Practice Questions Flashcards
What secondary condition requires the most medical management in adults with cerebral palsy? (a) Constipation (b) Pain (c) Cardiovascular disease (d) Osteoporosis
Answer: (b) Comments: In adults with cerebral palsy (CP) The incidence of pain is high (67%-82%) across all disability groups and appears to increase with age. It is multifactorial with musculoskeletal issues being of primary concern. Constipation issues persist into adulthood and adjustments to the bowel program may be needed. Adults with CP are not reported to have a higher risk of cardiovascular disease but are less likely to be screened than the general population. Osteoporosis is most common (up to 50% in some studies) in non-ambulatory persons, (Gross Motor Function Classification System, GMFCS levels 4, 5), and it increases with age.
What is the strongest single predictor of mortality in adults with pediatric onset disabilities? (a) Feeding problems (b) Presence of epilepsy (c) Inability to walk (d) Intellectual disability
Answer: (d) Comment: Feeding problems, epilepsy and inability to walk are conditions associated with pediatric mortality, but intellectual disability is the strongest single predictor of mortality in adults with pediatric onset disabilities. Intellectual disability affects a person’s ability to manage health care monitoring, exercise programs, nutrition, housing, and sexuality. These items are likely to be more closely monitored by parents and pediatricians for a child than by community workers and adult physicians caring for adults with pediatric onset disabilities.
What is the biggest barrier for participation in outdoor activities for children with physical disabilities? (a) Lack of an adult assistant (b) Rejection by physically able children (c) Need for aids such as braces or wheelchairs (d) Inaccessible playgrounds
Answer: (d) Comment: The environment is a significant barrier to play for children with physical disabilities. Fencing, sand, and inaccessible playground equipment relegate children with disabilities to an observational role and indicates they are not valued/welcomed. Younger children may see an adult assistant as a playmate but older children see them as intrusive and a hindrance in social situations. Physically able children are willing to include children with disabilities in their games but readily acknowledge limitations of playgrounds in accommodating them. Children often view their braces, walkers and wheelchairs as extensions of themselves and helpful in play situations.
Botulinum toxin injections into the hip adductors, hamstrings and gastrocsoleus in children with spastic cerebral palsy are shown to (a) have greater effect in children older than 10 years. (b) be better than serial casting in management of spastic equinus. (c) be ineffective in management of spastic equinus gait. (d) delay progression of hip displacement
Answer: (d) Commentary: Younger children with fewer physical limitations have more potential for improvement than older children with more physical limitations. Serial casting and botulinum toxin injections appear to have similar benefit. Spastic equinus gait is effectively improved with botulinum toxin injections into gastrocsoleus and hamstrings. Hip displacement can be delayed with botulinum toxin injections into hip adductors and hamstrings but does not affect long-term outcome. 2012
Which antispasticity drug used to treat a 3-year-old child with cerebral palsy binds to GABA receptors in the spinal cord to inhibit reflexes that lead to increased tone? (a) Clonidine (b) Tizanidine (c) Dantrolene (d) Baclofen
Answer: (d) Commentary: Baclofen binds to GABA receptors in the spinal cord to inhibit the reflexes that lead to increased tone. Clonidine is an alpha 2 agonist, as is tizanidine. Dantrolene works in the striated muscle at the level of the sarcoplasmic reticulum. All these drugs have pediatric application. Baclofen can be used beginning at age 2 years.
Pathological drooling in children with spastic quadriparetic cerebral palsy is (a) due to excessive saliva production. (b) unsightly, but has no medical significance. (c) associated with inefficient, uncoordinated swallowing. (d) associated with increased dental caries.
Answer: (c) Commentary: Pathological drooling is the unintentional loss of saliva either anteriorly over the lips or posteriorly over the back of the tongue. It is associated with an inefficient, uncoordinated swallow. Anterior drooling is normal in infants up to 18 months of age. Recent studies have shown that salivary production is similar to that of typical children without cerebral palsy. Medical complications of pathological drooling include chronic aspiration, pulmonary infections and skin irritation. Saliva is protective of dentition. 2011
A 6-year-old girl with Erb palsy since birth has an internal rotation deformity of her right shoulder. Her shoulder external range of motion, whether passive or active, is zero degrees. Right elbow, forearm, wrist, and hand function is good, latissimus dorsi strength is normal. Shoulder MRI shows glenoid dysplasia, but no shoulder dislocation. She writes with her right hand but is unable to reach the back of her head to fix her hair on the right. What is the most appropriate management by the physiatrist at this time? (a) Aggressive stretching of right shoulder internal rotators and strengthening of external rotators (b) Evaluation for compensatory strategies and assistive devices for independence in activities of daily living (c) Consultation with orthopedic surgeon (d) Observation until adolescence for anticipated further improvement
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When should one be concerned when observing a child with an asymmetric tonic neck reflex (ATNR, fencer position) with neck rotation, relative extension of the limbs on the chin side and flexion of the limbs on the occiput side? (a) In a 3-month-old infant who is able to move out of the fencer position (b) In a 5-month-old infant who is unable to move out of the fencer position (c) In a 6-month-old infant whose leg response to the stimulus is greater than the arm (d) In an infant of any age, since the ATNR is a primitive pathological reflex.
