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Amniotic Band Syndrome
Amniotic Band Syndrome
Syndrome
ABS
Dr Muhammad M El Hennawy
Ob/gyn specialist
59 Street - Rass el barr dumyat - egypt
www.drhennawy.8m.com
Typs Of ABS
A membrane formed at an early stage of pregnancy may cause
severe damage to the structure of the various tissues, resulting in
deformities in a large number of organs this often results in
intrauterine death. Defects in these cases usually include defects in
the location and structure of the heart, omphalocele, gastroschisis,
anencephalus, cleft face, hydrocephalus, etc.
A membrane formed at a later stage of pregnancy may not cause
damage, but if it does, this is usually to limbs. The entire limb, part
of it, or only digits can be involved, and the result can be limb
stumps or scars in one or more limbs. Often those fingers that are not
missing in the limb involved have signs of scarring or fusion.
Sometimes the damage is milder, manifesting as a localized annular
narrowing at the site where the membrane entrapped the limb, with
swelling distal to the ring due to lymphedema.
Incidence
Amniotic band syndrome (ABS) is an uncommon
fetal malformation with increasing prevalence
Amniotic banding affects approximately 1 in
1,200 to 1 in 15,000 live births.
It is also believed to be the cause of about 178 in
10,000 miscarriages.
About 80% of cases involve the hands and fingers
and a significant number of clubfoot cases are
correlated with ABS
Causes
Features or Stages
The constriction of appendages by amniotic
bands may result in:
Restrictions of the growth around the digits,
arms and legs
Constriction rings around the digits, arms and
legs - the bands will partially constrict the
blood flow in the affected appendage causing a
deep groove in the baby's skin
Swelling of the extremities distal to the point
of constriction (congenital lymphedema)
Amputation of digits, arms and legs
(congenital amputation)
Range of effects
The defect could be merely cosmetic, depending on the
severity of the constriction.
Deeper bands may cause lymphatic obstruction leading
to edema and vascular compromise, conditions that
require immediate release.
Abnormalities may occur distal to the constriction, such
as anterolateral bowing, hemihypertrophy,
pseudarthrosis, leg-length discrepancy, and teratologic
clubfeet. These conditions may lead to limited function
and movement.
Early in gestation, spontaneous abortions may result
from the encircling bands. If the constriction occurs
after development is almost complete, fissures,
acrosyndactylization, or intrauterine amputation occur
typically on the extremities.
ABS may affect the face with cleft lip or palate, asymmetric
microphthalmia or severe nasal deformity. Encephalocele
may be a manifestation of Amniotic Band Syndrome / ABS,
especially when eccentrically placed off the midline.
Abdominal wall defects - typically large defects with freefloating intestine but large enough for the lines to herniate
outside the abdomen, can also be the result of Amniotic
Band Syndrome / ABS.
The characteristic appearance of an aberrant sheet or band of
amnion attached to the fetus with resultant deformity and
restriction of motion allows a diagnosis of Amniotic Band
Syndrome / ABS to be made. Prenatal diagnosis is the
exception rather than the rule.
DD Of Amniotic Sheets
from Amniotic Bands
Amniotic sheets are the most common with an incidence of 0.6%.
They are thought to be caused by scarring inside the womb or
'senechiae' from previous operations, such as D&C. As the
membranes develop, they surround these sheets of scar tissue. Often
they aren't seen until later on in pregnancy, presumably following
rupture or compression by the growing baby. Amniotic sheets have
been found to be associated with an increased risk of early labour.
Amniotic bands are even less common, affecting 1 in 1200 (0.08%)
of all pregnancies. They are fibrous strands of membrane stretching
from the outer membrane surface into the amniotic cavity. They are
thought to originate when the inner membranes (amnion) rupture
without injury to the outer membrane (chorion). The ruptured
amnion remains as a plaque or fragments into bands which stretch
across the chorionic cavity. Amniotic bands are thought to happen
spontaneously or in association with trauma to the abdomen. There
has been the suggestion of a relationship between amniotic bands,
limb shortening and early chorion villous sampling (CVS).
