SPR Unknown #73-- FINAL

Maria Calvo-Garcia, M.D.

Keywords

spr unknown 73, abs, amniotic band


Publication Date: 2012-08-03

History

24 week fetus with right upper limb abnormality.

Findings

Fetal Ultrasound (fig. 1-3) and Fetal MRI Fiesta images (fig. 4-6) demonstrate a relatively short constricting amniotic band extending from the placental surface, close to the placental cord insertion, to the right wrist. There is significant edema of the right hand. Color and Pulse Doppler showed flow distal to the area of constriction. Umbilical cord vessels Doppler evaluation (not shown) was normal.

Diagnosis

Amniotic band sequence

Discussion

Amniotic band sequence (ABS) is a collection of fetal malformations associated with fibrous bands that entangle or entrap various fetal parts in utero [1]. The bands are usually thin (2-4 mm) and sometimes undulating. The hands and feet are involved in 80% of cases. A wide variety of limb abnormalities can result, including amputation, acrosyndactyly, oligodactyly, clubfoot or simply soft tissue constriction. Other abnormalities associated with amniotic bands include craniofacial clefts, neural tube and body wall defects [2]. Umbilical cord constriction by amniotic bands is less frequent but with potential lethal consequences [3]. When there is limb constriction, the prenatal natural history of ABS is typically progressing form distal edema to in utero limb amputation. It has been postulated that if the bands are diagnosed early, the affected patients may potentially benefit from in utero lysis of these bands [4].

The diagnosis of ABS is based on sonographic visualization of either amniotic bands associated with fetal deformation or deformities in a “random” nonembryonic distribution [5], [6]. Fetal MRI has recently been described as an adjunct to prenatal US and a useful complementary tool in cases where fetal surgery is contemplated [7]

Files

References

  1. Seeds, J.W., R.C. Cefalo, and W.N. Herbert, Amniotic band syndrome. Am J Obstet Gynecol, 1982. 144(3): p. 243-8.
  2. Goldfarb, C.A., A. Sathienkijkanchai, and N.H. Robin, Amniotic constriction band: a multidisciplinary assessment of etiology and clinical presentation. J Bone Joint Surg Am, 2009. 91 Suppl 4: p. 68-75.
  3. Heifetz, S.A., Strangulation of the umbilical cord by amniotic bands: report of 6 cases and literature review. Pediatr Pathol, 1984. 2(3): p. 285-304.
  4. Keswani, S.G., et al., In utero limb salvage: fetoscopic release of amniotic bands for threatened limb amputation. J Pediatr Surg, 2003. 38(6): p. 848-51.
  5. Mahony, B.S., et al., The amniotic band syndrome: antenatal sonographic diagnosis and potential pitfalls. Am J Obstet Gynecol, 1985. 152(1): p. 63-8.
  6. Burton, D.J. and R.A. Filly, Sonographic diagnosis of the amniotic band syndrome. AJR Am J Roentgenol, 1991. 156(3): p. 555-8.
  7. Neuman, J., et al., Prenatal imaging of amniotic band sequence: utility and role of fetal MRI as an adjunct to prenatal US. Pediatr Radiol, 2012. 42(5): p. 544-51.

6 images