Updated: 3/19/2018

[Blocked from Release] Constrictive Ring Syndrome

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Introduction
  • A malformation due to intrauterine rings or bands which constrict fetal tissue
    • the anatomy promximal to the constriction or amputation is normal
    • also referred to as Streeter dysplasia
  • Epidemiology
    • incidence
      • reported incidence varies between 1/1200 and 1/15000 live births
    • location
      • usually affects distal extremities
      • rare for only one ring to be present as an isolated malformation
    • risk factors
      • prematurity
      • maternal illness
      • low birth weight
      • drug exposure
  • Genetic
    • sporadic condition with no evidence of hereditary disposition
  • Pathoanatomy
    • exact etiology unknown but theories include
      • intrinsic anomaly in germ plasm resulting in the defects
      • intrauterine disruption during pregnancy
      • intrauterine trauma
  • Associated conditions 
    • club foot
      • most common
  • Prognosis
    • in rare cases, can cause limb amputation or death
Classification
 
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 patients get diagnosed at birth
  • Physical exam
    • check for distal pulses and perfusion
Imaging
  • Ultrasound 
    • intrauterine diagnosis can be made with ultrasound at end of first trimester 
Treatment
  • Operative
    • surgical release with multiple circumferential Z-plasties 
      • indications
        • if circulation is compromised by edema or limb has contour deformity
        • perform early (neonatal)
      • technique
        • acrosyndactyly is treated with distal release early in neonatal period
        • intrauterine band release can be done if limb is found to be at risk of amputation (rare)
 

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