CONGENITAL DIAPHRAGMATIC

HERNIA

Maj Asrar AhmadMBBS, FCPS

Anatomy

95 %

5 %

Embryology

Pathophysiology

Pathophysiology Pulmonary hypoplasia“compression theory”

- modeled in fetal lambs- rationale for early surgery

“global embryopathy”- modeled in newborn rats- rationale for new therapeutic ideas

Pulmonary hypertension - causes persistent fetal circulation

Incidence 1:2500-5000 live births 1100 cases in the U.S.

annually 80 % Left side

survival remains around ~65%

Diagnosis Antenatal:

U/S at ~20 weeks gestation ~60%

Polyhydramnios; intrathoracic stomach or liver; abdominal circumference; lung-to-head ratio

Presentation Shortness of breath Scaphoid abdomen

Three general presentations:Severe respiratory distress at the time of birth.Respiratory deterioration hours after delivery

Benefit from correction of hypoxemia and pulmonary hypertension

Feeding difficulties, chronic respiratory disease, pneumonia

10-20 % intestinal obstruction

Initial Management Oxygenate but avoid barotrauma

Intubate

Sedate

NGT for decompression

Medical Management Medical emergency not surgical

Pulmonary vasodilators Inotropes High frequency oscillatory ventilation ECMO Surfactant Antenatal steroids?

ECMO

ECMO

Surgical Management

Surgical Management

Surgical Management

Surgical Management

Developing Therapies Fetal surgery

PLUG fetal surgery

Growth factors