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Question 3541

Topic: 7. Hand and Wrist

A 45-year-old construction worker presents with chronic radial-sided wrist pain, weakness, and clicking following a fall onto an outstretched hand 5 years prior. Radiographs show advanced scapholunate advanced collapse (SLAC) wrist with severe radioscaphoid arthritis and preserved capitolunate joint. Conservative measures have failed. What is the most appropriate surgical treatment?

. Scapholunate ligament repair.
. Four-corner fusion (scaphoid excision and fusion of capitate, hamate, lunate, triquetrum).
. Proximal row carpectomy.
. Total wrist arthrodesis.
. Radial styloidectomy.

Correct Answer & Explanation

. Four-corner fusion (scaphoid excision and fusion of capitate, hamate, lunate, triquetrum).


Explanation

This patient has advanced SLAC wrist with severe radioscaphoid arthritis but a preserved capitolunate joint. Scapholunate ligament repair is only effective for acute, reducible injuries. Radial styloidectomy is insufficient. Total wrist arthrodesis is a salvage procedure for end-stage arthritis affecting all carpal joints, but it sacrifices all wrist motion. Proximal row carpectomy removes the diseased radioscaphoid joint but may lead to capitolunate arthritis over time. The most appropriate treatment for advanced SLAC wrist with preserved capitolunate articulation is a four-corner fusion (also known as scaphoid excision and capitolunate-hamate-triquetrum fusion). This procedure eliminates the painful radioscaphoid articulation, preserves a reasonable amount of wrist motion (primarily from the radiolunate joint), and is durable. It's preferred over total wrist fusion for patients who need to maintain some wrist motion.

Question 3542

Topic: 7. Hand and Wrist

A 50-year-old carpenter presents with chronic wrist pain and stiffness, particularly on the ulnar side, that has progressively worsened over 5 years. Radiographs show significant scapholunate advanced collapse (SLAC) wrist, characterized by a widened scapholunate gap, proximal migration of the capitate, and arthritic changes between the capitate and scaphoid, and radius and scaphoid (Stage II). Which of the following surgical procedures is the most appropriate option for symptomatic relief and functional improvement in this condition?

. Scapholunate ligament repair.
. Proximal row carpectomy (PRC).
. Total wrist arthrodesis.
. Scaphoid excision and four-corner fusion (4CF).
. Radioscapholunate arthrodesis.

Correct Answer & Explanation

. Scaphoid excision and four-corner fusion (4CF).


Explanation

For SLAC wrist Stage II, where there is arthritis between the scaphoid and capitate, and between the scaphoid and radius, but the radiolunate joint is preserved, Scaphoid Excision and Four-Corner Fusion (4CF), also known as triscaphe fusion, is a common and effective surgical option. This procedure involves excising the diseased scaphoid and fusing the capitate, hamate, triquetrum, and lunate. This provides a stable, pain-free midcarpal joint while preserving some wrist motion through the radiolunate articulation. Scapholunate ligament repair is for acute or subacute instability before arthritic changes. Proximal row carpectomy (PRC) is an alternative but is contraindicated if there are significant lunate or capitate chondral changes. Total wrist arthrodesis is a salvage procedure that eliminates all wrist motion. Radioscapholunate arthrodesis would fuse the radiolunate joint, which is still healthy in SLAC II.

Question 3543

Topic: 7. Hand and Wrist

A patient sustains a severe laceration to the anterior wrist, completely transecting the median nerve 2 cm proximal to the carpal tunnel. Despite the complete median nerve laceration, the patient retains a robust ability to oppose the thumb. Which of the following neural anomalies best explains this physical exam finding?

. Martin-Gruber anastomosis
. Marinacci anastomosis
. Riche-Cannieu anastomosis
. Berrettini anastomosis
. Linburg-Comstock anomaly

Correct Answer & Explanation

. Riche-Cannieu anastomosis


Explanation

The Riche-Cannieu anastomosis is a neural connection between the deep branch of the ulnar nerve and the recurrent motor branch of the median nerve in the hand. This anomaly allows ulnar nerve innervation of typical median-innervated thenar muscles (e.g., opponens pollicis), preserving thumb opposition despite a high median nerve injury. Martin-Gruber is a median-to-ulnar anastomosis in the forearm.

Question 3544

Topic: 7. Hand and Wrist

A 28-year-old male falls on an outstretched hand and complains of radial-sided wrist pain. Radiographs reveal a scapholunate gap of 4 mm and a scaphoid 'ring sign.' If left untreated, the carpal kinematics will become chronically altered. In this ensuing condition (DISI), what are the resultant resting positions of the scaphoid and lunate, respectively?

