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Congenital Anomalies

Dr. Laila Abu-Salem

By: Dr. Laila Abu-Salem

2014

Objectives
At the end of this lecture the students
will be able to describe and manage
the most common congenital
anomalies among children

By: Dr. Laila Abu-Salem

2014

Introduction
Congenital anomalies are evident in 23% of children at birth and may
reach to 6% by age 5 years by the
discovery of more anomalies.
Congenital anomalies are caused by
numerous factors ; genetic and
environmental.
By: Dr. Laila Abu-Salem

2014

Causes of Congenital

Drugs
Radiation
Viruses
Genetic trait

By: Dr. Laila Abu-Salem

2014

anomalies

Common Congenital

anomalies
in newborn

Respiratory:
1- Laryngeal stridor: noisy respiration because
abnormality of the larynx, the S&S mostly
appears when the child cries includes: cyanosis
dyspnea. Sternal retraction, intermittent
sucking, symptoms gradually disappears after
one year.

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

2- Choanal atresia:
congenital obstruction
of posterior nares at the
entrance to the nasopharynx. It may be
bilateral or unilateral

By: Dr. Laila Abu-Salem

2014

Choanal atresia

By: Dr. Laila Abu-Salem

2014

Congenital anomalies of
gastrointestinal system
1- Anomalies in the
mouth:

Cleft lip: a congenital


fissure of the upper lip
on the side of the
midline in the center of
nares. It may be
unilateral or bilateral

By: Dr. Laila Abu-Salem

2014

Cleft palate: Incomplete


fusion of the palate. The
condition may involve
the soft palate or hard
palate or both

By: Dr. Laila Abu-Salem

2014

2- Anomalies in the esophagus:


Esophageal atresia: there are many types the
most common is one in which the proximal
part ends in the closed pouch and the distal
part communicates with the trachea

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

Chlasia of the esophagus: neuromuscular


disorder in which the cardiac sphincter and the
lower portion of the esophagus are lax and
patent. S&S includes regurgitation, non
projectile vomiting immediately after feeding,
which leads to aspiration pneumonia and
esophageal structure

By: Dr. Laila Abu-Salem

2014

Anomalies of the
stomach and
dudenum
Pyloric stenosis:
hypertrophy of the
muscles surrounding
the pylorus that leads
to narrowing of the
pyloric canal
By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

Pyloric stenosis

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

Duodenal Obstruction

Duodenal obstruction: it
may be complete
(atresia) or incomplete
(stenosis) of the
duodenum.

By: Dr. Laila Abu-Salem

2014

Hiatus Hernia

Hiatus Hernia: it is
protrusion of the
stomach through the
hiatus in the diaphragm

By: Dr. Laila Abu-Salem

2014

Anomalies in the
intestine
Imperforate anus: it is
either: stenosis,
membranous, agenesis
(rectum has blind end) it
may be high or low, a
fistula between the
rectum and urinary tract
may be present
By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

Omphalocele: a
protrusion of the
abdominal cavity into
the base of the umbilical
cord through defect in
the anterior abdominal
wall

By: Dr. Laila Abu-Salem

2014

Intestinal atresia: It is
interruption in the
continuity of the
intestine which may
take the form of septum,
stenosis, atresia of
varying length or
multiple atresia at any
point of the small or
large intestine
By: Dr. Laila Abu-Salem

2014

Diaphragmatic hernia: is
a protrusion of the
viscera mainly intestine
through a defect in the
diaphragm in to the
chest cavity

By: Dr. Laila Abu-Salem

2014

Hirshsprungs disease:
congenital absence of
parasympathetic
ganglion nerve cells of a
part of intestine usually
in the distal end of the
descending colon

By: Dr. Laila Abu-Salem

2014

Intussusception:
invagination of a
portion of the bowel
into the portion
immediately distal to it,
the blood supply cut off
which leads to gangrene

By: Dr. Laila Abu-Salem

2014

Intussusception

By: Dr. Laila Abu-Salem

2014

Congenital Anomalies of the urinary


System

Epispadias: urethral
opening located on
dorsal of superior
surface of the penis
Hypospadias: urethral
opening located behind
glands penis or
anywhere along ventral
(lower) surface of penile
shaft
By: Dr. Laila Abu-Salem

