English case presentation Submitted to :

Fakhrurrazi Mr/Mrs

Congenital Diaphragmatic Hernia

Introduction

Congenital Diaphragmatic Hernia (CDH) is characterized by a defect in the

diaphragm leading to the protrusion ofabdominal contents into the thoracic cavity
affecting the normal development of the lungs. The condition may present as an
isolated lesion or as part of a syndrome. The incidence of CDH based on the
available literatureranges from approximately 0.8 - 5/10,000 births andvaries
across the population1 There is slightly higher male predominance and a lower
risk of isolated CDH reported among African-Americans 2. In spite of advances
made in the medical and surgical management of CDH, the mortality and
morbidity remain high 3. CDH infants also have a prolonged length of stay in the
hospital requiring multi-disciplinary approach for their management and follow-
up afterhospital discharge.

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CASE ILLUSTRATION

Baby Boy, 6 hours old, was hospitalized in Neonatal Intensive Care Unit
(NICU) of Dr. M Djamil Hospital Padang Since 20th November -17th Desember
2016 the patient was referred from Bukittingi Distric Hospital with notes
respitarory distres due to susp pneumonia neonatal. The chief complain was
breathlesness and grunting since born.

Present illness history (alloanamnesis was obtained from her father and
nurse). Normal birth weight neonates 3900 gr, birth length 52 cm, spontaneous
delivery helped by midwife, American Pediatric Gross Assessment Record
(APGAR) score 3 in fist minute and 5 in five minute (outhospital delivery).
Mother was fine, clearly amniotic fluid with normal amount. Aterm pregnancy
maturity appraisal 37-38 weeks. Breathlesness and grunting since born and
patients received 1 liter oxygen using nasal cannula from the operating room.
There is cyanosis relieved by oxygen. There is no fever , no seizure. Patients have
not been given breast feed or drink. Micturation and mekonium had been
present. Vitamin K injection had been given. No history of mother with fever,
pain when micturition and flour albus.

The patient has been taken to the emergency room Ahmad Muchtar
district hospital and referred with note respiratory distres due to suspct pneumonia
neonatal.

Family illness history

There is no family suffer similiar illness like this,

Pregnancy History

Patient was thirth child from 3 sibling. Last mother's pregnancy was 3
years ago and mother did not use any contraceptive drugs before. During
pregnancy the mother feels healthy, regularly checks pregnancy to the midwife,
never controls to obstetrician and never performs ultrasound during pregnancy.
Mothers regularly take vitamins given midwives and never consume drugs and
herbs, do not smoke and never get irradiation.

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 Mothers regularly eat during pregnancy, frequency 3 times per day, spend
1 serving / times. The last weight before labor 62 kg with weight gain during
pregnancy `15 kg .

Socio-Economic History and Environmental Conditions

Mother of 35 year old patient, moslem, high school education, working as

civil servant with income of Rp. 2,500,000 / month. Father of patient aged 40
years, moslem, high school education, working as as civil servant with income
Rp. 4,000,000 / month. Family lives in a semi permanent house with a cement
floor, consisting of 2 bedrooms, 1 living room and 1 bathroom. The yard is wide
enough, the source of drinking water comes from the well water, the garbage is
burned. Good home ventilation, electricity facilities from PLN. The cost of patient
care use goverment insurance.

Impression: Patients come from moderate socioeconomic class, with adequate

hygiene and environmental sanitation.

Immunization history

Patient has never been immunized since birth.

Physical Examination

Vital Sign
Heart rate : 155 times per minute, regularly
Respiratory rate : 70 times per minute, regular, no adequate
Downe score : 6 (takipneu, epigastric retraction, cyanosis relieved with
02, grunting heardwith stethoscope)
Temperature : 36.7 0C
Oxygen saturation : 75-80% without oxygen, 80-85 with oxygen 1 litre/i

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Nutritional & anthropometric status

Weight : 3900 grams (P50 - P90 Fenton curve)

Body length : 52 cm (P50 -P90 Fenton curve)
Head circumference : 35 cm (P50 Fenton curve)
Clinical impression : Suitable for neonates less than 38 weeks of age (Fenton
curve)

Physical examinations

Skin : Felt warm, cyanosis, no jaundice, no lanugo

Head : Round symmetry, no delivery lesi and deformity, fontanela major

opened, flat, 2x2 cm, head circumference 35 cm (normosefal)

Hair : Black, smooth and thin

Face : Symmetrical, does not dysmorphic

Eyes : Conjunctiva not pale, sclera not jaundice, palpebra not edema,

isokor pupil, 3 mm diameter, good light reflex

Ears : Pinna soft, fast recoil

Nose : Nasal Flare

Mouth : Oral cyanosis found, oral mucosa and wet lips

Neck : No enlarged lymph nodes are found

Chest : Symmetrical in both static and dynamic states, epigastric,

interkostal retraction.

