? 1679 ? Riverius recorded the first CDH
? 1761 ? Morgagni desribed types of CDH
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? 1905 ? Heidenhain repair CDH
? 1925 ? Hedbolm suggested that CDH led to
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pulmonary hypoplasia and early operationimprove survival
? 1946 ? Gross correct CDH < 24 hours of age
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? 1980-1990 ? delayed correction become
widely accepted
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Introduction
? 1 in 2000- 5000 births
? 95.8% posterolateral defects (Bochdalek)
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? 84.4% left side
? 13.2% right side
? 2.4% bilateral
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? Morgagni and pars sternalis hernias rare? 10-50% will have associated anomalies.
36
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36Embryology
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ANATOMYDiaphragmatic development- begins 4th
week
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1) Anterior central tendon --Septumtransversum (4 wk)- forms the
inferior portion of pericardial cavity
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and defines rudimentary
pleuroperitoneal membrane.
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2) Dorsolateral portion--Closure ofpleuroperitoneal canals and
formation of pleuroperitoneal
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membrane (6-8 wk).
3) Dorsal crura? dorsal esophageal
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mesentery4) Muscularisation of diaphragm- from
thoracic intercostal muscle group
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and post hepatic mesenchyme.
Failure of closure of pleuroperitoneal
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canal (9-10 wk)Posteriolateral lumbocostal trigone
(weak area)- by Bochdalek (1848)
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Pathophysiology
? Combination of events...
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A. Physical compression
B. Pulmonary hypoplasia
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bilateral y (ipsilateral>contralateral)-
Uncorrectable
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A. Smaller bronchi
B. Less branching
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C. Decreased alveolar SAC. Pulmonary hypertension-
potential y reversible
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A. Lack of pulmonary arterioles
B. Increased thickness of
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muscle? Abnormal y high vascular
reactivity of vessels
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? Right to left shunt
Diagnosis
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? Prenatally? U/S ? can diagnose 50%, 50% "normal",
? as early as 15 weeks
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? Polyhydramnios? Absent or intrathoracic stomach bubble
? Mediastinal or cardiac shift away from hernia
? Assess other organs
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? Amniocentesis for chromosomes, alphafetoprotein (18 weeks)
? Fetal MRI
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Diagnosis
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? Postnatal
? RD in the first 24hours.
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scaphoid abdomen(majority)
? After 24h with
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vomiting, cough,
cyanosis, gastric outlet
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obstruction, herniationof liver, spleen, bowel.
Prognostic Indicators
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? *Herniated organs (liver)
? *Lung to head ratio (LHR)
? <1 poor and <0.85 severe, 0.6-1.35
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61% survive, >1.35 100% survive
? Associated anomalies
? Birth weight and Apgar
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score ( the CDH study group)
? Measurement of L/T ratio
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L/T normal ~ 0.52, severe < 0.26? Others- Antenatal diagnosis<24wks,
polyhydramnios, position of stomach,
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right sided defect, Mcgoon's index,
Pulm. Artery Index, OI, MVI
Prenatal Diagnosis
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? Check for associated anomalies
? Plan for delivery at tertiary perinatal centre +/-
ECMO
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? Options for parents
? Prenatal therapies, termination, treatment after
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deliveryInvestigations
? Arterial blood gas
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? frequent arterial blood gas (ABG) measurements to assess for pH, PaCO2, and PaO2.
? PaO2 may be higher from a preductal (right-hand) sampling site.
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? Chromosome studies? frequent association with chromosomal anomalies (trisomy 13,18)
? If dysmorphic features are observed on examination, a consultation with a geneticist .
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? Serum electrolytes: Monitor serum electrolytes, ionized calcium, and glucose
levels initially and frequently. Maintenance of reference range glucose levels and
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calcium homeostasis is particularly important? ECHO
? Renal USG
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v Pulse oximetry
? Continuous pulse oximetry is valuable in the diagnosis and management
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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
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arteriosus
Management
? Spontaneous Vaginal Delivery, close to term
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? Initial resuscitation
? Supplemental O2
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? No bag & mask? ETT with mechanical ventilation
? Ventilation strategies*
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? Watch for pneumothorax (usually contralateral)
? NG tube
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? Fluid status? Inotropic support
? Delayed surgery ( until patient is stable).
