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Introduction
Amniotic band syndrome is a disease
of uncertain etiology with the reported
incidence of 1 in 15,000.[1] The early
detection and timely intervention give
rise to excellent outcome failing which
the outcome varies from a mere cosmetic
defectto evenfataldeath.
Case Report
An18‑year‑oldprimigravidawith18weeks
3 days’ period of gestation came with per
vaginal leak and complained of watery
discharge since one night which was foul
smelling and nonsticky. The previous
antenatalcheckupwasnotdone.Thepatient
had no other signicant medical history.
Obstetric examination had revealed uterus
of about 18 weeks in size with cephalic
presentation. Ultrasonography at the time
of examination had revealed multiple
anomalies. A stillborn baby was delivered.
After obtaining appropriate consent, the
fetus was sent for autopsy. The autopsy
examinationrevealed amalefetusweighing
around520g alongwith attachedumbilical
cord measuring 4 cm. Placenta measured
13 cm × 9 cm × 2 cm along with attached
umbilical cord measuring 18 cm. Grossly,
the placenta and umbilical cord were
Address for correspondence:
Dr. Manasi Gosavi,
Department of Pathology,
JNMC, KLE Academy of
Higher Education and Research,
Belgaum, Karnataka, India.
E-mail: srevatsa@gmail.com
Abstract
Amniotic band syndrome is a rare disorder with reported incidence of 1 in 15000. The spectrum
of the presentation includes a simple Streeter band on one side to multiple anomalies including
craniofacial malformation. In the present case, an 18‑year‑old primigravida with presented with
18 weeks 3 days’ period of gestation and complains of per vaginal leak.A male fetus weighing
520gwith attachedumbilical cordmeasuring 4cmwasreceived.External examinationof thefetus
revealed cleft lip with cleft palate, low‑set ears, clubfoot on the right side, amputation of left leg
2.3 cm away from the tibial tuberosity and left palm syndactyly. Microscopic examination of the
organswaswithinnormallimits.This caseispresented withanintentiontocontributetotheexisting
literatureand topresent therange ofanomaliesthatisseenwiththiscondition,with anemphasis on
earlydetection anddiagnosis.
Keywords: Amniotic deformity, adhesions, mutilations complex, amniotic band syndrome, Streeter
band
Amniotic Band Syndrome: A Case Report and Review of Literature
Case Report
Karthik Srevatsa,
Manasi Gosavi,
Ranjit Pratap
Kangle,
Krutika Mihirkumar
Joshi
Department of Pathology,
JNMC, KLE Academy of Higher
Education and Research,
Belgaum, Karnataka, India
How to cite this article: Srevatsa K, Gosavi M,
Ranjit P, Joshi KM. Amniotic band syndrome: A case
report and review of literature. J Sci Soc 2018;45:40‑2.
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unremarkable. External examination of the
fetus revealed cleft lip with cleft palate,
low‑set ears, clubfoot on the right side,
amputationofleftleg2.3cmawayfromthe
tibialtuberosityandleftpalmsyndactyly,as
depictedinFigures1a,2aand2b.Nobands
or membrane‑like structures were adherent
to the fetus or umbilical cord at the time
of receiving the autopsy. The fetus was
subjected to infantogram which revealed
a soft tissue shadow at the left axillary
region which is depicted in Figure 1b.
Grossly, the organs were found in situ and
normal. The microscopic examination of
the soft‑tissue shadow was suggestive of
cystic hygroma, rest of the microscopic
examination of organs were within normal
limits. Gross and histological examination
of the umbilical cord was within normal
limits.Placenta wasnot received.
Discussion
Amniotic membrane is essentially a fetal
epidermis such as layer. Amniotic band
syndrome is also called as congenital
construction band syndrome, amniotic
deformity, adhesions, mutilations
complex,[2] and Streeter band.[3] The
incidenceofamniotic band syndrometobe
around1in15,000toaround1in10,000.[1]
The spectrum of the presentation includes
a simple Streeter band on one side to
multiple anomalies including craniofacial
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DOI: 10.4103/jss.JSS_22_18
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Srevatsa, et al.: Amniotic band syndrome "case report and review of literature"
Journal of the Scientic Society | Volume 45 | Issue 1 | January-April 2018 41
malformation.Thetwotheoriesofamnioticband syndrome
include intrinsic theory and extrinsic theory.The intrinsic
model proposed by Streeter states that anomalies and
brous band have common origin because of improper
development of germinal disc in the early embryo. The
bands represent either sheets of macerated epidermis or
these are the result of circumscribed areas of imperfect
histogenesis.The aberranttissue representsmerelyresidues
of an abnormal developmental process and not a cause.
This theory is often used to explain major craniofacial
abnormalities, body‑wall defects, and internal organ
abnormalities.[3] The extrinsic model is widely accepted
with a sequence of events involving rupture of amnion
followed by loss of amniotic uid and extravasations
of parts of the fetus into the chorionic cavity. The exact
nature of anomalies depends on timing of rupture of band.
