FREE Orthopedics MCQS 2022 1651-1700

1651. (3973) Q4-8225:

Which of the following parameters is not a determinant of the Palmer classification of triangular fibrocartilaginous complex injuries:

1) Location of the lesion

3) Presence of lunatotriquetral ligament injury

2) Presence of ulnar head chondromalacia

5) Presence of ulnocarpal arthritis

4) Size of the lesion

The Palmer classification divides triangular fibrocartilage complex lesions into traumatic and degenerative. Traumatic subclassifications are based on the location of the ligament tear. In the degenerative tear, subclassifications are based on the degree of injury to the triangular fibrocartilage complex and associated chondral and ligamentous injury. Thus, the size of the lesion is not a parameter in the Palmer classification.

■Correct Answer:Size of the lesion

1652. (3974) Q4-8226:

What are the components of a Galeazzi fracture-dislocation:

1) Triangular fibrocartilage complex (TFC C ) tear, interosseous membrane tear, and radial shaft fracture

3) Interosseous membrane tear and radial shaft fracture

2) Ulnar shaft fracture, interosseous membrane tear, and TFC C tear

5) Wrist radial collateral ligament tear, dorsal intercarpal ligament tear, and ulnar shaft fracture

4) TFC C tear and radial shaft fracture

Only one in vitro study examined the soft tissue constraints of the Galeazzi fracture-dislocation pattern. Moore and colleagues performed a radial osteotomy at the pronator teres insertion of nine cadaveric forearms and then sectioned the TFC C and the interosseous membrane in alternating orders. They found that all three structures (TFC C , interosseous membrane, and radial shaft) must be injured to create a radial shortening of more than 10 mm in relationship to the distal ulna.

■Correct Answer:Triangular fibrocartilage complex (TFC C ) tear, interosseous membrane tear, and radial shaft fracture

1653. (3975) Q4-8227:

Who are the most common athletes to get medial epicondylitis of the elbow:

1) Tennis players

3) Swimmers

2) Golfers

5) Basketball players

4) Gymnasts

Although medial epicondylitis is called golferâs elbow, tennis players are more likely to have this condition. Medial epicondylitis can occur in any sport such as baseball pitching, javelin throwing, swimming, and gymnastics in which athletes place a significant valgus flexion force on their elbow.

■Correct Answer:Tennis players

1654. (3992) Q4-8244:

Indications for replantation include:

1) A crush injury in a patient who smokes

3) An ischemia time of 24 hours

2) A sharp amputation of the thumb in a 15-year-old patient

5) A single digit amputation proximal to the flexor digitorum superficialis (FDS) insertion

4) The use of an amputated part that was immersed in warm water for transport

A sharp amputation, particularly of the thumb, is the best indication for replantation. C rushed digits, prolonged ischemia time, poor condition of the severed part, and single digit loss proximal to the FDS insertion on the middle phalanx are relative contraindications to replantation.

 

■Correct Answer:A sharp amputation of the thumb in a 15-year-old patient

1655. (3993) Q4-8245:

An amputation through the wrist is an indication for attempted replantation.

1) True

2) False

An amputation through the wrist, palm, or forearm is an indication for attempted replantation. The caliber of the vessels and other structures provides a favorable environment for reconstruction.

 

■Correct Answer:True

1656. (3994) Q4-8246:

C are of an amputated part prior to replantation includes:

1) Painting the amputated part with povidone-iodine

3) Placing the amputated part in a warm saline bath

2) Immersing the amputated part in water

5) Putting the amputated part next to the patientâs body to keep it warm

4) Wrapping the amputated part with saline-soaked gauze and placing it in a plastic bag on ice

The appropriate care of an amputated part includes wrapping it in saline-dampened gauze and placing it on ice in a watertight bag. These actions preserve the tissues and slow cellular death until replantation is attempted. The part should not be immersed, painted with povidone-iodine, or kept next to the body.

 

■Correct Answer:Wrapping the amputated part with saline-soaked gauze and placing it in a plastic bag on ice

1657. (3995) Q4-8247: Replants are monitored by:

1) C olor

3) Doppler probes

2) Turgor

5) All of the above

4) Temperature measurements

Monitoring of replanted parts postoperatively is accomplished by clinical checks of color or turgor to indicate blood flow. Additional objective monitoring is performed by using Doppler probes to check flow or by measuring temperature differences between the replanted part as compared to other digits.

 

■Correct Answer:All of the above

1658. (3996) Q4-8248:

C omplications post-replantation include:

1) C old intolerance

3) Excessive laxity of the digit

2) Stiffness

5) All of the above

4) A and B only

C omplications after replantation include cold intolerance, which may improve over time, and stiffness of the replanted digit, which is generally due to immobility, tendon adhesions, and joint contracture.

 

■Correct Answer:A and B only

1659. (4003) Q4-8255:

Placing some tension on a flexor tendon repair increases the ultimate tensile strength of the repair.

1) True

2) False

Tension on the repair site of a flexor tendon laceration has been shown experimentally to increase the tensile strength of the repair.