Answer: (b) Commentary: The ATNR is typically present at birth and integrates between 4 and 6 months of age. An obligatory fencer position is abnormal at any age. A persistent or obligatory ATNR may be an early clue that a child has a disorder of motor control, most often cerebral palsy. 2010
A 1-year-old boy presents with marked weakness. Parents report a weak cry and cough since birth, and the child cannot sit independently. Exam findings include a bell-shaped thorax, hypotonia, some movement of the hands and feet but minimal movement at the hips and shoulders, and there are tongue fasciculations. The diagnosis is best confirmed by (a) genetic analysis. (b) muscle biopsy. (c) electromyography. (d) repetitive nerve stimulation
Answer: (a) Commentary: This child’s presentation is typical for spinal muscular atrophy (SMA) type I, also known as Werdnig-Hoffmann disease. SMA is the second most common neuromuscular disease of childhood, occurring with an incidence of 1:6,000. Deletion of the survival motor neuron gene leads to degeneration of anterior horn cells and can be detected in over 90% of children with SMA. Prior to availability of genetic diagnosis, EMG and muscle biopsies were utilized. Repetitive nerve stimulation has been used in the investigation of botulism and congenital myasthenia gravis but is not helpful in SMA. 2010
A 14-year-old girl with spastic quadriplegic cerebral palsy (CP) has driven a power wheelchair as her primary means of mobility since age 4 years. She recently sustained a fracture of her tibia with no known significant trauma. You anticipate that the bone mineral density (BMD) z score for her distal femur as measured on a dual-energy x-ray absorptiometry (DEXA) scan will be (a) increased relative to peers without spasticity. (b) increased relative to younger nonambulatory children with CP. (c) similar to younger ambulatory children with CP. (d) decreased relative to age-matched peers without CP.
Answer: (d) Commentary: Children with severe CP develop clinically significant osteopenia. Lower BMD z scores are associated with greater severity of CP as indicated by gross motor function, and these scores decrease with age. Rather than occurring primarily from actual losses in bone mineral, as in aging adults, the decreasing BMD z scores seen in older youths with CP occur because they have a slower rate of growth in bone mineral, relative to their healthy peers. 2010
You suspect that a 10-month-old boy’s subdural hematoma may be the result of child abuse. Which action is LEAST likely to be helpful in further evaluation? (a) Skeletal survey (b) Physical examination for cutaneous injuries (c) Hematology consultation (d) Ophthalmology consultation
Answer: (c) Commentary: The most common cause of serious brain injury in children younger than 1 year of age is abuse. In very young children subdural hematoma, subarachnoid hemorrhage, retinal hemorrhages and associated cutaneous, skeletal and visceral injuries are significantly more common among those with inflicted brain injury than in children with unintentional injury. 2010
Which ethnicity has the highest prevalence of neural tube defects?(a) Eastern European(b) Asian(c) African American(d) Hispanic
Answer: (d)Commentary: Even though neural tube defects have declined across all ethnicities, because of increased folate intake, the disparity between Hispanics and other ethnicities remains. While 50%-70% of neural tube defects can be prevented by adequate folic acid, genetic influences remain. 2012
What neurological level of spina bifida is associated with active plantar flexion, cavus foot deformities and neurogenic bowel and bladder? (a) thoracic (T2-12) (b) upper lumbar (L1-3) (c) lower lumbar (L4-5) (d) sacral (S1-2)
Answer: (d)Commentary: Bowel and bladder involvement is common at all levels, even in sacral lesions.Plantar flexion and inversion causes development of the cavus foot in sacral lesions. Lowerlumbar lesions develop unopposed ankle dorsiflexion leading to a calcaneous foot. Upper lumbarand thoracic lesions develop ankle plantar flexion contractures due to the inability to move theankle at all. 2012
Which pulmonary function parameters would you expect to increase in a child with advancedneuromuscular disease? (a) Total lung capacity (b) Vital capacity (c) Residual volume (d) Expiratory reserve volume
Answer:(c)Commentary: Patients with neuromuscular diseases (NMDs) demonstrate a restrictive patternwhen fractional lung volumes are measured. There is a reduction in total lung capacity, vitalcapacity, and the expiratory reserve volume. In contrast, residual volume (the volume of air thatremains in the lungs after a maximal, complete expiratory maneuver) can actually be elevatedwhen the respiratory muscles are too weak to deform the chest wall inward to deflate the lungsfully. These patterns will be exaggerated in children with NMDs who also develop scoliosis.Forced expiratory flows are typically reduced in proportion to lung volume so that the ratio of theforced expiratory volume in the first second (FEV1) to FVC is normal or high. 2012
A 3-year-old boy with upper lumbar spina bifida has bilaterally dislocated hips on x-ray during aroutine follow up visit. You advise his parents that he(a) needs surgery to relocate both his hips.(b) should have at least one hip relocated, surgically.(c) can still stand and walk despite the hip dislocations.(d) will not be able to sit comfortably in a wheelchair.
