetes who has recently undergone a

right below-knee amputation. The patient's son stops you in the hallway and inquires about hisfather's health status and prognosis for walking again. You have never met the patient's son before,and before answering the questions, you would first

(a) further review the patient's medical record and determine the patient's cardiac status.(b) perform a literature review of outcomes research in individuals with below-the-kneeamputations.(c) ask the patient for permission to discuss his health status with his son.(d) ask the son if the patient has a living will or a health care power-of-attorney.


189. (c) Maintaining confidentiality of patient information is important even when discussing health

information with family members. Before discussing the patient's health status with his son, theappropriate first step would be to ask the patient for permission. The other options listed would notbe appropriate initial management strategies.


199. A clinical trial can best be defined as a(a) retrospective study examining the natural history of a disease process.(b) prospective study that is randomized and double-blinded.(c) retrospective study with subjects selected on the basis of presence or absence of an illness.(d) prospective study comparing the effect of an intervention with a control.


199. (d) A clinical trial can best be defined as a prospective study that compares the effect and value of an

intervention with a control. A study measuring the natural history of a disease process is moreobservational in nature and can be either prospective or retrospective. Clinical trials are notnecessarily randomized or double-blinded. Clinical trials are prospective and not retrospective innature.


END OF 2006



2007



9. According to national databases of spinal cord injury (SCI), children under the age of 6 years are more likely to have which epidemiologic pattern of spinal cord injury?

(a) high tetraplegia, motor incomplete, occurred in motor vehicle accident (b) paraplegia, complete, occurred in motor vehicle accident (c) high tetraplegia, complete, caused by medical/surgical complications (d) paraplegia, motor incomplete, caused by medical/surgical complications


9. (b) According to databases of the Model SCI Systems and Shriner’s Hospital for Children, children under age 6 years are more likely than teenagers to sustain SCI in a motor vehicle accident. Their injuries are more likely to be T1 and lower, and they are more likely to have complete injuries.


49. Your 5-year-old patient with spastic tetraplegic cerebral palsy needs a wheelchair prescription. He is dependent for transfers, but cognitively normal. He is able to feed himself and uses a communication device. His family transports him in their car in an adapted car seat. On examination, he is unable to sit unsupported, but sits well with minimal support; he has no scoliosis, and his passive range of motion is full. Which elements would be best to include in his wheelchair prescription?

(a) Folding frame, sling seating (b) Adaptive stroller, linear seating (c) Tilt in space frame, custom seating (d) Rigid frame, contoured seating


49. (d) While this child is totally dependent for transfers, he only requires minimal support to sit upright and has no fixed deformities.

Custom seating should be used for those with fixed deformities.

A tilt-in-space frame should be used when children need to have their position in space changed frequently because of deformities or medical problems.

While it is tempting to prescribe a wheelchair with a folding frame for a family who transports a child in a car rather than a van, the child will be better positioned using contoured seating and a rigid frame. At age 5 years, the size of frame needed will be able to be transported in a car even without folding.

Adaptive strollers usually position the child in a reclined position and should be used as a backup to a wheelchair, which is not easily transported in an automobile, or for a child who can walk but periodically needs dependent mobility for fatigue or following seizures or for similar reasons.


99. On examination, a 3-month-old girl still has a Moro reflex, asymmetric tonic neck reflex, and plantar grasp reflex. She does not have any protective extension. You advise her parents that

(a) further diagnostic evaluation is indicated. (b) she requires a physical therapy evaluation. (c) she needs a neurology evaluation. (d) these reflexes are normal reflexes.


99. (d) These are normal reflexes in a 3-month-old child. The Moro and asymmetric tonic neck reflexes (ATNR) usually are integrated by approximately 6 months. The plantar grasp reflex is integrated by 12 to 14 months after walking has begun. Protective extension in sitting is seen anteriorly at 5 to 7 months, lateral at 6 to 8 months, and posterior at 7 to 8 months.


109. Which of the following is NOT a feature of central autonomic dysfunction in traumatic brain injury in children?

(a) Hypertension (b) Tachypnea (c) Rigidity (d) Hypothermia


109. (d) Central autonomic dysfunction occurs in some children following severe brain injury. It is characterized by hypertension, hyperpyrexia, rigidity, tachypnea, tachycardia, and diaphoresis. Various medications are used to treat this dysfunction, but no studies prove the value of one medication over another.

Remember: Pts with this condition after TBI have worse cognitive and motor outcomes at 1 yr

after injury.


139. A 2-month-old infant presents to you for evaluation of delayed development. He was the product of a normal term pregnancy, labor, and delivery. Birth weight was 3500 grams. He has had difficulty feeding since birth. Family history is negative for developmental problems. On physical examination, he is awake, but not alert. Weight is 3600 grams. Respiration is unlabored. He has poor head control and decreased tone throughout. Deep tendon reflexes are absent. What is the most likely diagnosis?