 

■Correct Answer:True

1660. (4004) Q4-8256:

Flexor tendon nutrition is derived from:

1) The vincula

3) Synovial diffusion

2) The pulleys

5) A and C only

4) All of the above

Flexor tendon nutrition in the uninjured state is derived via the vincula, which contain blood vessels for nutrition. Injured tendons obtain nutrition via diffusion of synovial fluid. The pulley system does not contribute to flexor tendon nutrition.

 

■Correct Answer:A and C only

1661. (4005) Q4-8257:

Immobilization as a postoperative therapy for flexor tendon repair is recommended for:

1) Lacerations that involve both the flexor digitorum superficialis tendon and the flexor digitorum profundus tendon

3) C hildren

2) 2-strand tendon repairs

5) Flexor tendon laceration in the thumb

4) Associated pulley rupture

As children have difficulties in following the detailed flexor tendon rehabilitation program that is recommended for adults after flexor tendon repair, it is advisable to completely immobilize them to protect the repair and avoid inadvertent rupture.

 

■Correct Answer:C hildren

1662. (4006) Q4-8258:

Optimization of early active motion protocols for flexor tendon rehabilitation includes:

1) Multi-strand repair

3) Using passive flexion of the finger to gain flexibility

2) Splinting the digit in extension

5) Ultrasound as an adjunct

4) Therapist hyperextension of the finger

The use of 6- and 8-strand repair techniques allow the flexor tendon repair to withstand the force applied by early active motion protocols. The addition of epitendinous tendon repair also strengthens the repair.

 

■Correct Answer:Multi-strand repair

1663. (4007) Q4-8259:

The splint for early active motion flexor tendon rehabilitation protocols includes:

1) A wrist flexion piece

3) A yoke over the affected finger

2) A dynamic extension outrigger

5) None of the above

4) A hinge at the wrist to allow a tenodesis effect

A hinge at the wrist, which provides a tenodesis effect, allows the patient to passively extend the wrist and flex the fingers in preparation for gentle muscle contraction of the fingers.

 

■Correct Answer:A hinge at the wrist to allow a tenodesis effect

1664. (4010) Q4-8262:

A 13-year-old boy tears his anterior cruciate ligament (AC L) while playing flag football. What is the preferred graft material for his AC L reconstruction:

1) Bone-patellar tendon-bone autograft

3) Four-strand hamstring tendon autograft

2) Quadriceps tendon autograft

5) Achilles tendon allograft

4) Four-strand hamstring tendon allograft

Due to the patients age, autograft is the preferred option. Also, due to the patientâs age, his growth plates are open and the surgeon is prohibited from using a graft with a bone construct due to the possible damage to the growth plate.

 

■Correct Answer:Four-strand hamstring tendon autograft

1665. (4021) Q4-8274:

What deformity can develop in a mistreated volar PIP joint dislocation?

1) Swan neck deformity

3) Boutonniere deformity

2) Extensor lag

5) Hyperextension deformity

4) Flexion contracture

With volar PIP joint dislocations, there is almost always a disruption of the central slip of the extensor tendon. Because the central slip is involved, the PIP joint will hold a flexed position, and the lateral bands will fall volar to the axis of rotation of the PIP joint. The lateral bands will then exacerbate the flexion at the PIP joint, and due to their pull on the terminal tendon at the insertion on the distal phalanx, the DIP joint will hyperextend. This results in a boutonniere deformity.

■Correct Answer:Boutonniere deformity

1666. (4022) Q4-8275:

What percentage of the articular surface must be involved in a dorsal PIP joint fracture dislocation for disruption of the collateral ligaments to occur?

1) 20%

3) 60%

2) 40%

5) 100%

4) 80%

The percentage of articular surface involved differentiates a stable dorsal PIP joint fracture dislocation from an unstable one. It is believed that when the involved fracture fragment is less than 40% of the articular surface, the insertion site of the collateral ligaments is not disrupted and the joint is stable. If more than 40% of the articular surface is fractured, then the insertion of the collateral ligaments is involved and the joint will subsequently be unstable.

■Correct Answer:40%

1667. (4023) Q4-8276:

Which structures are disrupted in a lateral PIP joint dislocation?

1) Volar plate by itself

3) Volar plate, both collateral ligaments and central slip

2) Volar plate and both collateral ligaments

5) Volar plate and lateral bands

4) Volar plate and one collateral ligament

When a lateral PIP joint dislocation occurs, failure probably begins with disruption of the collateral ligament either from its origin on the head of the proximal phalanx or its insertion on the base of the middle phalanx. The injury then proceeds through the accessory collateral ligaments and terminates with disruption of the insertion of the volar plate on the middle phalangeal base.

