Updated: 7/30/2017

Poland Syndrome

Topic
Review Topic
0
0
Questions
4
0
0
Evidence
5
0
0
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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  q 
      • result of absence or shortening of the middle phalanx 
      • simple complete syndactyly of the short digits
  • Epidemiology
    • 1 in 32,000 live births
    • occurs in 10% of syndactyly cases
  • Etiology
    • thought to be linked to subclavian artery hypoplasia
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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Questions (4)
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(OBQ12.191) Which of following malformations is most commonly associated with Poland's syndrome? Review Topic

QID: 4551
FIGURES:
1

Figure A

32%

(1330/4177)

2

Figure B

4%

(187/4177)

3

Figure C

17%

(699/4177)

4

Figure D

37%

(1543/4177)

5

Figure E

9%

(361/4177)

ML 5

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

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

QID: 1257
1

Apert syndrome

3%

(86/2867)

2

Poland's syndrome

5%

(153/2867)

3

Holt-Oram syndrome

9%

(254/2867)

4

Carpenter syndrome

8%

(225/2867)

5

Tay-Sach's Disease

74%

(2135/2867)

ML 3

Select Answer to see Preferred Response

PREFERRED RESPONSE 5
ARTICLES (5)
Topic COMMENTS (1)
Private Note