Pediatric neuromuscular disease

1. What is pseudohypertrophy and where is it seen?
2. What is Gower's sign?
3. What gait patterns are seen in neuromuscular disease?
4. What is the genetics behind Duchenne's muscular dystrophy?

Answers:
1. Seen in Duchenne's and Becker's MD, caused by increase in fat and connective tissue.
2. From proximal weakness of pelvic girdle muscles. Children compensate for hip extension weakness by moving hands up thighs into upright stance.
3. Toe walking/lumbar lordosis from hip extension weakness, Trendelenberg gait, steppage gait.
4. Abnormality of Xp21 gene loci, resulting in dystrophin deficiency.

Treatment of spina bifida

1. At what age is self-independent catheterization achieved?
2. What mental age is required for crutch walking?

Answers:
1. 5-6 years.
2. 2-3 years in a low lumbar, 4-5 years in low thoracic or upper lumbar.

Spina bifida

1. What is spina bifida occulta?
2. What is spina bifida cystica?
3. What is a common complication of myelomeningocele?
4. What is the incidence of urinary incontinence in spina bifida?
5. How does level of lesion correlate with IQ?

Answers:
1. Primarily vertebrae are affected with no herniation through the skin, usually occurring in the lumbosacral or sacral segments. It is not associated with Arnold-Chiari.
2. Designates meningocele, myelomeningocele, and other cystic lesions where the spinal canal contents herniate through the posterior vertebral opening.
3. Arnold-Chiari malformation, 90% of which are complicated by hydrocephalus.
4. 95% have incontinence, hypertonic bladder in thoracic lesions, hypotonic in sacral lesions.
5. Higher lesions result in lower IQ.

Reflexes in babies

1. What is asymmetric tonic neck reflex and when is it suppressed?
2. What is symmetric tonic neck reflex and when is it suppressed?
3. When is the palmar grasp reflex suppressed?
4. When is the plantar grasp reflex suppressed?

Answers:
1. When head turned to side, extremities extend on face side and flex on occiput side. This disappears by 6-7 months.
2. With neck flexion, arms flex and legs extend. With neck extension, arms extend and legs flex. This disappears by 6-7 months.
3. 5-6 months.
4. 12-14 months, when walking.

Cerebral palsy: associated deficits

1. What is an important indicator of whether a child with CP will walk?
2. What is an important indicator of intellectual potential?
3. What is the characteristic hearing loss in CP and in what type of CP is it most commonly seen?
4. What is the incidence of mental retardation in CP and in what types of CP is it most commonly seen?

Answers:
1. Being able to sit independently by age 2.
2. Speaking in 2-3 word sentences by age three.
3. Sensory neural impairment of hearing, most common with athetosis.
4. 50% incidence, most common in rigid atonic and severely spastic quadriplegia.

Cerebral palsy

1. What is the definition of cerebral palsy?
2. What are risk factors for CP?
3. What is the most common cause of CP?
4. What are the symptoms of spastic CP?
5. What is dyskinetic CP? What other deficiency is it associated with?

Answers:
1. CP is the result of a nonprogressive lesion to an immature brain (within first three years of life) and is the leading cause of childhood disability.
2. Prenatal intracranial hemorrhage, placental complications, gestational toxins, teratogens, TORCH infections, congenital brain malformations, maternal causes (seizures, hyperthyroidism), socioeconomic factors, reproductive inefficiency, prenatal hypoxia (from mult gestation, maternal bleeding or drug use).
3. Prematurity (birth < 32 weeks, wt < 2500 gm).
4. Hyperreflexia, clonus, Babinski, persistent primitive reflexes, overflow reflexes.
5. Extrapyramidal movement patterns secondary to abnormal regulation of tone, deficits in postural control, and coordination deficits. It is associated with a high incidence of sensorineural hearing loss.

Pediatrics: TBI

1. What are the leading causes of TBI in the pediatric population?
2. Does the presence of skull fracture indicate the severity of brain injury?
3. How does a Klumpke's palsy occur? What sort of syndrome is associated with it?


Answers:
1. Transportation related is #1, followed by falls, then sports, then assault.
2. No.
3. Violent upward pull on shoulder resulting in damage to C8-T1 cervical roots. Horner's syndrome is associated with Klumpke's palsy, related to injury to the superior cervical sympathetic ganglion.

Pediatric cancer

1. Where is the most common location of pediatric brain tumors?
2. What is Wilm's tumor?
3. What are the two most common types of malignant bone tumors and where are they commonly found?

