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Updated: 7/2/2021

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
Flashcards (10)
Cards
1 of 10
Questions (5)

(SBQ17SE.33) Figure A is a clinical photo of a 2 year old male with a right hand abnormality. In addition to his right hand deformity, he has a similar appearance of his left hand. He also has a cleft palate, craniofacial defects, and bilateral club feet. His upper extremities are otherwise normal proximal to his wrists. The diagnosis was suspected after a routine ultrasound. What is the inheritance pattern of this condition?

QID: 211468
FIGURES:

Autosomal dominant

22%

(409/1846)

Autosomal recessive

18%

(330/1846)

X-linked recessive

8%

(139/1846)

X-linked dominant

3%

(49/1846)

Sporadic

49%

(907/1846)

L 4 A

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(SBQ13PE.90) You are consulted on a newborn male inpatient who presents with the clinical sign shown in Figure A. All of the following are commonly associated with this syndrome EXCEPT?

QID: 5267
FIGURES:

Bronchopulmonary dysplasia

23%

(412/1795)

Cardiac defects

7%

(117/1795)

Cleft palate

14%

(244/1795)

Encephalocele

31%

(552/1795)

Rigid talipes equinovarus

25%

(442/1795)

L 5 D

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

QID: 3431
FIGURES:

Circumferential trunk involvement is more common than distal extremities involvement

1%

(19/3608)

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

8%

(282/3608)

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

2%

(68/3608)

There is a strong correlation with anterolateral tibial bowing

1%

(51/3608)

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

88%

(3162/3608)

L 1 B

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(SBQ04PE.74) A 2-year-old infant presents to your nationally-renowned pediatric hand clinic with the hand deformities shown in Figure A. As the foremost expert in the field, you counsel the parents that which of the following is associated with this patient's condition:

QID: 2259
FIGURES:

Unremarkable gestational history

31%

(555/1782)

Anterolateral tibial bowing

7%

(118/1782)

Congenital oblique talus

2%

(33/1782)

Congenital vertical talus

14%

(247/1782)

Congenital talipes equinovarus

46%

(819/1782)

L 5 D

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