congenital anomalies 2 (1)

8/10/2019 Congenital Anomalies 2 (1)

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Congenital Anomalies

Dr. Laila Abu-Salem

By: Dr. Laila Abu-Salem 2014


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Objectives

At the end of this lecture the students

will be able to describe and manage

the most common congenitalanomalies among children



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Introduction

Congenital anomalies are evident in 2-

3% of children at birth and may

reach to 6% by age 5 years by thediscovery of more anomalies.

Congenital anomalies are caused by

numerous factors ; genetic and

environmental.



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Causes of Congenital anomalies

Drugs

Radiation

Viruses

Genetic trait



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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.



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2- Choanal atresia:

congenital obstruction

of posterior nares at the

entrance to the naso-pharynx. It may be

bilateral or unilateral



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Choanal atresia



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Congenital anomalies of

gastrointestinal system1- 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 beunilateral or bilateral

http://images.google.com.eg/imgres?imgurl=http://www.craniofacialcenter.com/book/clefts/images/Cleft_11.jpg&imgrefurl=http://www.craniofacialcenter.com/book/clefts/Cleft_5.htm&h=242&w=320&sz=20&hl=ar&start=14&tbnid=_3a5-CFOiDPs8M:&tbnh=89&tbnw=118&prev=/images%3Fq%3DCleft%2Blip%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.craniofacialcenter.com/book/clefts/images/Cleft_11.jpg&imgrefurl=http://www.craniofacialcenter.com/book/clefts/Cleft_5.htm&h=242&w=320&sz=20&hl=ar&start=14&tbnid=_3a5-CFOiDPs8M:&tbnh=89&tbnw=118&prev=/images%3Fq%3DCleft%2Blip%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.mcg.edu/centers/cranio/images/6.jpg&imgrefurl=http://www.mcg.edu/centers/cranio/cleft.htm&h=434&w=288&sz=17&hl=ar&start=8&tbnid=ACTYOEPJdsruHM:&tbnh=126&tbnw=84&prev=/images%3Fq%3DCleft%2Blip%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DG


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Cleft palate: Incomplete

fusion of the palate. The

condition may involve

the soft palate or hardpalate or both

http://images.google.com.eg/imgres?imgurl=http://health.yahoo.com/media/healthwise/h5550987.jpg&imgrefurl=http://health.yahoo.com/topic/children/baby/article/healthwise/popup/zm2249&h=300&w=460&sz=25&hl=ar&start=4&tbnid=8ZQdKksNUTX1hM:&tbnh=83&tbnw=128&prev=/images%3Fq%3DCleft%2Bpalate%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.valleyhealth.com/images/image_popup/fl7_cleft_palate.jpg&imgrefurl=http://www.valleyhealth.com/Health_Library/mayo_catlinks.asp%3Fnavid%3D9%26sp%3D766&h=300&w=400&sz=19&hl=ar&start=7&tbnid=jYi51z-z7crlJM:&tbnh=93&tbnw=124&prev=/images%3Fq%3DCleft%2Bpalate%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.thefetus.net/images/article-images/face_and_neck/bilateral_cleft_anu_files/cleft%2520lip%2520and%2520palate%25201.jpg&imgrefurl=http://www.thefetus.net/page.php%3Fid%3D192&h=308&w=308&sz=12&hl=ar&start=2&tbnid=CblS_fclNS-aTM:&tbnh=117&tbnw=117&prev=/images%3Fq%3DCleft%2Bpalate%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DG


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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



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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



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Anomalies of thestomach anddudenum

Pyloric stenosis:hypertrophy of themuscles surroundingthe pylorus that leadsto narrowing of thepyloric canal



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Pyloric stenosis



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Duodenal Obstruction

Duodenal obstruction: it

may be complete

(atresia) or incomplete

(stenosis) of theduodenum.



