D.R.

Anna Kathrina P. Panlilio, EMT-B, RN, MD

TO PRESENT A CASE OF CONGENITAL
DIAPHRAGMATIC HERNIATION & DISCUSS:
-INCIDENCE
-CLINICAL MANIFESTATIONS
-DIFFERENTIAL DIAGNOSIS
-DIAGNOSIS
-MANAGEMENT
-PROGNOSIS
OBJECTIVES
D.R.
NB/MALE
DAVAO CITY
INFORMANT: MOTHER 90%
RELIABILITY
GENERAL DATA
GRUNTIN
G
MATERNAL HISTORY
 Born to a 26 year old G3P3 mother via NSVD
 Prenatal check up
 Local health center x 1 visit
 OB x 4 visits
 Non smoker, non alcoholic beverage drinker
 Compliant to multivitamins
 No other maternal illnesses
 PREVIOUS PREGNANCIES
Pregancy Mode of Year Age of Sex Birth Present
Order Delivery Gestation weight Status
G1 NSVD 2011 Full term M 2 kg Living
G2 NSVD 2018 Full term M 3 kg Living
G3 Present Pregnancy
PRESENT PREGNANCY
 1st trimester UTZ (12/02/19):
 Single live intrauterine pregnancy with good cardiac activity of 157 bpm
 12 weeks and 2 days by crown rump length
 EDC: 06/13/20
 2nd trimester UTZ (01/06/20):
UNREMARKABLE
 Single live intrauterine pregnancy in variable presentation with good cardiac and somatic activities
 Composite aging equivalent to 17 weeks and 2 days
 Anterior placenta, low lying, Gr. 1
 Adequate amniotic fluid volume (4.8cm)
 Fetal Weight is 169 grams
 3rd trimester UTZ (05/29/20):
 Single live intrauterine pregnancy in cephalic presentation with good cardiac and somatic activities
 Composite aging equivalent to 38 weeks and 1 day
 Anterior placenta, high lying grade III
 Adequate Amniotic fluid index (12.5cm)
 Fetal weight is appropriate for gestational age (12.5 grams)
LABOR AND DELIVERY
 Weakness of both lower extremities x 3 days
 Electrolyte imbalance - Hypokalemia (1.95MMOL/l)
 Few hours PTA, mother referred due to hypokalemia and hence this
admission
 Ongoing correction via IVF and PO during labor.
 Delivery via Normal Spontaneous Vaginal Delivery on June 2, 2020
AT BIRTH
• GOOD CRY

• GOOD ACTIVITY

UPON
TRANSFER
TO
WARMER
• CYANOSIS

• POOR ACTIVITY

• POOR CRY
10TH MIN
OF LIFE
• CYANOTIC

• IRREGULAR SHALLOW
RESPIRATIONS
• COOL EXTREMITIES

• POOR CRY

HOOKED TO FREE
FLOW OXYGEN
REFERRED TO PEDIA
15TH MIN
OF LIFE
HR >100bpm
Irregular breathing
Cyanosis
Some grimace, some flexion,
(-) seizure
Desaturation at 70%
Grunting

INTUBATED
ADMIT TO NICU
39 WEEKS
47cm
33cm

2.56kg

X
PHYSICAL EXAMINATION
 Vs: HR 128, Temp 36.5, RR 48
 Head circumference 33cm
 Chest circumference 30cm
 Length 47cm
 SKIN: warm, good turgor, cyanosis
 HEAD: normocephalic, intact scalp, flat fontanelles, (-) cephalhematoma
 EYES: anicteric sclerae, no discharge
 EARS: no deformities, no discharge
PHYSICAL EXAMINATION
 NOSE: no deformities, no discharge, alar flaring
 MOUTH: moist lips and mucosa, no cleft lip and palate
 NECK: symmetric, no masses, no deformities
 C/L: Intubated with asymmetrical chest expansion, good air entry, (+) deep
subcostal retractions, (+) decreased breath sound, right lung field, (-) desaturation
 LEFT UPPER EXTREMITY: 95%
 LEFT LOWER EXTREMITY: 96%
 RIGHT UPPER EXTREMITY: 96%
 LEFT LOWER EXTREMITIY: 93%

 CVS: adynamic precordium, regular rate and rhythm, no murmur

PHYSICAL EXAMINATION
 ABDOMEN: protuberant, soft, no palpable mass, dry and intact umbilicus, no
hepatomegaly

GENITAL: grossly male, descended testes

EXT: no deformities,strong central and distal pulses, CRT <2sec

ANUS: patent
 SALIENT FEATURES
HISTORY PHYSICAL EXAMINATION
Term Cyanosis
Delivered via NSVD Alar flaring
Apparently well at birth with noted Asymmetrical chest expansion Deep
progression of symptoms subcostal retractions
APGAR SCORE 8,9 Decreased breath sound on the right
BW: 2.56kg lung field
Poor activity (+) desaturation
Poor cry
TERM BABY BOY DELIVERED VIA
NORMAL SPONTANEOUS VAGINAL
DELIVERY; APGAR SCORE 8,9; BALLARD
SCORE 39 WEEKS, BIRTH WEIGHT
2.56KG, APPROPRIATE FOR
GESTATIONAL AGE, CONGENITAL HEART
DISEASE – CYANOTIC TYPE
ADMITTING IMPRESSION
 Cyanosis and Irregular breathing

