Streeter's dysplasia (amniotic band syndrome)
Objectives- Define Streeter's dysplasia (amniotic band syndrome)
- Describe the etiology of Streeter's dysplasia
- Describe the clinical spectrum of Streeter's dysplasia
- Describe treatment for Streeter's dysplasia
- Why are clubfeet associated with more proximal amniotic bands so difficult to treat?
Discussion
The terms, Streeter's dysplasia and amniotic band syndrome, are used interchangably to describe the condition of constricting bands around the limbs. It appears that the insult occurs very early in pregnancy, probably prior to 26 days, and is related to vascular supply. Associated defects with amniotic band syndrome include hydranencephaly, porencephaly, craniofacial abnormalities, and spinal dysraphism. These defects are compatible with malmigration of cephalic neural crest tissue which occurs at the same postconception time as development of amniotic band syndrome. Fetal hypoperfusion has been experimentally linked to this constellation of defects. The clinical appearance of amniotic band syndrome in the extremities is a constricting band which results in amputation in its severe forms, distal edema in moderate forms, or a constricting band without distal deficit in mild forms. Clubfeet associated with the more severe forms of amniotic band syndrome are very difficult to treat successfully. The bands can penetrate deep through the underlying tissue to bone replacing the normal tissue with constricting fibrous tissue. Traditionally, excision of the fibrous bands and Z plasty has been performed in stages for fear of interrupting the vascularity through the band. Recently, successful one stage releases have been described.
References
- Allington NJ, Kumar SJ, Guille JT. Clubfeet associated with congenital constriction bands of the ipsilateral lower extremity. Journal of Pediatric Orthopedics 1995;15(5):599-603.
- Bamforth JS. Amniotic band sequence: Streeter's hypothesis reexamined. American Journal of Medical Genetics 1992;44(3):280-7.
- Bourne MH, Klassen RA. Congenital annular constricting bands: review of the literature and a case report. Journal of Pediatric Orthopedics 1987;7(2):218-21.
- Greene WB. One-stage release of congenital circumferential constriction bands. Journal of Bone & Joint Surgery - American Volume 1993;75(5):650-5.
- Light TR, Ogden JA. Congenital constriction band syndrome. Pathophysiology and treatment. Yale Journal of Biology & Medicine 1993;66(3):143-55.
- Muguti GI. The amniotic band syndrome: single-stage correction. British Journal of Plastic Surgery 1990;43(6):706-8.
- Prabhu VC, Tomita T. Spinal cord tethering associated with amniotic band syndrome. Pediatric Neurosurgery 1999;31(4):177-82.
- Upton J, Tan C. Correction of constriction rings. Journal of Hand Surgery - American Volume 1991;16(5):947-53.
- Webster WS, Lipson AH, Brown-Woodman PD. Uterine trauma and limb defects. Teratology 1987;35(2):253-60.
- Wiedrich TA. Congenital constriction band syndrome. Hand Clinics 1998;14(1):29-38.
- Young ID, Lindenbaum RH, Thompson EM, Pembrey ME. Amniotic bands in connective tissue disorders. Archives of Disease in Childhood 1985;60(11):1061-3.
May 16-19, 2012 in Denver, CO
