Pediatrik

1) (OBQ12.58) Which of the following most accurately describes the cause of osteopetrosis?
Review Topic
QID: 4418
1.

Decreased expression of type I collagen

3% (66/2496)

2.

Decreased mineralization of osteoid matrix

6% (140/2496)

3.

Loss-of-function of GS alpha protein gene

6% (144/2496)

4.

Loss-of-function of carbonic anhydrase II gene

82% (2039/2496)

5.

Mutation of FGFR3

4% (90/2496)

PREFERRED RESPONSE 4
Osteopetrosis is characterized by defective osteoclastic resorption of immature bone caused
by a loss-of-function mutation in the carbonic anhydrase II gene.
Carbonic anhydrase II is an enzyme that converts carbon dioxide and water into H+ and
bicarbonate. The protons created are then transported across the ruffled border of osteoclasts,
leading to acidification and demineralization of bone matrix. There are AD and AR forms of
osteopetrosis, with the AR form usually being fatal in the first few years of life.
Scwartz et al. present a case report of an infant with osteopetrosis and no family history.
Diagnosis was made by generalized increased radiological bone density and by a bone biopsy
showing persistence of calcified cartilage. Measurement of his erythrocyte carbonic
anhydrase activity revealed a deficiency of CA II.
Illustration A shows CA II converting water and carbon dioxide to bicarbonate and protons
within an osteoclast. The protons are then shuttled across the ruffled border where bone is
resorbed.
Incorrect Answers:
Answer 1: Osteogenesis imperfecta is associated with a decrease in type I collagen (COL
1A1 and COL 1A2 genes)
Answer 2: Rickets is associated with a defect in mineralization of osteoid matrix caused by
inadequate calcium and phosphate
Answer 3: Fibrous dysplasia is associated with a GS alpha protein mutation
Answer 5: Achondroplasia is associated with a mutation of FGFR3 (fibroblast growth factor
receptor 3, on chromosome 4P)

2). (OBQ06.42) A 12-year-old boy with sickle cell anemia complains of 24 hours of pain in

the right thigh. He denies any traumatic injury. The right leg has tenderness with palpation of
the mid-thigh. The patient's temperature is 100.2 degrees F, and ESR is 45. Radiographs of
the femur are unremarkable. A radionuclide bone scan demonstrates abnormal uptake in the
mid-femur. A radionuclide bone marrow scan demonstrates decreased uptake within the
marrow. Which of the following is the best step in management? Review Topic
QID: 153
1.
Symptomatic care for his pain with NWB crutches, intravenous hydration, and
consultation with hematology
68% (212/310)

2.

Steroid injection of the quadriceps

0% (0/310)

3.

Two weeks of an oral cephalosporin and follow-up radiographs

1% (4/310)

4.

Bone biopsy for culture and intravenous antibiotics

19% (58/310)

5.

Surgical debridement, culture, and intravenous antibiotics

12% (36/310)

PREFERRED RESPONSE 1
Sickle cell crises resulting in bone infarcts can be difficult to differentiate from acute
osteomyelitis with physical exam and plain radiographs alone. This child is undergoing a
bone infarct as confirmed by his radionuclide scans.
Skaggs et al reviewed 79 cases of acute extremity pain in sickle cell patients. Radionuclide
bone marrow and bone scan was used to differentiate osteomyelitis from bone infarct. Four
cases of infection were diagnosed by normal uptake on the bone marrow scan and abnormal
uptake on the bone scan. These cases were confirmed osteomyelitis by positive culture.
Seventy cases were diagnosed as bone infarct by decreased uptake on the bone marrow scan
and abnormal uptake bone scan.

Chambers et al reviewed the charts of 2000 known sickle cell patients. Fourteen patients had
an episode of osteomyelitis or septic arthritis. Radiographs and bone scans were not helpful
in differentiating infection from an acute bone infarct. Salmonella was the most frequent
organism cultured from the osteomyelitis cases. The authors recommend bone aspiration or
biopsy in an sickle cell patient with extremity pain, swelling, and a fever greater than 38.2
degrees C.
3) (OBQ05.140) A 14 year-old girl has chronic foot pain which has failed to respond to

previous surgical coalition resection and soft tissue interposition. A radiograph of her foot is
shown in Figure A. A CT scan demonstrates a talocalcaneal coalition with almost complete
involvement of the subtalar joint. What is the treatment of choice? Review Topic
FIGURES: A
QID: 1026
1.