Answer: (c)Commentary: Due to the imbalance between hip flexors/adductors and hip extensor/abductors,hip dislocation occurs in 30%-36% of children with upper lumbar spina bifida. Unilateral hipdislocations tend to cause pelvic obliquity, difficulty with wheelchair positioning and standing,and surgery is advocated. However, bilateral hip dislocations do not generally require surgicalintervention, because a level pelvis and good range of motion are more important for ambulationthan located hips. 2012
Aside from motor vehicle crashes (MVC), which etiology is the most common cause of spinal cord injury in children younger than 5 years? A) Medical/surgical complications. B) Violence C) Sports D) Falls
A) medical/surgical complications. Motor vehicle crashes are the most common cause of spinal cord injury (SCI) in all pediatric age groups, accounting for 65% of SCI in children younger than 5 years. The second most common cause in of SCI in this age group is medical/surgical complications, accounting for 11.6% of spinal cord injuries, while the rate among older pediatric patients (age 16-21 years) is only 0.6%. More common in the older age groups are violence, sports injuries, and falls. For example, among children younger than 5 years, 8.7% experience SCI from violence, while 21.6% in the 16- to 21-year range fall victim to SCI for that reason. Sports account for 0.2 % of SCI in children below age 5, while the percentage in youngsters between the ages of 16 and 21 is 18.3%. Falls account for 6.5% of SCI in children younger than 5 years; 8.1 % of spinal cord injuries in the 16- to 21-year age group are attributed to falls, and 24% of adults over age 22 sustained their spinal cord injury in a fall. Because both physiology and behavior change with age in the pediatric population, the pediatric-onset SCI population exhibits distinct epidemiologic characteristics among different age groups and when compared to the adult-onset SCI population. 2014
The mother of a 7-year-old ambulatory child with cerebral palsy (CP) has just visited with the pediatric orthopedic surgeon. He has suggested a single-event multilevel surgery (SEMLS) for her child. This surgery would consist of tendon Achilles lengthenings (TAL), femoral varus derotation osteotomies (VDO) and rectus femoris transfers. She is worried that this is too much to do at once and wants your opinion. Your advice is that? A) he should have serial Tendo Achilles lengthenings (TAL) surgeries, not a single-event multilevel surgery B) femoral varus derotation osteotomies (VDO) should not be performed until after puberty C) single event mulitlevel surgery (SEMLS) is the most helpful orthopedic procedure for ambulatory children with CP older than 6 years D) split posterior tibial tendon transfers would be the surgery of choice in this case.
C is correct Multiple procedures during a single surgical event is shown to have less morbidity and better long-term outcomes than single procedures done over multiple, separate, surgical events often spanning many years. Single-event multilevel surgery (SEMLS) is defined as 2 or more bony or soft tissue procedures at 2 or more anatomic levels performed during a single surgical procedure. Multiple muscles and joints are targeted when function is the primary goal, because all these structures are interrelated for walking. 2014
A pattern of burns on the feet, buttocks and perineum of a 1 year old child is suggestive of a. sunburn b. playing with matches c. a house fire d. non-accidental trauma
d is correct. Non-accidental trauma accounts for an estimated 10% to 20% of pediatric burns and is more common in children younger than 2 years. Children with nonaccidental trauma are less likely to attend outpatient therapy and follow-up clinics 2014.