(a) Kugelberg Welander syndrome (b) Duchenne muscular dystrophy (c) Infantile botulism (d) Tetraplegic cerebral palsy


139. (d) This patient illustrates the diagnostic dilemma of the floppy infant. Causes of this problem include central nervous system lesions (both brain and spinal cord), myopathies, neuropathies, and neuromuscular junction problems.

This infant has had abnormalities since birth, which argues against infantile botulism.

Kugelberg Welander syndrome (also known as spinal muscular atrophy type 3) has onset during childhood, as does Duchenne muscular dystrophy.

Tetraplegic cerebral palsy often presents in infancy with floppiness and hyporeflexia, which later change to spasticity and hyperreflexia.


149. The most common musculoskeletal abnormalities seen in a child with L5 myelodysplasia with sparing of the L5 segment and above are

(a) cavus foot, early hip dislocation, hip and knee flexion contractures. (b) calcaneus foot, late hip dislocation, hip and knee flexion contractures. (c) cavus foot, late hip dislocation, hip adduction contractures. (d) calcaneus foot, early hip dislocation, hip adduction contractures.


149. (b) The child with L5 myelodysplasia typically has late hip dislocation, calcaneus foot, hip flexion contractures, and may have either knee extension or flexion contractures, depending on whether quadriceps (L2-4) or hamstrings (L4-S1) are stronger. Gluteus medius (hip abductor, L4-S1) and hip adductors (L1-3) are innervated higher than L5 and are typically balanced in L5 myelodysplasia. Late hip dislocation is due to either unbalanced hip musculature or spinal deformities.


169. An unusually high incidence of pressure ulcers is noted on your inpatient rehabilitation unit. As the medical director for the unit, you decide to implement a quality improvement process. The next best step in process would be to

(a) understand the cause of the skin breakdown. (b) select a strategy to decrease the incidence of pressure ulcers. (c) organize a team to investigate the problem. (d) reprimand the nurse manager for the unit.


169. (c) Various strategies can be used when implementing a quality improvement process. One widely accepted method is FOCUS PDCA. The steps in the process include finding an opportunity, organizing the team, clarifying the current process, understanding the causes of the variation, and selecting a strategy to implement it (FOCUS). Once this has been accomplished, then the strategy involves planning, doing, checking, and acting (PDCA). According to this process, the next best step in the scenario provided would be to organize a team to investigate the problem.


179. Preventable medical errors are

(a) rarely associated with significant morbidity. (b) most commonly a result of individual human error. (c) associated with no impact on patient satisfaction. (d) associated with patients’ loss of trust in the health care system.


179. (d) Preventable medical errors can result in lower levels of patient satisfaction and loss of trust in the health care system. Preventable medical errors often result in significant morbidity and even mortality. Estimates are that 44,000 to 98,000 people die each year as a result of medical errors that could have been prevented. These errors are frequently the result of system type errors rather than individual human error.


199. Investigators must address ethical considerations when designing and implementing research studies. One such consideration requires investigators to design protocols that will provide generalizable knowledge and ensure that the benefits of the research are proportionate to the risks assumed by the subjects. This ethical consideration is referred to as

(a) respect. (b) beneficence. (c) justice. (d) autonomy.


199. (b) Beneficence requires investigators to design protocols that will provide generalizable knowledge and ensure that the benefits of the research are proportionate to the risks assumed by the subjects.


END OF 2007



2008



9. You are asked to evaluate a 1-year-old patient who is not yet walking but is developmentally appropriate. Which reflex would you expect to find?

(a) Asymmetric tonic neck (b) Symmetric tonic neck (c) Palmar grasp (d) Plantar grasp


9. (d) The asymmetric tonic neck reflex (ATNR) and symmetric tonic neck reflex (STNR) are usually integrated by 6 to 7 months. Palmar grasp disappears by 5 to 6 months.

Plantar grasp is integrated when walking is achieved. The normal age of walking varies, but may be as late as 18 months.


59. Acquired subluxation or dislocation of the hips in spastic cerebral palsy is usually due to muscular imbalance with excessive tone of which muscles?

(a) Hip flexors and tensor fascia lata (b) Hip flexors and hip adductors (c) Rectus femoris and hip abductors (d) Tensor fascia lata and hip extensors


59. (b) Progressive changes associated with hip subluxation in patients with cerebral palsy result from the effects of neuromuscular imbalance on the growth and development of the hip joint. The primary problem is spasticity and muscular imbalance, and the musculoskeletal manifestations are secondary. Soft tissue abnormalities include a muscular imbalance between the stronger flexors and adductors, and the weaker extensors and abductors. A flexion-adduction contracture also shifts the center of rotation of the hip from the femoral head to the lesser trochanter, and the proximal femur is gradually displaced upward and outward.


