Clinically, there is distension of the abdomen without discolouration of the abdominal wall and without tenderness.  Feeds are often tolerated but gastric aspirate residuals are not uncommon.  The aspirates are usually undigested milk, and bilious aspirates should make you think of other causes.  Occasionally the distension can be so great as to cause respiratory embarrassment.

Radiographically, there is evidence of intestinal air, without features which would suggest other more sinister processes (i.e. intramural gas, free air, thick-walled bowel loops).

Treatment is to

• ensure that a large-bore intragastric tube is vented
• allow time off CPAP if possible
• encourage the baby to pass stool (using glycerine suppositories) if this is a problem

Causes include:

• Necrotising enterocolitis
• "Spontaneous" intestinal perforation (frequently in the terminal ileum)
• Gastric or duodenal ulcer erosion and perforation
• Association with postnatal steroid use

The use of indomethacin has been postulated to increase the incidence of bowel perforation.  However, large randomised studies have not shown any increase in infants treated with indomethacin.

The radiograph to the left shows extensive intraperitoneal air - so much so that this supine film clearly outlines air around the liver, the falciform ligament, and provides an air-contrast film of the bowel wall.  There is also air in the scrotum (where there were inguinal herniae).  The lower film demonstrates the placement of an intraperitoneal drain.

There is debate about whether an isolated perforation can be managed conversatively with peritoneal drainage.  This may be used as a temporary measure if the baby is too ill to be considered for a laparotomy, or for some infants this may be a definitive treatment.  There are presently trials designed to answer this question for both spontaneous perforations and perforated NEC (the NET and NEC STEPS trials).

Partial or complete duodenal obstruction may result from intrinsic causes or external compression.  Duodenal atresia results in complete obstruction. Radiographs show a characteristic picture of a "double bubble", with air filling a distended stomach and the blind-ended duodenum.  There is an association with Trisomy 21.

Partial obstruction may be caused by an intraluminal web, duodenal stenosis, annular pancreas, or congenital bands.

Duodenal obstruction may present in utero with polyhydramnios.  The antenatal ultrasound and plain radiograph images are of an infant with duodenal atresia who underwent a duodenoduodenostomy on the second day of life.  The karyotype was normal.

The presentation may be subtle (including intermittent episodes of vomiting in a baby otherwise feeding normally) or more obvious with signs of bowel obstruction or even life-threatening collapse.  Episodes of bile-stained vomiting in infants should be taken seriously.  There may be blood-stained stool.

Plain films may be normal or may show evidence of duodenal obstruction with a paucity of bowel gas through the rest of the abdomen (see top image to left).  Air-fluid levels suggesting obstruction are not usual with malrotation.  An airless abdomen is a sign of severe problems and usually indicates intestinal infarction.  Upper intestinal contrast studies are reliable in localising the ligament of Treitz. There may be a typical corkscrew constriction of the third portion of the duodenum (see images to the bottom left).

Treatment is surgical, with correction of the malrotation of the colon and therefore prevention of a midgut volvulus.  If a volvulus is suspected, an emergency laparotomy should be undertaken.

The bottom image shows the plain abdominal film of a preterm infant who presented with acute abdominal distension and cardiorespiratory collapse at a month of age.  She had been well, with no problems with feed tolerance, then presented acutely shocked and critically unwell. The radiograph shows dilated bowel loops, without evidence of intramural gas or free air.

Clinical presentation is usually with abdominal distension and bilious vomiting.  There are often many gas-filled bowel loops, but without air-fluid levels.  Air-fluid levels are thought not to occur because the meconium does not provide a good air-fluid interface.  There may be a "soapy" mass in the right lower quadrant.  A contrast enema will show a microcolon secondary to distal ileal obstruction.  Calcification may be seen on a plain radiograph if there has been meconium peritonitis.

Gastrograffin enemas may relieve the obstruction, but surgery may also be required.

The images to the left show a preterm infant who presented with marked abdominal distension at 3 days of life.  The top two images show his initial films.  Three is marked dilation of bowel loops.  No air is seen in the colon or rectum.

The lower two films showed progression to perforation, with overt free air under the diaphragm and a double air-contrast of bowel.  There is also free air in the scrotum, from tracking of air into the processus vaginalis.  At laparotomy, a meconium plug was found.

If the peritonitis occurs close to delivery, there may not be sufficient time to develop calcification, although there may be a significant chemical peritonitis.  Ascites may also be present.  Some infants will require surgery for persistent bowel obstruction or for adhesions.

The underlying pathology is an absence of ganglion cells in the mesenteric plexus of the colon. There is essentially an obstruction at the point of aganglionosis as peristalsis cannot progress beyond this point. The point where aganglionosis begins is most commonly at the rectosigmoid junction but can be seen in some cases as proximal as the small bowel. Up to 10% of cases involve the entire colon. Some cases are complicated by enterocolitis with progressive dilation of the colon, stasis of bowel contents with bacterial overgrowth, and acute fluid loss into the intestine with diarrhoea and dehydration. This can be severe enough to cause anuria and even death.  The diagnosis can be made by a rectal biopsy showing a lack of ganglion cells in the myenteric plexus of the colon.

Plain radiographs may show dilated loops of small bowel with air fluid levels. Although a transition zone (where the agangliosis starts) may be seen in older children, it is not usually seen in neonates. The transition zone is normal in calibre but the proximal bowel is dilated. If enterocolitis is present, the colon will appear dilated and there may be pneumotosis.

Antenatally, there may be polyhydramnios detected.  An ultrasound may confirm this, and the stomach may appear small.  Following the delivery, the baby may have excessive secretions which pool in the proximal pouch.  The baby will choke, cough, or regurgitate if fed. Abdominal distension is common if there is a distal fistula, as air moves through the fistula into the stomach.  If the baby requires ventilation, this may be exacerbated by positive pressure ventilation and cause further respiratory deterioration.

The initial diagnosis of a proximal pouch is confirmed by an inability to place a gastric tube into the stomach.  The tube is seen in the upper oesophagus and the pouch may be outlined by air. The presence of air in the abdomen indicates the presence of a distal fistula. For distal pouches or H-type fistulae, the diagnosis can be more difficult.

An image of the anatomical findings at bronchoscopy is shown to the left.

Be aware that a traumatic oesophageal perforation (commonly after a difficult intubation or other instrumentation of the pharynx) can give a similar radiological appearance.

Other associated anomalies are common and the baby should have an echocardiogram.  The echo should also identify which side the aortic arch is on, as this impacts on the surgical approach.

See also the inguinal, scrotal and genital guideline.

Inguinal hernie arise from persistence of a patent processus vaginalis (a projection of the peritoneum which accompanies the testis as it descends into the scrotum).  In females, the projection of peritoneum accompanies the round ligament.  Hydrocoeles are commonly seen with inguinal herniae, but can be isolated.  There can be diagnostic confusion between an incarcerated inguinal hernia and a hydrocoele.

Link to the hernia surgical guideline to view a video demonstrating how to reduce an inguinal hernia.

The radiograph to the left demonstrates air contained within bowel which has herniated into the left scrotum.  There is no evidence of bowel obstruction.