Download MBBS (Bachelor of Medicine, Bachelor of Surgery) 1st Year, 2nd Year, 3rd Year and Final year Pediatric Surgery 8 Congenital Diaphragmatic Hernia PPT-Powerpoint Presentations and lecture notes
CONGENITAL DIAPHRAGMATIC
HERNIA (CDH)
History
? 1679 ? Riverius recorded the first CDH
? 1761 ? Morgagni desribed types of CDH
? 1905 ? Heidenhain repair CDH
? 1925 ? Hedbolm suggested that CDH led to
pulmonary hypoplasia and early operation
improve survival
? 1946 ? Gross correct CDH < 24 hours of age
? 1980-1990 ? delayed correction become
widely accepted
Introduction
? 1 in 2000- 5000 births
? 95.8% posterolateral defects (Bochdalek)
? 84.4% left side
? 13.2% right side
? 2.4% bilateral
? Morgagni and pars sternalis hernias rare
? 10-50% will have associated anomalies.
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Embryology
ANATOMY
Diaphragmatic development- begins 4th
week
1) Anterior central tendon --Septum
transversum (4 wk)- forms the
inferior portion of pericardial cavity
and defines rudimentary
pleuroperitoneal membrane.
2) Dorsolateral portion--Closure of
pleuroperitoneal canals and
formation of pleuroperitoneal
membrane (6-8 wk).
3) Dorsal crura? dorsal esophageal
mesentery
4) Muscularisation of diaphragm- from
thoracic intercostal muscle group
and post hepatic mesenchyme.
Failure of closure of pleuroperitoneal
canal (9-10 wk)
Posteriolateral lumbocostal trigone
(weak area)- by Bochdalek (1848)
Pathophysiology
? Combination of events...
A. Physical compression
B. Pulmonary hypoplasia
bilateral y (ipsilateral
>contralateral)-
Uncorrectable
A. Smaller bronchi
B. Less branching
C. Decreased alveolar SA
C. Pulmonary hypertension-
potential y reversible
A. Lack of pulmonary arterioles
B. Increased thickness of
muscle
? Abnormal y high vascular
reactivity of vessels
? Right to left shunt
Diagnosis
? Prenatally
? U/S ? can diagnose 50%, 50% "normal",
? as early as 15 weeks
? Polyhydramnios
? Absent or intrathoracic stomach bubble
? Mediastinal or cardiac shift away from hernia
? Assess other organs
? Amniocentesis for chromosomes, alpha
fetoprotein (18 weeks)
? Fetal MRI
Diagnosis
? Postnatal
? RD in the first 24hours.
scaphoid abdomen
(majority)
? After 24h with
vomiting, cough,
cyanosis, gastric outlet
obstruction, herniation
of liver, spleen, bowel.
Prognostic Indicators
? *Herniated organs (liver)
? *Lung to head ratio (LHR)
? <1 poor and <0.85 severe, 0.6-1.35
61% survive, >1.35 100% survive
? Associated anomalies
? Birth weight and Apgar
score ( the CDH study group)
? Measurement of L/T ratio
L/T normal ~ 0.52, severe < 0.26
? Others- Antenatal diagnosis<24wks,
polyhydramnios, position of stomach,
right sided defect, Mcgoon's index,
Pulm. Artery Index, OI, MVI
Prenatal Diagnosis
? Check for associated anomalies
? Plan for delivery at tertiary perinatal centre +/-
ECMO
? Options for parents
? Prenatal therapies, termination, treatment after
delivery
Investigations
? Arterial blood gas
? frequent arterial blood gas (ABG) measurements to assess for pH, PaCO2, and PaO2.
? PaO2 may be higher from a preductal (right-hand) sampling site.
? Chromosome studies
? frequent association with chromosomal anomalies (trisomy 13,18)
? If dysmorphic features are observed on examination, a consultation with a geneticist .
? Serum electrolytes: Monitor serum electrolytes, ionized calcium, and glucose
levels initially and frequently. Maintenance of reference range glucose levels and
calcium homeostasis is particularly important
? ECHO
? Renal USG
v Pulse oximetry
? Continuous pulse oximetry is valuable in the diagnosis and management
of PPHN.
? Place oximeter probes at preductal (right-hand) and postductal (either
foot) sites to assess for a right-to-left shunt at the level of the ductus
arteriosus
Management
? Spontaneous Vaginal Delivery, close to term
? Initial resuscitation
? Supplemental O2
? No bag & mask
? ETT with mechanical ventilation
? Ventilation strategies*
? Watch for pneumothorax (usually contralateral)
? NG tube
? Fluid status
? Inotropic support
? Delayed surgery ( until patient is stable).