. Scaphoid flexed, Lunate flexed
. Scaphoid extended, Lunate flexed
. Scaphoid flexed, Lunate extended
. Scaphoid extended, Lunate extended
. Both bones remain neutral but translate ulnarly

Correct Answer & Explanation

. Scaphoid flexed, Lunate extended


Explanation

Scapholunate dissociation disrupts the scapholunate interosseous ligament. Normal carpal kinematics dictate that the scaphoid tends to flex and the triquetrum tends to extend. When the SL ligament tears, the scaphoid falls into pathologic flexion (volar tilt), while the lunate follows the intact lunotriquetral ligament into extension (dorsal tilt). This creates a Dorsal Intercalated Segment Instability (DISI) pattern.

Question 3545

Topic: 7. Hand and Wrist

A newborn presents with disproportionate short stature, postaxial polydactyly, and hypoplastic nails. An echocardiogram reveals an atrial septal defect. Radiographs show progressive distal shortening of the limbs (acromesomelia). What is the most likely diagnosis?

. Achondroplasia
. Ellis-van Creveld syndrome
. Jeune syndrome
. Metatropic dysplasia
. Cleidocranial dysplasia

Correct Answer & Explanation

. Ellis-van Creveld syndrome


Explanation

Ellis-van Creveld syndrome (chondroectodermal dysplasia) is characterized by short limbs, postaxial polydactyly, ectodermal defects (nail/tooth dysplasia), and congenital heart disease, most commonly a single atrium or ASD.

Question 3546

Topic: 7. Hand and Wrist

An infant presents with a remarkably narrow, bell-shaped thorax, short limbs, and polydactyly. Pelvic radiographs display a trident appearance of the acetabulum with spurs. The infant requires mechanical ventilation. Mutations affecting which cellular structure are most commonly responsible for this phenotype?

. Lysosomes
. Mitochondria
. Primary cilia
. Endoplasmic reticulum
. Golgi apparatus

Correct Answer & Explanation

. Primary cilia


Explanation

Asphyxiating thoracic dystrophy (Jeune syndrome) presents with a narrow, rigid thorax leading to severe respiratory distress. It belongs to the family of short-rib polydactyly syndromes, which are uniformly caused by defects in the primary cilia (ciliopathies).

Question 3547

Topic: 7. Hand and Wrist

A neonate is born with extremely short limbs, postaxial polydactyly, and a remarkably narrow, bell-shaped thorax. Radiographs show irregular metaphyses and handlebar-shaped clavicles. What is the most common cause of early mortality in this syndrome?

. Congenital heart block
. Renal failure
. Respiratory failure
. Hepatic cirrhosis
. Spinal cord compression

Correct Answer & Explanation

. Respiratory failure


Explanation

Asphyxiating thoracic dysplasia (Jeune syndrome) is characterized by a narrow thorax, short limbs, and metaphyseal irregularities. The severely restricted rib cage causes pulmonary hypoplasia, making respiratory failure the leading cause of early mortality.

Question 3548

Topic: 7. Hand and Wrist



A 45-year-old woman presents with the hand deformity shown, alongside dysphagia and severe cold intolerance in her fingers. Which autoantibody is most highly specific for her likely underlying condition?

. Anti-dsDNA
. Anti-centromere
. Anti-Jo-1
. Anti-Ro (SSA)
. Rheumatoid factor

Correct Answer & Explanation

. Anti-centromere


Explanation

The image and clinical vignette describe CREST syndrome (Limited Cutaneous Systemic Sclerosis), characterized by calcinosis cutis, Raynaud's, esophageal dysmotility, sclerodactyly, and telangiectasia. Anti-centromere antibodies are highly specific for CREST syndrome.

Question 3549

Topic: 7. Hand and Wrist

A 45-year-old female presents with tightening of the skin over her fingers, dysphagia, and painful calcium deposits in her digits. Hand radiographs are obtained.

Which autoantibody is most commonly associated with this patient's specific syndromic presentation?

. Anti-cyclic citrullinated peptide (Anti-CCP)
. Anti-dsDNA
. Anti-Ro (SSA)
. Anti-centromere
. Anti-Scl-70 (Anti-topoisomerase I)

Correct Answer & Explanation

. Anti-centromere


Explanation

This patient has CREST syndrome (limited cutaneous systemic sclerosis), characterized by Calcinosis cutis, Raynaud's, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. Anti-centromere antibodies are highly specific for CREST syndrome.

