congenital diaphragmatic hernia dr. s. parthasarathy md., da., dnb, md (acu), dip. diab.dca, dip....

Dr.S.Parthasarathy MD DNB PhD

Congenital diaphragmatic hernia

Dr. S. Parthasarathy MD., DA., DNB, MD (Acu),

Dip. Diab.DCA, Dip. Software statistics PhD (physio)

Mahatma gandhi medical college and research institute, puducherry, India


What is it ??

• Herniation of abdominal contents in the

thoracic cavity through a cong defect in the

diaphragm


How common ??

• Incidence:-• 1 in 2000 to 1 in 4000

• M:F : 2:1

• Lt : Rt : 5:1


Sites

80 % 10 %


Then what is eventration ??

• Eventration of diaphragm- • absence of muscular component of the

diaphragm, may be asymptomatic to s/s similar to Bochdalek hernia

• Reported percentages vary


Aetiology

• Unclear • 2 % familial • Genetic association - trisomies 13, 18 and 21.• Chromosome region 15q26 – necessary for

diaphragm development


40 % associated with other anomalies • Cardiac anomalies• Ductus arteriosus Septal defects, AV valve defects, Aortic arch

hypoplasia

• Musculoskeletal• Hypodactyly Long bone aplasia ,Talipes

• CNS• Microcephaly, Cerebral palsy

• Genitourinary• Hypospadias Renal dysplasia


Embryology • The diaphragm, lungs, and gastrointestinal tract

develop synchronously.

• Developing Diaphragm envelops the esophagus,

inferior vena cava, and aorta and fuses with the

foregut mesentery to form the posterior and medial

(membranous) portions of the diaphragm.

• Pleuro peritoneal canals fuse

• Failure of fusion --- CDH -- BUT


Pathogenesis

• Experimental evidence suggests that pulmonary

hypoplasia arises during the embryonic stage of

gestation, prior to the development of the fetal

diaphragm.

• Persistant pulmonary hypertension

• Transition ?? Endothelin !!

•


How do we know ??

• One dose of the herbicide nitrofen, when

administered to rodents in early pregnancy,

consistently induces pulmonary hypoplasia

and CDH in a high proportion of their

offspring.

• So pulmonary hypoplasia → CDH


It is similar to films

• A few people came to movies to see glamour

• But now glamour is the major determinant to suck people into its fold

• Pulmonary hypoplasia sucks ??

• Both sides problem in lungs – but contents worsen ipsilateral side


Think as a whole

• Its not a hole in the diaphragm

• Many problems noted

• Even sometimes – LV dysfunction is noted


What is inside ??

• underdeveloped airways, • Abnormal differentiation of type II pneumocytes, • reduced number of pulmonary arteries per unit

lung volume.• Intrapulmonary arteries become excessively

muscularized• React to vasoactive substances


Prenatal diagnosis

• USG - 24 weeks – stomach in the thorax easy

• But other contents and right sided may be difficult

• Look for associated cardiac anomalies

• Prognosis bad if other anomalies


lung-thorax transverse area ratio (LT ratio)

• ratio of right and left lung area to thorax area in a cardiac four-chamber view, to assess the severity of the pulmonary hypoplasia

• Serial measurements – important • < 0.25 – bad • fetal lung-to-head ratio– LHR<1.0 implies a

poor prognosis.


Antenatal treatment

• Surfactant therapy – proved use ?? • Steroids – betamethasone – beneficial

• Fetoscopic repair ?? • Fetoscopic balloon occlusion of trachea• Surfactant remains and expands • At the time of delivery – unplug trachea• patients may benefit if liver is not the content


After birth, can she be happy ??

• Normal or LSCS --=- NO PROBLEM

• BUT BE SURE TO BE TERM

Less mask ventilation


Physical examination

• scaphoid abdomen,

• bulging chest,

• decreased breath sounds

• distant or right-displaced heart sounds,

• bowel sounds in the chest• Cyanosis• Resp. distress .


Morgagni-type hernia

• Neonates with the Morgagni-type hernia

may present with less severe respiratory

compromise but with symptoms of bowel

obstruction.


bowel gas pattern in the chest, mediastinal shift


Blood gas values of infants


Pathophysiology

• Hypoxia – tachypnea • ↓• Hypocarbia – exhaustion • ↓• Hypercarbia – respiratory acidosis • ↓• Tissue hypoxia • ↓• Metabolic acidosis


Immediate intervention

• decompression of the stomach with an orogastric or nasogastric tube

• supplemental oxygen by mask.• No mask ventilation . • If cyanosis and hypoxemia persist, awake

intubation should be done to facilitate mechanical ventilation


• peak inspiratory pressures should not exceed 25 cm H2O.

