This document discusses amniotic band syndrome, a congenital birth defect caused by entanglement of fetal parts in fibrous bands in the uterus. It can result in deformities of limbs, organs and other parts. Clinical features may include constriction rings around limbs, amputation of digits or limbs, clubfoot, cleft lip/palate, and spinal deformities. Treatment involves reconstructive surgery after birth, while in rare cases fetal surgery may be attempted to prevent limb amputation. Prognosis depends on severity and location of bands.

1. PRESENTED BY
PANKAJ SINGH RANA
NURSE PRACTIONER
SRHU

2. 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 uterus.

3. 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.

4. Amniotic Band Syndrome -
Alternative Names
• 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
• TEARS (The Early Amnion Rupture Spectrum)
• amniotic deformity adhesions mutilations (ADAM).
• amniotic band sequence,
• congenital amputation,
• congenital constricting bands

6. CAUSES
• 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).

7. • 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

9. FEATURES
• 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)

10. CLINICAL MANIFESTATION
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. In the feet,
constricting bands most commonly involve the big toe
(hallux).

11. 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.

12. 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.

13. CLUBFOOT
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.

14. CLEFT LIP AND PALATE
 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

15. ENCEPHALOCELE
Encephalocele may be a manifestation of Amniotic Band
Syndrome / ABS, especially when eccentrically placed off
the midline. Abdominal-wall defects, typically large defects
with free- floating intestine but large enough for the lines
to herniate outside the abdomen, can also be the result of
Amniotic Band Syndrome / ABS.

16. AMPUTATED BIG TOE
• ABS is the most common cause of a congenital
amputation of a limb/digit

17. SPINAL DEFORMITIES
 The in spinal deformities Amniotic Band Syndrome
can be severe, manifesting as kyphotic lordosis or
scoliosis as well as severe rotational abnormalities,
even spinal amputation. Spinal deformity associated
with an abdominal-wall defect is particularly
suggestive of Amniotic Band Syndrome.

18. 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

19.  • 3D ultrasound and MRI can be used for more
detailed and accurate diagnosis of bands and the
resulting damage/danger to the fetus

20. 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

21. 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 uterus, 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

22. 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
mother’s 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

23. 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. .

24. 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

25. SYNDACTYLY - SURGICALLY SEPARATED
This photo is after two operations that separated his
webbed fingers (syndactyly).

26. 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 mm.daily

27. FOLLOW-UP
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
For patients with acrosyndactyly, hand function is
limited secondary to stiffness of the joints, but
reconstruction can result in good prehension and
grasp.

28. PROGNOSIS
The prognosis depends on the location and severity of the constricting
bands.
• 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.

29. 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.