Insaani Mukhlisah Supervisor : dr. DEWI ROBINAR, Sp.

A (K), IBCLC

 the

presence of an orifice in the diaphragm permits the herniation of abdominal contents into the thorax.

 CDH

is a rare condition that occurs in < 15:10000 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 pulmonary hypertension with high mortality.

 Defects

in fusion of these components were believed to give rise to the various diaphragmatic defects encountered

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

 Posterolateral:

Bochdaleks henia, most

common type. Anteromedial: Morgagnis hernia.

 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.

Genetic

CDH
Prematurity chromosom

Trisomy 21

Numerical chromosomal abnormalities

Trisomy 18

Trisomy 13,

del(1)(q41q42.12) del(4)(p16) WolfHirschhorn syndrome (WHS) Deletion del(8)(p23.1)

Structural chromosomal abnormalities

del(15)(q26.1q26.2)

Laboratory Examination

Physical Examination

History

 Genetic

counseling

Most cases are sporadic but there are some

reports of familial clusters that suggest multifactoral rather than autosomal recessive patterns of inheritance.

 Delayed

surgery

 has

gradually shifted from emergent repair to a policy of stabilization using a variety of ventilatory strategies before operation

ECMO Extra-corporeal membrane oxygenation useful adjunct treatment of CDH. Cannulation of both the right carotid artery and jugular vein 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

 Although

the lamb and rat models of CDH suggest surfactant deficiency this is controversial in humans.

 is

a specific phosphdiesterase-5 inhibitor shown to decrease pulmonary vascular resistance

 Name Age Gender Suku

: By. H : 9 hours : Male : Java

Dispneu and grunting

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.

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 good condition. HPHT 12-07-2012, 22-04-2013 TP.

 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.

 Pale

skin, weak muscle tone, movement is not active, whimpers, cold end extremity, takipneu (+), lethargy (+). Vital sign: HR : 164x/menit RR : 80 x/menit temperature : 36,7C
Birth

weigh Height

: 3080 gr : 49 cm

Nervous system : pale skin color, weak activity, lethargic, pupil 2mm/2mm, pupillary reflex (+ / +), seizures (-), weak muscle tone head : flat fontanella , normal suture, normal palatum, sianosis (-), low set ear (-) respiratory system : RR 80x/i, whimpers(+), nostril breathing (+), tightness (+), intercostal retractions (+), cyanosis (-), asymmetrical, the right kind of lagging, vesicular breath lower right, downe Score = 6

Cardiovaskular system : HR :164/menit S1 ,S2 normal, murmur (-), gallop (-)

Gastrointestinal system : normal Genitalia : Male Ekstremitas : simetris physical maturity (20), neuromuscular maturity (44), Ballard score (34), Estimated 36-38 weeks maturity

Diagnosis
Aterm neonates (37-38 weeks), pregnancy corresponding birth weight 3080 grams + enough + severe breathing + congenital diaphragmatic hernia

Treatment conservative therapy