Pulmonary Cardiac

Neonatal Transient
TGA TOF TAPVR
Pneumonia Tachypnea of the
Newborn
TRANSIENT TACHYPNEA OF
THE NEWBORN
RULE IN RULE OUT
Male History:
Term Delivered via NSVD
Apparently well at birth with noted BS: 39 weeks
progression of symptoms
Poor activity PE:
Poor cry Cyanosis
Alar flaring Desaturation
Deep subcostal retractions Asymmetrical chest expansion
Decreased breath sound on the right
lung field
NEONATAL PNEUMONIA
RULE IN RULE OUT
Cyanosis Uncommon in first 24 hours of
Alar flaring life
Subcostal retractions
Grunting
(+) Desaturation, O2 sat 70%
Asymmetrical chest expansion
with decreased breath sound,
right LF
TOTAL ANOMALOUS
PULMONARY VENOUS RETURN
RULE IN RULE OUT
Cyanosis No murmurs appreciated
Alar flaring
Subcostal retractions Cannot totally rule out
Grunting
TETRALOGY OF FALLOT
RULE IN RULE OUT
Cyanosis No murmurs appreciated
Increased respiratory effort
TRANSPOSITION OF THE
GREAT ARTERIES
RULE IN RULE OUT
Cyanosis Cannot totally rule out
Alar flaring
Subcostal retractions
Grunting
(+) Desaturation, O2 sat 70%
CONGENITAL
DIAPHRAGMATIC HERNIA
RULE IN RULE OUT
Cyanosis Cannot totally rule out
Alar flaring
Subcostal retractions
Grunting
(+) Desaturation, O2 sat 70%
Asymmetrical chest expansion
with decreased breath sound,
right LF
 FINDINGS SUGGESTIVE OF DIAPHRAGMATIC HERNIA WITH
CHEST X-RAY ATELECTASIS, RIGHT.
TERM BABY BOY DELIVERED VIA NORMAL
SPONTANEOUS VAGINAL DELIVERY; APGAR
SCORE 8,9; BALLARD SCORE 39 WEEKS, BIRTH
WEIGHT 2.56KG, APPROPRIATE FOR
GESTATIONAL AGE, CONGENITAL
DIAPHRAGMATIC HERNIA, RIGHT
WORKING IMPRESSION
1st DOL/HOSPITAL DAY A P

MANAGMENT
F: TFI 60
R: INTUBATED,
GOOD AIR ENTRY
TERM BABY BOY DELIVERED
VIA NORMAL SPONTANEOUS
VAGINAL DELIVERY; APGAR
STARTED IVF
ROUTINE NEWBORN CARE
ANTIBIOTICS STARTED
TACHYPNIC, (-) ALAR FLARING
SCORE 8,9; BALLARD SCORE OMEPRAZOLE STARTED
(+) DECREASE BS, RLF
LEFT UPPER EXTREMITY: 95% 39 WEEKS, BIRTH WEIGHT WORK-UP REQUESTED
LEFT LOWER EXTREMITY: 96% 2.56KG, APPROPRIATE FOR HOOKED TO MV”
RIGHT UPPER EXTREMITY: 96% GESTATIONAL AGE, FIO2 80 RR 40 PIP/PEEP 15/5 AC
LEFT LOWER EXTREMITIY: 93% CONGENITAL MODE
CXR: DIAPHRAGMATIC HERNIA, DIAPHRAGMATIC HERNIA, OGT INSERTED
RIGHT RIGHT REFERRED TO PEDIA SURGERY FOR
I: AFEBRILE, (-) SCLEREMA EVALUATION
C: DHS, (-) MURMUR, (-)
BRADYCARDIA
H: (-) PALLOR, (-) BLEEDING
M: NO HYPOGLYCEMIA, (-) JAUNDICE
O: (+) UO 3.1CC/KG/HR
BM X 1, (+) BLOODY OGT OUTPUT
N: AWAKE (-) SEIZURES
D: NPO
LABORATORY RESULTS
CBC Uncompensated metabolic
Hgb 141 Acidosis with
Acidemia overcorrected hypoxemia
Hct 0.4 Interpretation (VBG) (1 hour post MV set-up)
RBC count 3.4 Ph 6.85 7.11
WBC count 18.58 pCO2 59 41
pO2 56 129
Diff count
BE (B) -23.5 -15.8
Neutrophil 30 HCO3 10.3 13
Lymphocytes 56 O2 sat 58 98
Monocytes 10 TCO2 12.1 14.3
Eiosinophil 2
Basophil 2
Platelet 278
CRP 0.50 (-)
MANAGMENT
PEDIA SURGERY NOTES

