Volume 03 Issue 06-2023
38
International Journal of Medical Sciences And Clinical Research
(ISSN
–
2771-2265)
VOLUME
03
ISSUE
06
P
AGES
:
38-43
SJIF
I
MPACT
FACTOR
(2021:
5.
694
)
(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
ABSTRACT
Failure to thrive is the term used to describe the condition in which a young child fails to gain weight satisfactorily.
Common reasons for such poor weight gain are parental neglect or lack of food. On the other hand, a large number
of important gastrointestinal disorders may be responsible, including those associated with vomiting, such as food
intolerance or obstruction of the upper bowel by pyloric stenosis; disorders of digestion and absorption, including
celiac disease and cystic fibrosis; and bowel infections. Amniotic band syndrome (ABS) is a set of congenital
malformations attributed to amniotic bands that entangle fetal parts during intrauterine life, which results in a broad
spectrum of anatomic disturbances - ranging from minor constriction rings and lymphedema of the digits to complex,
bizarre multiple congenital anomalies incompatible with life.
KEYWORDS
Amniotic band syndrome, intrauterine amputation, congenital anomalies.
INTRODUCTION
In developed countries, SIDS (also called crib death or
cot death) accounts for 20 percent of deaths between
the ages of one month and one year. SIDS is a
categorization rather than an explanation, for the label
is given when no reason for death can be found from
the infant’s medical history or even after autopsy.
Most crib deaths occur in the first five months of life
and strike at home during the night. They are more
Research Article
CLASSIFICATION OF CONGENITAL AND ACQUIRED SYNDROMES FOUND
IN INFANCY
Submission Date:
June 02, 2023,
Accepted Date:
June 07, 2023,
Published Date:
June 12, 2023
Crossref doi:
https://doi.org/10.37547/ijmscr/Volume03Issue06-07
Sotvoldiyev Bekzodbek Maxamatmusayevich
Andijan State Medical Institute, Uzbekistan
Journal
Website:
https://theusajournals.
com/index.php/ijmscr
Copyright:
Original
content from this work
may be used under the
terms of the creative
commons
attributes
4.0 licence.
Volume 03 Issue 06-2023
39
International Journal of Medical Sciences And Clinical Research
(ISSN
–
2771-2265)
VOLUME
03
ISSUE
06
P
AGES
:
38-43
SJIF
I
MPACT
FACTOR
(2021:
5.
694
)
(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
common in the winter and in poor social
circumstances. A preceding minor respiratory infection
is common. This has prompted some investigators to
suggest that the underlying defect is the presence of a
virus in the bloodstream, leading to instability of
cardiac and respiratory mechanisms. Many other
hypotheses have been proposed to explain such
deaths, however, and it is likely that several different
causes may be involved.
Amniotic band syndrome (ABS) is a set of congenital
malformations attributed to amniotic bands that
entangle fetal parts during intrauterine life, which
results in a broad spectrum of anatomic disturbances -
ranging
from
minor
constriction
rings
and
lymphedema of the digits to complex, bizarre multiple
congenital anomalies incompatible with life (1,2).
Incidence of ABC is estimated in wide range of 1 : 1200
–
1 : 15000 live births (1,3,4-6) in 1 : 70 in stillborns (7)
and among abortuses as high as 178 : 10000 (2). Among
total of 3% major malformations in general population
(8), ABS is responsible for 1-2% (9). The aim of this paper
was to point out diagnostic and therapeutic approach
in newborns with ABS through the report of two cases.
Patient: female preterm newborn, second child in the
fourth pregnancy (1 healthy child, 2 artificial abortions)
in a healthy 28-year old mother. There were no trauma
or drug taking during pregnancy, no prenatal
chromosomopathy diagnostics, consangvinity or
malformations in the family. Pregnancy was regularly
controlled, had favourable course until premature
labour, ultrasound examination did not show any fetal
malformations.
Patient: male preterm newborn, born after 34
gestational weeks as a first child from a first, regularly
controlled pregnancy to a 27-year old healthy mother.
Pregnancy had favorable course until premature labor.