Lungs : Bronchovesikuler breath sounds decrease in right side, bowel

sounds not clearly, no ronki or wheezing

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Cardiac : No cordic visible, regular heart sound, no mur mur, no single

second heard sound

Abdomen : Not visible bulge, no scafoid, cord not found hiperemis and

consistency chewy, lien not palpable, abdominal circumference

30 cm, normal positive bowel sounds

Back : No sacral dimple

Periana l : No abnormalities

Genitalia : No abnormalities

Extremities : Warm acral, capillary fill time less than 2 seconds, no leg edema,

Moro reflex not examined, palmar grasp + / +, plantar grasp + / +,

symmetric movement, no paresis.

Laboratory finding were hemoglobin 15,1 gr/dl, leucocyte 16.200 /mm3

differential count 0/0/0/83/14/3 platelet 180.000/mm3.

Thorax X ray

Expertise : Photo condition is too soft, It appears inhomogenous lesions look like
intestinal images in the left hemithorac, heart pushing to the right, right
diaphragma not clear, left diaphragma was good. Sinus costofrenicus taper
Impression Diaphragmatic Hernia

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List of Problems:

1. Respiratory distress due to Congenital Diaphragmatic Hernia.

2. Congenital Diaphragmatic Hernia Susp Bochdalek Hernia

3. Nutrition is not yet adequate.

4. Immunization has not been performed.

Working Diagnosis

1. Normal birth weihgt 3900 gram, normal gestation age.

2. Congenital Diaphragmatic Hernia Susp Bochdalek Hernia

Management

1. Management of Emergencies
A. Airway: Maintains adequate airway, free airway from mucus
B. Breathing: intubated with manual ventilatioan 40-60x/i and prepare
ventilator installation with SIMV mode PEEP 6 , PIP 18, RR 40x/i, and
FiO2 40%, keep oxygen saturation more than 88% to maintain brain
oxygenation. Oral Gastric Tube instalation with decompression state
C. Circulation: administration of intravenous fluids, administration of
electrolyte maintenance and correction if electrolyte disturbance is
found.
D. Temperature treat in incubator, keep the temperature between 36.6 C
to 37.5 C.
E. Sugar: administration of parenteral nutrition while being fasting.
blood glucose examination cek serialy, keep GIR at least 6 mg /
kgBB / minute, 10% dextrose bolus if hypoglycemia is present.

2. Diagnostic Investigation Plan

a. Thorax X ray
b. Complete blood peripheral examination,
c. Blood Gas Analysis
d. Electrolyte

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 e. Blood cultures
3. Medical Treatment Plan
a. Ampicilin Sulbactam 2 x 200 mg ivB. Gentamisin 1 x 18 mg iv
b. Consult to surgery departemen for surgery treatment
4. Pediatric NutritionCare
a. Assessment: Chronological age: 1 days, 38 weeks' gestation, body
weight: 3900 grams (P50-P90 Fenton curve), body length: 52 cm
(P50-P90 Fenton curve), head circumference: 35 cm (P50 Fenton
curve), impression: Normal Birt weight, Normal gestational age
b. Number of calories to be given:
- RDA: 120 kcal / kgb / day
- RDA absolute: 3,9 x 120 kcal / day = 468 kcal / day. RDA
ideal: 3,2 kg x 120 kcal / day = 384 kcal / day
- BMR: 3,2 x 60 kcal / kg / day = 192 kcal / day
- Stress factor : 1.5
- BEE: BMR x stress factor = 192 kcal x 1.5 = 288
Kcal / day
c. Food formulation: Parenteral nutrition and breast milk
d. Route of giving: Intravenous and per enteral
e. Currently get: IVFD PG1 60 ml / kgBB / day containing dextrose
11% with GIR 4,5 ,
f. Nutrition plan: parenteral nutrition with fluid administration of
PG2 containing dextrose 11% with GIR 6,9 gram / kg / day protein
and 3 grams / kg / day. Total fluid given maximum 150 Ml /
kgBB / day. Enteral nutrition in the form of breast milk is given
gradually to achieve Recommended daily allowance (RDA)
according to drinking tolerance with a target of at least 120 kcal /
kg / day after patient can get oral feeding.
g. Monitoring and evaluation: acceptability, tolerance, calorie
increase gradually.