PLAN: EXPLORATORY LAPAROTOMY, REPAIR OF DIAPHRAGMATIC HERNIA RIGHT

FOR LABS:
- CBC BLOOD TYPING
- NA, K, CREA
- PT APTT
- ALBUMIN
- WHOLE ABDOMEN UTZ
- 2D ECHO
- BABYGRAM

- FOR PEDIA CLEARANCE AND REFERRAL TO ANESTHESIA FOR EARLY PREOP EVAL
- SECURE 50CC PRBC ALIQUOT OF PATIENT’S BLOOD TYPE FOR OR USE
- REINSERT OGT FR 8 OPEN TO DRAIN
PT PTT 06/03/2020 S. ELECTROLYTES 06/03/2020
PT Patient 18.8 Albumin 27.36
INR 1.44
CA 1.96
Pt % activity 60
CREA 96.19
PT control 15.9
K 2.57
APTT 47.2
MG 1.06
APTT control 30
NA 140.04
ALL WITHIN
NORMAL LIMITS HYPOALBUMINEMIA,
HYPOCALCEMIA, HYPOKALEMIA
 ULTRASOUND OF WHOLE
ABDOMEN
 DIAPHRAGMATIC HERNIA RIGHT
 SONORAPHICALLY NORMAL LIVER, GALLBLADDER, PANCREAS, SPLEEN AND
URINARY BLADDER
 BILATERAL HYDRONEPHROSIS GRADE 2 RIGHT, GRADE 1 LEFT
 No significant disparity in the size, shape and location of both kidneys, both exhibit
hypoechoic parenchyma echopattern, relative to that of the liver and the spleen. There is
minimal to moderate dilatation of both renal pelvis. Both ureters are not dilated. There is
neither evidence of mass nor calculus in the scans obtained. The urinary bladder is empty.

LENGTH (CM) WIDTH (CM) THICKNESS (CM) PARENCHYMAL

THICKNESS (CM)
RIGHT 3.8 2.21 2.73 0.92
KIDNEY
LEFT KIDNEY 4.0 2.04 2.06 0.94
 REFERRED SERVICES
IMPRESSION MANAGEMENT
CARDIO
UTZ 2D ECHO: START INOTROPES:
SEVERE PULMONARY ARTERIAL DOPAMINE DRIP
2DECHO HYPERTENSION
TRICUSPID REGURGITATION
SEVERE
PULMONIC REGURGITATION
SEVERE
PATENT FORAMEN OVALE
IDS EVALUATED AS NON-COVID
FOR OR CLEARANCE
PULMONOLOGY PREOPERATIVE EVALUATION HIGH RISK FOR
CONTEMPLATED
PROCEDURE
2ND DOL
F: TFI 70
R: INTUBATED,
GOOD AIR ENTRY
NO RETRACTIONS
LEFT UPPER EXTREMITY: 95%
LEFT LOWER EXTREMITY: 96%
RIGHT UPPER EXTREMITY: 96%
LEFT LOWER EXTREMITIY: 95%
I: AFEBRILE, (-) SCLEREMA
C: DHS, (-) MURMUR, (-)
BRADYCARDIA
H: (-) PALLOR, (-) BLEEDING
M: NO HYPOGLYCEMIA, (-)
JAUNDICE
O: (+) UO 2.8CC/KG/HR
BM X 1
N: AWAKE (-) SEIZURES
D: NPO
A P
TERM BABY BOY DELIVERED IVF CONTINUED WITH
VIA NORMAL SPONTANEOUS INCORPORATON OF CALCIUM AND
VAGINAL DELIVERY; APGAR KCL
SCORE 8,9; BALLARD SCORE ALBUMIN INFUSION X 3 DOSES
39 WEEKS, BIRTH WEIGHT 6HRS APART
2.56KG, APPROPRIATE FOR STARTED MIDAZOLAM DRIP (1)
GESTATIONAL AGE,
CONGENITAL
DIAPHRAGMATIC HERNIA,
RIGHT, HYDRONEPHROSIS,
GRADE 1, LEFT, GRADE 2,
RIGHT, SEVERE PULMONARY
ARTERY HYPERTENSION,
TRICUSPID REGURGITATION
SEVERE, PULMONIC
REGURGITATION SEVERE,
PFO, PDA
POST-CORRECTION RESULT REFERENCE
ALBUMIN 36.91 35.0-50.0
CALCIUM 2.22 2.23-2.58
POTASSIUM 2.61 3.6-5.1
MAGNESIUM 1.05 0.74-1.03
SODIUM 144.07 136.—144.0

