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Circumferential trunk involvement is more common than distal extremities involvement
1%
24/3946
Risk factors include late gestation (>44 weeks) and high birth weight (>3500g)
8%
314/3946
Incomplete circumferential bands not directly interfering with lymphatic circulation should be resected
2%
91/3946
There is a strong correlation with anterolateral tibial bowing
59/3946
Complete circumferential bands that interfere with lymphatic drainage can be treated with band excision and z-plasty.
87%
3428/3946
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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.
4.2
(21)
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