Amniotic band syndrome
Amniotic band syndrome
Classification and external resources
ICD-10 P02.8
ICD-9 762.8
OMIM 217100
DiseasesDB 32457
MedlinePlus 001579
eMedicine orthoped/561
MeSH D000652

Amniotic band syndrome (also known as "ADAM complex,"[1] "Amniotic band sequence,"[1] "Congenital constriction bands,"[1] 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.

Contents

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%.

Features

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 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.

Natural history

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

Ultrasound Scan ND 008.jpg

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

Treatment usually occurs after birth and where plastic and reconstructive surgery is considered to treat the resulting deformity.[2] Plastic surgery ranges from simple to complex depending on the extent of the deformity. Physical and occupational therapy may be needed long term.

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 are affected. This operation has been able to be successfully performed on fetuses as young as 22 weeks.[3] 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 a mother is at least 28 weeks gestation.[4] There are also several facilities in the United States that have performed successful amniotic band release surgery.

Prognosis

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 contributed 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.

See also

References

  1. ^ a b c d Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. 
  2. ^ 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. 
  3. ^ "Surgeons save unborn baby's legs". News Online (Australian Broadcasting Corporation). 2008-06-08. http://www.abc.net.au/news/stories/2008/06/08/2268374.htm. 
  4. ^ [1]
  • Walter JH, Goss LR, Lazzara AT (1998). "Amniotic band syndrome". The Journal of foot and ankle surgery : official publication of the American College of Foot and Ankle Surgeons 37 (4): 325–33. PMID 9710786. 

External links


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Look at other dictionaries:

  • amniotic band syndrome — n the highly variable group of physical abnormalities that can result from the formation of amniotic bands * * * early rupture of the amnion with formation of fibrous strands of amnion that may adhere to or compress parts of the fetus, resulting… …   Medical dictionary

  • amniotic band disruption complex — amniotic band syndrome …   Medical dictionary

  • amniotic band — n strands of amniotic tissue that are formed by premature rupture of the amnion and that become entangled esp. in the extremities of the developing fetus often limiting growth and resulting in various physical abnormalities (as limb or digit… …   Medical dictionary

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  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Amniotisches-Band-Syndrom — Klassifikation nach ICD 10 Q79.8 Sonstige angeborene Fehlbildungen des Muskel Skelett Systems Amniotische Schnürfurchen …   Deutsch Wikipedia

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