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Hiatus Hernia

Hiatus Hernia: it is

protrusion of the

stomach through the

hiatus in the diaphragm



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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 therectum and urinary tract

may be present



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Omphalocele: a

protrusion of the

abdominal cavity into

the base of the umbilicalcord through defect in

the anterior abdominal

wall



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Intestinal atresia: It is

interruption in the

continuity of the

intestine which maytake the form of septum,

stenosis, atresia of

varying length or

multiple atresia at anypoint of the small or

large intestine



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Diaphragmatic hernia: is

a protrusion of the

viscera mainly intestine

through a defect in thediaphragm in to the

chest cavity



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Hirshsprungs disease:

congenital absence of

parasympathetic

ganglion nerve cells of apart of intestine usually

in the distal end of the

descending colon

http://images.google.com.eg/imgres?imgurl=http://rad.usuhs.edu/medpix/tachy_pics/thumb/synpic32826.jpg&imgrefurl=http://rad.usuhs.edu/medpix/tf_case.html%3Fmode%3Dpt%26pt_id%3D10248&h=98&w=130&sz=2&hl=ar&start=22&um=1&tbnid=cGnkwzc1YIrAFM:&tbnh=69&tbnw=91&prev=/images%3Fq%3DHirshsprung%25E2%2580%2599s%2Bdisease%26start%3D20%26ndsp%3D20%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DNhttp://images.google.com.eg/imgres?imgurl=http://rad.usuhs.edu/medpix/tachy_pics/thumb/synpic32827.jpg&imgrefurl=http://rad.usuhs.edu/medpix/tf_case.html%3Fmode%3Dpt%26pt_id%3D10248&h=98&w=130&sz=2&hl=ar&start=21&um=1&tbnid=HBOIls6y3wWGMM:&tbnh=69&tbnw=91&prev=/images%3Fq%3DHirshsprung%25E2%2580%2599s%2Bdisease%26start%3D20%26ndsp%3D20%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DN


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Intussusception:

invagination of a

portion of the bowel

into the portionimmediately distal to it,

the blood supply cut off

which leads to gangrene



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Intussusception



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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



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Phimosis: stenosis of

preputial opening of

foreskin



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Hydrocele: fluid in the

scrotum

http://images.google.com.eg/imgres?imgurl=http://www.hope.edu/academic/kinesiology/athtrain/program/studentprojects/405-03/GI/hernia.jpg&imgrefurl=http://www.hope.edu/academic/kinesiology/athtrain/program/studentprojects/405-03/GI/Gastrointestinal.html&h=400&w=356&sz=19&hl=ar&start=3&um=1&tbnid=dhyR1vXe6136SM:&tbnh=124&tbnw=110&prev=/images%3Fq%3DInguinal%2Bhernia%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DG


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Inguinal hernia:

protrusion of the

abdominal content

through inguinal canal

http://images.google.com.eg/imgres?imgurl=http://www.hope.edu/academic/kinesiology/athtrain/program/studentprojects/405-03/GI/hernia.jpg&imgrefurl=http://www.hope.edu/academic/kinesiology/athtrain/program/studentprojects/405-03/GI/Gastrointestinal.html&h=400&w=356&sz=19&hl=ar&start=3&um=1&tbnid=dhyR1vXe6136SM:&tbnh=124&tbnw=110&prev=/images%3Fq%3DInguinal%2Bhernia%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://telesalud.ucaldas.edu.co/telesalud/Atlas%2520de%2520Cirug%C3%ADa%2520Pedi%C3%A1trica/Imagenes/Urologia/Canal%2520inguinal/Hernia%2520inguinal/hernia_inguinal.jpg&imgrefurl=http://telesalud.ucaldas.edu.co/telesalud/Atlas%2520de%2520Cirug%25C3%25ADa%2520Pedi%25C3%25A1trica/Espa%25C3%25B1ol/Urologia/hernia_inguinal.htm&h=524&w=851&sz=30&hl=ar&start=39&um=1&tbnid=A5du9ooKyfEtuM:&tbnh=89&tbnw=145&prev=/images%3Fq%3DInguinal%2Bhernia%26start%3D20%26ndsp%3D20%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DNhttp://images.google.com.eg/imgres?imgurl=http://www.childrenscentralcal.org/images/health_images/ei_0256.gif&imgrefurl=http://www.childrenscentralcal.org/content.asp%3Fpageid%3DP01998&h=226&w=400&sz=18&hl=ar&start=13&um=1&tbnid=f0vUbMd38dOegM:&tbnh=70&tbnw=124&prev=/images%3Fq%3DInguinal%2Bhernia%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.pediatriconcall.com/forpatients/commonchild/images/inguinal_hernia.jpg&imgrefurl=http://www.pediatriconcall.com/forpatients/commonchild/inguinal_hernia.asp&h=150&w=200&sz=26&hl=ar&start=12&um=1&tbnid=w0FZ8PysjVroRM:&tbnh=78&tbnw=104&prev=/images%3Fq%3DInguinal%2Bhernia%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DG


