Amniotic band syndrome

I have a four-month old son born with two fingers and a thumb. The doctor said he is otherwise completely healthy. The pediatrician who saw him in the hospital said he probably got his hands tangled in tissue, stopping the blood circulation. My question is: What is this called. My doctor told me it was Holt-Orum Syndrome, which I understand is caused by a genetic defect. That doesn't appear to be the case. Another doctor said it was described as ectrodactyly, but that describes his hands, not the cause. I have found some info on constricting bands syndrome, which sounds right, but it seems to involve a tear or break in the amniotic sac. Do you have any ideas what this is called, and where I might find some information on it?

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Robert Steele

Robert W. Steele, MD, is a board certified pediatrician at St. John's Regional Health Center in Springfield, MO. He graduated from medical... Read more

Amniotic Band Syndrome also known as Amnion Rupture Sequence is an uncommon process which begins when the amnion (the inner membrane of the placental structure) breaks. This exposes the fetus to fibrous bands from the amnion and chorion (another membrane of the placental structure) which may wrap around portions of the fetus. This is quite an uncommon event, and when it occurs, may cause death of the fetus due the amniotic fibrous bands wrapping around vital structures such as the head or umbilical cord. More commonly, the bands wrap around the extremities (arms, legs, fingers, and toes) and partially constricts them causing a deep groove which looks like the infant has a tight rubber band on. If the constriction is severe, however, it may cause complete amputation of the limb.

The problems that are experienced with this banding are dependent upon what portion of the body is effected and how tight the banding is. Occasionally plastic surgery is needed to correct the deep groove that is causing limited motion of the limb. However, banding has also been known to cause clubbed feet and fusion of fingers and toes which require additional intervention. This process is thought to occur most commonly during the first 12 weeks of pregnancy, and the cause remains unknown. Since the amnion rupture is a chance event, the risk of it occurring again with a future pregnancy is felt to be no greater than anyone else who hadn't had an amnion rupture. In other words, it is not genetic.

It is somewhat difficult to direct you to information about what your son has because the two diagnoses that have been given to you are completely different. Amniotic Band Syndrome is caused by a chance accidental tear in the amniotic sac, and most of the deformities are one sided. In other words, only one limb is usually affected. As you stated, Holt-Orum Syndrome is a genetic disorder which causes malformations of the fingers, hands, arms, and sometimes shoulders. And because it is a genetic defect, both sides of the body are affected. In addition, children with Holt-Orum commonly have a heart defect as well. If you would like more information on Holt-Orum Syndrome, an extensive (although somewhat technical) amount of information may be found here. In either case, your son will need physical and occupational therapy to make optimum use of his hands.

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