■Correct Answer:Volar plate and one collateral ligament

1668. (4024) Q4-8277:

A 22-year old right-hand dominant male college student consumes a large amount of alcohol and falls asleep for eleven hours with his right arm over a chair. When he awakens, he is unable to feel the dorsum of his hand and cannot extend his elbow, wrist, or the metacarpalphalangeal joints of his affected arm. Initial management should consist of

1) Upper Extremity Angiography

3) Non-Steroidal Anti-Inflammatory Drugs (NSAIDs)

2) Observation

5) Emergent Nerve Exploration, with repair if necessary

4) Electromyography

Saturday night palsy is the term used for external compression of the radial nerve, and commonly occurs following the clinical scenario described above. The accepted treatment is observation, as Sunderland et al, described complete relief in all seven patients with Saturday night palsy after a period of observation without any adjunctive treatments. Although NSAIDs can be used if pain is a symptom or there is significant edema, these are not necessarily needed. Electromyography may be indicated if there is no improvement in symptoms after a three to six month period of observation. Emergent nerve exploration is indicated only for open fractures with a known radial nerve injury. There is no role for angiography in treatment of Saturday night palsy.

■Correct Answer:Observation

1669. (186) Q5-293:

Flumazenil can be administered to reverse the effects of which of the following drugs:

1) Propofol

3) Midazolam

2) Fentanyl

5) Methohexital

4) Ketamine

Midazolam is a benzodiazepine. Flumazenil is the only commercially available benzodiazepine antagonist. The recommended dose is 0.01 mg/kg every minute until reversed to a maximum dose of 1 mg.

Fentanyl is an opioid and is reversible with naloxone. Flumazenil is a benzodiazepine antagonist and has no effects on the effects of opioids.

Propofol, ketamine, and methohexital have no known antagonists. 

■Correct Answer:Midazolam

1670. (187) Q5-294:

A 7-year-old boy fell off the jungle gym and landed on his outstretched right arm sustaining a forearm fracture in both bones. The fracture requires reduction. He was given a combination of fentanyl and midazolam for sedation. At his current level of sedation, he is protecting his own airway. His oxygen saturation has dropped slightly to 92% but is stable. He does not display any awareness or discomfort when the fracture is being manipulated. How would you define his current level of sedation:

1) Analgesia

3) Deep sedation

2) C onscious sedation

5) Dissociative amnesia

4) General anesthesia

Deep sedation is the level where most fracture reductions are performed. Patients who are deeply sedated do not respond to verbal or noxious stimuli; they may display both a decreased ability to protect their airway and decreased respiratory drive.

Analgesia is defined as affecting the sensation of pain, however, there is no change in overall awareness.

C onscious sedation is defined as a lessening of awareness, with maintenance of protective reflexes. Patients will respond appropriately to commands and awaken to verbal stimuli. Patients do not respond to commands or noxious stimuli.

With general anesthesia, patients will not respond to verbal or noxious stimuli. Additionally, they lose all of their respiratory drive and ability to protect their airway.

 

■Correct Answer:Deep sedation

1671. (188) Q5-295:

A 14-year-old girl with a history of multiple food allergies and severe asthma was involved in a motor vehicle accident and sustained an isolated right femur fracture. Which of the following medications is the best choice to control her pain:

1) Ketamine

3) Methohexital

2) Morphine

5) Midazolam

4) Meperidine

The goal in this patient is to provide safe, effective, and long-acting analgesia. Meperidine is recommended in this circumstance. It is an opioid that provides intermediate and long-term analgesia. Additionally, it does not cause the associated histamine release and bronchospasm that can occur in patients with asthma and atopia.

Ketamine would provide short-term analgesia, but would also alter the level of consciousness.

Morphine is well known for precipitating bronchospasm in patients with atopia and asthma. Therefore, it would not be the best choice in the scenario presented.

Methohexital (a barbiturate) and midazolam (a benzodiazepine) both cause decreased awareness and have no analgesic properties.

 

■Correct Answer:Meperidine

1672. (189) Q5-296:

Which of the following choices is the best for sedating an otherwise healthy child for a fracture reduction:

1) Morphine, diazepam

3) Fentanyl, diazepam

2) Meperidine, midazolam

5) Fentanyl, midazolam

4) Demerol, phenergan, and thorazine

The combination of fentanyl and midazolam is the closest we have to an ideal drug combination for conscious sedation. Both drugs have commercially available antagonists. Fentanyl and midazolam are both quick-onset and short-duration drugs. When using these two drugs together, there is a significant risk of respiratory depression. It is important to monitor patients closely.

 

■Correct Answer:Fentanyl, midazolam

1673. (190) Q5-297:

A 3-year-old child presents with an obvious right lower extremity abnormality. The left lower extremity has normal alignment and joint function. The right lower extremity displays a severely short thigh with a flexed hip. The right foot and leg are also abnormal. Radiographs reveal a right proximal femur with no femoral head or neck, as well as acetabular dysplasia. There is also fibular hemimelia and two missing lateral rays of the foot. The parents want treatment to preserve the function of the normal leg. The most appropriate treatment is:

1) Observation

3) Van Nes rotation-plasty

2) Right Syme's amputation with knee fusion and prosthetic fitting

5) Equinus prosthesis

4) Femoral lengthening

According to the Aitken classification, the patient has a type C proximal femoral focal deficiency (PFFD). In addition to a severe leg length discrepancy, a dysfunctional foot and ankle are also present. The patient would be best suited with an operative procedure to allow the development of ambulatory skills. The Syme's amputation with knee fusion is the standard procedure for severe PFFD and allows the patient to become proficient in the use of a prosthesis at an early age.

