Updated: 5/22/2021

Poland Syndrome

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  • Introduction
    • A congenital disorder characterized by
      • unilateral chest wall hypoplasia
        • due to absence of sternocostal head of pectoralis major
      • hypoplasia of the hand and forearm
      • symbrachydactyly and shortening of middle fingers
        • result of absence or shortening of the middle phalanx
        • simple complete syndactyly of the short digits
  • Epidemiology
    • Incidence
      • 1 in 32,000 live births
  • Etiology
    • Pathophysiology
      • thought to be linked to subclavian artery hypoplasia
    • Associated conditions
      • occurs in 10% of syndactyly cases
  • Presentation
    • Physical exam
      • extent of hand and chest involvement varies
      • chest deformities
        • hypoplasia or absence of the pectoralis major, pectoralis minor, deltoid, serratus anterior, external oblique, and latissimus dorsi
        • Sprengel’s deformity
        • scoliosis
        • dextrocardia
        • absence or underdevelopment of the breast
      • hand deformities
        • syndactyly
        • hypoplasia or absence of metacarpals or phalanges
        • absence of extensors or flexor tendons
        • carpal coalition or hypoplasia
        • radioulnar synostosis
        • nail agenesis
  • Imaging
    • CT scan
      • will show absent perctoralis major
  • Treatment
    • Operative
      • syndactyly release
        • indications
          • performed in most patients
        • technique
          • complete syndactyly release produces skin deficiency that requires skin grafting
          • perform only one side of the digit at a time to avoid vascular complications
          • local flap is created for commisure reconstruction followed by interdigitating zigzag dorsal and palmar flaps along the medial and lateral aspect of the digit
  • Complications
    • Skin graft failure
    • Excessive tension
    • Improper flap planning
    • Digital artery injury
    • Web creep
    • Nail deformity

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(OBQ12.191) Which of following malformations is most commonly associated with Poland's syndrome?

QID: 4551
FIGURES:
1

Figure A

31%

(1534/4873)

2

Figure B

4%

(211/4873)

3

Figure C

16%

(793/4873)

4

Figure D

38%

(1856/4873)

5

Figure E

9%

(420/4873)

L 5 B

Select Answer to see Preferred Response

(OBQ04.152) Syndactyly is associated with all of the following conditions EXCEPT?

QID: 1257
1

Apert syndrome

3%

(111/3593)

2

Poland's syndrome

6%

(199/3593)

3

Holt-Oram syndrome

9%

(330/3593)

4

Carpenter syndrome

8%

(305/3593)

5

Tay-Sach's Disease

73%

(2630/3593)

L 3 C

Select Answer to see Preferred Response

Evidence (8)
VIDEOS & PODCASTS (1)
EXPERT COMMENTS (1)
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