HYPOCALCEMIA, HYPOKALEMIA
 SURGERY
PROCEDURE INTRA-OP FINDINGS OPERATIVE TECHNIQUE
UTZ
3RD DOL 3X3 CM DEFECT NOTED AT PATIENT SUPINE UNDER GA
RIGHT HEMIDIAPHRAGM ASEPSIS AND ANTISEPSIS
2DECHO DRAPING DONE
EXPLORATORY HERNIATED RIGHT
TRANSVERSE RIGHT UPPER QUADRANT
LAPAROTOMY HEPATIC LOBE AND SMALL
INCISION DOWN TO PERITONEUM
BOWEL SEGMENT - ALL
PRIMARY REPAIR VIABLE
NOTED A 3X3 CM DEFECT AT THE RIGHT
OF CDH RIGHT HEMIDIAPHRAGM WITH HERNIATED SMALL
BLOOD LOSS: MINIMAL BOWL AND RIGHT HEPATIC LOBE
ALL ARE VIABLE, HERNIAL SAC EXCISED
IJ CATHETER PRIMARY REPAIR DONE WITH SILK 3-0 ATR
INSERTION, RIGHT LUNGS EXPANDED, PNEUMOTHORAX DRAIN
WITH TUBE SUBMERGED IN WATER BOTTLE
PRIOR TO COMPLETE LIGATION
CLOSURE
DRESSING
4TH DOL A P
1ST POST-OP DAY
SURGERY
F: TFI 200
R: Intubated 60/40/14/4 AC
TERM BABY BOY DELIVERED VIA
NORMAL SPONTANEOUS VAGINAL
MAINTAINED ON IVF AND ON
NPO
MODE DELIVERY; APGAR SCORE 8,9; MAINTAINED MECHVENT SETUP
I: AFEBRILE, (-) BALLARD SCORE 39 WEEKS, BIRTH MEDS
UTZ
SCLEREMA WEIGHT 2.56KG, APPROPRIATE FOR
C: (-) MURMUR, GESTATIONAL AGE, CONGENITAL
SHIFT AMPICILLIN TO
2DECHO
(-) BRADYCARDIA DIAPHRAGMATIC HERNIA, RIGHT, CEFOTAXIME
DOPAMINE (10) HYDRONEPHROSIS, GRADE 1, LEFT, SHIFT GENTAMICIN TO
H: (+) PALLOR, GRADE 2, RIGHT, SEVERE AMIKACIN
(-) BLEEDING PULMONARY ARTERY MIDAZOLAM DRIP (1)
M: (-) JAUNDICE HYPERTENSION, TRICUSPID DOPAMINE DRIP (10)
(-) HYPOGLYCEMIA REGURGITATION SEVERE, FENTANYL
O: (+) UO 2,3CC/KG/HR PULMONIC REGURGITATION OMEPRAZOLE
(+) BM SEVERE, PFO, PDA
FOR REPEAT LABORATORY
N: (+)SPONT MOVEMENT, S/P EL REPAIR OF CDH RIGHT, IJ
(-) SEIZURE CUTDOWN RIGHT WORK-UP
MIDAZOLAM (1) FOR BLOOD TRANSFUSION
D: NPO
PNEUMOTHORAX, RIGHT WITH
COMPLETE LEFT LUNG ATELECTASIS
MANAGMENT
PEDIA SURGERY
PROCEDURE DONE:

CLOSED TUBE THORACOTOMY, RIGHT

 REPEAT
CHEST X-RAY
POST CTT
INSERTION,
RIGHT
• RESOLVING
PNEUMOTHORAX, RIGHT
• CONSIDER RE-
EXPANSION
PULMONARY EDEMA
• SUGGESTIVE OF
MINIMAL
PNEUMOPERICARDIUM
• RECENT SOFT TISSUE
EMPHYSEMA, RIGHT
LATERAL CHEST WALL
4 TH
DOL, 1 POST OP DAY
ST

RESULT REFERENCE
ALBUMIN 24.74 35.0-50.0
CALCIUM 2.13 2.23-2.58
POTASSIUM 3.26 3.6-5.1
MAGNESIUM 0.92 0.74-1.03
SODIUM 132.24 136.—144.0

HYPOALBUMINEMIA, HYPOCALCEMIA, HYPOKALEMIA,

HYPONATREMIA
 COURSE IN THE WARD
5th DOL DIAGNOSIS MANAGEMENT
2nd POST-OP DAY
5TH DOL (1ST POST-OP DAY) CONGENITAL DIAPHRAGMATIC MULTIPLE ELECTROLYTE
HERNIA, RIGHT, IMBALANCE CORRECTION
HYDRONEPHROSIS, GRADE 1,
HYPOALBUMINEMIA (24.74) LEFT, GRADE 2, RIGHT, SEVERE
ON GOING
HYPOCALCEMIA (2.13) PULMONARY ARTERY CONTINUED INOTROPES
HYPOKALEMIA (3.26) HYPERTENSION, TRICUSPID AND FENTANYL
HYPONATREMIA (132.24) REGURGITATION SEVERE, CONTINUED WEANING FROM
PULMONIC REGURGITATION MECHANICAL VENTILATION
SEVERE, PFO, PDA, S/P EL REPAIR ANTIBIOTICS CONTINUED
OF CDH RIGHT, IJ CUTDOWN
RIGHT, PNEUMOTHORAX
RESOLVING S/P CLOSED TUBE REFER TO NEPHRO SERVICE
THORACOSTOMY RIGHT (6/6/20), FOR EVALUATION AND CO-
MULTIPLE ELECTROLYTE MANAGEMENT
IMBALANCE
(HYPOALBUMINEMIA,
HYPOCALCEMIA, HYPOKALEMIA,
HYPONATREMIA)
 ULTRASOUND OF WHOLE
ABDOMEN
 DIAPHRAGMATIC HERNIA RIGHT
 SONORAPHICALLY NORMAL LIVER, GALLBLADDER, PANCREAS, SPLEEN AND
URINARY BLADDER
 BILATERAL HYDRONEPHROSIS GRADE 2 RIGHT, GRADE 1 LEFT
 No significant disparity in the size, shape and location of both kidneys, both exhibit
hypoechoic parenchyma echopattern, relative to that of the liver and the spleen. There is
minimal to moderate dilatation of both renal pelvis. Both ureters are not dilated. There is
neither evidence of mass nor calculus in the scans obtained. The urinary bladder is empty.