revision coalition resection and extensor digitorum brevis interposition

12% (82/658)

2.

revision coalition resection and fat interposition

7% (44/658)

3.

tibiotalocalcaneal arthrodesis

5% (36/658)

4.

talonavicular arthrodesis

4% (27/658)

5.

triple arthrodesis

71% (467/658)

PREFERRED RESPONSE 5
For symptomatic coalition before degenerative changes have occurred, resection is the usual
treatment; however, this is not indicated if the patient has failed previous coalition resection
surgery, and has greater than 50% involvement of the posterior facet of the subtalar joint.
Triple arthrodesis involves fusion of the subtalar, calcaneocuboid, and talonavicular joints
and is the most effective procedure for fixed hindfoot and forefoot deformities. Subtalar
fusion can be performed in select cases with no significant hindfoot deformity. This
procedure is contraindicated in young children (10-12 years) because of the limitation it puts
on foot growth. Wilde et al found fair or poor results in all ten feet with preoperative CT
scans showing an area of relative coalition to be >50% and heel valgus > 16 degrees.
Scranton reported consistently successful resections of talocalcaneal coalitions if the coalition
was less than one-half of the surface area of the talocalcaneal joint and there was no
degenerative arthritic changes in the talonavicular joint.

4) (OBQ04.74) All of the following are clinical features of neurofibromatosis type I (NF-I)
EXCEPT? Review Topic
QID: 1179
1.

freckling in the axillae

4% (10/272)

2.

optic glioma

11% (30/272)

3.

2 or more Lisch nodules

3% (8/272)

4.

talipes equinus

81% (220/272)

5.

cafe' au lait spots

1% (3/272)

PREFERRED RESPONSE 4
NF-1 is an autosomal dominant disorder, with approximately 50% of cases due to a new
mutation. It is the most common genetic disorder caused by a new mutation of a single gene.
The entity is quite common, affecting 1 in 4000, and is one of the most common dominantly
inherited gene disorders in humans. According to the NIH Consensus Development
Conference Statement (1987) the diagnostic criteria for NF-1 are met in an individual if two
or more of the following are found: 1) Six or more caf-au-lait macules over 5 mm in greatest

diameter in prepubertal individuals and over 15 mm in greatest diameter in postpubertal

individuals. 2) Two or more neurofibromas of any type or one plexiform neurofibroma. 3)
Freckling in the axillary or inguinal region. 4) Optic glioma. 5) Two or more Lisch nodules
(iris hamartomas). 6)
A distinctive osseous lesion such as sphenoid dysplasia or thinning of long bone cortex with
or without pseudarthrosis. 7) A first-degree relative (parent, sibling, or offspring) with NF-1
by the above criteria. According to the Crawford article, common orthopaedic manifestations
are "spinal deformity, congenital tibial dysplasia (congenital anterolateral tibia bowing and
pseudarthrosis), and disorders of excessive bone and soft-tissue growth".
5 ) (OBQ05.129) Figure A shows a lateral radiograph of an 9-month old's dorsiflexed foot.

What is the first line treatment for this condition?

Review Topic

FIGURES: A
QID: 1015
1.

Observation

8% (54/664)

2.

Serial casting

47% (311/664)

3.

Manipulation under anesthesia followed by a single casting

1% (8/664)

4.

Surgical re-alignment

2% (16/664)

5.
Serial manipulation and casting followed by surgical release and talonavicular
reduction with pinning
41% (275/664)

PREFERRED RESPONSE 2
The radiographs show hindfoot parallelism between the talus and calcaneus which is
characteristic of congenital talipes equinovarus, also known as clubfoot. This question
emphasizes the importance of the talocalcaneal angle. From a testing perspective, it is
important to be able to differentiate clubfoot from congenital vertical talus (CVT) on
radiographs. Vertical talus will not show the parallelism between the talus and calcaneus seen
with clubfoot and the navicular (which is not ossified in infants) will not be aligned with the
talus even on attempts to reduce with plantarflexion. Meary's angle and the talocalcaneal
angle are shown for clubfoot and vertical talus in Illustration A and B respectively.
Roye, et al. stated that all radiographs of the foot should simulate weight bearing. The two
most commonly used measurements are the lateral and AP talocalcaneal angles, which
display parallelism.
Laaveg et al reviewed the long term treatment of congenital clubfoot and found that
functional results were satisfactory in both appearance and function in 88.5% of 104 clubfeet.