The primary defect may be due to an early rupture of the
amnion caused by an intrinsic weakness, inammation,
and trauma. This would permit uid leakage, leading to
the introduction of the fetus into the chorionic cavity.The
chorionreabsorbsthis uidandstimulates theproliferation
of mesenchymal bands. These entangle the fetus and limit
itsmovements, withsubsequent mechanicalconstrictions.[4]
The various anomalies that are associated with amniotic
band syndrome include clubfoot, choanal atresia,
gastroschisis, omphalocele, bladder exstrophy, imperforate
anus and anencephaly, facial clefting, asymptomatic
microphthalmia, incomplete or absent cranial calcication,
andanteriorabdominalwall defects.[5]
Other abnormalities include syndactyly, lymphedema,
clubfoot, phalangeal hypoplasia, and limb‑length
discrepancy. Pseudoarthrosis, metatarsus adductus,
peripheral nerve palsy, dystrophic nails, cleft lip, and
palateskin‑tubepediclesvisceralbodywallmalformations
and eccentric craniofacial synostosis defects are other
associations. Congenital brain abnormalities, cardiac
malformations, short stature, spina bida, reported
in the literature probably are most likely to represent
incidental ndings.[5] Fetal death associated with amniotic
band strangulation of the umbilical cord has also been
reported.[6] Family history is not contributory, and the
syndrome occurs in no particular association with known
genetic or chromosomal disorders, and the syndrome
is almost always sporadic in nature.[5] Risk factors
for amniotic band syndrome include young maternal
age, unplanned pregnancy, hyperthermia, nonsteroidal
anti‑inammatory drug usage. First pregnancy is more
Figure 1: (a) Black solid arrowhead depicting clubfoot, black arrow depicting
autoamputation of the left lower limb, white solid arrowhead depicting
autoamputation of the left upper limb. (b) Infantogram depicting soft-tissue
shadow in the left axillary region
b
a
Figure 2: (a) Depicting cleft palate. (b) White solid arrowhead depicting cleft
lip, black arrow depicting autoamputation of the left upper limb
b
a
Table 1: Patterson’s diagnostic criteria for congenital
ring constrictions
1.Simpleringconstriction
2.Ringconstrictionsaccompaniedbydeformityofthedistalpart
withorwithoutlymphedema
3.Ringconstrictionsaccompaniedbyfusionofdistalpartsranging
fromfenestratedorterminalsyndactylyto“exogenous”syndactyly
4.Intrauterineamputations
Oneormorecriteriamustbepresent
Table 2: Malformations helpful in timing the amniotic
rupture
Event in normal
organogenesis
Date of
structure
determination
Resulting
malformation when
process is interrupted
Formationof
frontonasalprocess
28days Proboscis
Limbbudding 28days Absentextremity
Flexionoftheembryo 28days Omphalocelewith
abdominalwall
deciency
Fusionofmaxillaryand
medialnasalprocess
35days Cleftlip
Perforationofnasal
passages
45days Choanalatresia
Closureofpalate 9weeks Cleftpalate
Returnofintestinesto
abdominalcavity
10weeks Omphalocele
Eruptionofscalphair 16weeks Lackofnormalhair
whorl
Formationofdermal
ridges
18weeks Altereddermalpattern
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Srevatsa, et al.: Amniotic band syndrome "case report and review of literature"
42 Journal of the Scientic Society | Volume 45 | Issue 1 | January-April 2018
likely to have these defects, and the exact reason for this
is not clear; however it is thought that the vascularity of
a multi gravid uterus is likely to be better than that of a
Primi gravid uterus.[7,8] Antenatal risk factors associated
with amniotic band syndrome include prematurity of less
than 37 weeks, low birth weight of less than 2,500 g,[5]
maternalillnessduringpregnancy,maternaldrugexposure,
andmaternalhemorrhage/trauma.[9]
It is difcult to visualize the amniotic bands in the rst
trimester also more difcult is to identify these bands if
they are present in the extremities. The ultrasonographic
analysis allows for the detection of amniotic band
syndrome prenatally by visualization of amniotic sheets
or bands attached to the fetus. It is difcult to visualize
the amniotic bands in the rst trimester, and it is more
difcult to identify these bands if these are present in the
extremities. However, in the second and third trimesters
of pregnancy, it is relatively easy to detect the major
anomaliesofamniotic band syndromebyits characteristic
features and restriction of motion. The sequential
ultrasonographic examinations, along with restricted fetal
movements, help in correct diagnosis of amniotic band
syndrome in utero. Amniotic band syndrome should be
consideredwhencharacteristic asymmetricfetal anomalies
are visualized ultrasonographically irrespective of the
presence or absence of brous membranes.[5] Table 1
describes the diagnostic criteria for amniotic band
syndrome.[10]
The rupture of amniotic band leads to entrapment of fetal
partsbythestickymesodermalbrandswhicharisefromthe
chronic side of amnion. Once the band is formed, there is
decreasedbloodsupplyandsubsequentamputation.Table2
describes the usual malformations that help in timing the
ruptureof amnioticband.[11]
Treatment
Shallow grooves or bands unless associated with
neurovascular compromise require no operative treatment.
Deep constriction bands must be surgically released
immediately to prevent the risk of auto‑amputation or
gangrene.
Overall, the goal is to improve functionality and to
minimize additional problems as the child grows. Parental
counseling is recommended to convey that there is no
knownassociatedriskfor subsequentpregnancies.
Conclusion
Amniotic band syndrome can result in various anomalies.
This case report intends to add to the literature available
regardingthesameand highlightstheroleof fetal autopsy
indocumenting theanomaliespresent.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
given his/her/their consent for his/her/their images and
otherclinical informationto bereported inthe journal.The
patients understand that their names and initials will not
be published and due efforts will be made to conceal their
identity,butanonymitycannotbeguaranteed.
Financial support and sponsorship
Nil.
Conicts of interest
Therearenoconictsofinterest.
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5. TheJournal ofFoot andAnkleSurgery1998;37:325‑33.
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11. HigginbottomMC,Jones KL,HallBD, SmithDW.Theamniotic
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