Amniotic band constriction

Amniotic band syndrome

A baby's hand affected by amniotic band syndrome
Classification and external resources
Specialty pediatrics
ICD-10 P02.8
ICD-9-CM 762.8
OMIM 217100
DiseasesDB 32457
MedlinePlus 001579
eMedicine orthoped/561
MeSH D000652

Amniotic band constriction (also known as "amniotic band syndrome", "ADAM complex", "Amniotic band sequence", "Congenital constriction bands" and "Pseudoainhum"[1]) is a congenital disorder caused by entrapment of fetal parts (usually a limb or digits) in fibrous amniotic bands while in utero.

Signs and symptoms

The constriction of appendages by amniotic bands may result in:

  1. Constriction rings around the digits, arms and legs
  2. Swelling of the extremities distal to the point of constriction (congenital lymphedema)
  3. Amputation of digits, arms and legs (congenital amputation)

A strong relationship between ABS and clubfoot (also called "talipes") exists. A 31.5% of associated clubfoot deformity and ABS can be correlated with 20% occurring bilaterally. Other abnormalities found with ABS include: clubhands, cleft lip, and/or cleft palate, and hemangioma.

Decreased fetal movement could be a sign of a serious problem which may include ABS. 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.

Cause

To explain the cause of ABS, there are two main theories.

The amniotic band theory is that ABS occurs due to a partial rupture of the amniotic sac. This rupture involves only the amnion; the chorion remains intact. Fibrous bands of the ruptured amnion float in the amniotic fluid and can encircle and trap some part of the fetus. Later, as the fetus grows but the bands do not, the bands become constricting. This constriction reduces blood circulation, hence causes congenital abnormalities. In some cases a complete "natural" amputation of a digit(s) or limb may occur before birth or the digit(s) or limbs may be necrotic (dead) and require surgical amputation following birth.

The vascular disruption theory: Because the constricting mechanism of the amniotic band theory does not explain the high incidence of cleft palate and other forms of cleft defects occurring together with ABS, this co-occurrence suggests an "intrinsic" defect of the blood circulation.

Diagnosis

Amniotic band syndrome is often difficult to detect before birth as the individual strands are small and hard to see on ultrasound. Often the bands are detected indirectly because of the constrictions and swelling upon limbs, digits, etc. Misdiagnosis is also common, so if there are any signs of amniotic bands, further detailed ultrasound tests should be done to assess the severity. 3D ultrasound and MRI can be used for more detailed and accurate diagnosis of bands and the resulting damage/danger to the fetus.

Treatment

In rare cases, if diagnosed in utero, fetal surgery may be considered to save a limb which is in danger of amputation or other deformity. This typically would not be attempted if neither vital organs nor the umbilical cord were affected. This operation has been successfully performed on fetuses as young as 22 weeks.[2] The surgery took place at Melbourne's Monash Medical Centre in Australia and is believed to be the earliest surgery of its type, as surgeons usually hold off on operating until the woman is in week 28 of gestation. There are also several facilities in the United States that 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.[3] Plastic surgery ranges from simple to complex depending on the extent of the deformity. Physical and occupational therapy may be needed long term.

Prosthetics may help some ABS sufferers to live more functional lives. 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.[4]

Prognosis

Amniotic Band Syndrome, 26-year-old Male

The prognosis depends on the location and severity of the constricting bands. Every case is different and multiple bands may be entangled around the fetus.

Bands which wrap around fingers and toes can result in syndactyly or amputations of the digits. In other instances, bands can wrap around limbs causing restriction of movement resulting in clubbed feet. In more severe cases, the bands can constrict the limb causing decreased blood supply and amputation. Amniotic bands can also sometimes attach to the face or neck causing deformities such as cleft lip and palate. If the bands become wrapped around the head or umbilical cord it can be life-threatening for the fetus.

The number of cases of miscarriage that can be attributed to ABS is unknown, although it has been reported that it may be the cause of 178 in 10,000 miscarriages.

Prevention

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. The cause of amnion tearing is unknown and as such there are no known preventative measures.

Epidemiology

Amniotic banding affects approximately 1 in 1,200 live births. It is also believed to be the cause of 178 in 10,000 miscarriages. Up to 50% of cases have other congenital anomalies including cleft lip, cleft palate, and clubfoot deformity. Hand and finger anomalies occur in up to 80%.

People living with ABS

Among the more notable people living with ABS are:

See also

References

  1. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
  2. ↑ "Surgeons save unborn baby's legs". News Online (Australian Broadcasting Corporation). 2008-06-08.
  3. ↑ Gabos PG (2006). "Modified technique for the surgical treatment of congenital constriction bands of the arms and legs of infants and children". Orthopedics 29 (5): 401–4. PMID 16729738.
  4. ↑ "3-D Printer Brings Dexterity To Children With No Fingers". News Online. 2013-06-18.
  5. ↑ Sophie Wells website http://www.sophiewells.com/
  6. ↑ Superhands http://www.superhands.us/pages/casillas.html
  7. ↑ Single-handed art http://www.singlehandedart.com/the-artist
  8. ↑ 'Despite setbacks, swimmer takes head-first approach to reaching Paralympic gold' http://www.local12.com/ara/hottopics/story/Despite-setbacks-swimmer-takes-head-first/VpNtHBpp0U2YTLDQu0TmSQ.cspx
  9. ↑ http://www.bjjheroes.com/bjj-fighters/jean-jacques-machado-bjj-fighter-wiki
  10. ↑ Monica Price website http://specialneedsfamilyfun.com/family/monica.htm

External links

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