Answers:
1. Posterior fossa.
2. Wilm's tumor or nephroblastoma develops in the renal parenchyma in children 2-5 years, and may be associated with congenital anomalies.
3. Osteosarcoma most common, followed by Ewing's sarcoma. Osteosarcoma is in the metaphysis of long bones, most commonly in the distal femur. Ewing's arises in long and flat bones, including pelvis, typically in diaphysis.

Pediatric burns

1. What is the rule of 9s in children?
2. What does placing a burn victim in a comfortable position promote?
3. What is the prime location for acute decubiti in children?

Answers:
1. For a child <1 year, 9% is taken from the legs and added to the head. For each subsequent year, 1% is returned to the legs until 9 years old.
2. Contracture.
3. Occiput.

Rheumatic fever and hemophilia

1. What are the major Jones criteria for Rheumatic fever?
2. What are the minor Jones criteria for Rheumatic fever?
3. What is the hallmark of hemophilia?

Answers:
1. Two major or one major and two minor required: Carditis, polyarthritis, chorea, erythema marginatum, subQ nodules.
2. Fever, arthralgia, elev ESR or CRP, prolonged PR interval.
3. Hemarthrosis.

Juvenile RA

1. What is the most common connective tissue disease in children?
2. What eye condition is associated with pauciarticular type I JRA? What percentage of patients develop this?
3. Which JRA subtype has the most severe arthritis?
4. What JRA subtype is associated with pauciarticular type II?

Answers:
1. Juvenile rheumatoid arthritis?
2. Iridocyclitis in 50%.
3. Polyarticular rheumatoid factor positive: severe arthritis in >50%.
4. HLA-B27.

pediatric LE amputation

1. What is the most common congenital lower limb deformity?
2. What is partial proximal femoral focal deficiency?
3. When can a child start using a motorized WC?
4. What is the most common complication after amputation in a child?

Answers:
1. Fibular longitudinal deficiency or fibula hemimelia.
2. Absence of the development of the proximal femur, resulting in stunting or shortening of the entire femur.
3. 5-6 years.
4. Terminal overgrowth.

NOTE

This method of studying is no longer working for me, so from now on, I am only going to focus on book icons. I will do 4-5 book icons per entry.

(I don't know if anyone is reading this blog or cares. If you're reading, please drop a comment so I know you're out there!)

Transhumeral congenital deficiency

1. At what age can a body-powered elbow be used?
*2. What is the Krukenberg procedure?
3. What is the Vilkke procedure?

Answers:
1. 2-3 years.
2. Reconstruction of forearm for children with absent hands by separation of ulna and radius.
3. Attaches a toe to the residual limb.

Congenital limb deficiency

1. When does limb development occur in utero?
2. What is the preferred classification system for limb deficiency?
3. What are five syndromes associated with limb deficiency?
*4. What is the most common congenital limb deficiency?
*5. When should the initial upper extremity prosthetic first be fitted? When should a terminal device first be fitted?

Answers:
1. First trimester, day 26 of gestation till 8 weeks.
2. ISPO, which divides deformities into transverse and longitudinal.
3. TAR syndrome, Fanconi's syndrome, Holt-Oram Syndrome, Baller-Gerold Syndrome, VACTERL.
4. Left terminal transradial.
5. First fitting when child achieves sitting balance at 6-7 months. TD provided at 11-13 months, when child begins to walk and performs simple grasp.

Pediatrics: hip pain

*1. What is the most common hip-related cause of limping and pain in children?
2. How is toxic synovitis of the hip diagnosed? How is it treated?
3. What is Legg-Calve-Perthes disease? What is the treatment and prognosis?
4. What is slipped capital femoral epiphysis? What population is it seen in?
5. What is the treatment of SCFE?

Answers:
1. Toxic synovitis.
2. Diagnosis is with limited IR of hip on exam, increased WBC and ESR, normal X-ray. Treatment is rest and NSAIDs, avoid full activity.
3. Avascular necrosis of the ossification center of the femoral head. Conservative treatment includes rest and abduction brace vs. varus osteotomy. There is a good prognosis if detected early with less than 50% femoral head involvement.
4. SCFE is separation of proximal femoral epiphysis through the growth plate, often seen in obese children, preadolescent/adolescent.
5. Treated with surgical pinning to prevent further epiphyseal displacement.

Pediatrics: elbow and knee injury

1. What is nursemaid's elbow? How is it treated?
2. What is Little Leaguer's elbow and how is treated?
3. What is Osgood-Schlatter's disease? How is it diagnosed and treated?