Monitoring Plan

1. Vital signs and clinical manifestations (vital signs, evaluation of

respiratory distress and congenital diaphragmatic hernia)
2. Laboratory evaluation: septic work up (complete peripheral blood test, and
blood culture),
3. Surgery evaluation : preparation pre and after surgery.

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 4. Evaluation of parenteral nutrition and enteral nutrition as well as
assessment of drinking tolerance and plan of vitamin and iron
supplementation after fullfeed patient.
5. Periodic monitoring of body weight (daily), body length (weekly) and
head circumference (weekly).
6. Screening plans for high-risk infant morbidity screening, such as vision,
hearing.
7. Evaluate developments using the Fenton curve and denver developmental
screening test (DDST II).

Immunization plan

The immunization is adjusted to the recommendation of Indonesian

Pediatric Association. Immunizations in premature infants are given according to
chronological age. Hepatitis B1 immunization is delayed until the baby is stable.

Follow Up

1st day (November 20st 2016)

1st day (November 20st 2016)
S Breathlessness decreased, Spontaneous breath adequate with suport
ventilator SIMV mode PEEP 6 , PIP 18, RR 40x/i, and FiO2 40%, no
desaturated, still fasting, no vomitus, no abdominal bloated, mixturation
and nobdefecation yet . Normal with fluid balance -20cc, diuresis 1,5
cc/bw/hour.
O Patients less active, respiratory rate 60-70 times per minute, heart rate
150-156 times per minute, regular, 37 C, oxygen saturation 92-96%
preductal and post ductal, body weight 3900 grams
Skin: No jaundiced.

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 Eyes: no palpebra edema, no pale conjunctiva, no sclera jaundice .
Chest: symmetrical chest movement, minimal epigastric retraction seen,
lung bowel sounds positif in sinistra, heart not found abnormalities.
Abdomen: no bulge no scafoid, no distension, normal bowel sound
Ekstemitas: warm akral, capillary refill time <2 sec
A Respiratory distres stable with ventilator mode SIMV
Stable Diaphragmatic Hernia.
P SIMV Ventilator PEEP 6, PIP 18, RR 60x/i, FiO240%
IVFD PG180 ml/bw/day
Fasting
Ampicilin Sulbactam 2 x 200 mg iv
Gentamisin 1 x 18 mg iv
Ask planing from child surgery departement
E Blood gas analysis : pH 7,34, pCO2 45, pO2 47, HCO3- 22,7, BE -3,
Saturation 81%. Monitor saturation 96 %
Impression : Normal with hipoxemia couse mix venous blood.

Blood Electrolyte : Sodium 137 mmol/L, Potassium 3.5 mmol/L, Total

Calsium 6.8 mmol/L,
Impression : Hypocalsemia
Action:
Calsium Coretion use calsium gluconas
Cek Calsium after corection
Calsium post corection 8,6 mmol/L, (in normal limit)

Random Blood Sugar : 69 mg/dL (in normal limit)

2th- 7th day (November 21st-25th 2016)

S Still Breathlessness, spontaneous breath adequate with suport ventilator

SIMV mode PEEP 6 , PIP 18, RR 40x/i, and FiO2 decrease 25%, no
desaturated, still fasting, no vomitus, no abdominal pediatric surgery
departement plant to operated tomorow, prepare pateint tolerance,
mixturation was normal, defecation 1 x/ 1-2 day, with fluid balance +

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 35cc, diuresis 3 cc/bw/hour.
O Patients less active, respiratory rate 50-65 times per minute, heart rate
140-146 times per minute, regular, 37 C, oxygen saturation 90-95%,
body weight 3800 grams
Skin: Lok jaundiced, kramer grade II
Eyes: no palpebra edema, no pale conjunctiva, no sclera jaundice .
Chest: symmetrical chest movement, lung bowel sounds positif in
sinistra, heart not found abnormalities.
Abdomen: no bulge, sacfoid abdomen, no distension, normal bowel
sound
Ekstemitas: warm akral, capillary refill time <2 sec
Bilirubin-induced neurologic dysfunction (BIND) score: 0 (Mental
status: 0, Cry pattern: 0, Muscle tone: 0), Downe score: 1
A Neonatorum Jaundice Kramer III due to suspect breast feeding Joundice
Respiratory distres stable
Stable Diaphragmatic Hernia.
P SIMV ventilator PEEP 6, PIP 18, RR 60x/i, FiO225%
IVFD PG2150 ml/bw/day
Lipid 3 g/kg/hari
Fasting
Ampicilin Sulbactam 2 x 200 mg iv (6 day)
Gentamisin 1 x 18 mg iv (6 day)

E Hemoglobin 15, gr/dl, leucocyte 16.000 /mm3platelet 180.000/mm3.

