Updated: 3/19/2018

Constrictive Ring Syndrome (Streeter's Dysplasia)

Topic
Review Topic
0
0
Questions
2
0
0
Evidence
5
0
0
https://upload.orthobullets.com/topic/6082/images/40_moved.JPG
https://upload.orthobullets.com/topic/6082/images/40_moved.JPG
https://upload.orthobullets.com/topic/6082/images/type 1 abs.jpg
https://upload.orthobullets.com/topic/6082/images/abs photo2.jpg
https://upload.orthobullets.com/topic/6082/images/abs_hand_2 photo.jpg
https://upload.orthobullets.com/topic/6082/images/abs photo.jpg
Introduction
  • Amniotic band syndrome occurs when loose fibrous bands of ruptured amnion adhere to and entangle the normal developing structures of the fetus.
    • also referred to as
      • amniotic disruption sequence 
      • constriction ring syndrome
      • premature amnion rupture sequence
      • Streeter's dysplasia
  • Epidemiology
    • incidence
      • 1:15,000 live births
    • demographics
      • affects males and females equally
    • location
      • occurs in hands and fingers 80% of the time 
        • greater than 90% occur distal to wrist
  • Pathophysiology
    • no firmly established etiology 
    • most accepted theory is that the disrupted amnion releases fibrous membranous strands which wrap around the developing limb in a circumferential fashion  
  • Genetics
    • inheritance pattern
      • sporadic and not hereditary
  • Associated conditions 
    • orthopaedic
      • clubfoot 
      • syndactyly 
    • nonorthopaedic
      • cleft palate
      • cleft lip
      • craniofacial defects
  • Prognosis
    • related to location and severity of constricting bands
Classification
 
Patterson Classification
Type I  • Simple constriction ring
 
Type II  • Deformity distal to ring (hypoplasia, lymphedema)
 •  Edema may or may not be present


Type III  • Fusions distally (syndactyly, acrosyndactyly)
Type D  • Amputation
 
Degrees of Constrictive Ring Syndrome
Simple constriction rings Mild ring with no distal deformity or lymphedema  
Rings with distal deformity Ring may cause distal lymphedema in association with deformity  
Acrosyndactyly Fusion between the more distal portions of the digits with the space between the digits varying from broad to pinpoint in size.

Amputations Loss of limb distal to ring
 
Presentation
  • Symptoms
    • most patient asymptomatic and diagnosed at birth
  • Physical exam
    • normal anatomy proximal to constriction ring
    • bands perpendicular to longitudinal axis of the digit or limb
      • most common presentation
    • central digits more commonly affected
    • amputations distal to constriction site can be found
    • when no amputations present look for
      • secondary syndactyly
      • bony fusions
        • may observe sinus tracts proximally between digits
Imaging
  • Ultrasound 
    • intrauterine diagnosis can be made with ultrasound at end of first trimester 
Treatment
  • Nonoperative
    • observation
      • indications
        • Type I (simple constriction ring)
  • Operative
    • excision or release of constriction band
      • indications
        • Type I with compromise of digital circulation
    • circumferential Z-plasties 
      • indications
        • Type II 
          • distal deformities present 
    • surgical release of syndactyly
      • indications
        • Type III with distal fusions
    • reconstruction of involved digits or limb (i.e., lengthening of bone, deepening of web space)
      • indications
        • Type IV to improve function
Complications
 
 

Please rate topic.

Average 2.9 of 8 Ratings

Thank you for rating! Please vote below and help us build the most advanced adaptive learning platform in medicine

The complexity of this topic is appropriate for?
How important is this topic for board examinations?
How important is this topic for clinical practice?
Questions (2)
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(OBQ11.8) Figure A depicts a child with a congenital abnormality. Which of the following is true regarding this condition? Review Topic

QID: 3431
FIGURES:
1

Circumferential trunk involvement is more common than distal extremities involvement

0%

(11/2651)

2

Risk factors include late gestation (>44 weeks) and high birth weight (>3500g)

7%

(197/2651)

3

Incomplete circumferential bands not directly interfering with lymphatic circulation should be resected

2%

(46/2651)

4

There is a strong correlation with anterolateral tibial bowing

1%

(29/2651)

5

Complete circumferential bands that interfere with lymphatic drainage can be treated with band excision and z-plasty.

89%

(2349/2651)

ML 1

Select Answer to see Preferred Response

PREFERRED RESPONSE 5
ARTICLES (5)
Topic COMMENTS (0)
Private Note