6) (OBQ10.1) Which of the following must be done whenever a non-ambulatory infant

presents to the ER with a diaphyseal long bone fracture?

Review Topic

QID: 3089
1.
Immediate consultation with child protective services and possible admission to the
hospital
99% (1786/1809)

2.

Order serum vitamin D levels

0% (3/1809)

3.

Order serum calcium and phosphorus levels

0% (6/1809)

4.

MRI of the cervical spine

0% (6/1809)

5.

Perform genetic testing for COL1-A1 and COL1-A2

0% (5/1809)

PREFERRED RESPONSE 1
Each of the answers could be performed in this scenario as part of a diagnostic evaluation.
However, missing a case of child abuse could result in further abuse of the child or even
death, making this the most important issue to address.
Banaszkiewicz et al performed a retrospective review over a five year period on children < 1
year of age who presented to the ER with a fracture. They found that 28% of the time, abuse
was underestimated at the time of evaluation. They recommend admitting all patients in this
age group with any fracture and consulting child protection services.

7) (OBQ06.75) Plantar flexion of the first ray is the initial deformity seen in which

condition?

Review Topic

QID: 186
1.

Oblique talus

2% (17/825)

2.

Kohler's disease

2% (17/825)

3.

Charcot-Marie-Tooth

90% (746/825)

4.

Calcaneovalgus foot

4% (31/825)

5.

Juvenile hallux valgus

1% (12/825)

PREFERRED RESPONSE 3
Charcot-Marie-Tooth (CMT) disease is a hereditary motor sensory neuropathy. These patients
often develop a pes cavovarus deformity (Illustration A) with the intial stage being plantar
flexion of the 1st ray.
A weakened tibialis anterior / peroneus brevis is overpowered by a stronger peroneus longus /
posterior tibialis and results in a plantar flexed first metatarsal and pronated forefoot as
described in the article by Mosca. Hindfoot varus deformity develops secondarily. As the
deformities become more fixed, an equinus deformity can also develop.
Coleman block testing as described in CORR from 1977 can help determine if the hindfoot
varus deformity is fixed or flexible. Hindfoot flexibility is noted by placing the lateral foot on
a block to remove the plantar flexed first ray from contributing to the deformity and
observing the hindfoot moving to a more neutral position.

8) (OBQ09.141) A 3-year and 6-month old child fell while playing with friends 2 hours ago

and has avoided bearing weight on the right leg since that time. The child is afebrile and
exam reveals tenderness along the distal tibial shaft with no significant swelling. Radiographs
are shown in Figure A and B. What is the most appropriate treatment? Review Topic
FIGURES: A
QID: 2954
1.

MRI of the tibia

1% (8/1318)

2.

Aspiration of the tibia

0% (3/1318)

3.

Referral to child services

3% (33/1318)

4.

Long leg cast application

96% (1264/1318)

5.

Serum vitamin D, calcium, and phosphate levels

1% (7/1318)

PREFERRED RESPONSE 4
The clinical presentation and radiographs are consistent with a toddler's fracture of the tibia
and the next step in management includes long leg cast application.
As described by Mellick et al this scenario is classic for the toddler's fracture; a toddler-aged
child with avoidance of weightbearing after a fall. The radiographs demonstrate a nondisplaced spiral fracture of the distal tibial shaft. There are no signs/symptoms of infection, so
MRI and aspiration are not appropriate. Metabolic work-up is not warranted for this common

type of fracture. Child abuse should be suspected with long bone fractures of the lower
extremity in child who are not yet ambulatory (not this case).

9) (OBQ05.135) A 10-year-old boy presents with medial foot pain that is severe enough that

it limits his daily activities such as walking to school. Physical exam shows tenderness in the
medial forefoot 3 cm anterior and inferior to the medial malleolus. A radiograph is shown in
Figure A. Nonoperative treatment including orthotics and cast immobilization was attempted
for three months without success. What is the most appropriate next step in treatment?
Review Topic

FIGURES: A
QID: 1021
1.

No treatment needed-return to class

1% (14/1935)

2.

Continue serial casting

1% (13/1935)

3.

