Constriction ring syndrome

It is a malformation due to intrauterine bands or rings that produce deep grooves in (most commonly distal) extremities such as fingers and toes.

In rare cases the constriction ring can form around other parts of the fetus and cause amputation or even intrauterine death.

[5][6][7] The precise configuration of the bands, lymphedema, and character of the amputations are not predictable and vary with each individual patient.

[10] This theory is reinforced by the clinical presentation of the constriction rings with other internal visceral and systematic anomalies.

[citation needed] Individual strands are small and hard to see on ultrasound, so bands are detected indirectly because of the constrictions and swelling upon limbs or digits.

Misdiagnosis is also common, so if there are any signs of amniotic bands, further detailed ultrasound tests should be done to assess the severity.

It is rare but possible for the membrane to become wrapped around the placenta or the neck of the baby in the womb causing strangulation and death.

ADAM complex is the association of limb defects (caused by constriction rings) and certain craniofacial clefts[8][14][15] "Adams–Oliver syndrome is often mislabeled as CRS and consists of cutis aplasia of the scalp in which a longitudinal defect can vary in size and can often be associated with full-thickness skullcap loss.

[8] The constriction ring syndrome is a complex collection of asymmetric congenital anomalies, in which no two cases are exactly alike.

The CRS involves strands which obstruct the lymphatic vessels and thus causing fluid retention, distal of the affected extremity.

[8] Hands with fused fingers need to be released in phases to preserve the distal blood supply.

Because the result is a transverse amputation that cuts off the vascular supply to the developing extremity,[2] the actual constriction ring is not seen.

[7][12][17][20] Large reported series reveal an incidence between 5% and 15% of craniofacial malformations with clefting of the lip or palate.

[14][21][22][23][24][25][26] Amniotic band syndrome is considered an accidental event and it does not appear to be genetic or hereditary, so the likelihood of it occurring in another pregnancy is remote.

[citation needed] Surgical correction is recommended when a constriction ring results in a limb contour deformity, with or without lymphedema.

"[28] For the direct closure of the defect after dissecting a constriction band there are two techniques: In rare cases, if diagnosed in utero, fetal surgery may be considered to save a limb that is in danger of amputation or other deformity.

[29] Monash Medical Centre in Melbourne, Australia, as well as multiple facilities in the United States, have performed successful amniotic band release surgery.

Treatment usually occurs after birth and where plastic and reconstructive surgery is considered to treat the resulting deformity.

The price and complexity of these prosthetics vary dramatically, but advances in 3-D printing have helped to increase the availability of artificial fingers while reducing their cost of production.

In other instances, bands can wrap around limbs causing restriction of movement resulting in clubbed feet.

Amniotic bands can also sometimes attach to the face or neck causing deformities such as cleft lip and palate.

Amniotic band syndrome of the hand in a 26-year-old male