2014

Phimosis: stenosis of
preputial opening of
foreskin

By: Dr. Laila Abu-Salem

2014

Hydrocele: fluid in the


scrotum

By: Dr. Laila Abu-Salem

2014

Inguinal hernia:
protrusion of the
abdominal content
through inguinal canal

By: Dr. Laila Abu-Salem

2014

Inguinal hernia

By: Dr. Laila Abu-Salem

2014

Polycystic kidney

Polycystic kidney:
enlarged kidney filled
cysts if the condition
bilateral, the infant will
not pass urine but if it is
unilateral may be
missed until later life.

By: Dr. Laila Abu-Salem

2014

Wilms tumor

Wilms tumor:
malignant tumor of the
kidney that arise from
an embryonic structure

By: Dr. Laila Abu-Salem

2014

CNS Congenital Defect

Spina Bifida: is a
defective closure of the
vertebral column. The
consequences of the
defect depends on the
site & the extent of the
defect.

By: Dr. Laila Abu-Salem

2014

Spina Bifida Occulta:


the defect mostly
involving the 5th lumber
and 1st sacral vertebra
are affected with no
protrusion of the
interspinal contents. The
skin over the defect may
reveal a dimple, lipoma,
tuft of hair
By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

Meningocele

Meningocele: Visible
saclike mass on the back
which contains spinal
fluids & meninges.

By: Dr. Laila Abu-Salem

2014

Meningomyelocele:
more serious defect
in which the spinal
cord, and/or nerve
roots as well as
meningeal covering
protrude through the
defect.
By: Dr. Laila Abu-Salem

2014

Hydrocephalus

Hydrocephalus: An
imbalance between the
production of CSF and
its absorption into the
circulation due to
defect in the
cerebrospinal dilate

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

Orthopedic Anomalies

Clubfoot: flexion at the


ankle with inversion of
the heel and fore foot

By: Dr. Laila Abu-Salem

2014

Torticollis

Torticollis: lateral
inclination and a
rotation of the head
away from the midline
of the body with
limitation of range of
motion of the neck

By: Dr. Laila Abu-Salem

2014

Torticollis

By: Dr. Laila Abu-Salem

2014

Congenital Hip Dislocation

Congenital Hip
Dislocation:the femur head
is completely dislocated
from the acetabulum. The
infant shows limited
disability to abduct the hip,
asymmetry of the gluteal
skin fold & inguinal creases
with shortening of the
affected leg

By: Dr. Laila Abu-Salem

2014

Hip Dislocation

By: Dr. Laila Abu-Salem

2014

Surgery related differences between


young children &adult
Metabolic rate is higher in children than adult
So they need to be fed frequently
Healing is faster in children than adult
Less analgesic is needed in children
Child lacks the reserve physical resources
fluid & electrolytes is serious in children

By: Dr. Laila Abu-Salem

2014

General aspect of pre & post


operative care

Transportation of the newborn should be:


Safe
In a heated incubator
Accompanied with O2. suctioning, proper
observation & intervention whenever
appropriate
All pertinent information
By: Dr. Laila Abu-Salem

2014

Pre-operative care

Psychological preparation
Be free from respiratory infection except in
emergency situations
NPO before operation with consideration of
age (3-4 hours for neonates)
Prepare the skin at the site of operation
Check the mouth
Remove pins from childs hair
By: Dr. Laila Abu-Salem

2014

Cont. Pre-operative care

Clean, loose, warm hospital gown


Check identification band
Pre-medication
Empty bowel &bladder (use enema only if
prescribed)
Clean nostrils
Let the child to take favorite toy and allow the parent
to accompany their child
Tell the parent where to wait
By: Dr. Laila Abu-Salem

2014

Post-Operative care

Close observation; Airway and V.S


Keep the child warm
Keep the child on side until become alert
Check wound, I.V fluid, urinary output, observe the
skin for: Temp., color & sings of signs of shock
Start oral fluid as tolerated while infusion is on if;
aspirate is clear, peristaltic movements are heard,
gases are passed. Then soft diet according the child's
age
Sedative as order
Ambulation according the childs age &the type of
operation
By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

By: Dr. Laila Abu-Salem

2014

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