Excision of the medial prominence of the navicular, including the synchondrosis

95% (1836/1935)

4.

ORIF of the navicular non-union

3% (51/1935)

5.

Bone stimulator for the navicular non-union

0% (7/1935)

PREFERRED RESPONSE 3
This patient has a painful accessory navicular that has failed conservative treatment. Excision
of the medial prominence of the navicular is indicated as a second line of treatment.
The accessory navicular is a normal variant seen in up to 12% of the population. Repetitive
microtrauma at the level of the synchondrosis may lead to medial foot pain. If non-operative
management fails, excision of the accessory navicular is indicated. Often, advancement of the
posterior tibial tendon is completed with accessory navicular excision (Kidner procedure).
The theory is that this helps restore the pull of the tendon and prevents medial arch sag.
Kopp et al. retrospectively reviewed symptomatic patients treated with accessory navicular
excision and anatomic repair of the posterior tibialis tendon. Thirteen of 14 patients were
pain-free. No patients had activity limitations and all were satisfied with the outcome.
Bennett et al. retrospectively reviewed symptomatic patients treated with accessory navicular
excision and anatomic repair of the posterior tibialis tendon. Good and excellent results were
obtained in 90% of patients.
Figure A shows an AP radiograph of the foot that demonstrates an accessory os of the
navicular.
Incorrect Answers:
Answers 1, 2: Given that this patient has exhausted conservative measures, surgical
intervention is the next best step.
Answers 4, 5: This is not a navicular fracture.

10 ) (OBQ09.178) A 9-year-old boy fell off of a swing set and injured his left elbow.

Radiographs are shown in Figures A and B. Open reduction and internal fixation of this
fracture is indicated secondary to which of the following: Review Topic
FIGURES: A
QID: 2991
1.

Displacement of greater than 5 mm

5% (36/707)

2.

An incarcerated fragment in the ulnohumeral joint

86% (609/707)

3.

2+ valgus laxity seen with manual stressing

1% (6/707)

4.

To prevent cubitus valgus deformity

3% (19/707)

5.

High risk of symptomatic non-union of fragment

5% (36/707)

PREFERRED RESPONSE 2
Medial epicondyle avulsion fractures can be treated with open reduction and internal fixation
when the fractured fragment is incarcerated in the ulnohumeral joint or if there is significant
ulnar nerve dysfunction. Figures A and B show a displaced medial epicondyle fracture that is
incarcerated in the ulnohumeral joint, thus requiring ORIF.
Lee et al studied 25 pediatric patients who had undergone ORIF of a medial epicondyle

fracture and found no correlation between surgical outcome and injury mechanism,
displacement, interval from injury to surgery, dislocation, fixation method, or duration of
immobilization. All patients in this study had good to excellent outcomes.
Farsetti et al compared pediatric patients with medial epicondyle fractures with displacement
> 5mm who received a long arm cast without reduction, ORIF, and excision. They found no
difference in long term results between patients treated with ORIF and those who received
non-op treatment, while those treated with excision had poor long term results.

11) (OBQ13.23) You are the team physician for a collegiate football team and receive

weekly injury reports from the athletic trainer. All players with sickle-cell trait are listed at
the bottom to remind all on-field personnel that they may need which of the following?
Review Topic
QID: 4658
1.

Oxygen supplementation and oral or IV hydration

94% (2114/2245)

2.

Additonal layers of warm clothes

1% (24/2245)

3.

Increased pain medication

0% (7/2245)

4.

Avoidance of non-steroidal anti-inflammatory medicines

2% (51/2245)

5.

Days of rest due to increased joint pain

2% (40/2245)

PREFERRED RESPONSE 1
Players with a blood test indicating the presence of sickle-cell trait (SCT) are at risk for
exertional sickling collapse which responds initially to rest, hydration and oxygen.
SCT is not a disease but a condition, resulting from inheritance of one gene for sickle
hemoglobin (S) and one gene for normal hemoglobin (A).
The vital concern is exertional sickling collapse, which can be fatal, occurs in a variety of
sports, and is a leading cause of death in college football.
According to the review by Eichner, sickling collapse is an intensity-associated syndrome
that differs from the other common causes of collapse. The best approach in college football
may be tailored precautions to prevent sickling collapse and enable athletes with SCT to
thrive. Other clinical concerns in SCT are compartment syndromes and lumbar myonecrosis,
splenic infarction, gross hematuria, hyposthenuria, and venous thromboembolism.
Kark et al. reviewed all cases of sudden death occurring among 2 million enlisted recruits
during basic training in the U.S. Armed Forces from 1977 to 1981. They concluded that
"recruits in basic training with the sickle-cell trait have a substantially increased, agedependent risk of exercise-related sudden death unexplained by any known preexisting
cause".
Incorrect Responses:
2. no research to support need for warmer clothes and cases occur year round.
3-4. these are typical of sickle cell disease, not SCT.
5. important in patients with renal impairment which is not typically seen in SCT.

