Sprengel's Deformity: Pathogenesis, Omovertebral Bone & Associated Syndromes | Part 33

Correct Answer: C

Rationale: Figure 13.5 description lists "Klippel–Feil’s syndrome" as one of the most common associated malformations. Options A, B, D, and E are not listed as commonly associated with Sprengel's deformity in the provided text. The main distractor, A, is a connective tissue disorder not directly linked here.

Correct Answer: C

Rationale: The text states that failure of descent can be caused by "great intrauterine pressure, due to either an increased or a diminished amount of amniotic fluid." Options A, B, D, and E are not mentioned as specific causes of intrauterine pressure leading to Sprengel's deformity in the provided text. The main distractor, E, is a general cause of congenital anomalies but not specifically linked to intrauterine pressure in this context.

Correct Answer: B

Rationale: Figure 13.3 description states, "The left scapula is elevated, and its size is smaller than that at the opposite side." Options A, C, D, and E contradict this observation. The main distractor, A, is the opposite of the correct finding.

Correct Answer: C

Rationale: The text describes the omovertebral bone as a "trapezoid-shaped structure of cartilage or bone." Options A, B, D, and E are incorrect compositions. The main distractor, A, is only one possible component if it's a fibrous band, but the structure itself is described as cartilage or bone.

Correct Answer: C

Rationale: Figure 13.5 description lists "absent or fused ribs" as one of the most common anomalies in the thoracic rib cage associated with Sprengel's deformity. Options A, B, D, and E are either unrelated conditions or not specifically listed as common associations in the provided text. The main distractor, B, is a chest wall deformity but not specifically listed as a common rib anomaly.

Correct Answer: D

Rationale: The definition states, "Sprengel’s deformity... is defined as a congenital abnormal elevation of one or both scapulas." Options A, B, C, and E are too restrictive or incorrect. The main distractor, C, is partially correct but misses the possibility of bilateral involvement.

Correct Answer: B

Rationale: The text states, "Second possible etiology is that the changes in the shape and size of the scapula are an arrest of development due to improper or defective muscular tension." Options A, C, D, and E are not mentioned as etiologies in the provided text. The main distractor, A, is a general cause of bone anomalies but not specifically mentioned here.

Correct Answer: C

Rationale: Figure 13.3 description states, "The asymmetry of the shoulder is caused by the upward and forward displacement of the scapula." Options A, B, D, and E are either unrelated conditions or not the primary cause of shoulder asymmetry in Sprengel's deformity as described. The main distractor, D, can be an associated anomaly but is not the direct cause of the shoulder asymmetry itself.

Correct Answer: D

Rationale: The text states the omovertebral bone "may be attached to the vertebral border of the scapula in a number of ways: by bony continuity, by cartilaginous union, or by a fibrous band." Muscular attachment is not listed as a type of union for the omovertebral bone itself. The main distractor, E, is incorrect because muscular attachment is not listed.

Correct Answer: C

Rationale: Figure 13.5 description lists "cervical spina bifida" as one of the common associated malformations. Options A, B, D, and E are either unrelated conditions or not specifically listed as common associations in the provided text. The main distractor, E, is a known cervical spine issue but not explicitly listed here.

Correct Answer: C

Rationale: The text outlines two main possibilities for pathogenesis: "first, that it is a failure of descent" and "Second possible etiology is that the changes in the shape and size of the scapula are an arrest of development due to improper or defective muscular tension." Options A, B, D, and E are not described as primary pathogenic theories in the provided text. The main distractor, D, is a general genetic concept but not the specific mechanism described.

Correct Answer: B

Rationale: Figure 13.3 description states, "The asymmetry of the shoulder is caused by the upward and forward displacement of the scapula." Options A, C, D, and E describe incorrect displacement directions. The main distractor, D, gets the upward component correct but the anterior-posterior incorrect.

Correct Answer: B

Rationale: Figure 13.5 description lists "chest-wall asymmetry" as one of the most common associated malformations. Options A, C, D, and E are either unrelated conditions or not specifically listed as common associations in the provided text. The main distractor, A, is a chest wall deformity but not specifically mentioned as a common association here.

Correct Answer: C

Rationale: Sprengel's deformity is defined as a congenital abnormal elevation of one or both scapulas from the normal anatomic position, precisely matching the clinical presentation. Winged scapula involves prominence of the medial border, not necessarily elevation. Scapulothoracic dissociation is a traumatic injury. Klippel-Feil syndrome is an associated condition, not the primary scapular deformity itself. Pectoralis major agenesis affects the chest wall and shoulder contour but not primarily scapular elevation.