Question 3550

Topic: 7. Hand and Wrist
A 4-year-old girl is diagnosed with multifocal Langerhans cell histiocytosis. She presents with lytic skull lesions and exophthalmos. What endocrine abnormality is classically associated with this clinical triad?
. Hypothyroidism
. Hyperparathyroidism
. Diabetes insipidus
. Cushing syndrome
. Precocious puberty

Correct Answer & Explanation

. Diabetes insipidus


Explanation

The classic Hand-Schüller-Christian triad of multifocal LCH includes lytic skull lesions, exophthalmos, and diabetes insipidus. The diabetes insipidus results from histiocytic infiltration of the pituitary stalk or hypothalamus.

Question 3551

Topic: 7. Hand and Wrist

A 42-year-old woman presents with tightening of the skin on her face and hands, Raynaud's phenomenon, and dysphagia. She notes painful ulcerations at the tips of her fingers.

Which of the following antibodies is associated with an increased risk of developing pulmonary arterial hypertension in this specific disease subset?

. Anti-Scl-70
. Anti-centromere
. Anti-Jo-1
. Anti-Ro/SSA
. Anti-Sm

Correct Answer & Explanation

. Anti-centromere


Explanation

Anti-centromere antibodies are highly associated with the limited cutaneous form of systemic sclerosis (CREST syndrome). This specific subset has a significantly increased risk of developing isolated pulmonary arterial hypertension compared to patients with diffuse disease.

Question 3552

Topic: 7. Hand and Wrist
A 4-year-old child presents with a triad of exophthalmos, diabetes insipidus, and multiple lytic skull lesions. This classic clinical triad is indicative of which variant of Langerhans cell histiocytosis?
. Eosinophilic granuloma
. Hand-Schüller-Christian disease
. Letterer-Siwe disease
. Hashimoto-Pritzker disease
. Erdheim-Chester disease

Correct Answer & Explanation

. Hand-Schüller-Christian disease


Explanation

Hand-Schüller-Christian disease is a chronic disseminated form of LCH. It is classically characterized by the clinical triad of diabetes insipidus, exophthalmos, and lytic bone lesions of the skull.

Question 3553

Topic: 7. Hand and Wrist

A child from an isolated Amish community presents with disproportionate short stature, postaxial polydactyly of the hands, and dysplastic nails. Which of the following cardiac defects is most classically associated with this specific skeletal dysplasia?

. Tetralogy of Fallot
. Ventricular septal defect
. Common atrium or Atrial septal defect (ASD)
. Coarctation of the aorta
. Transposition of the great arteries

Correct Answer & Explanation

. Common atrium or Atrial septal defect (ASD)


Explanation

Ellis-van Creveld syndrome (Chondroectodermal Dysplasia) features short limbs, postaxial polydactyly, and nail/teeth dysplasia. Cardiac anomalies occur in 50-60% of patients, classically a single atrium or ASD.

Question 3554

Topic: 7. Hand and Wrist

A newborn presents with disproportionate short stature, postaxial polydactyly, hypoplastic nails, and a congenital heart defect. Which cardiac anomaly is most frequently associated with this specific skeletal dysplasia?

. Tetralogy of Fallot
. Common atrium (single atrium)
. Coarctation of the aorta
. Transposition of the great arteries
. Ventricular septal defect

Correct Answer & Explanation

. Common atrium (single atrium)


Explanation

Ellis-van Creveld syndrome (chondroectodermal dysplasia) is an autosomal recessive condition commonly found in the Amish population. It is strongly associated with cardiac defects, most notably a common atrium or atrial septal defects.

Question 3555

Topic: 7. Hand and Wrist

A neonate is evaluated for disproportionate short stature, postaxial polydactyly of the hands, and dysplastic fingernails. An echocardiogram is ordered due to a high association of a specific congenital heart defect with this syndrome. Which cardiac defect is most classically associated with Ellis-van Creveld syndrome?

. Tetralogy of Fallot
. Transposition of the great arteries
. Ventricular septal defect
. Atrial septal defect (common atrium)
. Coarctation of the aorta

Correct Answer & Explanation

. Atrial septal defect (common atrium)


Explanation

Ellis-van Creveld syndrome (chondroectodermal dysplasia) typically presents with short stature, postaxial polydactyly, and ectodermal defects (nails/teeth). Nearly 50-60% of patients have congenital heart disease, most commonly an atrial septal defect or a single atrium.

Question 3556

Topic: 7. Hand and Wrist

A patient presents with a distal humerus fracture. During the initial physical examination, you note an inability to extend the wrist and fingers, along with sensory loss in the dorsal forearm and hand. Which nerve injury is most likely?

