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.
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.
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)
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)