Answers:
1. Displacement of the radial head and neck distal to the annular ligament, with normal hand function and elbow X-ray. Treatment is by supination of the forearm.
2. Medial epicondylitis from repetitive stress on the apophysis of the medial humeral epicondyle ossification center, usually from baseball throwing. Treatment is rest.
3. Common self-limited disease of anterior tibial tubercle, which results in microfractures in the apophyseal cartilage and pain. X-ray may be normal or reveal fragmentation of ossified portion of the tibial tubercle. Treatment is activity restriction for 4-8 weeks, especially activities requiring deep knee bends.

Congenital torticollis

*1. What is congenital torticollis and what is the most common cause?
2. What does X-ray show in torticollis?
3. What is the treatment?

Answers:
1. Head is tilted laterally toward one shoulder with chin rotated away. Most common cause is fibrosis of the SCM, possibly due to birth trauma and ischemia. Other causes include hemivertebra or atlantoaxial rotary subluxation.
2. Rotation of C1-C2.
3. Stretching exercises. If cervical ROM is regained by one year, facial asymmetry will resolve. If torticollis is persistent or there is a hemivertebra, surgery is needed.

Pediatrics: leg and hip

1. When does genu varus resolve in infants?
*2. What is Blount's disease? What group is it most common in? What is the treatment?
3. What is developmental dysplasia of the hip? How common is it and what factors increase risk?
4. What is Galeazzi's test?
5. What are the Barlow and Ortolani tests?
6. What is the treatment of DDH?

Answers:
1. 12-18 months.
2. Abnormal function of medial portion of proximal tibial growth plate, causing bowing in proximal tibia, most common in obese African-Americans who walk early. Treatment is usually osteotomy of the proximal tibia and fibula.
3. DDH includes hip subluxation, hip dislocation, and acetabular dysplasia. It occurs in 1/1000 births, more common in breech and females.
4. Flexion of hip and knees bilaterally and look at level of knees. If one knee is lower than the other, this implies DDH.
5. Barlow involves pushing laterally on the hip with the leg in a flexed and adducted position, resulting in hip dislocation. Ortolani test reduces the hip back in place by pushing anteriorly with the long finger over the greater trochanter to left the femoral head over the posterior lip of the acetabulum, which should result in a palpable clunk if positive.
6. In the first few months of life, infant can be treated with closed reduction and Pavlik harness or hip spica cast. Surgery may be needed if diagnosed at walking age.

Pediatrics: feet and toes

1. What is metatarsus varus?
2. What is talipes equinovarus (clubfoot) and what percent require correction?
3 What is talipes calcaneovalgus?
4. What conditions are associated with cavus foot?

Answers:
1. Adduction of forefoot on hindfoot with heel in normal position. 85% correct by age 3-4 years.
2. Equinus or PF of foot, varus or inversion deformity at heel, forefoot varus. 50% require surgical correction.
3. excessive DF of ankle and eversion of foot, usually due to intrauterine position.
4. Poliomyelitis, Charcot-Marie-Tooth, Friedrich's ataxia. Usually associated with claw toes.

Spondylolisthesis in children

*1. What are the most common causes of spondylolisthesis in children? At what levels?
2. What is the difference between isthmic and dysplastic spondy?
3. What determines the need for treatment?

Answers:
1. Dysplastic and isthmic are most common. The most frequent level is L5-S1, then L4-5.
2. Isthmic is the result of a slip at a previoius spondy or a pars defect. Dysplastic has lenthening of the lamina and is more likely to cause cauda equina compression.
3. Over 50% slippage requires surgical fusion, less than 50% depends on severity of symptoms. Surgery rarely needed for <25% slippage.

Scheuermann's kyphosis

1. What is Scheuermann's kyphosis?
2. What do X-rays show?
3. What is the treatment?

Answers:
1. Three or greater consecutive vertebrae are wedged greater than 5 degrees.
2. Irregular vertebral endplates and protrusion of disc material into vertebral bodies (Schmorl's nodes). There is also narrowed disc space and anterior wedging.
3. No physical exercises if there is pain with irregular vertebral bodies, TLSO for pain control, rest, ice, gentle stretching, NSAIDs.

Evaluation and treatment of scoliosis

1. What is the Adams test?
2. What is the Cobb method?
3. When are PFTs done? What is the most common abnormality on PFTs?
4. What is the treatment for scoliosis based on the degree of curvature?
5. How much does curvature generally progress during adolescent growth?