Impresion : in normal limit.

Protombin time 12,5 s, Activated partial tromboplastin time 45 s

Impresion : in normal limit.

First blood culture : Klebsiella sensitif to meropenem

Action : Change antibiotics after surgery.

8th day (November 26th 2016)

S Operation has been performed, intra operatif showing defects on the

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 back left diaphragm diameter 5 cm. Looks yeyunum, ileum, and colon
tranversum entered, Intestinal was intact no hematoma and perforation
found And performed repair hernia diaphragm. No Spontaneous after
surgery . Patient with suport Ventilator CMV mode PEEP 6 , PIP 18, RR
50x/i, and FiO2 50%, no desaturated, still fasting, no vomitus, no
abdominal bloated,mixturation was normal with fluid balance + 10cc,
diuresis 4,1 cc/bw/hour.
O Patients no active, respiratory rate 50 times per minute, heart rate 155
times per minute, regular, 36,5 C, oxygen saturation 90-95%, body
weight 3800 grams
Eyes: no palpebra edema, no pale conjunctiva, no sclera jaundice .
Chest: symmetrical chest movement, same both of lung, no bowel sound
Abdomen: no bulge, no distension, no bowel sound
Ekstemitas: warm akral, capillary refill time <2 sec

A Bochdalek diaphragmatic hernia post repair

No spontauneous breathing couse sedation
P SMV ventilator PEEP 6, PIP 18, RR 50x/i, FiO250%
IVFD PG2150 ml/bw/day
Lipid 3 g/kg/hari
Fasting
Meropenem 3 x 160 mg iv
Cek Hemoglobin, leukosit, trombosit, electrolite, blood gas
analysis after surgery

E Hemoglobin 13,8, gr/dl, leucocyte 8.500 /mm3platelet 80.000/mm3.

Impresion : Decrease trombosit without s ign of bleeding

Action : Observation sign bleeding

Blood Electrolyte : Sodium 135 mmol/L, Potassium 4,1 mmol/L,

Calsium 9,1 mmol/L,
Impresion : in normal limit

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 Blood gas analysis : pH 7,34, pCO2 34, pO2 71, HCO3- 19,7,7, BE -7,
Saturation 91%.
Impresion : Hopoxemia
Action : Incrase Fio2 60% keep oxygen saturation more than 90 %

9th day (November 27th 2016)

S Spontaneous breath become adequate, ventilation mode has changed
from CMV to SIMV, no desaturaed and ventilator setting has been
gradually decreased and weaning ventilator to nasal ncpap, with peep 7
Fio2 21%. No brethlesnes, no fever, no vomiting, no bleedeing sign
mixturation was normal with fluid balance + 30cc, diuresis 4,5
cc/bw/hour.
O Patients active, respiratory rate 55 times per minute, heart rate 145
times per minute, regular, 36,5 C, oxygen saturation 90-95%, body
weight 3900 grams
Eyes: no palpebra edema, no pale conjunctiva, no sclera jaundice .
Chest: symmetrical chest movement, same both of lung, no bowel sound
Abdomen: no bulge, no distension, no bowel sound
Ekstemitas: warm akral, capillary refill time <2 sec

A Improvement condition
P NCPAP 7 FiO221%
IVFD PG2150 ml/bw/day
Lipid 3 g/kg/hari
Tropik feeding 8 x 3 cc/ ogt
Meropenem 3 x 160 mg iv

10th- 25th day (November 28th 2016- Desember15th )

S Spontaneous breath is getting stronger, weaning NCPAP 7 days after
surgery. Improved tolerance of drink. Micturation and defecation within
normal limits. No fever. Good surgical wound. Body weight
increase.The baby look healthy and has good appetite. The patient had
been allowed to get discharged
O Patients active, respiratory rate 50 times per minute, heart rate 145
times per minute, regular, 36,5 C, oxygen saturation 90-95%, body

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 weight 4200 grams
Eyes: no palpebra edema, no pale conjunctiva, no sclera jaundice .
Chest: symmetrical chest movement, same both of lung, no bowel sound
Abdomen: no bulge, no distension, no bowel sound
Ekstemitas: warm akral, capillary refill time <2 sec