Management
? Mechanical Ventilation
? May require high setting, high FiO2
? High OI (poor survival) (OI= MAP?FiO2?100/PaO2)
? Paralysis/ sedation
? Arterial and venous access
? IV antibiotics, IV fluids, correct acidosis, glucose
maintainence
? NG tube
? Inotropic support
? Temperature maintainence
? Role of Tolazoline( a- receptor blocker),CCB, endothelin
receptor blockers, Sildenafil, Nitric Oxide
? Role of surfactant- ??
Ventilation Strategies
? Prevent conditions that raise pulmonary
vascular resistance (hypoxemia, acidosis,
hypotension and hypercarbia)
hyperventilation to control hypoxemia
Aim: pH > 7.45, PCO2 < 35 , PaO2 > 60
Avoid barotrauma.
Wung et al. showed that some of the mortality in CDH infants was infact due
to ventilator-induced lung injury
? Gentle ventilation with permissive hyper-capnia
? Low PIP, pressure-limited ventilation, minimal set
RR, minimal sedation and tolerance of high PCO2
upto 60 survival>90
? Goal- Preductal PO2> 60mmHg and SaO2> 90%
and PCO2< 60mmHg
Surgical Repair
? First repair in 1940 (Ladd & Gross)
? 1970's ? Early intervention
? Emergent surgery to alleviate the compression of the
"good" lung
? Current practice allows a period of stabilization prior
to repair
? CDH is a physiologic emergency, not a surgical emergency
? Pulmonary hypertension is the primary determinant of
mortality
? Wait until weaning / low ventilatory settings or Pulm. HTN
abated or stabilised on ECMO
Surgical Repair
Surgical repair- options
? Open (subcostal incision) / thoracoscopic repair
? Abdominal/ thoracic
? Primary repair after reduction of contents with
interrupted non absorbable sutures (Prolene)
? Reconstructive options:
Pre renal fascia, surrounding ribs, Prosthetic patch,
split abd. Muscle flap/ LD flap (thoracic)
? Skin closure and creation of ventral hernia/
temporary silo
? ICD ? not required (only when
bleeding/col ection/air leak)
Long term outcome
? GERD
? Neurological outcome- CNS and Ear
? Chronic lung disease (obst./rest. Airway- 50%
survivors)
? Growth issues
? Chest wall deformities, scoliosis
Newer Advances
1) In utero
? Surgical repair - >70% mortality
? Tracheal ligation or occlusion (FETO)
? 27-28 weeks GA
? Causes distention and secondary hyperplasia
? Good results in 40% of patients
2)Ventilation strategies ? HFOV, NO, ECMO, Liquid
ventilation (PFC)
3) Lung transplantation
4) Growth factors- combined TRH & steroids
MCQ's
1) In CDH, the most common defect is
a. Anterior morgagni
b. Posteriolateral Bochdalek
c. Right sided defect
d. Pars sternalis
2) CDH is a/an
a. Physiologic/ medical emergency
b. Surgical emergency
c. Both a and b
d. OPD case
3) 2kg newborn child Day 1, with respiratory
distress, Scaphoid abdomen, Bowel sounds
+nt in left chest and X-ray chest s/o bowel
loops present in left chest with mediastinal
shift to right. The first intervention you do in
emergency
a. Bag and mask
b. Iv Fluids and antibiotics
c. ETT and ventilation
d. Ng tube insertion
4) Antenatal Usg s/o Polyhydramnios and absent
stomach bubble, most prabable diagnosis s/o
a. PUV
b. Malrotation
c. CDH
d. TEF with EA
5) Day 4 Child with CDH on ventilator with high
settings, ECHO s/o PDA, referred from NiCU
for surgical opinion
a. Immediate surgery
b. Wait further
c. ECMO
d. PDA ligation
6) Child with CDH on ventilator requires
a. Aggressive ventilation with high PIP
b. Gentle ventilation with permissive
hypercapnia
c. High RR
d. High tidal volume
7) CDH associated with better prognosis is
a. Liver up
b. Premature
c. LHR > 1
d. Right sided
8) Antenatal review to pediatric surgery OPD,
G1P1,30wks pregnant with CDH child with
polyhydramnios
a. Termination of pregnancy
b. Prenatal steroids and delivery at 34 wks
c. SVD near term at hospital
d. Refer to genecist
9) Surgery for CDH
a. Reduction of contents and primary repair
b. Primary repair after content reduction with
ventral hernia creation
c. Prosthetic Patch repair
d. Thoracoscopic repair
e. All of the above
10) Recent advances in treatment of CDH
antenatally
a. ECMO
b. HFOV
c. FETO
d. NO
This post was last modified on 08 April 2022