Trauma
during
pregnancy,
prenatal
fetal
karotypisation, cerclage, drug taking, consanguinity or
malformations in family were denied. Routine
ultrasound examination did not show any fetal
malformations. Birth weight was 2250 grams (50
percentils), birth length 46 cm (50 percentils), head
circumference 29,5cm (10 percentils), Apgar score 8/9.
Immediately after birth, the following malformations
were observed: cleft lip and palate, left-sided uvula,
absent lower wall of the left nostril. On the right hand,
third finger was shortened with the presence of two
constriction rings; bellow lower ring the presence of
edema. On the upper part of the right foot there were
amniotic band mark, syndactylia of II and III, and IV and
V right tows; right tows were shortened with
hypoplastic nails. Besides, there were some more
minor malformations present: asymmetric and
malformed ears, funnellike bended thoracic cage,
bilateral klyndactily of the fifth finger, sandal creases
on the feet. Karyotyp was normal male (46, XY). All
performed examinations (ultrasound of central
nervous
system
and
abdominal
organs,
Volume 03 Issue 06-2023
40
International Journal of Medical Sciences And Clinical Research
(ISSN
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2771-2265)
VOLUME
03
ISSUE
06
P
AGES
:
38-43
SJIF
I
MPACT
FACTOR
(2021:
5.
694
)
(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
echocardiografy, oftalmoscopic examination) showed
normal anatomy of examined organs. Plastic surgeon
and orthodontist were consulted.
ABS ethiopathogenesis is still unknown, but there are
two main theories (10-13). Widely accepted "extrinsic
model”, proposed by Torpin and Faulkner in 1966
explains defects genesis by rupture of the amnion in
early pregnancy, with forming of amniotic bands and
amniotic liquid loss, followed by extrusion of all or
parts of the fetus into the chorionic cavity. Bands
entrap the parts of the growing fetus, and fetus' limbs
and other div parts become entangled and subjected
to compression, which compromises fetal circulation
and also his growth and development with consecutive
disturbances of functions and anatomy. The intrinsic
model was proposed by Streeter in 1930 and suggests
that the anomalies and the fibrous bands have a
common origin, caused by a perturbation of
developing germinal disc of the early embryo. Beside
ABS etiopathogenesis, risk factors which start such
sequence of events are also poorly known. Most cases
of ABS are not of genetic origin, and there is no
recurrence in siblings or children of affected adults.
However, there are some reports of amniotic band
syndrome among families with collagen disorders,
more specifically Ehler-Danlos syndrome (2,6), in other
diseases that involve connective tissue, e.g. in
osteogenesis imperfecta , and in one case of
epidermolysis bullosa congenital (14,15). Some other
possible etiologic factors besides inheritance were
explored in several studies. Some studies found
connection between ABS and mother's age (especially
primiparas under the age of 25 (12,15), prematurity (16),
abdominal trauma (2,15), unsuccessful abortion (17),
intrauterine contraception (2), cerclage (18), chorionic
villus sampling (17-20), amniocentesis (2,17-20),
malformations of the uterus (15), some drugs taking,
like ergotamine (21), acetaminophen (12), misoprostol
(21). However, there is no firm evidence of definite
causality for any of these factors, and therefore a great
number of authors considers ABS as defects with
sporadic occurrence, with no gender prevalence, and
no strong risk factors (1). Several studies confirm this
opinion, with evidence that most of the cases of ABS
have no risk factors in prenatal anamnesis. Both our
patients have only prematurity in their family and
prenatal anamnesis. Amniotic band syndrome has very
polymorphic clinical findings, because type of
deformities depends on the time of amniotic rupture
during pregnancy and part of the fetal div which is
entangled in amniotic bands. Early amniotic rupture,
during first 45 days, leads to the most severe cranio-
facial and visceral malformations (5). Every part of the
fetal div can be damaged, but most often
extremities, especially upper extremities. Most often
there are minor defects, such as constriction rings or
digit amputations; but, even minor defects are multiple
in 77% of cases (4). Abnormalities of the extremities can
be expressed in several ways: constriction rings of the
Volume 03 Issue 06-2023
41
International Journal of Medical Sciences And Clinical Research
(ISSN
–
2771-2265)
VOLUME
03
ISSUE
06
P
AGES
:
38-43
SJIF
I
MPACT
FACTOR
(2021:
5.