In a child this young, a rotation-plasty may not be the best option due to a propensity to de-rotate and the weakness of the underdeveloped right ankle.

For severe PFFD with unstable or dysplastic joints, lengthening is not the best option. If the leg length discrepancy were predicted to be <20 cm and a hip joint were present, then lengthening would be a better choice.

If the child had a mild leg length discrepancy and was a candidate for lengthening, then an equinus prosthesis would be a reasonable choice until appropriate age for lengthening.

 

■Correct Answer:Right Syme's amputation with knee fusion and prosthetic fitting

1674. (191) Q5-298:

The most common associated abnormality with proximal femoral focal deficiency (PFFD) is:

1) Tibial hemimelia

3) Absent radius

2) Fibular hemimelia

5) C ardiac defects

4) C ongenital scoliosis

Up to 45% of proximal femoral focal deficiency (PFFD) cases have coexisting fibular hemimelia.

Tibial hemimelia may also have associated abnormalities of the distal femoral physis leading to a varus knee, but PFFD is more common.

Absent radius is typically associated with thrombocytopenia agenesis radius (TAR) syndrome. C ongenital scoliosis and cardiac defects are not commonly reported with PFFD.

 

■Correct Answer:Fibular hemimelia

1675. (192) Q5-299:

Osteogenesis imperfecta (OI) is caused by defects in:

1) C alcitonin

3) Type II collagen

2) Type I collagen

5) Osteoclasts

4) Type X collagen

Both quantitative and qualitative defects in type I collagen lead to the various types of osteogenesis imperfecta (OI). Several hundred different collagen I mutations have been found in patients with OI. The less severe forms of OI are caused by mutations in which the defective gene product is not incorporated into collagen, so that formation of cells using the unimpaired strands can continue.

■Correct Answer:Type I collagen

1676. (193) Q5-300:

Genetic transmission of osteogenesis imperfecta (OI) is best described as:

1) Autosomal dominant

3) Sex-linked dominant

2) Autosomal recessive

5) Not genetically transmitted

4) Autosomal dominant and autosomal recessive

Depending on the particular mutation involved, osteogenesis imperfecta can be either autosomal dominant or autosomal recessive. Osteogenesis imperfecta occurs because of a defect in type I collagen. The genes for type I collagen are found on chromosomes 7 and 17, and are therefore not sex-linked.

 

■Correct Answer:Autosomal dominant and autosomal recessive

1677. (194) Q5-301:

Which type of osteogenesis imperfecta (OI) is most likely to be confused with child abuse:

1) Type I

3) Type III

2) Type II

5) Type I and Type II

4) Type IV

Osteogenesis imperfecta (OI) type IV can be mild, with normal sclera, normal teeth, and variable levels of hearing impairment. Such cases can be easily mistaken for child abuse.

While mild forms of OI type I do exist, the presence of blue sclera in all patients throughout life, and a high percentage of hearing impairment and dentinogenesis imperfecta help to rule out a diagnosis of child abuse.

C hild abuse is not a consideration in OI type II because it is an extremely severe form of the disease and results in perinatal death.

OI type III is a severe progressively deforming form. Over one half of patients have fractures and deformities at birth. Short stature, kyphoscoliosis, and triangular faces help to distinguish OI type III from child abuse.

 

■Correct Answer:Type IV

1678. (195) Q5-302:

The most common neurologic complications of osteogenesis imperfecta (OI) is:

1) Intracranial hemorrhage

3) Brainstem compression

2) Subdural hematoma

5) Herniated nucleus pulposes

4) Mental retardation

Basilar impression with brainstem compression is a neurologic sequela of the deforming types of osteogenesis imperfecta (OI). It can be identified by noting that the tip of the dens projects 7 mm or more above McGregor's line on plain film.

■Correct Answer:Brainstem compression

1679. (196) Q5-303:

C riteria for diagnosis of neurofibromatosis 1 (NF1) include all the signs below except:

1) C afé-au-lait spots

3) Axillary or inguinal freckling

2) Lisch nodules

5) A distinctive osseous lesion

4) Neurologic deficit

While neurologic deficit may be associated with some of the spinal deformities in neurofibromatosis 1 (NF1), it does not constitute one of the diagnostic criteria.

The diagnostic criteria for NF1 were established by The C onsensus Development C onference on Neurofibromatosis at the National

Institutes of Health in 1987:

Six or more café-au-lait spots, at least 15 mm in diameter in adults and 5 mm in children. Two or more neurofibromas of any type or one plexiform neurofibroma.

Freckling in the axillae or inguinal regions (C rowe sign). Two or more iris hamartomas (Lisch nodules).

A distinctive osseous lesion, such as sphenoid dysplasia or thinning of long bone cortex, with or without pseudarthrosis. A first-degree relative with NF1 by the above criteria.