LENGTH (CM) WIDTH (CM) THICKNESS (CM) PARENCHYMAL

THICKNESS (CM)
RIGHT 3.8 2.21 2.73 0.92
KIDNEY
LEFT KIDNEY 4.0 2.04 2.06 0.94
Click icon to add picture
Figure 2 The Society for Fetal Urology grading system for postnatal
hydronephrosis

GRADING OF
HYDRONEPHROSIS
:
GRADE O: NO DILATATION
GRADE 1: URINE BARELY SPLITS
SINUS
GRADE 2: FULL PELVIS MAJOR
CALYCES DILATED
GRADE 3 UNIFORMLY DILATED
MINOR CALYCES, PARENCHYMA
SPARED
GRADE 4: SIMILAR TO GRADE 3
Timberlake, M. D. & Herndon, C. D. A. (2013) Mild to moderate postnatal hydronephrosis—grading systems BUT WHEN COMPARED WITH THE
and management NORMAL CENTRALATERAL
Nat. Rev. Urol. doi:10.1038/nrurol.2013.172
KIDNEY, THERE IS PARENCHYMAL
THINNING
 COURSE IN THE WARDS NEPHRO DIAGNOSIS
6TH DOL (3RD POST-OP BILATERAL HYDRONEPHROSIS
DAY) GRADE 1 LEFT, GRADE 2 RIGHT
PROBABLY SECONDARY TO
BILATERAL CAKUT, CONSIDER: POSTERIOR
HYDRONEPHROSIS GRADE URERTHRAL VALVE,
2 RIGHT, GRADE 1 LEFT URETEROPELVIC JUNCTION
OBSTRUCTION BILATERAL,
VESICOURETHRAL REFLUX;
RULE OUT TRANSIENT
HYDRONEPHROSIS
MANAGEMENT
NEPHROLOGY NOTES
REPEAT UTZ ON 7TH DOL
-IF REPEAT UTZ SHOWS
RENAL PELVIC DIAMETER
>15MM OR RPD >10MM WITH
PERIPHERAL CALICYEAL
DILATATION – FOR VCUG
-IF REPEAT UTZ SHOWS NO
DILATION OF URETER AND
RPD <15MM, WILL DO
REPEAT UTZ AT 6 WEEKS
OLD
VCUG ONCE FREE FROM
INFECTION
BP MONITORING Q SHIFT
SERUM ELECTROLYTES AND
CREATININE MONITORING
COURSE IN THE WARDS DIAGNOSIS MANAGEMENT
7TH DOL (3RD POST-OP DAY) PULMONARY ARTERIAL WEANED FROM MIDAZOLAM
HYPERTENSION CONTINUED OTHER
FULL PULSES, TRICUSPID REGURGITATION MANAGEMENT
(-) BRADYCARDIA , SEVERE
PULMONARY REGURGITATION
CRT<2 SECONDS SEVERE,
CARDIO PLAN
PATENT FORAMEN OVALE,
PATENT DUCTUS ARTERIOSUS WEAN FROM DOPAMINE
MONITOR HEART RATE
REFER FOR HR <120BPM
ATRAUMATIC HEAD, NO GENETICS PLAN:
MASSES, SYMMETRIC FACIAL
FEATURES CURRENTLY NO
EQUAL CHEST EXPANSION, RECOGNIZABLE SYNDROME;
RALES BIBASAL LUNG
FIELDS, SUGGEST KARYOTYPING TO
ADYNAMIC PRECORDIUM, RULE OUT CHROMOSOMAL
DISTINCT HEART SOUNDS, NO STRUCTURAL ABNORMALITY
MURMUR OR ANEUPLOIDY
NONDISTENDED ABDOMEN,
FULL PULSES, CRT<2
SECONDS,GROSSLY MALE
GENITALIA, NO DEFORMITIES
COURSE IN THE WARDS DIAGNOSIS MANAGEMENT
8TH DOL (4TH POST-OP TERM BABY BOY DELIVERED VIA PATIENT WAS EXTUBATED
DAY) NORMAL SPONTANEOUS AND HOOKED TO NASAL
VAGINAL DELIVERY; APGAR CANNULA AT 2LPM
INTUBATED WITH EQUAL SCORE 8,9; BALLARD SCORE 39
AIR ENTRY WEEKS, BIRTH WEIGHT 2.56KG,
ADEQUATE CHEST RISE APPROPRIATE FOR GESTATIONAL
NO DESATURATION AGE, CONGENITAL
(+) CTT, RIGHT WITH DIAPHRAGMATIC HERNIA, RIGHT,
OSCILLIATIONS HYDRONEPHROSIS, GRADE 1,
LEFT, GRADE 2, RIGHT, SEVERE
ABG PULMONARY ARTERY
METABOLIC ACIDOSIS HYPERTENSION, TRICUSPID
WITH ADEQUATE REGURGITATION SEVERE,
OXYGENATION PULMONIC REGURGITATION
SEVERE, PFO, PDA
S/P EL REPAIR OF CDH RIGHT, IJ
CUTDOWN RIGHT
PNEUMOTHORAX, RIGHT-
RESOLVING, S/P CTT INSERTION,
RIGHT
SERUM ELECTROLYTES
MONITORING RESULT REFERENCE
BUN 19.91 2.9-7.1
CREA 219.30 (2.5mg/dL) 57-113
CL 93.1 101.0-111.0
SODIUM 131.38 136.—144.0
MAGNESIUM 0.95 0.74-1.03
POTASSIUM 3.47 3.6-5.1
CALCIUM 3.00 2.23-2.58

