congenital hernia diphragmatica

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

congenital hernia diphragmatica

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