12 ) (OBQ05.23) A 30-month-old boy has worsening bilateral bowleg deformities, and

radiographs depicting Langenskiold stage II are shown in Figure A. The most appropriate
initial management should consist of which of the following? Review Topic
FIGURES: A
QID: 60
1.

Observation

26% (174/664)

2.

Bracing with knee-ankle-foot orthoses

57% (380/664)

3.

Bracing with ankle-foot orthoses

4% (27/664)

4.

Proximal tibia/fibula valgus osteotomy with bar resection

5% (33/664)

5.

Proximal tibia/fibula valgus osteotomy with hemiepiphysiodesis

7% (46/664)

PREFERRED RESPONSE 2
Initial management of infantile Blount's disease in children less than 3 years old consists of
full time bracing with a knee-ankle-foot orthosis.
Infantile Blount's disease is pathologic cause of genu varum in children less than 3 years of
age. Radiographically, a widened and irregular medial physeal line and an irregularly ossified
and medially sloped epiphysis are seen. Initial treatment is non-operative in children less than
3 years of age. Operative management in children older than 3 is considered if non-operative
management has failed, or in Langenskiold classes V and VI, where there is a congenital bar
across the physis. When osteotomy is required, overcorrection to at least 5 degrees of valgus
should be done.
Richards et. al. reported successful outcomes following bracing for an average of 9 months in
patients 3 years and younger with Langenskiold stage II or less. Patients with bilateral disease
more commonly required osteotomy.
Figure A shows bilateral infantile Blount's disease, with varus angulation of the tibial
metaphysis and beaking of the medial metaphysis.

13 ) (OBQ08.150) In patients older than 12-months of age with developmental dysplasia of

the hip, all of the following physical exam findings are likely present EXCEPT?
Topic

Review

QID: 536
1.

Limited hip abduction

3% (20/789)

2.

Positive Ortolani maneuver

83% (657/789)

3.

Abnormal leg lengths on Galeazzi testing

2% (18/789)

4.

Trendelenburg gait

7% (57/789)

5.

Pelvic obliquity

5% (36/789)

PREFERRED RESPONSE 2
There are many exam maneuvers which are used in the diagnosis of developmental dysplasia
of the hip (DDH). Exam findings differ based on the age of the patient.
Vitale et al emphasize that physical exam findings associated with DDH in a child older than
12-months can be different than those seen in the newborn. Specifically, limited hip
abduction, a positive Galeazzi test, a positive Trendelenburg gait, and asymmetry of hip
abduction are all useful exams tests that are likely to be positive. The Barlow and Ortolani
maneuvers are of limited use in older children (> 6 months) because the soft tissues about the
hip tighten.
Illustration V demonstrate how the Barlow and Ortolani test are performed.

14 ) (OBQ10.265) Which of the following pediatric congenital disorders is caused by a

glycine substitution in the procollagen molecule?

Review Topic

QID: 3316
1.

Scurvy

8% (107/1364)

2.

Osteogenesis imperfecta

80% (1098/1364)

3.

Fibrous dysplasia

5% (69/1364)

4.

Diastrophic dysplasia

4% (49/1364)

5.

Ochronosis (alkaptonuria)

3% (38/1364)

PREFERRED RESPONSE 2
Osteogenesis imperfecta (OI) is caused by a defect in Type I collagen (COL1A1 and
COL1A2) that causes abnormal cross-linking via a glycine substitution in the procollagen
molecule. This defect leads to decreased collagen secretion, bone fragility, wormian bones,
short stature, scoliosis, tooth defects, hearing defects, and ligamentous laxity.
The article by Cole explains that OI includes a wide spectrum of disease and has various
inheritance patterns (autosomal dominant or autosomal recessive). Fibrous dysplasia is
caused by a germ line defect in the Gsa protein. Scurvy is caused by a vitamin C deficiency
that causes a decrease in chondroitin sulfate synthesis and subsequent defect in intracellular
hydroxylation of collagen peptides. Diastrophic dysplasia is caused by a mutation of a gene
coding for a sulfate transport protein. Ochronosis (alkaptonuria) is caused by a defect in the
homogentisic acid oxidase system.
15) (OBQ06.113) What is the most common complication following surgical fixation for the

fracture shown in Figure A in an 8-year-old boy?