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Management
? Mechanical Ventilation
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? May require high setting, high FiO2? High OI (poor survival) (OI= MAP?FiO2?100/PaO2)
? Paralysis/ sedation
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? Arterial and venous access
? IV antibiotics, IV fluids, correct acidosis, glucose
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maintainence? NG tube
? Inotropic support
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? Temperature maintainence
? Role of Tolazoline( a- receptor blocker),CCB, endothelin
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receptor blockers, Sildenafil, Nitric Oxide? Role of surfactant- ?
Ventilation Strategies
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? Prevent conditions that raise pulmonaryvascular resistance (hypoxemia, acidosis,
hypotension and hypercarbia)
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hyperventilation to control hypoxemia
Aim: pH > 7.45, PCO2 < 35 , PaO2 > 60
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Avoid barotrauma.Wung et al. showed that some of the mortality in CDH infants was infact due
to ventilator-induced lung injury
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? Gentle ventilation with permissive hyper-capnia
? Low PIP, pressure-limited ventilation, minimal set
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RR, minimal sedation and tolerance of high PCO2upto 60 survival>90
? Goal- Preductal PO2> 60mmHg and SaO2> 90%
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and PCO2< 60mmHg
Surgical Repair
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? First repair in 1940 (Ladd & Gross)? 1970's ? Early intervention
? Emergent surgery to alleviate the compression of the
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"good" lung
? Current practice allows a period of stabilization prior
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to repair? CDH is a physiologic emergency, not a surgical emergency
? Pulmonary hypertension is the primary determinant of
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mortality
? Wait until weaning / low ventilatory settings or Pulm. HTN
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abated or stabilised on ECMOSurgical Repair
Surgical repair- options
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? Open (subcostal incision) / thoracoscopic repair? Abdominal/ thoracic
? Primary repair after reduction of contents with
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interrupted non absorbable sutures (Prolene)
? Reconstructive options:
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Pre renal fascia, surrounding ribs, Prosthetic patch,split abd. Muscle flap/ LD flap (thoracic)
? Skin closure and creation of ventral hernia/
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temporary silo
? ICD ? not required (only when
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bleeding/col ection/air leak)--- Content provided by FirstRanker.com ---
Long term outcome? GERD
? Neurological outcome- CNS and Ear
? Chronic lung disease (obst./rest. Airway- 50%
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survivors)
? Growth issues
? Chest wall deformities, scoliosis
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Newer Advances1) In utero
? Surgical repair - >70% mortality
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? Tracheal ligation or occlusion (FETO)
? 27-28 weeks GA
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? Causes distention and secondary hyperplasia? Good results in 40% of patients
2)Ventilation strategies ? HFOV, NO, ECMO, Liquid
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ventilation (PFC)
3) Lung transplantation
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4) Growth factors- combined TRH & steroidsMCQ's
1) In CDH, the most common defect is
a. Anterior morgagni
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b. Posteriolateral Bochdalekc. Right sided defect
d. Pars sternalis
2) CDH is a/an
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a. Physiologic/ medical emergencyb. Surgical emergency
c. Both a and b
d. OPD case
3) 2kg newborn child Day 1, with respiratory
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distress, Scaphoid abdomen, Bowel sounds
+nt in left chest and X-ray chest s/o bowel
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loops present in left chest with mediastinalshift to right. The first intervention you do in
emergency
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a. Bag and mask
b. Iv Fluids and antibiotics
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c. ETT and ventilationd. Ng tube insertion
4) Antenatal Usg s/o Polyhydramnios and absent
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stomach bubble, most prabable diagnosis s/o
a. PUV
b. Malrotation
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c. CDHd. TEF with EA
5) Day 4 Child with CDH on ventilator with high
settings, ECHO s/o PDA, referred from NiCU
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for surgical opinion
a. Immediate surgery
b. Wait further
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c. ECMOd. PDA ligation
6) Child with CDH on ventilator requires
a. Aggressive ventilation with high PIP
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b. Gentle ventilation with permissivehypercapnia
c. High RR
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d. High tidal volume7) CDH associated with better prognosis is
a. Liver up
b. Premature
c. LHR > 1
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d. Right sided8) Antenatal review to pediatric surgery OPD,
G1P1,30wks pregnant with CDH child with
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polyhydramnios
a. Termination of pregnancy
b. Prenatal steroids and delivery at 34 wks
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c. SVD near term at hospitald. Refer to genecist
9) Surgery for CDH
a. Reduction of contents and primary repair
b. Primary repair after content reduction with
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ventral hernia creation
c. Prosthetic Patch repair
d. Thoracoscopic repair
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e. All of the above10) Recent advances in treatment of CDH
antenatally
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a. ECMO
b. HFOV
c. FETO
d. NO
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