Answers:
1. Have patient bend forward with legs straight at knees. Posterior trunk is prominent at convex side of curve.
2. On radiograph, draw line through superior endplate of apex of curve, then inferior endplate of bottom of curve, calculate angle.
3. If thoracic scoliosis is >50-60 deg, there may be PFT abnormalities. Most common is decreased VC.
4. 1-20 degrees: observe. 20-40 degrees: brace if idiopathic, surgery if neuromuscular. >40 deg: surgery.

Scoliosis

1. What is functional scoliosis? How does it differ from structural scoliosis?
2. What are the three periods of idiopathic scoliosis?
3. What is the most common presentation of congenital scoliosis? What other abnormality is it often associated with?
4. What neuromuscular diseases are commonly associated with acquired scoliosis?

Answers:
1. Functional scoliosis is due to malpositioning or unilateral paraspinal muscle pull, associated with back pain and muscle spasm. Unlike structural scoliosis, it is reversible.
2. Infantile: 0-3 yrs. Juvenile: 4 yrs-puberty onset. Adolescent: puberty to just prior to epiphyseal closure.
3. Hemivertebra in which lateral half of vert fails to form. Commonly associated with unilateral renal agenesis.
4. CP (spastic quadriplegia), Duchenne's MD (after WC bound), spinal bifida, spinal muscular atrophy.

Development and growth

1. How long does it take for the birth length to double? For the birth weight to triple?
2. How many fontanels are present at birth and when do they close?
3. How many ossification centers are present in an infant? What is the first bone to calcify in utero?
4. What is the earliest marker of abnormal neurological maturation?

Answers:
1. Birth length doubles in four years. Weight triples in one year, quadruples in two years.
2. Six fontanels (anterior, posterior, 2 sphenoid, 2 mastoid). Anterior normally closes at 10-14 mo, posterior at 4 months.
3. 5 ossification centers: distal femur, proximal tibia, calcaneus, talus, cuboid. Clavicle is the first to calcify in utero, during fifth fetal week.
4. Obligatory or persistent primitive reflexes.

Chromosomal syndromes

1. What are signs associated with Down Syndrome (Trisomy 21)?
2. What are signs associated with Turner Syndrome (45X)?
3. What are signs associated with Klinefelter Syndrome (47XXY)?
4. What infectious agents that cause interuterine malformations?
5. What drugs and maternal diseases are teratogenic?

Answers:
1. Mongoloid slant of eyes, Brushfield spots on iris, protruding tongue, low set auricles, excess nuchal skin, Simian lines, incurving of 5th fingers, increased distance between first toes, mottling of skin, hypotonia, heart disease (VSD).
2. Triangular face, abnormal ears, webbed neck, broad chest, wide niples, short stature, cubitus valgus, primary amenorrhea, CHD (coarctation of aorta), normal IQ, infertility.
3. Tall, small testicles, gynecomastia, eunuchoid build, mild mental retardation, infertility.
4. Rubella, CMV, toxoplasma, herpes, varicella.
5. Alcohol, cocaine, anticonvulsants, Vit A, DM, phenylketonuria.

Knee orthoses

1. What are the functions of a flexible knee orthosis?
2. What is the Swedish knee cage?
3. What is the Lenox-Hill derotation orthosis?

Answers:
1. Comfort for patients with OA, knee sprains, mild edema, proprioceptive feedback, kinesthetic reminder, minimal mechanical support, retains body heat, and stabilizes patellar tracking.
2. Controls minor to mod knee hyperextension, may be articulated or nonarticulated.
3. Controls knee axial rotation in addition to AP and mediolat, and is used for prevention and management of sports injuries (ACL).

KAFOs/HKAFOs

1. What are the purposes of a KAFO?
2. What are indications for a HKAFO?
*3. What is a Scott-Craig orthosis?
*4. What is a reciprocal gait orthosis?

Answers:
1. Control genu recurvatum, valgum, and varum, provide skeletal support following surgery or fracture, knee flexion contractures, prevent knee buckling.
2. Hip flexion/extension instability, hip adduction/abduction weakness, hip internal/external rotation instability.
3. For standing and ambulation in paraplegics of level L1 or higher, allowing unsupported standing. Ambulation is achieved using crutches and walker with swing-to or swing-through gait.
4. Used for upper lumbar paralysis in which active hip flexion is preserved. Hip joints are coupled together with cables to provide mechanical assistance to hip extension. Ambulation requires two crutches or walker with a four-point gait.