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7/29/2019 Congenital Hernia Diphragmatica
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Insaani Mukhlisah
Supervisor : dr. DEWI ROBINAR,Sp.A (K), IBCLC
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the presence of an orifice in the diaphragm
permits the herniation of abdominal
contents into the thorax.
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CDH is a rare condition that occurs in < 1-
5:10000 births . It seems to be slightly
more frequent in males and less frequent
in blacks.
The lungs are hypoplastic and have
abnormal vessels that cause respiratory
insufficiency and persistent pulmonaryhypertension with high mortality.
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Defects in fusion of
these components were
believed to give rise to
the various
diaphragmatic defects
encountered
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The diaphragm forms from the fusion of the septum
transversum, the paired pleuroperitoneal membranes,
the mesenchyme that arises adjacent to the esophagus,
and the ingrowth of muscles from the body wall
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Posterolateral: Bochdaleks henia, most
common type.
Anteromedial: Morgagnis hernia.
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Most diaphragmatic defects involve the
posterior and lateral aspects of the
diaphragm, such as the posterolateral or
Bochdalek CDH (80-90%)
In about 5%, the anterior retrosternal or
parasternal diaphragm, referred to as the
Morgagni-Larrey hernia.
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CDH
Genetic
chromosomPrematurity
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Numericalchromosomalabnormalities
Trisomy 21
Trisomy 18
Trisomy 13,
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Structuralchromosomalabnormalities
Deletion
del(1)(q41-q42.12)
del(4)(p16) Wolf-Hirschhorn
syndrome (WHS)
del(8)(p23.1)
del(15)(q26.1-q26.2)
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History
PhysicalExamination
LaboratoryExamination
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Genetic counseling Most cases are sporadic but there are some
reports of familial clusters that suggest
multifactoral rather than autosomal recessivepatterns of inheritance.
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Delayed surgery
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has gradually shifted from emergent repair
to a policy of stabilization using a variety of
ventilatory strategies before operation
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ECMO
Extra-corporeal membrane oxygenation useful adjunct
treatment of CDH.
Cannulation of both the right carotid artery and jugularvein and connection to a circuit with a membrane gas
exchange chamber allows oxygenation and CO2
disposal without participation of the lung which is
preserved from any pressure insult
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Although the lamb and rat models of CDH
suggest surfactant deficiency this is
controversial in humans.
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is a specific phosphdiesterase-5 inhibitor
shown to decrease pulmonary
vascular resistance
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Name : By. H
Age : 9 hours
Gender : Male
Suku : Java
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Dispneu and grunting
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Neonate 9 hours old born on April 4 2013 at 2 pm in Andini Hospital
by SC on oblique position indication, 02 resuscitation performed until
delivery
State of the patient whimpers after birth (+), Apgar score 6/8,
retraction (+), tightness (+), lethargy (+), cyanosis (+), cold
extremity(+), vomiting (+) frequency 2 times contains milk formula,
residual amnion fluid is clear, Neo K injection (+), eye ointment (+),
has not given breast milk, formula already given, birth injury (-), BAB
(-), BAK (-), seizures (-), bloating (+), yellow (-), shortness of (+)
The patient was taken to the installation of neonates because
whimpers, the patient arrived at the installation of neonates at 22:41
pm.
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G2P1A0H1, maternal age 34 years, 38-39 weeks
gestational age, history of hypertension (-), DM (-),
leukorea (-), fever (-), drinking alcohol (-), smoking (-),
taking medications (- ), traumatic (-). ANC regularly every
month to obstetricians, 6x ultrasound, fetal goodcondition. HPHT 12-07-2012, 22-04-2013 TP.
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Two days before birth, Patient ANC to
obstetricians and baby sonogram was
oblique. Planned elective SC April 4, 2013
at 12:30 SC begins. Babies born April 4 At
14.00.
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Pale skin, weak muscle tone, movement isnot active, whimpers, cold end extremity,takipneu (+), lethargy (+).
Vital sign:HR : 164x/menitRR : 80 x/menit temperature : 36,7C
Birth weigh : 3080 grHeight : 49 cm
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Nervous system : pale skin color, weak activity,lethargic, pupil 2mm/2mm, pupillary reflex (+ / +),seizures (-), weak muscle tone
head : flat fontanella , normal suture, normalpalatum, sianosis (-), low set ear (-)
respiratory system : RR 80x/i, whimpers(+),nostril breathing (+), tightness (+), intercostal
retractions (+), cyanosis (-), asymmetrical, the rightkind of lagging, vesicular breath lower right,downe Score = 6
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Cardiovaskular system :
HR :164/menit
S1 ,S2 normal, murmur (-), gallop (-)
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Gastrointestinal system : normal
Genitalia : Male
Ekstremitas : simetris
physical maturity (20), neuromuscular
maturity (44), Ballard score (34), Estimated
36-38 weeks maturity
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DiagnosisAterm neonates (37-38 weeks),
pregnancy corresponding birth weight
3080 grams + enough + severebreathing + congenital diaphragmatic
hernia
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Treatment conservative therapy
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