. Ulnar nerve
. Median nerve
. Radial nerve
. Musculocutaneous nerve
. Anterior interosseous nerve

Correct Answer & Explanation

. Radial nerve


Explanation

Inability to extend the wrist and fingers (wrist drop, finger drop) along with sensory loss in the dorsal forearm and hand is pathognomonic for radial nerve injury. While the radial nerve is more commonly injured with humeral shaft fractures, it can be affected by high-energy distal humerus fractures or iatrogenically during lateral surgical approaches. Ulnar nerve injury typically affects intrinsic hand muscles, sensation on the ulnar side. Median nerve injury affects forearm pronation, wrist flexion, thumb opposition, and sensation in the radial 3.5 digits. Musculocutaneous nerve injury affects elbow flexion. Anterior interosseous nerve affects deep forearm flexors and does not cause sensory loss.

Question 3557

Topic: Nerve & Tendon

A patient with a distal humerus fracture and persistent ulnar nerve palsy post-operatively shows no signs of improvement after 6 weeks. What is the most appropriate next step in management?

. Continue observation for up to 6 months
. Start high-dose oral corticosteroids
. Perform a diagnostic ultrasound of the nerve
. Consider surgical exploration and anterior transposition of the ulnar nerve
. Refer for psychological counseling for chronic pain

Correct Answer & Explanation

. Consider surgical exploration and anterior transposition of the ulnar nerve


Explanation

Persistent ulnar nerve palsy after 6 weeks post-operatively, especially if it was noted acutely and has not shown signs of recovery, warrants consideration of surgical exploration and anterior transposition of the ulnar nerve. This is to address potential nerve entrapment by scar tissue, hematoma, or hardware, or to rule out nerve laceration. While some neuropraxias can recover over months, 6 weeks without any improvement is a strong indication for intervention, as further delay can lead to irreversible nerve damage. Observation for 6 months is too long. Corticosteroids are not indicated. Ultrasound can be helpful but surgical exploration is definitive. Psychological counseling is premature.

Question 3558

Topic: Nerve & Tendon

When performing a posterior approach with an olecranon osteotomy, the ulnar nerve is typically identified and protected. What is the usual fate of the ulnar nerve after fracture fixation?

. It is resected to prevent future entrapment.
. It is always left in situ in the cubital tunnel.
. It is commonly transposed anteriorly, either subcutaneously or submuscularly.
. It is repaired to the triceps muscle.
. It is rarely identified, as it is deep to the bone.

Correct Answer & Explanation

. It is commonly transposed anteriorly, either subcutaneously or submuscularly.


Explanation

After identification and protection during distal humerus fracture surgery, the ulnar nerve is commonly transposed anteriorly. This is done to prevent potential entrapment in scar tissue, hardware, or malunion post-operatively, as well as to accommodate for any changes in the cubital tunnel anatomy during fixation. It can be transposed subcutaneously or submuscularly (under the flexor-pronator mass). Resecting the nerve is highly detrimental. Leaving it in situ risks entrapment. It is definitely identified as it's superficial in the cubital tunnel. It is not repaired to the triceps.

Question 3559

Topic: Nerve & Tendon

What is the primary goal of restoring the carrying angle of the elbow during distal humerus fracture repair?

. To improve cosmetic appearance
. To prevent ulnar nerve compression
. To ensure proper biomechanics and function of the elbow joint
. To facilitate early wound healing
. To reduce the risk of heterotopic ossification

Correct Answer & Explanation

. To ensure proper biomechanics and function of the elbow joint


Explanation

The carrying angle is the slight valgus angle formed by the long axis of the humerus and the ulna when the arm is extended and supinated. Restoring the physiological carrying angle during distal humerus fracture repair is crucial for ensuring proper biomechanics and function of the elbow joint. Failure to do so can lead to cubitus varus or cubitus valgus deformities, causing instability, pain, altered gait mechanics, and potentially delayed ulnar nerve palsy (cubitus valgus). While cosmetics are a factor, biomechanics and function are paramount. Ulnar nerve compression is a risk of cubitus valgus, not the sole reason for restoration. It doesn't directly affect wound healing or HO risk.

Question 3560

Topic: Nerve & Tendon

In the setting of a distal humerus fracture, which aspect of ulnar nerve management is typically not part of standard practice for a posterior approach?

. Identification and isolation of the nerve
. Intraoperative nerve monitoring (e.g., EMG)
. Routine primary repair of the nerve
. Anterior transposition if it's at risk of impingement or re-entrapment
. Gentle handling and protection throughout the procedure

Correct Answer & Explanation

. Routine primary repair of the nerve


Explanation

Routine primary repair of the ulnar nerve is typicallynotpart of standard practice unless there is a clear transection or severe laceration. For most cases, the nerve is identified, isolated, gently handled, protected, and often transposed anteriorly if at risk. Intraoperative nerve monitoring is increasingly used in complex cases. The goal is to prevent injury, and if a neuropraxia occurs, to manage it with observation or neurolysis/transposition, not primary repair unless completely severed.