69. In children with spastic cerebral palsy, which approach strengthens weak muscles?

(a) Ankle-foot orthotics (b) Tendon transfer surgery (c) Intrathecal baclofen (d) Functional training


69. (d) Children with cerebral palsy often have weakness as part of their disorder. Treatments such as bracing, tendon lengthening or transfers, and medications such as botulinum toxin or intrathecal baclofen add to this weakness. Strengthening programs or functional training programs can help to strengthen weak muscles.


79. The family of your 15-year-old patient who had a severe traumatic brain injury 6 weeks ago asks you if they may feed their son. You observe that the patient is agitated at times, has a hoarse voice, and drools. You try to feed him applesauce and notice that he seems to swallow part of it and does not cough. The most likely finding on the videofluoroscopic swallowing study will be

(a) Silent aspiration (b) Reflux (c) Coughing and gagging (d) Normal swallow


79. (a) The lack of coughing in a patient with neurologic impairment when presented with food may mean a normal swallow, but is more likely to mean silent aspiration. A normal videofluoroscopic swallowing study is unlikely in a patient with a TBI who is drooling and hoarse. Hoarseness may be a sign of reflux, but in a child with a TBI is more likely to mean vocal cord abnormality.


139. A 3-year-old child has a high thoracic spinal cord injury. At age 10, which of the following is the most likely complication?

(a) Severe lordosis without scoliosis (b) Scoliosis requiring surgical treatment (c) Deep venous thrombosis (d) Heterotopic ossification


139. (b) Children who sustain cervical or high thoracic spinal cord injuries at an early age are at high risk of developing progressive scoliosis that requires surgical management.


149. The most common congenital limb deficiency is

(a) right transtibial limb deletion. (b) right transradial limb deletion. (c) left transtibial limb deletion. (d) left transradial limb deletion.


149. (d) The most common congenital limb deficiency is the left midlength transradial deficiency.

Remember:It is thought to be caused by a clot which occludes the artery, resulting in resorption of the distal limb, often leaving nubbins of fingers at the end of the stump.


169. Relevant to investigational studies, the principle of justice requires that

(a) harm be minimized and benefits maximized. (b) informed consent includes full disclosure of risks and benefits. (c) research subjects be treated fairly and selected equitably. (d) investigators respect the privacy of research subjects.


169. (c) The principle of justice requires subjects to be treated fairly and selected equitably.

The principle of beneficence ensures that researchers minimize harm and maximize benefits associated with research involvement.

The principle of autonomy ensures that informed consent includes full disclosure of risks and benefits and that the privacy of subjects is respected.

Remember:Social justice was about being “fair” with the allocation of health care resources


179. The process of developing and adopting quality standards for clinical practice

(a) is dependent on providers being mandated for reporting data. (b) is ineffective in changing physician practice patterns. (c) allows health insurance providers to deny reimbursement for

care. (d) helps to form expectations for safety among both providers

and consumers.


179. (d) The process of developing and adopting quality standards for clinical practice helps to form expectations for safety among both providers and consumers.


END OF 2008



2009



59. As compared to children with severe traumatic brain injuries, children with severe anoxic encephalopathy are more likely to have

(a) rigidity and decreased rate of regaining consciousness. (b) rigidity and increased rate of regaining consciousness. (c) hypotonia and decreased rate of regaining consciousness. (d) hypotonia and increased rate of regaining consciousness.


Answer: A Commentary: Compared to children with severe traumatic brain injury, children with severe anoxic encephalopathy are less likely to regain consciousness; they also have shorter survival time, and often have profound rigidity.

Note:Think about the spasticity from the anoxic injury that causes spastic tetraplegic cerebral palsy


79. Which maternal factor is associated with an increased risk of spina bifida?

(a) Anticonvulsant medications during pregnancy (b) Upper socioeconomic class (c) Alcohol ingestion during pregnancy (d) Folic acid 4mg/day prior to and during pregnancy


Answer: A Commentary: The etiology of spina bifida is multifactorial. Both polygenic inheritance and environmental influences contribute. Several studies have shown that the incidence of spina bifida is reduced if food is fortified with folic acid or if mothers take folic acid prior to conception and during pregnancy. Recommended doses of folic acid are 0.4 mg/day in women who are not at high risk and 4 mg /day in women at high risk (eg, those with a family history of spina bifida). Some studies have also implicated lower socioeconomic class and in utero exposure to anticonvulsant medications as being risk factors. Maternal alcohol ingestion is not related to an increased risk of spina bifida in a baby.


99. Children with L4-5 spina bifida are most likely to have

(a) equinus foot. (b) cavus foot. (c) knee flexion contractures. (d) knee extension contractures.