694
)
(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
soft tissue accompanied by distal edema, shortenage
of the limb or intrauterine limb amputation,
amputation of the digits (most often II, III and IV finger)
and toes, syndactyly, hypoplasia of the digits, foot
deformities, pseudoarthrosis, periferal nerve palsy
(1,21). If bands compress the fetal head or face,
different
cranio-facial
disturbances
appear
–
asymmetric face clefts, orbital defects (anophtalmos,
microphtalmos, enophtalmos), corneal abnormalities,
central nervous system malformations (anencephaly,
encephalocoele, asymmetric meningocoele), calvaria
defect. Amniotic bands can also cause abdominal wall
defect and abdominal organs extrophy (1), chest wall
defect with heart extrophy (6), umbilical cord
strangulation with often lethal outcome (1). Amniotic
rupture and consecutive olygoamnion can, by
mechanical pressure on the fetus, cause deformities
such as metatarsovarus, scoliosis (5) or hip dislocation
(1). Because of such a wide spectrum of possible
anomalies
and
many
combinations
of
their
simultaneous appearance, there are no two identical
cases of ABS (1). Beside all previously mentioned
malformations caused by amniotic bands itself, a
subset of cases manifest additional findings that are
not consistent with that mechanism, such as
congenital
heart
defects,
renal
anomalies,
hemangiomas, imperforate anus, polydactyly, septo-
optic dysplasia, typical cleft lip and palate (10). ABS can
be diagnosed prenatally by ultrasound, which can
sometimes show amniotic bands, but more often
malformations consistent with ABS, as well as
olygoamnion and reduction of foetal movements (22).
ABS can be diagnosed as early as 12 gestational weeks
(23); in the second trimester of gestation most of ABS
defects could be seen during routine ultrasound
examinations (22). The most important ultrasound
diagnostic criteria are visible amniotic bands,
constriction rings on extremities and irregular
amputations of fingers and/or toes with terminal
syndactily. Mild defects, however, are less likely to be
diagnosed prenatally, in which case defects are seen
after birth (23). Latest ultrasound techniques
–
three-
dimensional
and
fourdimensional
ultrasound
contribute to more sensitive prenatal diagnostics of
ABS, and in complicated cases foetal magnetic
resonance can be helpful (3). Placenta and amnion
examination after the delivery should be obligatory
part of the newborns health evaluation because it can
show presence of amniotic bands, among other things
(3,17,19). Physical examination is the main way of
postnatal diagnostic of ABS, with usage of addition
searches in order to establish potential malformations
of different organs and div parts: ultrasound,
echocardiography, X-ray. ABS must be considered in
differential diagnosis of all complex or asymmetric
malformations, especially those on extremities, face
and div walls. ABS should be differentiated from the
whole spectra of symmetric fusion defects of middle
div line (19). In differential diagnosis some rare
findings, such as amniotic folds, complex extremity-
Volume 03 Issue 06-2023
42
International Journal of Medical Sciences And Clinical Research
(ISSN
–
2771-2265)
VOLUME
03
ISSUE
06
P
AGES
:
38-43
SJIF
I
MPACT
FACTOR
(2021:
5.
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)
(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
div wall, extraamniotic pregnancy should be taken
into
consideration
(2).
Exclusion
of
chromosomopathies in ABS cases is of great
importance, because of informing the parents of
future recurrence risk, which is very low for ABS (19).
Therapy of ABS is mostly surgical, with an individual
approach to every single case. Interdisciplinary
consulting and work is very often needed (plastic
surgeon,
orthopedic
surgeon,
orthodontist,
ophthalmologist, neurosurgeon…) (1). Lately, there
have been some attempts of prenatal ABS treatment -
foetoscopic laser cutting of amniotic bands, before
their compression on the fetus makes malformations
(25). In cases when foetal anomalies incompatible with
life are prenataly seen, pregnancy termination is
advised (2).
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International Journal of Medical Sciences And Clinical Research
(ISSN
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VOLUME
03
ISSUE
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P
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:
38-43
SJIF
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MPACT
FACTOR
(2021:
5.
694
)
(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
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