 

■Correct Answer:Neurologic deficit

1680. (197) Q5-304:

The most common osseous abnormality in neurofibromatosis 1 (NF1) is:

1) C ongenital tibial dysplasia

3) Valgus deformity of the ankle

2) Scoliosis

5) Dysplasia of posterior cranial fossa

4) Macrodactyly

Of the many orthopedic manifestations of neurofibromatosis 1 (NF1), including kyphoscoliosis, lordoscoliosis, spondylolisthesis, congenital tibial dysplasia, segmental hypertrophy, cystic bone lesions, and subperiostial bone proliferation, scoliosis is the most common.

 

■Correct Answer:Scoliosis

1681. (198) Q5-305:

The origin of "dumbbell lesions" found in radiographs of patients with neurofibromatosis 1 (NF1) is:

1) Flattening of the intervertebral disk with enlargement on the lateral borders

3) Neurofibromas or meningoceles that protrude through spinal foramina

2) Thinning of the midshaft of the tibia giving this bone the appearance of a dumbbell

5) Two closely spaced cysts within a bone

4) Subperiostial bone proliferation

Intraspinal lesions, such as neurofibromas and meningoceles, that protrude through the neural foramina give the radiologic appearance of a "dumbbell lesion."

Flattening of the intervertebral disk with enlargement of the lateral borders is not a feature of neurofibromatosis 1 (NF1). Tibial dysplasia seen in patients with NF1 lead to anterolateral bowing and does not give the appearance of a dumbbell. While subperiostial bone proliferation is seen in NF1, it does not give the appearance of a dumbbell.

Bone cysts are a recognized complication of NF1, but are not the origin of the dumbbell lesions seen on radiographs. 

■Correct Answer:Neurofibromas or meningoceles that protrude through spinal foramina

1682. (199) Q5-306:

Which of the following treatments is contraindicated as treatment for kyphoscoliosis in neurofibromatosis 1 (NF1):

1) Laminectomy over the apex of the kyphosis

3) Bracing

2) Halo traction

5) Posterior spinal fusion

4) Anterior spinal fusion

Laminectomy is contraindicated because the cord is usually compressed anteriorly and resection removes bone necessary for fusion.

■Correct Answer:Laminectomy over the apex of the kyphosis

1683. (200) Q5-307:

A 1-year-old girl presents with a right lower extremity abnormality. Her parents report that she has been attempting to stand, but she has not yet walked. C linically, she has a stiff, flexed, varus right knee with an obvious leg length discrepancy. Her ankle is also in a varus position. She does not spontaneously flex or extend the knee from its flexed position. Radiographs show that she has complete tibial hemimelia. The best choice of treatment at this time for the condition is:

1) Observation

3) Limb lengthening

2) Syme amputation

5) Brown procedure

4) Knee disarticulation

Knee disarticulation eliminates the malformed knee and ankle, allows the use of a prosthesis at an early age to promote ambulation development, and has good long-term results.

Observation is a poor option due to the severity of the deformity and the need for treatment to develop ambulation. Syme's amputation does not address the deformity of the knee.

In general, joint malformation or instability precludes lengthening procedures.

The Brown procedure centralizes the fibula at the knee and includes a Syme's amputation for the abnormal ankle. However, a functioning quadriceps is a prerequisite and there is a high likelihood of flexion contracture postoperatively.

 

■Correct Answer:Knee disarticulation

1684. (201) Q5-308:

All of the following are consistent with tibial hemimelia (TH) except:

1) Hypoplastic distal femur

3) Ankle varus

2) Absent extensor mechanism

5) Knee flexion contracture

4) Knee valgus

All of the answers are consistent with tibial hemimelia (TH) except for knee valgus. The knee is typically in varus due to a present fibula in TH. The foot is typically also in equinovarus, and the leg segment is shortened. Knee disarticulation is the best treatment for a complete TH.

■Correct Answer:Knee valgus

1685. (202) Q5-309:

A newborn boy presents with an abnormal right lower extremity. The right ankle is at the same level as the midshaft of the right tibia. The foot is also abnormal, and appears to be in a position of equinus and valgus. Radiographs confirm the equinovalgus of the foot, as well as absence of two lateral rays. There is also an absence of the fibula and anterolateral bowing of the tibia. Treatment of the lower extremity should consist of:

1) Trans-tibial amputation

3) Observation

2) Limb lengthening

5) Tibial osteotomy

4) Syme amputation

C urrently, the Syme's amputation (or modifications thereof) is the standard treatment for type II fibular hemimelia (FH). The procedure is usually delayed until the child is pulling to stand so that the child can begin walking with a prosthesis. The procedure has reports of excellent long-term results.

Trans-tibial amputation in a young child is not a good option due to secondary overgrowth of the distal limb residual and need for multiple revision surgeries.

Limb lengthening may be an option for a child with a mild type I fibular hemimelia with minimal foot and ankle deformity, however, this is not an option for type II FH because of the severe abnormality of the foot and ankle.