HYPOKALEMIA, HYPONATREMIA, HYPERCALCEMIA,

ELEVATED CREATININE
NEONATAL ACUTE KIDNEY
INJURY
 AKI is typically defined as a decrease in glomerular filtration rate (GFR),
which is traditionally defined by an increase in serum creatinine (SCr).
 Clinically, neonatal AKI has been most commonly defined as an SCr greater
than 1.5 mg/dL (133 micromol/L) or
 An increase of at least 0.2 to 0.3 mg/dL (17 to 27 micromol/L) per day from a
previous lower value.
REPEAT ULTRASOUND OF
KUB
 KUB UTZ: BILATERAL HYDRONEPHROSIS GRADE I
 RIGHT RPD 1.12 CM
 LEFT RPD 1.13 CM
 Prenatally Diagnosed Hydronephrosis
• Mild to moderate unilateral • Severe unilateral • Moderate-to-severe bilateral
• Mild bilateral • Mild-to-moderate unilateral in male • Mild-to-moderate bilateral in
with distended bladder male with distended bladder

Renal Ultrasound after 7 days Renal Ultrasound after 48 hours Renal Ultrasound on 1st postnatal day

RPD <15mm RPD ≥15 mm OR RPD<15m RPD>10mm OR RPD≤10mm

and no dilated RPD > 10 to <15 mm + peripheral m OR and Dilated ureter(s) and no dilated
ureter calyceal dilatation OR Dilated ureter no ureter ureter

Amoxicillin prophylaxis Follow-up renal US at 1

Follow-up renal US at 6 weeks, 10 mg/kg/day week, 6 weeks, 3 months,
3 months, then 6 monthly till then 6 monthly till age 2
age 2 years then yearly# MCUG years, then yearly#

Exclude obstruction if: Primary VUR

• No VUR present
REFERRAL TO • Features of bladder outlet obstruction
SURGERY
DMSA scan Stop antibiotics
Features of Exclude obstruction:
• MAG3 renogram with furosemide*
obstruction No obstruction
• Consider MRU if UVJ obstruction is suspected
HYDRONEPHROSIS ACCORDING
TO SEVERITY
 <10MM : NORMAL OR MILD HYDRONEPHROSIS
 10-15MM : MODERATE HYDRONEPHROSIS
 >15MM : SEVERE HYDRONEPHROSISL; THESE INFANTS ARE AT GREATEST RISK
FOR SIGNIFICANT RENAL DISEASE, WHICH MAY REQUIRE SURGICAL
CORRECTION
 Prenatally Diagnosed Hydronephrosis
• Mild to moderate unilateral • Severe unilateral • Moderate-to-severe bilateral
• Mild bilateral • Mild-to-moderate unilateral in male • Mild-to-moderate bilateral in
with distended bladder male with distended bladder

Renal Ultrasound after 7 days Renal Ultrasound after 48 hours Renal Ultrasound on 1st postnatal day

RPD <15mm RPD ≥15 mm OR RPD<15m RPD>10mm OR RPD≤10mm

and no dilated RPD > 10 to <15 mm + peripheral m OR and Dilated ureter(s) and no dilated
ureter calyceal dilatation OR Dilated ureter no ureter ureter

Amoxicillin prophylaxis Follow-up renal US at 1

Follow-up renal US at 6 weeks, 10 mg/kg/day week, 6 weeks, 3 months,
3 months, then 6 monthly till then 6 monthly till age 2
age 2 years then yearly# MCUG years, then yearly#

Exclude obstruction if: Primary VUR

• No VUR present
REFERRAL TO • Features of bladder outlet obstruction
SURGERY
DMSA scan Stop antibiotics
Features of Exclude obstruction:
• MAG3 renogram with furosemide*
obstruction No obstruction
• Consider MRU if UVJ obstruction is suspected
WORKING IMPRESSION
Term Baby Boy delivered via Normal Spontaneous Vaginal Delivery
APGAR score 8,9; Ballard Score 39 weeks; Birthweight 2.56
kilograms; Appropriate for Gestational Age; Congenital
Diaphragmatic Hernia, Right; Pneumothorax Resolved; Severe PAH;
Tricuspid Regurgitation, Severe; Closing PDA, PFO; bilateral
hydronephrosis grade 1; AKI secondary to sepsis - resolved; s/p
exploratory laparotomy, repair of CDH right, IJ cutdown right
(6/5/20, SPMC); S/P CTT Insertion right (6/6/20, SPMC); S/P
femoral cutdown right, (6/26/20); HCAP improving, Neonatal sepsis
(S. Hominis 6/27/20)-improving
CONGENITAL
DIAPHRAGMATIC
HERNIA
Click icon to add picture

WHAT IS
CONGENITAL
DIAPHRAGMA
TIC HERNIA
(CDH)?
 CONGENITAL
DIAPHRAGMATIC HERNIA
  There is failure of one or both of the pleuroperitoneal membranes to
close the pericardioperitoneal canals
 The peritoneal and pleural cavities are continuous with one another
along the posterior body wall allowing abdominal viscera to enter the
pleural cavity.
 This complicates 2.3-2.8 per 10,000 live births
EMBRYOLOGY
 The diaphragm is derived from the fusion of the following structures:
1. Septum Transversum
2. Pleuroperitoneal Membranes
3. Muscular components from the lateral and dorsal body walls
4. Dorsal Mesentery of the Esophagus
Review-of-medical-embryology/chapter-48-development-of-the-diaphragm
Review-of-medical-embryology/chapter-48-development-of-the-diaphragm
Review-of-medical-embryology/chapter-48-development-of-the-diaphragm
Review-of-medical-embryology/chapter-48-development-of-the-diaphragm
EMBRYOLOGY
The repositioning of the
diaphragm is caused by rapid
growth of the dorsal part of
the embryo (vertebral
column), compared with that
of the ventral part.
The phrenic nerves supply
the diaphragm with its motor
and sensory innervation.
 27%