FIGURES: A
QID: 299
1.

Coxa valga

5% (12/257)

2.

Chondrolysis

4% (10/257)

Review Topic

3.

Stiffness

4% (9/257)

4.

Clinically significant limb length discrepancy

5% (13/257)

5.

Avascular necrosis

82% (212/257)

PREFERRED RESPONSE 5
The radiograph demonstrates a pediatric basicervical femoral neck fracture. Femoral neck
fractures in the pediatric population are associated with a high rate of osteonecrosis. They are
divided into epiphyseal, transcervical, basicervical and intertrochanteric. Fracture
displacement, age over ten years and an epiphyseal or transcervical fracture pattern are risk
factors for AVN.
Moon et al found the rates of AVN were as follows: Delbet type I=38%, II=28%, III=18%,
and IV=5%, where I=Epiphyseal, II=Transcervical, III=Basicervical, IV=Intertrochanteric.
Hughes et al discussed several complications. Coxa vara, not coxa valga, occurs about 2030% of the time and is more common in fractures treated nonoperatively.
Incorrect Answers:
Chondrolyis has been reported sporadically, but is always found concomitantly with AVN.
Stiffness is not commonly seen after surgical fixation. Premature physeal closure can occur
with or without surgery but because the femoral head epiphysis is only responsible for 13%
of the ultimate length of the femur, growth disturbances rarely result in limb length
discrepancies exceeding 2 cm. If displaced, the fracture requires open reduction, hematoma
evacuation and internal fixation with pins. Whether or not the pins should cross the physis is
still subject to debate.

16 ) (OBQ08.232) Which of the following muscles would be affected if a 6-month-old child

sustains a birth-related brachial plexopathy affecting C5 nerve root?

Review Topic

QID: 618
1.

Trapezius

5% (29/643)

2.

Triceps

0% (3/643)

3.

Biceps

94% (603/643)

4.

Interossei

1% (4/643)

5.

Flexor digitorum profundus

0% (3/643)

PREFERRED RESPONSE 3
The biceps and brachialis muscles are supplied by C5-6 and as such a brachial plexopathy
affecting the C5 nerve root would affect the child's ability to perform elbow flexion and
forearm supination. The deltoid is also supplied by C5 and palsy would lead to deficient
shoulder abduction. Finally, extensor carpi radialis longus and brevis are supplied by C5-6
and palsy would result in weak wrist extension.
The trapezius is supplied by the spinal accessory nerve (cranial nerve XI) and palsy would
lead to scapular winging. The interossei are supplied by T1, the triceps by C6-7, and the
flexor digitorum profundus by C7-8.

Waters studied the natural history of brachial plexus palsy and the effects of treatment
including microsurgical repair, tendon transfer, and derotational osteotomy.
Smith et al evaluated 170 patients to determine prognosticators for recovery of function in
brachial plexopathy. They found that prolonged neurological recovery or a greater level of
initial injury were each associated with worse long-term shoulder function.
17 ) (OBQ06.90) A 13-month-old boy has shortening and deformity of the right leg. A

clinical photograph and radiograph are provided in Figures A and B. Which of the following
findings are indicative of a good outcome with a tibiotalar Syme amputation? Review
Topic
FIGURES: A
QID: 201
1.

35 degree flexion contracture

2% (8/532)

2.

Absence of tibiofibular synostosis

12% (65/532)

3.

Bilateral involvement

0% (2/532)

4.

Full functional quadriceps strength

85% (450/532)

5.