A Improvement condition
P Discharged
E Second blood cultute : steril

LITERATURE REVIEW

Review
Etiology
The etiology of CDH largely remains unclear and currently is thought to
be multifactorial. The majority of the cases have an isolated diaphragmatic defect
presenting with pulmonary hypoplasia and persistent pulmonary hypertension of
newborn (PPHN). CDH can be associated with cardiac, gastrointestinal,
genitourinary anomalies or with chromosomal aneuploidy such as trisomies.
Multiple genetic factors along with environmental exposures and nutritional
deficiencies have been proposed to be the possible etiologies for CDH 4.
Studies in rodent models have pointed towards a disturbance in Vitamin A
pathway 5. Nitrofen, a herbicide, when administered to pregnant rodents, results in
CDH in the majority of offspring. Similar effects were seen in WT1 and COUP-
TFII mutant mouse models. Studies in neonates with CDH have shown low
retinol and retinol-binding protein levels from cord blood samples6.

Pathology

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 Location (Fig. 1): Postero-lateral hernias also known as Bochdalek hernias
are the most common type (7075%) with the majority occurring on the left side
(85%) and less frequently on the right side (13%) or bilateral (2%). Anterior
defects or Morgagni hernias (2328%) and central hernias (27%) are the other
types. Size (Fig. 2): The diaphragm begins to develop at approximately 4 weeks of
gestation and is fully formed by 12 weeks . The defect may range from a small
opening of the posterior muscle rim to complete absence of diaphragm. 7
The embryologic basis of CDH remains controversial. It was thought
initially that the defect happened secondary to failure of different parts of the
diaphragm to fuse resulting in a patent pleuroperitoneal canal 7. This, in turn,
allows the gut to enter the thoracic cavity when it returns from the extraembryonic
coelom of the umbilicus. Another speculation is that lung hypoplasia may be the
primary causal factor in the pathophysiology of diaphragmatic hernia. If the
development of lung bud is disturbed, there is an impaired development of a post
hepatic mesenchymal plate (PHMP) that is closely related to the development of
lung, resulting in a defective diaphragm. Evidence from electron microscopy in a

14
rat model of CDH further supports the fact that when the development of the
PHMP is impaired, a diaphragmatic defect occurs. 7,8
A weakness in the diaphragm can cause diaphragmatic eventration and
may be mistaken for a diaphragmatic hernia. Diaphragmatic eventration is more
common on the right side and is not associated with severe lung hypoplasia.
While a complete absence of the diaphragm may occur resulting in diaphragmatic
agenesis and severe lung hypoplasia. Irrespective of the basis, a defect in the
diaphragm causes the abdominal viscera to herniate into the thoracic cavity
resulting in abnormal lung development. The defect also leads to abnormal fetal
breathing movements resulting in the void of stretch induced lung maturation 4.
Thus the major underlying pathophysiology of CDH appears to be a combination
of lung immaturity and hypoplasia that leads to PPHN. This may be further
aggravated by left ventricular underdevelopment and right ventricular hypertrophy
resulting in ventricular dysfunction9,10.

Lung hypoplasia/immaturity
Lung hypoplasia occurs on the ipsilateral side of herniation, with the
contralateral side being affected to a variable extent (Fig. 3). Hypoplasia was
initially thought to be secondary to physical compression of the lung by
abdominal contents arresting lung development. Recently, a two-hit hypothesis
has been proposed based on rat model explaining the lung injury in CDH (Fig. 4).
According to this hypothesis, the initial insult occurs during the stages of
organogenesis resulting in bilateral hypoplasia, followed by compression of the
ipsilateral lung secondary to the herniation of the abdominal viscera at later
stages. This theory explains the variability of lung hypoplasia on the contralateral
side. The interference results in decreased branching of the bronchioles and
pulmonary vessels leading to acinar hypoplasia. The terminal bronchioles are
decreased with thickening of alveolar septa. The lung is relatively immature and
hypoplasia of pulmonary vasculature leads to PPHN11

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Pulmonary hypertension in CDH
In CDH, the total pulmonary vascular bed is reduced with decreased
number of vessels per unit of lung. In addition, pulmonary vascular remodeling
with medial hyperplasia and peripheral extension of the muscle layer into small
arterioles is evident The paucity of pulmonary vasculature and remodeling of the

16
vessels contribute to the fixed or irreversible component of PPHN in CDH ..
Altered vasoreactivity possibly due to an imbalance of autonomic innervation
(increased sympathetic and decreased parasympathetic) , and/or impaired
endothelium-dependent relaxation of pulmonary arteries or an imbalance between
vasoconstrictor and vasodilator mediators may contribute to the reversible
component of PPHN. Following birth, a combination of pulmonary arterial
hypertension, right ventricular hypertrophy and/or failure, and left ventricular
hypoplasia with pulmonary venous hypertension results in severe PPHN
unresponsive to conventional management 12.