Other Bands
Other less common types of band-like appearing
structures may also be observed on obstetric
sonogram.
These include: chorio-amniotic separation (normal
finding in the 1st trimester up to 16 weeks),
velamentous cord insertion, uterine fusion
abnormalities (bicornuate, septate uterus, etc.), and
remaining membranes after demise of a twin.
In these situations, correlation of ultrasound features
with patient's clinical history can be useful.
The following diagram can be utilized to aid diagnosis when a band-like structure is visualized in the uterus.
The authors advise caution, however, as there may be some overlap of appearances in the different categories
Ultrasound Appearance: Usually thin membranelike strands criss-crossing the amniotic sac and
attached to fetal body parts. (ABS)
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.
Fetal Treatment
In utero limb salvage: fetoscopic release of amniotic bands for
threatened limb amputation.
Fetoscopic laser release of amniotic bands in extremity ABS
offers the potential to prevent limb amputation
A small camera is inserted through the mothers abdomen and
uterus into the amniotic sac in order to see and cut the bands.
Cutting the amniotic bands is performed utilizing a fetoscopic
technique.
The procedure is typically performed under regional anesthesia.
Early fetoscopic release may prevent amputation and allow
improved limb development.
Child Treatment
Indications for intervention depend on the medical
stability of the child and on the neurovascular status of
the limb.
Bands that only cosmetically affect the superficial skin
generally do not require any intervention.
Only the tight constriction bands, resulting in gross
lymphedema, vascular compromise, or both necessitate
immediate surgical release.
Surgery also is indicated for patients with syndactyly or
acrosyndactyly that compromises hand function. Thumb
amputation (which is rare), club feet, cleft lip, and cleft
palate require reconstruction, but these procedures can
be performed electively at a later time.
Prognosis
Acrosyndactyly
Acrosyndactyly is a more
complex type of syndactyly. The
fingers had separated but a band
formed around the fingers
causing them to refuse during
development.
These images are the left and
right hand of a newborn.
Banded Foot
When bands wrap around limbs during
development they can constrict the limb
causing decreased blood supply and
amputation.
Banding on Leg
This image shows a banding line on newborn's
leg.
Clubfeet Photo
A strong relationship between ABS and
clubfoot exists.
A 31.5% of associated clubfoot deformity and
ABS can be correlated with 20% occurring
bilaterally.
Nubbins
This is typical of Congenital Transverse
Deficiency, a form of ABS.
A short below-the-elbow amputation. The
fingers may be represented only by nubbins or
dimpling found on the end.
Syndactyly - at birth
A hand at birth, the banding line from the wrist to
the pinky finger and around the wrist. The three
fingers are webbed together (syndactyly).
The three fingers are small, with abnormal finger
nails, and missing some joints.
There is also some lymphedema (swelling at the
joint) of the index finger middle joint.
Clubfoot Treatment
The accepted method of treating clubfeet is by
the Ponseti method of Serial Plaster Casting.
Treatment should be started right away. The
initial treatment consists of manipulating the
foot to get it to the best position possible, and
then holding the correction in a cast.
Summary
Amniotic Band Syndrome can be difficult to diagnose.
Ultrasound does not usually reveal it.
Even though the incidence of the condition is very low,
obstetricians need to be aware of the possibility of umbilical
cord constriction by an amniotic band.
The presentation of a patient with decreased fetal heart rate and
a history of amniocentesis must to be monitored closely. There is
a high fetal death rate associated with this diagnosis. When the
fetus is at a viable gestational age, immediate delivery may be
indicated.
When Intrauterine Demise does occur, amniotic band syndrome
should be included as one of the possible etiologies. Thorough
evaluation of the placenta and cord is necessary to rule out
constriction of the cord by Amniotic bands.
Although the finding of Amniotic Band Syndrome may not
change the outcome identifying a definitive cause for the parents
may provide a small measure of closure in the context of this
tragic event.