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Amniotic Band

Syndrome
ABS

Dr Muhammad M El Hennawy

Ob/gyn specialist
59 Street - Rass el barr dumyat - egypt

www.drhennawy.8m.com

?What is Amniotic Band Syndrome


It is a set of congenital birth defects
believed to be caused by entrapment of fetal
parts (usually a limb or digits) in fibrous
amniotic bands while in utero.

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.

Amniotic Band Syndrome


- Alternative Names
Some of these are different names for ABS and some are other syndromes that are often confused with ABS.
Misdiagnosis is apparently common.

Amniotic Deformity, Adhesions and Mutilations


Amniotic band disruption complex or sequence
Amniotic bands and sheets
Annular constriction bands
Congenital ring constriction
Constriction Band Syndrome and Amniotic Constriction Band Syndrome
Intrauterine amputation
Streeter's dysplasia
TEARS (The Early Amnion Rupture Spectrum)
Congenital Transverse Defects or Deficiency
Limb-body Wall Complex
Amniotic deformity adhesions mutilations (ADAM).
ADAM Complex
Amniotic band sequence,
Amniochorionic mesoblastic fibrous strings
Congenital amputation,
Congenital constricting bands
Tissue bands

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

Prenatal risk factors


No distinct sex predilection has been
determined.
Nearly sixty percent of the cases have some
sort of abnormal gestation history.
Prenatal risk factors associated with amniotic
band syndrome include prematurity (<37
weeks), low birth weight (<2500 g), maternal
illness (during pregnancy), maternal drug
exposure and maternal hemorrhage/trauma.

Causes

Amniotic Band Syndrome is not genetic (i.e. not inherited).


It is extremely unlikely that ABS will affect a future pregnancy.
To date, no prenatal factors have been associated with ABS
The primary event is a rupture of the amniotic membrane and its detachment
from the chorion with amniotic fluid leaking through the tear (spontaneously
or after trauma to the lower abdomen ).
As a result, the fetus can move digits or limbs through this tear and exit the
amniotic cavity (partially or completely).
The outer surface of the amnion, and to a lesser degree the naked chorion,
produce mesodermic fibrous strings which may entangle and entrap different
fetal organs, leading to constriction and amputation anomalies.
These deductions agree with the increased frequency of constriction bands
located more distally on the digits, hands and feet, as well as with the varying
severity of constrictions, ranging from slight grooves in the skin to actual
amputation of the digit or limb
However, this theory does not explain the association of amniotic bands with
damage to internal organs and other severe anomalies.

The Timing Of The Rupture


The timing of the rupture is believed to
occur between 28 days after conception to
18 weeks of gestation.
Late bands can occur and present at birth,
even after a normal ultrasound was
performed earlier in the pregnancy.

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 affects the hands in almost 90% of cases. The


distal portion of the extremities is most often
involved, especially in the longer central fingers of
the hand. The thumb and small finger are rarely
involved, presumably because of their shorter
lengths. In the feet, constricting bands most
commonly involve the big toe (hallux).
Lymphatic and vascular compromise may result
from severe band compression. Immediate surgical
release is required if at birth the child presents with
a swollen, engorged digit or limb.
More often, the constricted digit or limb has been
amputated in utero. Acrosyndactyly occurs after
digital separation is complete, but the fingers get
twisted by bands and eventually join together.

Clubfoot occurs in up to 25% of cases of


amniotic banding. In half of occurrences
of clubfoot, a tight band wraps around the
peroneal nerve, which causes muscle
imbalance and clubfoot.
Constriction bands across the face and
head may result in facial clefts. Cleft lip
and palate require reconstruction when
the child is approximately 3 to 6 months
of age.

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.

The findings in Amniotic Band Syndrome / ABS may be


limited to isolated defects, including isolated facial cleft,
digital amputation or mild elephantiasis of an extremity
beyond a constrictive band. These features may be
difficult to diagnose using ultrasound because the
detailed fetal visualization required is beyond the scope
of routine obstetrical ultrasound examinations.
At the worst end of the spectrum, the fetus may be so
severely deformed by the amniotic bands that the spine
is contracted and organs are formed in perplexing and
bizarre proportions. The head may be completely
misshapen or absent.
The bands responsible for these deformities are rarely seen
and a presumptive diagnosis of Amniotic Band
Syndrome / ABS is made based on the commonly
associated deformities.

