DIAPHRAGMATIC HERNIA

SUDARSHAN PANDEYINTERN , KUSMS

DEVELOPMENT OF DIAPHRAGM

DEVELOPMENT OF LUNGSFactor affecting Lung

Development• Lung liquid• Amniotic fluid volume• Intra thoracic space

availability• Fetal breathing

DEFINITION• A diaphragmatic hernia is defined as a communication

between the abdominal and thoracic cavities with or without abdominal contents in thorax.

TYPES

ETIOLOGY • Congenital• Traumatic

ANATOMICALLY• Hiatal (esophageal hiatus)• Paraesophageal • Retrosternal (Morgagni)• Posterolateral ( Bochdalek)

CONGENITAL DIAPHRAGMATIC HERNIA

CONGENITAL DIAPHRAGMATIC HERNIA

EPIDEMIOLOGY

• INCIDENCE: 1/2000- 1/5000 live births

• M:F – 1:2• Left side more common

(85%)• B/L <5%• Sporadic( most cases) • Familial ( autosomal

recessive, multifactorial)

ASSOCIATED ANOMALIES• CNS lesions• Omphalocele• Esophageal atresia• Cardiovascular lesions• Part of trisomy 21, trisomy

13, trisomy 18, fryns,

SIGNS AND SYMPTOMS• Respiratory distress (tachypnea,

grunting, cyanosis, use of accessory muscles – cardinal sign

• Scaphoid abdomen • Increased chest wall diameter• Bowel sound heard in chest

with decreased/absent breath sounds on side of hernia

• Shifting of apex beat contralateral side of hernia

DELAYED PRESENTATION• Often right side• Regurgitation• Vomiting( d/t intestinal

obstruction)• Incarcination of intestine• ischemia sepsis

and shock

DIAGNOSIS • Prenatal USG at 16-24 WOG:

polyhydramnios, chest mass, mediastinal shift, gastric bubble in thoracic cavity

• After delivery: Chest X Ray with nasogastric tube

• Barium study• Echocardiography • Amniocentesis

X RAY UPPER GI STUDY

PROGNOSTIC IMAGING FEATURE

• Lung to head size ratio(LHR)• LHR<1 : POOR SURVIVAL• LHR>1.4: INCREASED

SURVIVAL• Liver in thoracic cavity: poor

prognosis

LHR= LUNG AREA/HC

Differential Diagnosis• Cystic lung lesion( pulmonary sequestration, cystic adenoid

malformation Pulmonary sequestration• Eventration of Diaphragm• Bronchogenic cyst• Neurogenic Tumors• Primary Lung Sarcoma

TREATMENT

INITIAL MANAGEMENT• Aggressive respiratory support

( rapid endotracheal intubation, sedation

• NG tube insertion and urinary catheterization needed

• Pre- and postductal oxygenation and arterial pressure have to be monitored continuously by umbilical arterial catheter placement

• Prolonged Bag and mask contraindicated

GOAL• Pre ductal SaO2>= 85%• PIP<25 cm of water• Permissive hypercapnia

PaCO2 (45- 60 mm Hg)

VENTILATORY STRATEGIES• Conventional mechanical

ventillation• HFOV• ECMO

GOAL• Oxygenation without

barotrauma• PIP=<25 cm of water• Rate 30-60 breathes/min

CMV vs HFOV

Nitric oxide• Selective pulmonary

vasodilator

ECMO(Extracorporeal Membrane Oxygenation)

• Lower limit of weight required>=2000g

• Can be venoarterial and venovenous

SURGICAL REPAIR• Mostly after 48 hrs after

stabilization and resolution of pulmonary hypertension

• Delayed in newborn on HFOV• Most common approach :

subcostal approach• Laparoscopic and thoracoscopic

repair• Native tissue vs patch(GORE-

TEX, porous polytetrafluroethylene patch)

Relative indicators for stability• Requirement of

conventional ventilation only

• Low PIP• FiO2<50%

RECENT ADVANCES

• Liquid ventilation : Tracheal installation of perfluorocarbon (PFC) to replace nitrogen as a carriage for oxygen and carbon dioxide.

• Fetal surgery: Human Tracheal occlusion (fetoscopic endoluminal tracheal occlusion, FETO)

COMPLICATION

• GERD (50%)• Intestinal Obstruction (20%)• Recurrent diaphragmatic hernia (5-20%)• Delayed growth in 1st 2 years of life• Neurocognitive defect (more common in

infants requiring ECMO)• Pectus excavatum• Scoliosis

PROGNOSIS

POOR PROGNOSIS

• Overall survival of CDH 67%• Spontaneous fetal demise: 7-10%

• Associated major anomalies• Symptoms before 24 hrs of age• Severe pulmonary hypoplasia• Need for ECMO

REFERENCES

• Nelson- Text Book of Paediatrics-19th edition• Langman’s Medical Embryology- 11th edition

THANK YOU