Correct Answer: C

Rationale: The clinical text explicitly states that Sprengel's deformity "is often associated with other anomalies of the skeletal system and other organs." This makes option C the most accurate. Options A, B, D, and E contradict this fundamental characteristic of the deformity.

Correct Answer: C

Rationale: One of the possibilities for the pathogenesis of Sprengel's deformity is a failure of descent caused by "great intrauterine pressure, due to either an increased or a diminished amount of amniotic fluid." Options A, B, D, and E are not described as pathogenic mechanisms in the provided text.

Correct Answer: B

Rationale: The clinical text states, "One third of patients has omovertebral bone." This makes option B the correct answer. The omovertebral bone is a significant associated anomaly, but not universally present.

Correct Answer: C

Rationale: The text describes the omovertebral bone as a "trapezoid-shaped structure of cartilage or bone." This directly matches option C. Other options describe different shapes or compositions not consistent with the provided information.

Correct Answer: C

Rationale: The text states that the omovertebral bone "extends from the superomedial border of the scapula." This makes option C the correct origin point. The other options are incorrect anatomical locations for its origin.

Correct Answer: D

Rationale: The text specifies that the omovertebral bone extends "to the spinous processes, lamina, or transverse processes of the cervical spine." This directly corresponds to option D. The other options refer to incorrect vertebral regions or structures.

Correct Answer: B

Rationale: The text states that the omovertebral bone attaches to the cervical spine, "most commonly the fourth to seventh cervical vertebrae (C4-C7)." This makes option B the correct answer. The other options refer to incorrect cervical or thoracic levels.

Correct Answer: B

Rationale: The clinical context for Fig. 13.3 states, "The asymmetry of the shoulder is caused by the upward and forward displacement of the scapula." This directly matches option B. The image itself visually supports this description of the elevated and anteriorly positioned scapula.

Correct Answer: B

Rationale: The clinical context for Fig. 13.3 states, "The left scapula is elevated, and its size is smaller than that at the opposite side." This directly supports option B. The image visually depicts the affected scapula appearing smaller.

Correct Answer: A

Rationale: The clinical context for Fig. 13.3 states, "The scapula is rotated about the sagittal axis, bringing the upper medial angle away from the spinal column and the lower angle close to it." This description perfectly matches option A. The image helps visualize this rotational component.

Correct Answer: C

Rationale: The text lists "a defective musculature, unable to draw the scapula caudally" as a possible cause for the failure of descent. This directly supports option C. The other options are not mentioned as pathogenic mechanisms in the provided text.

Correct Answer: B

Rationale: The text states, "Second possible etiology is that the changes in the shape and size of the scapula are an arrest of development due to improper or defective muscular tension." This directly matches option B. The other options are not mentioned as etiologies in the provided text.

Correct Answer: C

Rationale: The clinical context for Fig. 13.5 lists "Klippel–Feil’s syndrome" as one of the most common associated anomalies involving the cervicothoracic vertebrae. The other options are either not listed as common associations or are not primarily cervicothoracic vertebral anomalies in this context.

Correct Answer: B

Rationale: The clinical context for Fig. 13.5 explicitly states, "The most common anomalies are absent or fused ribs, chest-wall asymmetry..." This directly supports option B. The other options are not listed as common associated thoracic rib cage anomalies in the provided text.

Correct Answer: C

Rationale: The clinical context for Fig. 13.5 lists "Klippel–Feil’s syndrome" as a common associated malformation. Klippel-Feil syndrome is characterized by congenital fusion of two or more cervical vertebrae, leading to a short neck and restricted motion, which aligns with the vignette. The other syndromes are not specifically listed as common associations with Sprengel's deformity in the text.

Correct Answer: B

Rationale: The clinical context for Fig. 13.5 lists "congenital scoliosis" as one of the most common associated malformations. The vignette describes a lateral curvature of the spine, which is characteristic of scoliosis. The other options are not listed as common associations with Sprengel's deformity in the provided text.

Correct Answer: B

Rationale: The clinical context for Fig. 13.5 lists "cervical spina bifida" as one of the most common associated malformations. Spina bifida involves a defect in the posterior elements of the vertebrae. The other options are not listed as common associations with Sprengel's deformity in the provided text.

Correct Answer: B

Rationale: The clinical context for Fig. 13.3 states, "The asymmetry of the shoulder is caused by the upward and forward displacement of the scapula." This directly translates to the affected shoulder appearing higher and more anterior, matching option B. The image visually confirms this characteristic asymmetry.