Observation as a form of treatment for a child with a type II FH will be unsuccessful because eventually surgical intervention will be needed due to the leg length discrepancy and prosthetic fitting issues.

Tibial osteotomy may occasionally be required for the tibial bowing, however, the bowing usually resolves on its own. This procedure does not address the deformity of the foot and ankle.

 

■Correct Answer:Syme amputation

1686. (203) Q5-310:

Fibular hemimelia (FH) can be associated with which of the following abnormalities:

1) Femoral shortening

3) Equinovalgus foot

2) Anterior cruciate ligament (AC L) deficiency

5) All of the above

4) Tarsal coalition

All of the stated abnormalities can be found with femoral hemimelia. It is estimated that 15% of cases have femoral deficiency. C ommonly seen in the condition are tarsal coalition, anterior cruciate ligament deficiency, and an equinovalgus foot.

■Correct Answer:All of the above

1687. (204) Q5-311:

A 7-year-old boy presents with bilateral high arches. His parents report that they are having difficulty finding shoes that comfortably fit him. The patient denies any foot pain. The father had similar problems with his feet and was diagnosed with a "mild" neurologic condition. On exam, the child has bilateral pes cavus with a supple hindfoot. Treatment of the feet at this time should consist of:

1) Soft tissue procedures alone

3) Triple arthrodesis

2) Soft tissue procedures and calcaneal osteotomy

5) Observation

4) Bracing

The child has a supple deformity secondary to C harcot-Marie-Tooth disease that will progress if untreated. Soft tissue procedures, which may consist of claw toe correction, plantar release, and possibly tendon transfer, are recommended for children younger than 8 years old who have a supple hindfoot.

The calcaneal osteotomy is reserved for patients with a rigid hindfoot.

Triple arthrodesis is a salvage procedure reserved for a fixed, painful foot in older children.

Bracing and observation are not preferred options due to the progressive nature of the disease, and the lack of ability to apply corrective forces to the foot in cavus.

 

■Correct Answer:Soft tissue procedures alone

1688. (205) Q5-312:

A 17-year-old man with C harcot-Marie-Tooth disease (C MT) presents with pain in his right foot. He has had no treatment for the foot in the past. On exam, he is noted to have a rigid pes cavus with hindfoot varus, as well as some weakness in the anterior tibialis and peroneal muscles. Radiographs display the above deformity with degenerative changes in the subtalar joint. Treatment of the foot should consist of:

1) Observation

3) Triple arthrodesis

2) Nonsteroidal anti-inflammatory drugs (NSAIDs)

5) Soft tissue release and calcaneal osteotomy

4) Soft tissue release and tendon transfers

The patient has a rigid, painful deformity with radiographic signs of arthritis. A triple arthrodesis is his best chance at a pain-free, plantigrade foot.

Observation will not solve his pain due to the deformity and degenerative changes in the foot.

Nonsteroidal anti-inflammatory drugs (NSAIDs) may help with his pain, however, the degeneration in the foot will continue to progress.

Because the patient has a rigid deformity, soft tissue procedures will not alleviate the pain.

 

■Correct Answer:Triple arthrodesis

1689. (206) Q5-313:

Which ancillary test is not helpful in the diagnosis of C harcot-Marie-Tooth disease (C MT):

1) Electromyography (EMG)

3) Nerve biopsy

2) Nerve conduction velocity (NC V)

5) Muscle enzymes

4) Muscle biopsy

C harcot-Marie-Tooth disease (C MT) is a neuropathic process resulting in muscle atrophy, therefore, muscle enzyme studies will not be helpful.

Electromyography (EMG) will confirm the diagnosis by displaying increased amplitude and duration of signals, both of which are indicative of a neuropathic process.

Nerve conduction velocity (NC V) will also confirm the diagnosis by displaying decreased motor and sensory conduction velocities.

Nerve biopsy can be helpful by showing loss of myelinated fibers and fibrosis. Muscle biopsy will show diffuse atrophy, fibrosis, and adipose tissue within muscle.

 

■Correct Answer:Muscle enzymes

1690. (207) Q5-314:

Which of the following is not a feature of the foot deformity in C harcot-Marie-Tooth disease (C MT):

1) Hindfoot valgus

3) Plantarflexed 1st metatarsal

2) Forefoot pronation

5) Interphalangeal (IP) joint flexion

4) Metatarsophalangeal (MTP) joint hyperextension

Hindfoot varus develops to counter forefoot pronation due to weakness of evertors with preservation of inverter muscle strength.

The first metatarsal plantarflexes relative to the other metatarsals, leading to pronation of the forefoot.

Plantarflexion of the first metatarsal occurs as part of the windlass mechanism as the intrinsics and plantar fascia contract. As the intrinsics weaken, the toe extensors pull the metatarsophalangeal (MTP) joint into hyperextension as part of the claw toe deformity.

When the MTP joint hyperextends, the strength of the long toe flexors pulls the interphalangeal joint into flexion contributing to the claw toe deformity.

