Constrictive Ring Syndrome

Introduction

A malformation due to intrauterine rings or bands which constrict fetal tissue
- the anatomy promximal to the constriction or amputation is normal
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)

TAG

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

Review Topic

FIGURES: A

1. Circumferential trunk involvement is more common than distal extremities involvement
2. Risk factors include late gestation (>44 weeks) and high birth weight (>3500g)
3. Incomplete circumferential bands not directly interfering with lymphatic circulation should be resected
4. There is a strong correlation with anterolateral tibial bowing
5. Complete circumferential bands that interfere with lymphatic drainage can be treated with band excision and z-plasty.

PREFERRED RESPONSE ▶

DISCUSSION: The image and vignette describe a patient with constriction band syndrome (CBS). In the case of lymphatic obstruction or vascular compromise, the treatment of CBS is band excision. There are many terms used to describe this phenomenon. However, the etiology is the entanglement of fetal parts in the amniotic membrane.

Foulkes et al reviewed 71 cases of congenital constriction band syndrome (CCBS). They found the average patient had three involved limbs, with a predilection for distal, central digits of the upper extremity. There was a strong correlation with abnormal gestation and clubfoot. Treatment included distraction osteogenesis and free osteocutaneous transfer.

Goldfarb et al reviewed amniotic constriction band syndrome (ABS), highlighting its association with annular constriction of multiple extremities. They classified ABS into classic (disruptions and deformations) and non-classic (malformations). ABS is due to disruptions (amputations, acrosyndactyly), deformations (oligohydraminos, scoliosis, talipes equinovarus) and malformations (body-wall defects, cleft lip/palate). As there is moderate overlap between the classic and nonclassic, additional research into the underlying cause is being investigated.

Green described a one-stage release of circumferential constriction bands in three patients. The advantages of this technique are the decreased need for anesthesia and subsequent procedures as well as facilitating postoperative care.

Kawakura et al reviewed the intrinsic and extrinsic theories of (CBS). The most common manifestations are distal extremity involvement, intrauterine amputations and acrosyndactyly. Excision of bands and mobilization of subcutaneous adipose tissue as described by Upton is seen in Illustration A.

Incorrect Answers:

Answer 1: Distal extremities are more affected than the trunk
Answer 2: Risk factors include low birth weight (<2500g), prematurity (<37wks), maternal drug exposure, trauma during pregnancy and attempted fetal termination during the first trimester.
Answer 3: Shallow bands that do not interfere with circulation or lymphatic drainage do not need to be released.
Answer 4: There is a strong correlation with clubfoot, not anterolateral bowing.

Illustrations: A

REFERENCES:

1. Foulkes GD, Reinker K. Congenital constriction band syndrome: a seventy-year experience. J Pediatr Orthop. 1994 Mar-Apr;14(2):242-8. PMID:8188842 (Link to Abstract)

2. Goldfarb CA, Sathienkijkanchai A, Robin NH. Amniotic constriction band: a multidisciplinary assessment of etiology and clinical presentation. J Bone Joint Surg Am. 2009 Jul;91 Suppl 4:68-75. PMID:19571071 (Link to Abstract)

3. Greene WB. One-stage release of congenital circumferential constriction bands. J Bone Joint Surg Am. 1993 May;75(5):650-5. PMID:8501079 (Link to Abstract)

4. Kawamura K, Chung KC. Constriction band syndrome. Hand Clin. 2009 May;25(2):257-64. Review. PubMed PMID: Kawamura K, Chung KC. Constriction band syndrome. Hand Clin. 2009 May;25(2):257-64. PMID:19380064 (Link to Abstract)

Question Authors:

Terrill Julien MD, John Badylak MD, Ben Taylor MD,

Please Rate Educational Value!

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Average 3.0 of 8 Ratings

Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions, Personal Observations, and Biomechanic Studies)

Greene WB. One-stage release of congenital circumferential constriction bands. J Bone Joint Surg Am. 1993 May;75(5):650-5. PMID:8501079 (Link to Abstract)
Kawamura K, Chung KC. Constriction band syndrome. Hand Clin. 2009 May;25(2):257-64. Review. PubMed PMID: Kawamura K, Chung KC. Constriction band syndrome. Hand Clin. 2009 May;25(2):257-64. PMID:19380064 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012

Undefined

Foulkes GD, Reinker K. Congenital constriction band syndrome: a seventy-year experience. J Pediatr Orthop. 1994 Mar-Apr;14(2):242-8. PMID:8188842 (Link to Abstract)
Goldfarb CA, Sathienkijkanchai A, Robin NH. Amniotic constriction band: a multidisciplinary assessment of etiology and clinical presentation. J Bone Joint Surg Am. 2009 Jul;91 Suppl 4:68-75. PMID:19571071 (Link to Abstract)

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