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This one is about: Constriction Band Syndrome
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CONSTRICTION BAND SYNDROME
by Joanne Green
Of the many cleft defects, most are, at least in some part, genetically related. That is, there is either a genetic cause, or a genetic predisposition toward clefting. This is even more probably if the cleft is part of some identifiable syndrome. That, however, is not the case with Constriction Band, or Amniotic Banding, Syndrome.
Constriction Band Syndrome is, in fact, the result of a pre-birth accident. It happens when, for some reason or another, there is a rupture in the amniotic sac during pregnancy. The Amniotic sac then falls away. Most often, under those circumstances, the fetus will spontaneously abort and the pregnancy ends in miscarriage. However, there are cases in which, against all odds, the fetus continues to develop without the protection of the amniotic sac. Without the protective sac, much can happen to the fetus during the remainder of gestation. First, the baby is in danger of interference by the placenta. The Chorionic portion of the placenta is made up of long fibers. In a normal pregnancy, there is a barrier between the baby and the fibers of the Chorion. But when that barrier is absent, the thread-like fibers float, with the baby, in the amniotic fluids and wrap themselves around the appendages of the baby. If they become entangled and fail to slip back off again, they can constrict the development of that appendage, much the way a metal ring can constrict the growth of a young tree. The constriction can result in any one of a number of conditions. First, the constriction itself will look much like a tight rubber band, but the crease caused by the band is permanent. It may or may not affect the growth and development of the limb beyond the band.
In the most extreme case, if the band is entangled early in the pregnancy and if it is entangled tightly enough, the constriction can cause a gestational amputation of the limb. Or the limb, while still attached, may be tiny and shriveled, and useless as a limb. Typically, such a limb is amputated after birth. In the least extreme cases, the banding can cause nothing more than an unsightly band.
The interference of the bands can also cause syndactyly (fusing of the bones and/or tissues of the fingers or toes). In extreme cases, all four fingers of the hand may be fused. In minor cases, two toes may be "webbed" together.
Not only is the fetus in danger of the interference of the placenta, but the placental blood flow, again, in the chorion, is in danger of interference by the baby. With a disruption in the placental blood flow at a critical point of development, birth defects may occur. Fingers or toes may be shortened or "snubbed". Hemangioma may be formed in alarmingly large numbers. And, if the rupture occurred early enough in the pregnancy, clefting may occur. The lack of an amniotic sac will result in there being very little amniotic fluid during gestation as well. With less that the optimal amount of fluid, the developing baby will settle low and heavy in the womb. This abnormal positioning may cause the child to develop clubbed feet.
A child born with constriction band syndrome will likely present a lot of cosmetic problems, including missing, malformed or syndactyly fingers or toes; congenitally amputated arms or legs; clubbed feet; numerous hemangioma; and/or cleft lip and palate. Likewise, however, a child born with constriction band syndrome is not likely to be mentally retarded, or to have life-threatening problems associated with any of the vital organs.
Also, because amniotic banding is the result of a pre-birth accident, it is not genetically caused. A child born with constriction band syndrome is not going to "pass it along" to future generations.
All of the birth deformities associated with constriction band syndrome are the result of another problem - specifically, the absence of the amniotic sac during gestation. None will get "worse" after birth. Each of the deformities is treated appropriately after birth.
Syndactyly fingers and toes can be surgically separated. Fortunately, the child's thumb is rarely affected by syndactyly, probably due to the fact that during gestation the fetus often keeps the thumb tucked and out of the way of the floating, fibrous tissues of the chorion. With opposability, the child's hands, while they may look a little different, will be functional. The banding itself will also be surgically released by way of a Z-plasty operation that will actually surround the limb. The Z-plasty is an effective way to release the band and restore any circulation that may have been compromised. However, the resulting scar is fairly obvious and looks "different".
Clubbed feet are treated through a process of casting and training the developing bones. If casting alone is not effective, some surgery may be indicated.
Most hemangioma resulting from constriction band syndrome are the "raised raspberry-type" and pose no threat to the child. That type of hemangioma, which is a concentration of blood vessels on the surface of the skin, will fade and shrink, and eventually disappear altogether without any intervention. If the hemangioma are the subcultaneous, cavernous type they may or may not present more problems and should be watched closely.
It is not uncommon for a cleft-affected child with constriction band syndrome to have, in fact, a very wide, very severe cleft. That cleft is repaired in the same way and according to the same time line as any other cleft. That is, the lip is generally closed at about 3 months of age; the palate is generally closed at or about the child's first birthday.
Because most developing fetuses do not survive the rigors of development without the protective amniotic sac, constriction band syndrome is a rare disorder. However, when it does occur, it should be considered the result of a pre-birth accident. Each of the injuries caused by the accident should be attended to with the appropriate treatment decision.
------------------------------------------------------Joanne Green is the mother, by adoption, of three cleft-affected children. Her son, Joey, was born with Constriction Band Syndrome. Joanne is also the Editor of WIDE SMILES Magazine.