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Updated: May 15 2023

Constrictive Ring Syndrome (Streeter's Dysplasia)

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  • summary
    • Constriction Ring Syndrome, also known as Streeter's Dysplasia or Amniotic band syndrome, is a congenital condition that occurs when loose fibrous bands of ruptured amnion adhere to and entangle the normal developing structures of the fetus.
    • Diagnosis is made clinically at birth with the presence of bands perpendicular to the longitudinal axis of the digit or limb with normal anatomy proximal to the constriction ring. 
    • Treatment is usually the release of the bands or digital reconstruction depending on the severity of digital compromise.
  • Epidemiology
    • Incidence
      • 1:15,000 live births
    • Demographics
      • affects males and females equally
    • Anatomic location
      • occurs in hands and fingers 80% of the time
        • greater than 90% occur distal to wrist
  • Etiology
    • 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
        • syndactyly
      • nonorthopaedic
        • cleft lip
        • craniofacial defects
        • cardiac defect
        • Encephalocele
  • 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 IV
      • 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
  •  Extremity amputation 
  • Prognosis
    • Related to location and severity of constricting bands
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