Family history of tibial hemimelia

1% (4/532)

PREFERRED RESPONSE 4
The clinical scenario and images demonstrate tibial hemimelia. An amputation below the
knee has greater likelihood of success if there is a functional quadriceps and no flexion
contracture of the knee. Otherwise, a knee disarticulation may be necessary.
Kalamchi et al review 24 cases of tibial hemimelia to establish a classification scheme. Type I
is total absence of the tibia. Type II is distal absence of the tibia. Type III is distal deficiency
with tibiofibular diastasis. Assessment of the quadriceps function and degree of flexion
contracture is crucial to following the appropriate treatment option.

18) (OBQ06.255) A 5-year-old boy has a history of being treated with the Ponseti technique

for a unilateral clubfoot. What muscle most commonly causes a dynamic deformity in the
swing phase of gait following Ponseti casting? Review Topic
QID: 266
1.

Tibialis Posterior

12% (43/347)

2.

Tibialis Anterior

74% (258/347)

3.

Gastrocnemius

8% (27/347)

4.

Peroneus Longus

5% (16/347)

5.

Peroneus Brevis

0% (1/347)

PREFERRED RESPONSE 2
In clubfoot patients older than 12 to 18 months, a dynamic swing phase supination deformity
may develop as a result of medial overpull of the anterior tibialis tendon. Incomplete
reduction of the navicular onto the talar head results in changing the anterior tibialis muscle
from predominately a strong dorsiflexing to a strong supinating force. If uncorrected, this
may lead to dynamic deformity during the swing phase of gait.
The study by Ippolito et al found that at long term followup children treated with Ponseti's
manipulation technique and cast immobilization followed by an open heel-cord lengthening
had better outcomes than those that underwent Ponseti technique and extensive posteromedial
release.
The study by Kuo et al reviewed 71 feet who had residual dynamic clubfoot deformity that
underwent either split or complete anterior tibial tendon transfer. Both treatments resulted in
increased dorsiflexion and eversion ROM, eversion strength, and correction of forefoot
adduction and supination. They concluded there was little clinical signficance between the
two surgical approaches.
The article by Ponseti describes his method for the treatment of congenital clubfoot
deformity. The cavus deformity is corrected by supination of the fore part of the foot. The
varus deformity of the hindfoot is corrected with displacement or lateral rotation of the
navicular, together with the cuboid and the anterior aspect of the calcaneus, without pronation
of the foot. The foot position is maintained in a toe-to-groin plaster cast with the knee flexed
90 degrees and the foot in maximum external rotation. The equinus is corrected last, by
dorsiflexion of the foot with the heel in valgus angulation. A tendo-achilles tenotomy can
facilitate correction of equinus. To decrease the risk of recurrence, a Denis Brown splint is
utilized full time for several months and then converted to part-time wear.
19) (OBQ05.241) Which of the following injuries is associated with the highest risk of

morbidity and mortality in a pediatric trauma patient?

QID: 1127
1.

Pelvic fracture

34% (258/768)

2.

Scapula fracture

12% (93/768)

3.

Spine fracture

53% (407/768)

Review Topic

4.

Femur fracture

1% (9/768)

5.

Tibia fracture

0% (0/768)

PREFERRED RESPONSE 3
Buckley et al reviewed 805 pediatric trauma patients who sustained 953 fractures and
dislocations to determine the relationship between skeletal injury and trauma score, injury
severity score, length of hospital stay, hospital charges, and mortality. The femur was the
most commonly fractured bone, and overall mortality was 3% in the group studied. The
trauma patients with associated scapula or pelvic fractures had an 11% mortality rate,
whereas those patients with spine fractures had a 16% mortality rate. Femur and tibia
fractures had a mortality rate of 2% and 1% respectively. The authors concluded that central
musculoskeletal injuries (spine, clavicle/scapula, and pelvis) were associated with the longest
hospital stays and intensive care unit admissions, as well as the highest Injury Severity
Scores, hospital charges, and mortality rates. Tepas et al reviewed the records of 4,400
children with traumatic head injuries, and compared them to head injuries reported from an
adult trauma registry. They found that overall children have a lower mortality than adults, and
that CNS injury is the predominant and most common cause of pediatric traumatic death.
20 ) (OBQ12.39) A 28-year-old African-American male with a history of Sickle Cell

Disease complains of progressive left hip pain for the past two years. He denies any causative
injuries. His images are shown in Figures A and B. Which of the following mechanisms is
most likely responsible for his symptoms? Review Topic
FIGURES: A
QID: 4399
1.

Blood disorder due to abnormal hemoglobin S alleles

91% (2180/2396)

2.