The spinal deformities in Amniotic Band Syndrome can


be severe, manifesting as kyphotic lordosis or scoliosis
as well as severe rotational abnormalities, even spinal
amputation. While spinal deformity can be seen in other
syndromes, severe spinal deformity should suggest
Amniotic Band Syndrome / ABS.
Spinal deformity associated with an abdominal-wall
defect is particularly suggestive of Amniotic Band
Syndrome.
While the typical appearance of an omphalocele is
possible, the more common defect is a large slash-like
defect of both the thoracic and abdominal cavities with
evisceration.

These defects are associated with exteriorized


bowel, liver and sometimes heart without an
enveloping membrane. When associated with
limb abnormalities, this is characteristic of the
limb-body-wall complex form of Amniotic Band
Syndrome.
Deformation of the calvarium is another group of
anomalies characteristic of Amniotic Band
Syndrome.
If complete, the fetus may appear anencephalic. If
partial, the fetus may appear to have an
encephalocele.

?How is ABS diagnosed


ABS is very difficult to diagnose.
Prenatal ultrasound may not be able to identify the
bands as the individual strands are small and hard to
see on ultrasound but only see the results of the fusion
such as missing or deformed limbs.
Misdiagnosis is also common, up to 70% of all
amniotic bands seen on prenatal ultrasound disappear
upon repeat ultrasound. If the ultrasound demonstrates
amniotic bands with free fetal movement and lack of
fetal attachment to the band, they are termed innocent
amniotic bands and pose little risk to the fetus.
3D ultrasound and MRI can be used for more detailed
and accurate diagnosis of bands and the resulting
damage/danger to the fetus.

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.

Approach to Evaluating Band-like Structures

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)

First trimester transvaginal sonogram showing an


interesting synechia
Ultrasound Appearances:
Shelf-like thick band communicating along it's length with the
uterine wall along it's length. In other words, a synechia has a base
and a free edge. This appearance is caused by a combination of the
fibrous synechia itself, and the complete wrapping of fetal
membranes around the synechia.

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.

Interventions and Treatment


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

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.

Special Considerations For Delivery


Type of delivery - Typically, pregnancies with ABS do not
require cesarean delivery. The need for fetal intervention should
not impact the mode of delivery. The delivery plan will be
carefully discussed.
Place of delivery - If all the prenatal monitoring suggests that
the baby is doing well, the baby can be delivered at the hospital.
However, the hospital should be prepared to handle any
immediate needs of the newborn and have a neonatal intensive
care unit with the capability to provide specialized care.
Time of delivery - Unless there are signs of serious
complications of ABS, there is no reason to intentionally induce
an early delivery. The team at the Center may recommend early
delivery for pregnancies that appear to be in danger

Follow-up and prognosis


All patients with ABS should be monitored regularly until skeletal
maturity is reached, because of the potential for recurrence of the
rings and for secondary contractures that may develop
The prognosis is good for limbs affected by isolated superficial
extremity bands. Aside from cosmetic irregularities, no functional
defects are usually present. Deeper bands may be associated with
progressive problems leading to lymphatic and neurovascular
compromise that requires operative intervention.
For patients with acrosyndactyly, hand function is limited secondary
to stiffness of the joints, but reconstruction can result in good
prehension and grasp. Children whose limbs have been amputated in
utero usually adapt well to their physical limitations, and aside from
fitting with a prosthesis, little often needs to be done. In children
with a transverse deficiency proximal to the ankle joint, a prosthesis
is required for full function.

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.

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.

Amputated Big Toe


ABS is the most common cause of a
congenital amputation of a limb/digit.

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.

Cleft Lip & Palate


Oral clefting occurs when the tissues of the
lip and/or palate of a fetus don't grow
together early in pregnancy.
Children with clefts often don't have
enough tissue in their mouths, and the tissue
they do have isn't fused together properly to
form the roof of their mouths.

The umbilical cord of the dead twin is completely


amputated.

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.

Overlapping, Underlapping Toes


Congenital Overlapping toes are characterized
by one toe lying on top of an adjacent toe.
Congenital Under lapping toes usually involve
the fourth and fifth toes.

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.

ABS affecting the leg above


ankle.

Left hand with constriction


bands, dystrophic nails and
fusion of digits II-V by
amniotic membrane.

Right foot with stunted


growth of all digits,
dystrophic nails,
constriction bands and
partial syndactyly of
digits II to V.

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.

Syndactyly - Surgically Separated


This photo is after two operations that
separated his webbed fingers (syndactyly).

Distraction Augmentation Manoplasty


This surgery creates growth at the rate of an
inch of bone a month, to create new palms
and to lengthen the fingers. It consists of the
insertion of a device through the bone
which is a complex group of bars, nails and
steel screws. It requires screwing several
turns per day to reach a growth rate of up to
1 ml. daily.

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.