 

■Correct Answer:Hindfoot valgus

1691. (368) Q5-491:

Which of the following etiologies is not thought to be associated with pseudarthrosis of the clavicle:

1) Prominent cervical ribs

3) C leidocranial dysplasia

2) Failure of fusion of the medial and lateral clavicular ossification centers

5) Stress lesion from dominant arm

4) Pressure from the higher position of the right subclavian artery

Several theories have been proposed to explain the rare phenomenon of isolated pseudarthrosis of the clavicle. The most accepted theory is pressure from the higher riding right subclavian artery. Pseudarthrosis has also been described in patients with prominent cervical ribs. Finally, some believe that this condition is caused by failure of fusion of the medial and lateral ossification centers of the clavicle. C leidocranial dysplasia may be associated with pseudarthrosis of the clavicle. There is no evidence that they are related to stress.

■Correct Answer:Stress lesion from dominant arm

1692. (369) Q5-493:

The most common presenting symptoms of congenital pseudarthrosis of the clavicle are:

1) Enlarging mass and history of pseudoparalysis at birth

3) Minimally painful or painless prominence with no history of trauma

2) Refusal to use arm, palpable defect, and pain

5) Weakness in the distribution of the upper trunk of the brachial plexus

4) Limitation of abduction to less than or equal to 90°

The most common presentation of pseudarthrosis of the clavicle is a painless mass in right the clavicle. There is no history of trauma, and the child uses the extremity normally, with minimal pain, and with no signs of instability.

There is no history of pseudoparalysis involved with congenital pseudarthrosis. Arm use is nearly normal, except for aching with activity.

There is minimal restriction of motion with the pseudarthrosis, which is one reason it is sometimes not diagnosed until later in childhood.

There has not been neurologic impairment with this condition.

 

■Correct Answer:Minimally painful or painless prominence with no history of trauma

1693. (370) Q5-494:

Which of the following statements regarding congenital pseudarthrosis of the clavicle is not true:

1) C ongenital pseudarthrosis of the clavicle occurs primarily on the right, rarely bilaterally, and when occurring on the left, congenital pseudarthrosis of the clavicle is usually associated with dextrocardia.

3) C ongenital pseudarthrosis of the clavicle is thought to occur due to pressure on the clavicle from the subclavian artery or prominent cervical ribs.

2) C ongenital pseudarthrosis of the clavicle is strongly associated with neurofibromatosis and patients often have other pseudarthroses, with the tibia being the most commonly affected site.

5) Impairment of the upper trunk of the brachial plexus rarely develops over time.

4) Treatment for congenital pseudarthrosis of the clavicle involves intercalary bone grafting, plate, and screws.

Pseudarthrosis of the clavicle occurs primarily on the right side. The right predominance has been attributed to pressure on the right clavicle from the subclavian artery and occasionally cervical ribs. Treatment is straightforward with intercalary bone grafting with plate and screws fixation. There is no association with neurofibromatosis and/or pseudarthrosis of the tibia.

Unlike pseudarthrosis of the tibia, congenital pseudarthrosis of the clavicle is rarely, if ever, associated with neurofibromatosis

Pressure from the subclavian artery or prominent cervical ribs have been used to explain the observation that the pseudarthrosis is almost always located on the right, and if bilateral, it is associated with bilateral cervical ribs

Bone graft with plate fixation is the usual treatment for these cases, although there have been reports of success in younger children from simply suturing the periosteum of the two ends together

Brachial plexus impairment rarely develops in the untreated pseudarthrosis.

 

■Correct Answer:Impairment of the upper trunk of the brachial plexus rarely develops over time.

1694. (373) Q5-497:

Which of the following laboratory findings are consistent with ankylosing spondylitis:

1) Elevated erythrocyte sedimentation rate (ESR), positive antinuclear antibody (ANA), negative rheumatoid factor (RF), and HLA- DR4

3) Elevated ESR, negative ANA, positive RF, and HLA-B27

2) Normal ESR, negative ANA, negative RF, and HLA-B27

5) Normal ESR, positive ANA, positive RF, and HLA-B27

4) Elevated ESR, negative ANA, negative RF, and positive HLA-B27

Diagnostic work-up for an inflammatory autoimmune condition should include an erythrocyte sedimentation rate (ESR), antinuclear antibody (ANA), rheumatoid factor (RF), haplotype, and Lyme titer. The laboratory results most consistent with ankylosing spondylitis are an elevated ESR at the time of an acute exacerbation, negative ANA and RF, and a haplotype of HLA- B27.

■Correct Answer:Elevated ESR, negative ANA, negative RF, and positive HLA-B27

1695. (376) Q5-500:

Which of the following clinical features distinguishes homocystinuria from Marfan syndrome:

1) Lens dislocation

3) C hest wall abnormalities

2) Scoliosis

5) Delayed intellectual development

4) Tall stature

Patients with Marfan syndrome do not typically have defects in intellectual functioning, while patients with homocystinuria typically do show signs of delayed intellectual development.

Patients with Marfan syndrome and homocystinuria both develop lens dislocations, scoliosis, chest wall abnormalities, and tall stature.

