ECTOPIA

CORDIS

NIKHI
LXII G

CERTIFICATE
This is to certify that Nikhil Kr. Gautam , a
Student of class XIIG of the Kendriya Vidyalaya
A. G. C. R. Colony , session 2015-2016,
has satisfactorily completed the required
Biology project work as per the syllabus of
Standard XII in the laboratory of the school.
Date:
Chemistry Teacher
signature
(Mrs. Vandana Singh)

ACKNOWLEDGEMENT
I am over helmed in
all
humbleness
and
gratefulness to acknowledge my depth to those who
have helped me to put these ideas , well above the
level of simplicity and into something concrete.
I would like to express
my special thanks of
gratitude to my teacher Mrs. Vandana Singh who
gave me this golden opportunity to do this
wonderful project on topic ECTOPIACORDIS Which
also helped me in doing lots of research and I came
to know about many things . I am really thankful to
them.
I would like to thank my parents who helped me a lot
in gathering different info. and guiding me to make this
project , despite of their busy schedules.
Thanking you,

INDEX
What

is Ectopia Cordis ?
Discussion & Discovery.
Causes of ectopia cordis .
Epidemiology .
Case Report : Thoraco-Abdominal Ectopia
Cordis .
Pathology .
Anomalies associated with ectopia cordis .
Treatment & Repair .
Prognosis & Survival .
References .

What is Ectopia Cordis ?

oEctopia

cordis is a Latin word, which

literally means outside and
heart.
oEctopia cordis (EC) is the presence of a
live , beating heart outside the thorax
And
is one of the most
unique congenital anomalies . The
reported point prevalence is 5 to 8 per
millio live births . To our knowledge ,
91 cases of EC
have been
documented
in
published articles.
oBecause
of
its rarity and
other associated abnormalities , EC
is a challenging
congenital
anomaly.

Discussion & Discovery.

oBecause

of its rarity and other associated abnormalities , EC Is a

challenging congenital anomaly.
o In complete EC the heart is entirely outside the thoracic cavity with or
without
a pericardial
covering , and constitutes a
neonate
emergency.
o In partial EC, the
heart can be seen to pulsate through the
skin.
Regarding the 91 known published cases , the heart was uncovered in
41%, covered with serous membran in 31%, an d covered by skin in 27%.
oThe first report of EC was by Haller in 1706 , & it was classified
into
different types by Weese in 1818 & Todd in 1836 . Ectopia cordis
may
be grouped into cervical (3%) , cervicothoracic
(<1%) ,
thoracic
(60%),thoracoabdomina l (7%), or abdominal type (30%). Thoracoabdominal
EC has a better prognosis, whilst only
a few patients with
the thoracic type have survived and
the cervicaltype is
not compatible with life.
o Hypothese s regarding the aetiology of each EC
class
involve
abnormalities in thedevelopment of mesoderm
during early
embryogenesis . The purported defects includethe maturation failure of
several midline mesodermal components, including the heart, sternum,
rectus abdominis , diaphragm , and endocardium.

oThe

failure of cephalic anterior

folds to fuse in a timely manner
may result in the commonly
associated anomalies foundwith
EC. In the event of a true EC, the
failure of sternal fusion appears
to be secondaryto a primary
malposition of the heart itself. A
failureof the heart to descend
into the thorax during thethird
week of gestation may leave it
trapped abovethe closing upper
portion of the sternum, creating
either a cervical or
cervicothoracic ectopia.
The estimated prevalence rate
of ectopia cordis is 0.079/10,000
births and is said to occur more
in female infants.

Epidemiology .
oEctopia

Ccordis is a rare disease with incidence

of 5.5 to 7.9 cases per million births . Until
2001 , 267 cases have been reported , most
of them (95%) associated with other heart
diseases .
oAccording to the position of the misplaced heart
, ectopia cordis can be classified into five types :
1) CERVICAL :- It is the EC in which the heart is
located in the neck with sternum that is usually
intact .
2) THORACICO-CERVICAL:- It is the EC in which
the heart is partially in the cervical region but
the upper portion of sternum is split .
3) THORACIC:- It is the EC in which the
sternum is completely split or absent , and the
heart lies partially or completely outside the
thorax .

4) THORACO-ABDOMINAL:- It is the EC in which usually

accompanies Cantrells syndrome that causes defects involving
the diaphragm , abdominal wall , pericardium , heart and the
lower sternum . The Cantrells syndrome that is also known
as the pentalogy of Cantrell (PC) .
5) ABDOMINAL :- It is the EC in which the heart passes through
adefect in the diaphragm to enter the abdominal cavity .
Thoracic

and Thoraco-abdominal ectopia cordis constitute the

vast majority of known cases .

CASE REPORT :

Thoraco-Abdominal Ectopia

Cordis : A Rare Entity

Given by Ashwin V Apte
Department of Surgery , Peoles Collage of Medical Sciences and Research Center , Bhanpur , Bhopal

full term 2 hours old male boy weighing 2.4 kg was shifted to our hospital with ectopia
cordis . He was born to a26 years old mother by caesarian section due to fetal distress.
there was no history of consanguineous marriage,