Ventricular dysfunction
Ventricular dysfunction is observed in some patients with severe PPHN
due to CDH. During fetal life, the ductus arteriosus serves as a pop-off value and
limits right ventricular strain. After birth, remodeled pulmonary vasculature in
CDH results in pulmonary hypertension and leads to right ventricle (RV)
dysfunction. This is more pronounced after birth when there is excessive strain on
the right ventricle. Abnormalities of the left ventricle (LV) have been reported in
.
infants with CDH When compared to neonates with other causes of PPHN,
infants with left sided CDH had significantly lower left ventricular mass assessed
by echocardiography. Reduced left ventricular output has been documented in left
sided and right sided CDH . The reduced left ventricular mass contributes to
functional LV hypoplasia and may result in increased left atrial pressure and
pulmonary venous hypertension (Fig. 5) ,9,10

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Diagnosis
Prenatal diagnosis by ultrasound detects more than 50% of CDH cases at a
mean gestational age of 24 weeks . Three-dimensional ultrasound imaging, fetal
echocardiography and fetal magnetic resonance imaging (MRI) are other prenatal
diagnostic modalities used in assessing the severity and outcome of CDH. Left
sided CDH may be characterized by the presence of heterogeneous mass which
may be stomach filled with fluid or intestines. In contrast, isolated right-sided
CDH is extremely difficult to diagnose by ultrasound if the liver is the only organ
that has herniated.13
Indirect signs such as a shift in cardiac axis, identifying the gall bladder
and vasculature in the liver using Doppler may aide in the diagnosis. MRI has
been found to be useful in detecting fetal anomalies and can be a valuable adjunct
to evaluate the position of the liver and estimating
lung volume. Associated cardiac and neural tube defects may affect the outcome
of infants with CDH 13
Associated syndromes and anomalies requiring genetic
work up
Most common associated chromosomal abnormalities are the trisomies 18,
13 and 21. Chromosomal aneuploidies such as monosomy X, tetrasomy 12 p,
tetraploidy 21 have also been associated with CDH. CDH is the most common
finding in Fryns syndrome. CDH can also be part of Pentalogy of Cantrell, Apert,

18
Brachmann-Cornelia De Lange, Beckwith-Wiedemann, CHARGE, Coffin-Siris,
Goldenhar sequence, Simpson- Golabi-Behmel, Stickler, Pierre Robin sequence
and VACTERL4
Once diagnosed, the patient should be referred to a tertiary care center for
further prenatal workup and management. A multi-disciplinary prenatal consult
involving the obstetrics, neonatology, pediatric surgery, genetics at a center that
has expertise in managing infants with CDH and extracorporeal membrane
oxygenation (ECMO) are imperative. In addition, if an MRI was done, radiology
is also involved in the multi-disciplinary prenatal consult.

Fetal predictors of outcomes

Major determinants of the outcomes in CDH are i) the presence of
associated anomalies especially heart disease and ii) extent of lung hypoplasia and
(iii) position of the liver 13.
The prognosis of isolated CDH is generally better than CDH complicated
by multiple anomalies. Populationbased studies report higher survival for isolated
CDH compared to CDH with anomalies14. Metkus et al. reported higher survival
for CDH detected after 25 weeks by ultrasound. This has not been validated and in
the true sense, herniation that occurs before 25 weeks tends to have severe lung
hypoplasia compared to herniation after 25 weeks 15.
Liver herniation (liver-up) is associated with worse prognosis. Earlier
studies have reported 100% survival without liver herniation (liver-down) as
compared to 56% with liver herniation15.
Metkus et a 15. used the ratio of the contralateral lung size compared with
the head circumference to come up with the lung-to-head ratio (LHR, Fig. 6) to
assess the severity of pulmonary hypoplasia and to predict postnatal outcome in
fetuses with CDH. Since these measurements differed by gestational age and were
not found to be consistent across centers observed to expected lung-tohead ratio
(O/E LHR) was studied which was independent of gestational . LHR ratio is often
used along with liver herniation to predict outcome