■Correct Answer:Delayed intellectual development

1696. (377) Q5-501:

Patients with homocystinuria undergoing lower extremity and spinal surgery must be warned of an increased risk of which complication:

1) Aortic root dissection

3) Venous thromboembolic disease

2) High output heart failure

5) Spontaneous pneumothorax

4) Prolonged ventilator dependence

Arterial and venous thromboembolic disease is common in patients with homocystinuria. Patients are at increased risk for this major complication when undergoing any surgical procedure.

Unlike patients with Marfan syndrome, patients with homocystinuria do not develop aortic root dilation, aneurysms, mitral valve prolapse with high output heart failure, or spontaneous pneumothoraces.

Underlying lung pathology is not a feature of homocystinuria, therefore, these patients are not at an increased risk for prolonged ventilatory support.

 

■Correct Answer:Venous thromboembolic disease

1697. (378) Q5-503:

What is the molecular disturbance responsible for the development of homocystinuria:

1) Translocation of chromosome 11 and 22

3) Methionine deficiency

2) Accumulation of cerebrosides in the reticuloendothelial system

5) Pyridoxine deficiency

4) Deficiency of cystathionine b-synthase

The metabolic disturbance responsible for homocystinuria is a deficiency of cystathionine ß-synthase.

A translocation of chromosome 11 and 22 is a feature of some patients with Ewingâs tumor. Accumulation of cerebrosides in the reticuloendothelial system is found in Gaucherâs disease.

Methionine is present in excessive quantities in homocystinuria because it cannot be converted to cysteine due to the deficiency of cystathionine ß-synthase.

Pyridoxine deficiency is a secondary feature of the disorder, but it is not the primary cause. 

■Correct Answer:Deficiency of cystathionine b-synthase

1698. (435) Q5-573:

Which form of chronic inflammatory arthritis is more common in boys than in girls?

1) Polyarticular

3) Systemic onset

2) Pauciarticular

5) Monoarticular

4) Seronegative spondyloarthropaty

Overall, juvenile rheumatoid arthritis (JRA) is much more common in girls. In pauciarticular JRA, the ratio is 4:1 female. In polyarticular JTA, it is 3:1 female, and in systemic JRA the ratio is 1:1. Seronegative spondylarthropathy is more common in males.

The incidence of polyarticular JRA is 3 times higher in girls than in boys. The incidence of pauciarticular JRA occurs 4 times more often in girls than in boys. Girls and boys are equally affected by systemic onset JRA.

■Correct Answer:Seronegative spondyloarthropaty

1699. (436) Q5-574:

Which of the following subtypes of juvenile rheumatoid arthritis (JRA) results in the highest risk of developing iritis:

1) Systemic JRA

3) Polyarticular JRA

2) Pauciarticular JRA

5) Eye involvement is not a problem in JRA

4) All are at approximately equal risk

Patients with pauciarticular juvenile rheumatoid arthritis (JRA) have the highest risk of developing iritis, cataracts, and blindness. Polyarticular JRA has the second highest risk. Patients with systemic JRA rarely develop iritis. All newly diagnosed JRA patients should be acutely evaluated and closely followed by an ophthalmologist.

Systemic-onset JRA has the lowest risk of uveitis There is a distinct difference among the types of JRA in terms of risk of eye involvement. Eye involvement is one of the problems physicians should be alert for in order to make an early diagnosis and prevent cataracts and blindness.

■Correct Answer:Pauciarticular JRA

1700. (437) Q5-575:

In the evaluation of a child with possible inflammatory arthritis, which tests should be ordered for possible juvenile rheumatoid arthritis:

1) C BC , ANA, RF, ESR, C RP, ophthalmology consult

3) C BC , echocardiogram, ANA, RF, and C RP

2) RF, C RP, ANA, ESR, and C BC

5) RF and synovial fluid analysis

4) HLA-typing, ophthalmology consult, ANA, RF, and C BC

Juvenile rheumatoid arthritis (JRA) is primarily a clinical diagnosis; however, routine screening tests should be ordered as part of a routine work-up. All patients should have a C BC , RF, ANA, ESR, C RP, and ophthalmology consult to look for eye involvement. An echocardiogram is not necessary unless a pericardial rub or other cardiac symptoms are present. HLA associations have been made with the different sub-types of the disease; however, this test is not necessary or diagnostic.

The C BC helps to rule out hematologic malignancy and assess the patientâs general health.

The ANA looks at the possibility of systemic lupus erythematosus and, if positive at low titer, heralds an increased risk of uveitis.

A positive RF helps rule in JRA and makes the risk of later erosive disease more likely.

ESR and C RP are helpful for monitoring disease activity. Ophthalmology consultation will help to rule out or detect uveitis at an early stage.

HLA typing is not indicated in the typical patient with JRA; it may be useful in the older male with axial symptoms. Synovial fluid analysis yields inflammatory fluid in this condition, but no information specific for the diagnosis.

■Correct Answer:C BC , ANA, RF, ESR, C RP, ophthalmology consult