Paediatrics: Congenital abnormalities: chest

Congenital abnormalities: chest

Congenital diaphragmatic hernia

The incidence of CDH is 1/2400. The main problem is not the diaphragmatic hernia, but rather the associated pulmonary hypoplasia that is often severe and determines prognosis. The most common type of diaphragmatic defect is posterolateral (Bochdalek) and left-sided, occur-ring in 90% (see Figs 23.3 and 23.4).

•   Antenatal screening: most CDHs are identified on antenatal US, but the prognosis for these foetuses is poor (i.e. 20% survive).

•   Birth: if the diagnosis is not made antenatally and the baby presents at birth, clinical findings may include respiratory distress, scaphoid abdomen, and apparent dextrocardia. The prognosis for survival is 760%.

•   Coincidental: 710% of CDHs are discovered during early childhood, including most anterior (Morgagni) defects. The prognosis is excellent.

Neonatal management

•   Initial management consists of sedation, paralysis, endotracheal intubation, and mechanical ventilation with 100% O₂.

•   NGT placement and avoid bag–mask–valve ventilation.

•   If oxygenation is good and pulmonary hypoplasia is not severe, repair of the diaphragmatic defect is undertaken after a few days either by primary suture or insertion of a prosthetic patch.

Hiatus hernia (HIH)

HIH refers to herniation of the stomach into the chest through the oe-sophageal hiatus in the diaphragm. The lower oesophageal sphincter also moves and becomes incompetent. Most children with HIH present with gastro-oesophageal reflux (GOR). Two types of hiatus hernia are recog-nized (Fig. 23.5):

•   sliding (common);

•   rolling or paraoesophageal (rare).

Management

•   Diagnosis is made radiologically by barium meal.

•   Treatment comprises management of the GOR, initially medically.

•   Surgery is reserved for children who fail to respond to medication, complicated reflux (e.g. peptic strictures), and paraoesophageal hernias (because of the risk of incarceration and infarction of the herniated stomach). Surgery involves repair of the hiatus hernia and a fundoplication to prevent GOR.