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Management

PRENATAL TREATMENT

Severe and extremely severe diaphragmatic hernias have poor outcomes

and the affected patients may be of fered fetal endoscopic tracheal occlusion
(FETO). The primary goal of FETO is to minimize pulmonary hypoplasia and
reduce mortality. Harrison et al were the first to introduce the concept in animal
model by deflation of a previously inflated intra-thoracic balloon. Since its
clinical introduction, FETO has been performed for severe cases at 2628 weeks
and for moderate cases at 3032 weeks of gestation. Tracheal occlusion leads to
the accumulation of the lung fluid, which causes increased lung tissue stretch and
accelerated growth. On the other hand, it reduces the number of type II
pneumocytes and surfactant expression. This is the major rationale for balloon
retrieval at 3334 weeks of gestation. Prenatal balloon removal is also associated
with lower morbidity and better survival rate16

POSTNATAL TREATMENT

Some newborns with severe CDH may require extracorporeal membrane

oxygenation. It consists of the cannulation of both carotid arteries and jugular vein

20
and their connection to the circuit with a membrane gas exchange chamber. It
allows oxygen and carbon dioxide exchangewithout involving the lungs. ECMO
use is typically restricted to infants > 2 kg and gestational age > 34 weeks in the
absence of significant intracranial hemorrhage, chromosomal anomalies or other
congenital anomalies. There is a not survivable subset of CDH fetuses due to
extremely severe pulmonary hypoplasia for whom ECMO may be futile. Some
authors reported reduced number of patients treated with this method due to weak
evidence of its real benefits in such neonates. Postnatal therapy is complex and
includes immediate intubation, small-volume, high-frequency oscillatory
ventilation, and intensive pharmacological treatment, including nitric oxide use. 16

CASE ANALYSIS
It has been reported a case a new born boy baby with Congenital
Diaphragmatic Hernia (CDH) bochdalek hernias tipe. CDH occurs in
approximately 1 in 3000 live births and results in high neonatal morbidity and
mortality. A Bochdalek hernia is a congenital abnormality in which an opening

21
exists in the infants diaphragm, allowing normally intra-abdominal organs
(particularly the stomach and intestines) to protrude into the thoracic cavity
compressing the lung and displacing the mediastinum to the opposite side. The
Bochdalek hernias is the most common type CDH (7075%) with the majority
occurring on the left side (85%).7 Bochdalek hernias have a ratio of 3:2 of males
to females. 2 Infants born with a Bochdalek hernia have a high mortality rate due
to respiratory insufficiency. Between 25-60% of infants die from a Bochdalek
hernia. 7

The clinical profile of respiratory distress reported in this case may

progress to severe or impending respiratory failure . The symptoms found
breathlessness, severe retraction, cyanosis relieved by oxygen, mild decrease in air
entry, and grunting audible by sthetoscope with total Down Score was 6. During
physical examination seen respiratory failure., bowel sounds in the right chest
clearly on second day and excavated or scafoid abdomen on second day
hospitalized .. During the radiography we noted a hemithorax filled like structures
bowel.

In emergency management we instalation oral gastric tube with

decompression, intubated with manual ventilatioan, keep oxygenation, and
prepare ventilator installation. CDH severe cases is recommended an immediate
oxygenation. In Delivery room (DR) management deliveries should be conducted
at centers with capabilities of managing an infant with CDH and associated
complications. Resuscitation in the DR is based on neonatal resuscitation program
guidelines. All infants with CDH or suspected CDH need an
orogastric/nasogastric tube with suction to decompress the bowel. Bag-mask
ventilation should be avoided. The majority of these infants (especially with a
prenatal diagnosis of CDH) require intubation in the delivery room. A pre-ductal
pulse oximeter is placed on the right upper extremity as soon as possible. We
should be careful with assisted ventilation in order to maintain a low inspiratory
pressure. Ventilation using a T-piece resuscitator is preferred to avoid high airway
pressures. Peak inspiratory pressure (PIP) should be preferably below 25 cm H2O
to avoid damage to the hypoplastic/immature lung and , to avoid damage or

22
breakage of the contralateral lung. Oxygen can be titrated to maintain preductal
saturations recommended by NRP. In some institutions, preductal saturations >
70% are accepted for the first 12 h if pH and arterial carbondioxide for PaCO2
are within normal limits. 17

In this case we use ventilation mode Synchronized Intermittent

Mandatory Ventilation (SIMV) because baby's spontaneous breath is still strong.
We use PEEP 6, PIP 18 with target tidal volume to 5-6ml/kg to prevention of
ventilator-induced lung injury and keep oxygen saturation more than 88%. The
optimal ventilation mode for infants with CDH and hypoplastic lungs is not
known. Many centers initiate conventional mechanical ventilation (CMV) for
respiratory support and optimize ventilation by adjusting PIP and respiratory rate.
The recently concluded VICI (Ventilation in infants with congenital diaphragmatic
hernia) trial compared CMV and high-frequency oscillatory ventilation (HFOV)
as the initial mode of ventilation in CDH. There was no statistically significant
difference in the combined outcome of mortality or bronchopulmonary dysplasia
(BPD). It is important to note that guidelines for initial settings for CMV in this
study included low positive end-expiratory pressure (PEEP) (3 to 5 cm H2O) and
PIP (20 to 25 cm H2O). These findings suggest that an initial attempt at CMV is
reasonable for patients with CDH.17