• FIO2 should be adjusted so that preductal arterial saturations (SaO2 ) are >85%.


Options • A-a PO2 gradient is more than 500mmHg is

predictive of bad prognosis • 400 – 500 – doubtful • < 400 -- OK • Preductal 20 to 30 mm Hg O2 more • Shunt is where ?? – PDA or PFO • NaHCo3, ventilation ,• pulm. Vasodilators (inh. NO) – more useful in

Right heart failure • It corrects PHT but not hypoxia


• Maintain temperature • IV fluids, • Fentanyl infusion

• high-frequency oscillatory ventilation (HFOV)• Not more than 15 cm – no proper trials


Before surgery

• Hypoxemia • Hypercarbia • Acidosis • Should be corrected before surgery • NO PPH

• Emergent to intervene • But emergent to operate ?? • May wait for 7 to 10 days


One more index

• oxygenation index

• [FIO2x mean airway pressure x 100/PaO2].) Values of oxygenation index in excess of 40 predict mortality greater than 80%.

• 0.5 * 25 * 0.5• = 6.25


Extracorporeal membrane oxygenation

• Yes useful if IPPV fails • Veno venous and veno arterial • Membrane oxygenator • Beware of heparin in ECMO • But long term survival and morbidity were

worse in those cases


ECMO


Other bad prognostic indicators

1. symptoms severe enough to require intubation immediately after birth

2) <1000g3)< 33 weeks gestational age4) PaCo2 > 50 mm Hg


Anaesthetic options


Specific problems of neonates:• Anatomical problems : difficult venous and difficult airway

access

• - Physiological problems : high metabolic rate, limited

pulmonary, cardiac and thermo regulatory reserve ,

impaired renal and hepatic function.

• - Pharmacological problems:

• multi-system immaturity contrast to adult

• RAVI

• ( reserve, rate, airway, venous, immaturity)


B- Specific problems of prematurity (less than 37 weeks)

• - Perioperative hypoglycemia. – • Hypothermia.• Intracranial haemorrhage• - Congestive heart failure. • Retinopathy.• - Respiratory distress.

• HHHH RR - pneumonic


As Prof. mentions

In neonates -- Take care of

• oxygenation, • Temperature • IV fluids


Anaesthetic management

• Laboratory tests

• ABG, CBC, electrolytes, blood sugar, blood type,

and cross-match for blood products.

• Temperature corrected

• Upper limb IV access √ – reduction of hernia

may obstruct IVC


Premed and monitors

• No premedication

• Monitors for • RS, ( pre and postductal SPO2)• CVS ( CVP- preferably femoral access )• Temperature


Induction

• Awake intubation • Sevo induction and intubation • Relaxant ??

• No problem if already intubated by paediatrician • No nitrous before reduction – Air -O2- agent• Ventilate – high frequency, 25 – 30 cm pressure • Permissive hypoxemia


Again it’s the same

In neonates -- Take care of

• oxygenation, • Temperature • IV fluids

• IV fentanyl 1 – 3 Mic gm / kg


Intraop problems

• Closure

• Sudden hypotension and desaturation

• 1. tension pneumothorax – contralateral – ICD • 2. IVC compression – silastic patch closure • Pneumothorax can also happen ipsilaterally


The basic fluid schedule

• 5% dextrose in one-fourth to one-half strength saline are given at 4 mL/kg/hour.

• Intraoperative evaporative and third space losses are replaced with Ringer's lactate or saline at approximately 6 to 8 mL/kg/hour.

• Each milliliter of blood loss is replaced with 3 mL of Ringer's lactate or 1 mL of 5% albumin


• Do not extubate

• But beware of transport


Can go bad again • Pulmonary hypoplasia

• Associated congenital defects

• Inadequate preoperative preparation.

• Pneumothorax

Ineffective postoperative management.

Hemorrhage, tension pneumothorax, inferior vena

cava compression, persistent fetal circulation,

excessive suction on chest tube


• 30 – 60 % mortality


Long-term follow-up

• Gastroesophageal reflux occurs in up to 62%

of patients,

• 56% are below the 25th percentile for weight

• 32% require a gastrostomy,

• 19% require fundoplication


Summary

• Definition • Incidence • Types • Prenatal • Bad prognosis • Pre anaesthesia • Anaesthesia • Post op


Thank you all

congenital diaphragmatic hernia dr. s. parthasarathy md., da., dnb, md (acu), dip. diab.dca, dip....

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