Progressive slippage of physis though the hypertrophic zone

1% (25/2396)

3.

Osteomyelitis most likely due to Salmonella species

5% (112/2396)

4.

Accumulation of glycosaminoglycan breakdown products

2% (45/2396)

5.

COL5A1 or COL5A2 mutation

1% (19/2396)

PREFERRED RESPONSE 1
The clinical presentation and images are most consistent with left hip osteonecrosis as a result
of coagulation and vascular occlusion caused by sickle cell anemia.

Sickle cell disease is a genetic disorder of hemoglobin synthesis characterized by 2 abnormal

hemoglobin S alleles. Under low oxygen conditions the affected blood cells become "sickle
shaped" and unable to pass through vessels. This results in vascular occlusion that may have a
variety of clinical consequences depending on the body part affected.
Hernigou et al. review the natural history of symptomatic osteonecrosis in adults with sickle
cell disease. Once symptomatic , osteonecrosis of the hip in sickle cell disease has a high
likelihood of progressing and leading to femoral head collapse. Deterioration can be rapid
and in most patients operative intervention is necessary to prevent further collapse or
alleviate intractable pain.
Mont et al. performed a literature review on the natural history of untreated asymptomatic
osteonecrosis of the femoral head. Their findings supported that asymptomatic osteonecrosis
had a high prevalence of progression to symptomatic femoral head collapse. Small, medially
located lesions had a low rate of progression, while medium to large sized osteonecrotic
lesions did progress in a substantial number of patients. They recommended consideration of
joint-preserving surgical treatment in asymptomatic patients with a medium-sized or large,
laterally located lesion.
Figure A shows an AP pelvis with left hip osteonecrosis. Figure B shows a T2 coronal MRI
with left hip osteonecrosis. Illustration A shows an example of a hemoglobin molecule which
has become "sickle shaped," and as a result is unable to pass through vessels efficiently.
Incorrect Answers:
Answer 2: Progressive slippage of the physis though the hypertrophic zone describes slipped
capital femoral epiphysis.
Answer 3: These radiographs are most consistent with osteonecrosis of the femoral head.
There is an increased incidence of Salmonella osteomyelitis in patient with Sickle Cell
disease, but Staphylococcus aureus is still the most common organism.
Answer 4: Accumulation of glycosaminoglycan breakdown products describes lysosomal
disorders.
Answer 5: COL5A1 or COL5A2 mutation describes the mutation of Ehlers Danlos
syndrome.

21 ) (OBQ09.208) The parents of a wheelchair-bound 8-year-old boy with cerebral palsy

present with difficulty during diaper changes and with hygiene care. His physical exam
demonstrates 5 of hip abduction on the left hip and 15 on the right. An AP pelvis radiograph
is shown in figure A. What is the most appropriate treatment? Review Topic
FIGURES: A
QID: 3021
1.

Bilateral botox injections and physical therapy

16% (65/407)

2.

Nighttime Pavlik harness

2% (8/407)

3.

Bilateral abductor release and valgus femoral osteotomies

12% (50/407)

4.

Bilateral adductor release, varus femoral osteotomies and acetabuloplasties

65% (264/407)

5.

Observation with repeat radiograph in 6 months

5% (19/407)

PREFERRED RESPONSE 4
Children with spastic cerebral palsy develop hip subluxation as the result of long term muscle
imbalance. Subluxation can progress to hip dislocation with resulting difficulties in seating,
hygiene or personal care, and/or hip pain. The patient has bilateral dislocated/near dislocated

hips with acetabular dysplasia.

Flynn and Miller provide a thorough review of hip disorders in patients with cerebral palsy
including etiology, physical exam and treatment guidelines. Recommendations included hip
reconstruction for children over 4 years of age with severe subluxation or dislocation if
severe degenerative changes are absent. Hip reconstruction consists of a one-stage soft-tissue
lengthening with varus derotational femoral osteotomy and possible acetabuloplasty.
Spiegel and Flynn also have provided a comprehensive review of hip dysplasia in patients
with cerebral palsy. The article discusses early intervention to prevent complications
associated with subsequent hip dislocations. Surgical intervention in patients older than 4
years with hip dislocations was once again hip reconstruction with soft-tissue lengthening,
femoral osteotomies and acetabuloplasties in patients in patients with marked acetabular
abnormalities.