Amniotic band syndrome occurs when loose fibrous bands of ruptured amnion adhere to and entangle the normal developing structures of the fetus. also referred to as amniotic disruption sequence, constriction ring syndrome, premature amnion rupture sequence, Streeter’s dysplasia.
It is a malformation due to intrauterine bands or rings that give deep grooves in, most commonly, distal extremities like fingers and toes.
Causes of this syndrome is based on three theories
- Clinical presentation of the constriction rings with other internal visceral and systematic anomalies. Because of these other anomalies the names “Constriction Ring Syndrome”.
- Spontaneous rupture of the amnion occurs early in the second trimester, the separation of amnion from chorion produces many small, thin strands that can become entangled within digits and toes.
- The involvement of intrauterine trauma. Intrauterine trauma could be something like amniocentesis, or something like a fetal surgery.
Sign and symptoms of this syndrome are:
- Constriction rings around the digits, arms and legs.
- Swelling of the extremities distal to the point of constriction (congenital lymphedema).
- Amputation of digits, arms and legs (congenital amputation).
Diagnosis of constriction ring syndrome can be confirmed with an ultrasonography.
Treatment is only Surgical correction when a constriction ring results in a limb contour deformity, with or without lymphedema. Some of the surgeries are :
Triangular flaps; For this technique the circumference between the two borders must be measured. Depending on the difference the number of triangular flaps can be decided. With a triangular flap you can create more skin.
Z/W-plasty; “Z-plasty is a plastic surgery technique that is used to improve the functional and cosmetic appearance of scars. It can elongate a contracted scar or rotate the scar tension line. The middle line of the Z-shaped incision (the central element) is made along the line of greatest tension or contraction, and triangular flaps are raised on opposite sides of the two ends and then transposed.”