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Inguinal hernia



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Polycystic kidney

Polycystic kidney:

enlarged kidney filled

cysts if the condition

bilateral, the infant willnot pass urine but if it is

unilateral may be

missed until later life.

http://images.google.com.eg/imgres?imgurl=http://www.waxy.org/random/images/weblog/dads_kidneys.jpg&imgrefurl=http://www.waxy.org/archive/2002/05/31/dads_kid.shtml&h=850&w=1280&sz=201&hl=ar&start=4&um=1&tbnid=ObmmCJvs4aLm_M:&tbnh=100&tbnw=150&prev=/images%3Fq%3Dpolycystic%2Bkidney%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.waxy.org/random/images/weblog/dads_kidneys.jpg&imgrefurl=http://www.waxy.org/archive/2002/05/31/dads_kid.shtml&h=850&w=1280&sz=201&hl=ar&start=4&um=1&tbnid=ObmmCJvs4aLm_M:&tbnh=100&tbnw=150&prev=/images%3Fq%3Dpolycystic%2Bkidney%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.valleyhealth.com/images/image_popup/r7_kidneyscompared.jpg&imgrefurl=http://www.valleyhealth.com/Health_Library/mayo_catlinks.asp%3Fnavid%3D21%26sp%3D345&h=306&w=400&sz=11&hl=ar&start=7&um=1&tbnid=GtJK4Z00A1HNVM:&tbnh=95&tbnw=124&prev=/images%3Fq%3Dpolycystic%2Bkidney%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DG


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Wilms tumor

Wilms tumor:

malignant tumor of the

kidney that arise from

an embryonic structure

http://images.google.com.eg/imgres?imgurl=http://www.ispub.com/xml/journals/iju/vol3n2/renal-fig2.jpg&imgrefurl=http://www.ispub.com/ostia/index.php%3FxmlPrinter%3Dtrue%26xmlFilePath%3Djournals/iju/vol3n2/renal.xml&h=483&w=642&sz=233&hl=ar&start=37&um=1&tbnid=sTlSqxKtMXbDSM:&tbnh=103&tbnw=137&prev=/images%3Fq%3DWilm%2527s%2Btumor%26start%3D20%26ndsp%3D20%26svnum%3D10%26um%3D1%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DN


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CNS Congenital Defect

Spina Bifida: is a

defective closure of the

vertebral column. The

consequences of thedefect depends on the

site & the extent of the

defect.



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Spina Bifida Occulta:

the defect mostly

involving the 5thlumber

and 1st

sacral vertebraare affected with no

protrusion of the

interspinal contents. The

skin over the defect mayreveal a dimple, lipoma,

tuft of hair

http://www.spineuniverse.com/displaygraphic.php/409/shutack_fig2-BB.gifhttp://images.google.com.eg/imgres?imgurl=http://www.spineuniverse.com/displaygraphic.php/410/shutack_fig3-BB.gif&imgrefurl=http://www.spineuniverse.com/displayarticle.php/article234.html&h=210&w=257&sz=11&hl=ar&start=3&tbnid=CkaReguvGciKWM:&tbnh=92&tbnw=112&prev=/images%3Fq%3DSpina%2BBifida%2BOcculta%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DG


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Meningocele

Meningocele: Visible

saclike mass on the back

which contains spinal

fluids & meninges.