A review of autopsies of 68 infants with CDH showed significant

pulmonary injury (alveolar damage, hyaline membrane formation,
pneumothoraces in 2/3 of autopsies) secondary to mechanical ventilation where
18
53 infants were switched to HFOV in a median time of 15 h from birth In view
of preventing volutrauma and barotrauma, a gentler approach to ventilation is
19,20
preferredin infants with CDH. CMV mode with PIP usually below 25 cm
H2O and PEEP 5 cm H2O targeting preductal saturations of >85%, post-ductal
saturations of >70% and PaCO2 of 4560 mmHg are used to initiate ventilation.
Many centers switch to HFOV or jet ventilation as a rescue therapy if the
ventilator targets cannot be achieved on CMV. 19

The patient performed surgery on the 8th day of treatment after pediatric
surgery was ready to operate. Intra operative showing defects on the back left

23
diaphragm diameter 5 cm. Looks yeyunum, ileum, and colon tranversum entered.
Intestinal was intact no hematoma and perforation found. We debate with
pediatric surgery because baby got long fasting and risk intestinal vilous atrophy.
Reduction in mucosal mass occurs largely within 48 hours, whereas the process of
villus atrophy is complete within 72 hours of TPN. Surgical repair should be
performed only after stabilization, through subcostal laparotomy with reduction of
hernial contents into the abdominal cavity and closure of the diaphragmatic
defect. There are generally two aproach, early repair < 72 hours, delayed repair >
72hours. Reducing herniated contents back to abdomen to permit expansion of
the compressed lung, but does not result in immediate improvement in pulmonary
hipertension.16

The patient didnt do echocardiogram because patient no transportable. An

echocardiogram is the best non-invasive test to assess cardiac function and
pulmonary pressures in an infant with CDH and is usually done within the first 24
hour and followed up as needed. But in this patient we didnt found clinical
presentation of pulmonal hypertension like prominent right ventricular impulse, a
single second heard sound, mur mur of tricuspid insuficiency, and diferential
oximeter reading preducktal and postductal saturations 21

Empirical antibiotic therapy should be provided if suspected sepsis and

invasive treatment in hight risk neonate. Several multicentre studies have found
that the combination of penicillin (ampicillin) and aminoglycoside (gentamicin)
antibiotics is an effective empirical therapy option for high-risk neonates. 22 A
study by The National Laboratory Surveillance Data in the United Kingdom found
that almost all (94 %) Cultures obtained from neonates with SNAD are sensitive
to penicillin and gentamicin and 100% sensitive to amoxicillin and cefotaxime. A
combination of ampicillin and gentamicin may be given before blood cultures are
obtained and if within 48 to 72 hours there is no clinical improvement, the
cephalosporin group may be used as a second treatment option. 23 Patients were
given empirical antibiotic therapy in the form of ampicillin sulbactam and
gentamicin for 6 days, but on the seven day found klebsiela germ sensitif to
meropenem. Antibiotic replaced with meropenem after surgery.

24
Duration of antibiotics is determined by the type of germs that cause. Sepsis
caused by Gram positive bacteria is given antibiotics for 10-14 days, whereas
sepsis caused by Gram negative bacteria is given antibiotics for at least 21 days. 24
Siani, et al reported that short-term antibiotic treatment in infants with negative
blood culture results as effectively With extended antibiotics for up to 7 days.

Respiratory issues, nutritional problems, neurodevelopmental delays,

hernia recurrence and orthopedic deformities. The American Academy of
Pediatrics (AAP) came out with guidelines for follow-up of infants who are
discharged with CDH A multidisciplinary approach with long-term follow-up is
required for these infants. Respiratory morbidities include chronic lung disease,
rebound pulmonary hypertension, obstructive pulmonary disease and infection.
Nutritionalproblems include gastro esophageal aversion to oral feeds, gastrostomy
tube feeding and failure to thrive. Neurological and development problems range
from physical disability to neurocognitive and functional delays. Hearing loss is
common in these infants. Orthopedic deformities such as pectus and scoliosis are
seen in patients post CDH repair 17

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