3% 70%
TYPES OF HERNIA
GENETICS
 Isolated CDH comprises approximately 60% of cases.
 Most cases of CDH are sporadic, although a small number of family pedigrees
with Mendelian inheritance patterns have been identified
 Congenital heart disease is the most common extra-respiratory anatomic
abnormality associated with CDH.
Click icon to add picture CARDIAC
ANOMALIES IN
PATIENTS WITH
CONGENITAL
DIAPHRAGMATIC
HERNIA AND
THEIR PROGNOSIS
Significant heart defect is associated
with 10.6% of CDH.
Survival for patients with HD is
significantly lower than for patients
with normal cardiac anatomy.
No survivors of CDH coupled with
TGA
EARLY DETECTION
(ANTENATAL)
 59% detected in prenatal ultrasound
 Highest: 20–22 weeks
 Based on either direct signs, such as bowel loops, stomach and/or liver parts
displaced into the thoracic cavity, or indirect signs, such as an abnormal
position of the heart with mediastinal shift.

UNREMARKABLE
Click icon to add picture
CLINICAL
FEATURES
Tachypnea
Increased respiratory effort
Hyperinflated chest
Scaphoid abdomen
Auscultation: poor air entry
on affected side.
Peristalsis on the affected side
Shift of cardiac sounds
Signs of pneumothorax
Other anomalies
DIAGNOSTICS:
 Prenatal Ultrasonography
 Imaging study (CXR)
 2d echocardiography
 Blood work-up: Blood culture, CBC PC
Click icon to add picture

CHEST X-RAY

FINDINGS SUGGESTIVE OF
DIAPHRAGMATIC HERNIA
WITH LUNG ATELECTASIS,
RIGHT
Click icon to add picture

BABY GRAM

An important clue to the

correct diagnosis is the
course of the nasogastric
tube and the absence of
paucity of gas-filled bowel
loops within the abdomen
PATHOPHYSIOLOGY
 Postnatal
 Pulmonary parenchymal insufficiency –
There is a small functional lung mass and a few conducting air passages
and developed alveoli.
 Pulmonary hypertension –
They are predisposed to persistent pulmonary hypertension of the
newborn (PPHN).
Blood is shunted away from the lungs through the foramen ovale and patent
ductus arteriosus (PDA) which further promotes acidosis and hypoxia.
Click icon to add picture

PULMONARY
HYPERTENSIO
N
They are predisposed to
persistent pulmonary
hypertension of the
newborn (PPHN).

Blood is shunted away

from the lungs through
the foramen ovale and
patent ductus arteriosus
(PDA) which further
promotes acidosis and
hypoxia.
Click icon to add picture PULMONARY
PARENCHYMA
L
INSUFFICIENC
Y
There is a small
functional lung mass
and a few
conducting air
passages and
developed alveoli.
CLINICAL FEATURES BASED
ON SEVERITY OF THE DEFECT
 Severe respiratory distress at the time of birth (severe hypoplasia)
 Respiratory deterioration hours after delivery (honeymoon period)
 Feeding difficulties, Chronic respiratory disease
 MANAGEMENT
 Early and appropriate respiratory support is warranted. Stabilization with
endotracheal intubation is required.
 Prolonged mask ventilation, enlarging the stomach and small bowel, must be
avoided.
 A naso-orogastric tube must be placed immediately for decompression.
 Arterial and central venous lines are mandated
 Preductal arterial oxygen saturation value of >85% is the minimum goal
 Gentle ventilation with permissive hypercapnia reduces lung injury

Nelson Textbook of Pediatrics, 21st ed, Kliegman, et.al. 2019

 MANAGEMENT
 Echocardiography to measure pulmonary and system vascular pressures and to
determine presence of cardiac dysfunction.
 Inotrope use for left ventricular dysfunction
 No study has proved surfactant administration to be beneficial.
 ECMO or HFOV use

Nelson Textbook of Pediatrics, 21st ed, Kliegman, et.al. 2019

EXTRACORPOREAL MEMBRANE
OXYGENATION (ECMO)
 This machine pumps blood from the patient’s body to an artificial lung (oxygenator) that adds
oxygen to it and removes carbon dioxide. Thus, it replaces the function of the person’s own
lungs. The ECMO machine then sends the blood back to the patient via a pump with the same
force as the heart, replacing its function.

HIGH FREQUENCY VENTILATION

A machine that delivers small volumes of respiratory gas at an extremely rapid rate (300-1500

breaths per min or 5-25 hertz)
MANAGEMENT
SURGICAL

Timing — the timing of surgery has shifted from early surgical intervention to

delaying surgical correction until the patient has been stabilized medically

In particular, surgery is usually delayed in neonates with more severe forms of

pulmonary hypoplasia and pulmonary hypertension who require additional
medical care, which may include ECMO
SURGICAL MANAGEMENT
MANAGEMENT
SURGICAL
PRIMARY REPAIR
This is done if the opening in the diaphragm is small with adequate diaphragmatic muscle. A single layer of
non absorbable horizontal mattress sutures can be used.