http://images.google.com.eg/imgres?imgurl=http://www.neurocirugia.com/intervenciones/meningocele/mielom1.jpg&imgrefurl=http://www.neurocirugia.com/intervenciones/meningocele/Meningocele.htm&h=312&w=453&sz=18&hl=ar&start=4&tbnid=VwsFHxQC3UeYcM:&tbnh=87&tbnw=127&prev=/images%3Fq%3DMeningocele%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://library.med.utah.edu/WebPath/jpeg3/PERI094.jpg&imgrefurl=http://library.med.utah.edu/WebPath/PEDHTML/PED027.html&h=330&w=504&sz=70&hl=ar&start=1&tbnid=nFG7fagly-ZH7M:&tbnh=85&tbnw=130&prev=/images%3Fq%3DMeningomyelocele%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DG


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Meningomyelocele:

more serious defect

in which the spinal

cord, and/or nerveroots as well as

meningeal covering

protrude through thedefect.

http://images.google.com.eg/imgres?imgurl=http://library.med.utah.edu/WebPath/jpeg3/PERI094.jpg&imgrefurl=http://library.med.utah.edu/WebPath/PEDHTML/PED027.html&h=330&w=504&sz=70&hl=ar&start=1&tbnid=nFG7fagly-ZH7M:&tbnh=85&tbnw=130&prev=/images%3Fq%3DMeningomyelocele%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.sgrh.com/dept/paedsurg/image016.jpg&imgrefurl=http://www.sgrh.com/dept/paedsurg.htm&h=155&w=207&sz=5&hl=ar&start=5&tbnid=WzSUZxYafgSkTM:&tbnh=79&tbnw=105&prev=/images%3Fq%3DMeningomyelocele%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DG


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Hydrocephalus

Hydrocephalus: An

imbalance between the

production of CSF and

its absorption into thecirculation due to

defect in the

cerebrospinal dilate



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Orthopedic Anomalies

Clubfoot: flexion at the

ankle with inversion of

the heel and fore foot



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Torticollis

Torticollis: lateral

inclination and a

rotation of the head

away from the midlineof the body with

limitation of range of

motion of the neck



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Torticollis



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Congenital Hip Dislocation

Congenital Hip

Dislocation:the femur head

is completely dislocated

from the acetabulum. The

infant shows limiteddisability to abduct the hip,

asymmetry of the gluteal

skin fold & inguinal creases

with shortening of theaffected leg



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Hip Dislocation


S l t d diff b t
http://images.google.com.eg/imgres?imgurl=http://www.aafp.org/afp/20061015/1310-f2.jpg&imgrefurl=http://www.aafp.org/afp/20061015/1310.html&h=412&w=240&sz=22&hl=ar&start=5&tbnid=SqJZayg4ExEr4M:&tbnh=125&tbnw=73&prev=/images%3Fq%3DCongenital%2BHip%2BDislocation:the%2B%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.aafp.org/afp/20061015/1310-f3.jpg&imgrefurl=http://www.aafp.org/afp/20061015/1310.html&h=191&w=270&sz=14&hl=ar&start=6&tbnid=Sm5vI3SUdVEniM:&tbnh=80&tbnw=113&prev=/images%3Fq%3DCongenital%2BHip%2BDislocation:the%2B%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DGhttp://images.google.com.eg/imgres?imgurl=http://www.aafp.org/afp/20061015/1310-f2.jpg&imgrefurl=http://www.aafp.org/afp/20061015/1310.html&h=412&w=240&sz=22&hl=ar&start=5&tbnid=SqJZayg4ExEr4M:&tbnh=125&tbnw=73&prev=/images%3Fq%3DCongenital%2BHip%2BDislocation:the%2B%26gbv%3D2%26svnum%3D10%26hl%3Dar%26rlz%3D1T4SHCN_enEG218EG219%26sa%3DG


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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


G l t f & t


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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



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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



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Cont. Pre-operative care

Clean, loose, warm hospital gown

Check identification band

Pre-medication

Empty bowel &bladder (use enema only ifprescribed)

Clean nostrils

Let the child to take favorite toy and allow the parentto accompany their child

Tell the parent where to wait



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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 theskin 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 ofoperation



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B D L il Ab S l 2014

congenital anomalies 2 (1)

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