Answer: D Commentary: The knee extensors (quadriceps) are innervated at the L3-4 level, while the knee flexors (hamstrings) are innervated at the L5-S1 level. A child with L4-5 preserved level would have quadriceps muscles that work, while hamstrings will either be weak or absent. Foot muscles are innervated at the L5-S2 levels.

Equinus and cavus feet result from asymmetric pull of foot muscles, which would be seen in sacral levels of spina bifida.

So:Knee extensors = L3-4Knee flexors = L5-S1


109. A 6-month-old child with L4 spina bifida presents to your clinic. He also has shunted hydrocephalus. Other than repair of his back and shunt placement, his past medical history has been negative. On examination, you find that he has full hip flexion against gravity and knee extension strength is at least 4/5. The infant has no movement around the ankle. Feet are in neutral position. Hip examination is symmetric. Which prediction is most accurate in this patient?

(a) The child is likely to be a functional community ambulator by age 5 years. (b) The child is likely to be only a household ambulator. (c) The child is likely to learn to crutch walk by 18 months. (d) The child is likely to be only a wheelchair user.


Answer: A Commentary: This child has a strong quadriceps muscle and no deformities noted at 6 months of age. He is reported to be healthy. The best early predictor of ambulation in children with spina bifida is a strong quadriceps muscle. Negative predictors are spine and lower extremity deformities and obesity. Children do not typically learn to use crutches until 3 to 5 years of age or older.


119. Which statement is true about the asymmetric tonic neck reflex (ATNR) or the symmetric tonic neck reflex (STNR)?

(a) The STNR is present at birth and fades away by 1 year. (b) The STNR provides postural stability as the child goes from crawling to

standing. (c) The ATNR appears about 6 months of age and fades away by 1 year. (d) The ATNR is obligatory in all children at certain ages.


Answer: B Commentary: The ATNR is also known as the fencer position and is a neonatal reflex that disappears by 6 months of age. It is never obligatory in normal children. The STNR appears about 6 months and disappears by 1 year. It provides postural stability as the child makes the transition from crawling to standing.

Asymmetric tonic neck reflex


The transition up into a crawling position is assisted by the emergence of the STNR which enables extension of the arms and flexion of the legs at the same time. However, the STNR has to be 'switched off' before the baby can crawl forward as this involves a combination of flexion and extension - e.g. in a cross-pattern crawl the right arm and left leg flex while the left arm and right leg extend.


129. Your 3-year-old patient with cerebral palsy has a Gross Motor Function Classification System (GMFCS) Level IV. Family is asking you what to expect she will be able to do when she is a teenager. Which activity is the highest level she is most likely to attain as a teenager?

(a) Independent ambulation in the household (b) Independent ambulation in the community (c) No independent mobility in the household (d) Wheelchair use in the community


Answer: D Commentary: The Gross Motor Function Classification System (GMFCS) classifies mobility of people with cerebral palsy from I to V. Level I is independent ambulation indoors and outdoors with no assistive device. Level IV requires wheelchair for household and community mobility.

Remember:The Gross Motor Functional Measure (GMFM) evaluates motor changes over time in children with cerebral palsy. It includes activities in prone and supine positions, rolling, sitting, crawling, kneeling, standing, walking, running, and jumping.


159. In terms of continuous quality improvement, a sentinel event is defined as

(a) a benchmark event that sets the standard for patient care. (b) an occurrence that requires dismissal of personnel. (c) a single occurrence that is highly problematic or socially

unacceptable. (d) an event that results in the opening of a new hospital

program.


Answer: C Commentary: In terms of continuous quality improvement, a sentinel event is defined as a single occurrence that is highly problematic or socially unacceptable. Sentinel events will typically trigger an in-depth root cause analysis to determine the cause of the event as well as potential solutions. The focus of these investigations is to evaluate the processes and systems that are in place rather than to focus blame on individual practitioners.


189. What is the primary advantage of a body powered upper limb prosthesis compared to a myoelectric prosthesis?

(a) Greater sensory feedback (b) Moderate or no harnessing (c) Less body movement to operate (d) Enhanced cosmesis


Answer: A Commentary: The advantages of body powered upper limb prostheses include the following factors: moderate cost, most durability, highest sensory feedback, and a variety of prehensors available for various activities.

Their disadvantages are that they require the most body movement to operate, have the most harnessing and require increased energy expenditure to use.

Myoelectric and/or switch controlled upper limb prostheses have the following advantages: they require moderate to no harnessing, require fewer body movements to operate, have moderate cosmesis, provide more function in proximal areas and, in some cases, provide a stronger grasp.

Battery powered prostheses are the heaviest and most expensive prostheses. They also require the most maintenance, provide limited sensory feedback and require extended therapy time.


END OF 2009



2010



pediatric rehab 2001-2009

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