PATCH REPAIR
Opening in the diaphragm is large or a significant portion of the diaphragm is absent. The surgeon will
require additional tissue or material to close the defect. A Gore-Tex patch repair is often required in
patients with large CDHs in whom increased tension using a primary repair compromises total thoracic
compliance

MUSCLE FLAP REPAIR

Closing of the hole with a flap of muscle. The internal oblique or latissimus dorsi from the infant’s back or
abdomen is used.
MANAGEMENT
SURGICAL
RECURRENCE
- 10-20%
- Common in cases where a prosthetic patch is used
- Should be considered in any child with a history of CDH who presents with new
gastrointestinal or pulmonary symptoms
PROGNOSIS
 The postnatal survival rate at tertiary centers has improved, with
reported rates of 70 to 92 percent. This increased survival rate
appears to be a result of the shift from early surgical intervention to
intensive preoperative supportive care aimed at avoiding lung
injury, followed by surgical correction. 
AAP/NEO REVIEWS

 CDH Scoring equation (Brindle et al)

 Low birth weight (1)
 Low 5 minute APGAR score (1)
 Unknown APGAR score (out born) (2)
 Cardiac anomalies (2)
 Chromosomal abnormalities (1)
 Significant pulmonary hypertension (2)
AAP/NEO REVIEWS

Interpretations (RISK OF DEATH)

 0 – LOW RISK
 1-2 – INTERMEDIATE RISK
 >/=3 – HIGH RISK
 NEO REVIEWS

 iNO administration, a selective pulmonary vasodilator, improves

pulmonary blood flow augmenting gas exchange.
 A report from the Congenital Diaphragmatic Hernia Registry did not
find that surfactant improves outcomes in CDH.
 Other diagnostics recommended: Echocardiography, ultrasonography,
cranial ultrasound.
 Medical management: milrinone, sildenafil
Congenital Diaphragmatic Hernia: Updates and Outcomes, Benjamin, J . Et.al. NeoReviews. Vol 12.
August 2011
 NEO REVIEWS

 The ideal time for repair remains unknown.

 The use of chest tubes after repair for draining accumulated fluid is no
longer considered routine
 Long term morbidity is highest in those who require patch repair and those
treated with ECMO.
 Complications: Chronic lung disease, pulmonary hypertension, GERD,
feeding difficulties and failure to thrive, neurodevelopmental outcomes
Congenital Diaphragmatic Hernia: Updates and Outcomes, Benjamin, J . Et.al. NeoReviews. Vol 12.
August 2011
 PRE-OPERATIVE STABILIZATION AND DELAYED
REPAIR
 With the discover of hypoplastic lung and that abnormal arteriolar
muscularization and resulting pulmonary hypertension, delayed surgical
repair was introduced.
 Cartlidge et al concluded that the use of preoperative stabilization in 17
patients was responsible for a decrease in mortality from 88% to 47%. Sakai
et al demonstrated that lung compliance often deteriorates markedly after
repair
 Currently, most infants who have CDH are stabilized before operative
intervention. The mean age at the time of surgery for infants not treated with
extracorporeal membrane oxygenation (ECMO) is 73 hours (range, 0 to 443
h).

Congenital Diaphragmatic Hernia: The Neonatologist’s Perspective., Meurs, KV, Short, BL., NeoReviews,
1999
NEO REVIEWS
The VICI Trial is the only randomized, prospective, multicenter trial comparing CMV to HFOV.
One hundred seventy-one patients were randomized to receive either CMV or HFOV, and the
remainder of each patient’s treatment followed guidelines set by the CDH EURO Consortium.
Baseline characteristics were similar, and there was no difference in the primary outcome of
death or bronchopulmonary dysplasia. Secondary outcomes favored CMV, suggesting that CMV
may be associated with improved hemodynamics compared to HFOV, as used in the VICI trial,
in patients with CDH. We recommend starting with CMV, but HFOV remains a reasonable
option for those patients who require excessive peak inflation pressure on CMV.

Congenital Diaphragmatic Hernia: Maximizing Survival, Mark F. Weems, Tim Jancelewicz and Hitesh S. Sandhu

NeoReviews December 2016
NEO REVIEWS
 Permissive hypercapnia is now preferred over induced respiratory alkalosis, and most experts
feel comfortable allowing PCO2 values as high as 60 to 70 mm Hg (8–9 kPa). In our practice,
we target PCO2 values of 50 to 65 mm Hg (6–8.6 kPa) if the pH values are greater than 7.20.
However, acidosis is known to increase pulmonary vascular resistance, and in some infants,
adequate oxygenation may not be achieved until a more normal pH is established.

Congenital Diaphragmatic Hernia: Maximizing Survival, Mark F. Weems, Tim Jancelewicz and Hitesh S. Sandhu

NeoReviews December 2016
D
 Single-institution studies have reported that best oxygenation index on day 1 of life (BOI-d1)
is the most reliable postnatal predictor of survival in CDH. 
 This multicenter study showed, that except from the defect side, all the prenatal variables
studied have predictive value but the most useful is BOI-d1.
THANK YOU!