Clinical History
A 4-month-old baby girl presented with intractable vomiting and dyspnoea for 2 days. Her birth and medical history was unremarkable. Urgent babygram followed by water soluble contrast meal were performed.
Imaging Findings
Supine chest radiograph (Figure 1) showed a distended gas-filled structure in the left thoracic cavity (black arrow indicating the herniated gastric wall), displacing the mediastinum to the right. Water-soluble contrast meal (Figure 2, Right lateral projection only; frontal film not shown) confirmed a massively distended stomach extending into the chest. The greater curvature was located superiorly (white triangles), and the small beak of contrast (grey arrow) suggested a point of twist. No contrast filling of the pylorus or duodenum was demonstrated despite changing of body positions, suggestive of complete obstruction.
Emergency laparotomy confirmed organoaxial gastric volvulus complicating a congenital left diaphragmatic hernia (CDH). The diaphragmatic hernia was manually reduced and repaired followed by gastropexy.
Discussion
Background
Acute gastric volvulus is extremely rare in the paediatric population. The majority of reported cases present at less than 1-year of age with left-sided congenital diaphragmatic hernia (CDH). A CDH can predispose to gastric volvulus because of abnormal elongation or absence gastrophrenic and gastrosplenic ligaments which are connected to the left diaphragm [1]. Gastric volvulus is classified as organoaxial, mesenteroaxial, or combined. In organoaxial volvulus, the axis of stomach rotation occurs along a line joining the esophagogastric junction and pylorus. In mesenteroaxial volvulus, the stomach rotates on a line passing through greater and lesser curvatures [1, 2].
Clinical Perspective
The rotated stomach is prone to ischemia, which is potentially life-threatening (mortality can reach 80%) [3]. A high level of suspicion should be raised in infants presenting with distressing abdominal pain/distension, intractable vomiting, or inability to pass a nasogastric tube (i.e. the Bordchart’s triad) [3].
Imaging Perspective
Abnormal gaseous lucency in the left lung +/- abnormal position of the nasogastric tube on radiograph suggests the diagnosis, alongside with double gastric bubble or a distended stomach despite nasogastric tube decompression +/- lying in a horizontal plane [4]. The diagnosis can then be confirmed by a prompt water-soluble-contrast upper gastrointestinal study (findings described in Figure 2). Contrast CT may be helpful to delineate anatomical relationship and look for complications (e.g. gastric strangulation, perforation, peritonitis, shock) but this should not delay prompt operative treatment [1]. MRI has a limited role due to potential delay of treatment.
Outcome
The mainstay of treatment is emergency open or laparoscopic surgery with detorsion of the volvulus, reduction of the herniated content, closure of the diaphragmatic defect and fixation of the stomach to the anterior abdominal wall. Occasionally needle decompression of the stomach may be required to ease reduction of the herniated stomach [1, 5].
Take Home Message
In summary, this case illustrates a rare but classical presentation of acute gastric volvulus in congenital diaphragmatic hernia in an infant. A high level of suspicion, careful assessment of the initial chest radiograph and a prompt water-soluble upper GI study are crucial for timely diagnosis.
Differential Diagnosis List
Organoaxial gastric volvulus complicating a congenital left diaphragmatic hernia
Congenital pulmonary airway malformation
Small bowel or gastric hernia without volvulus
Final Diagnosis
Organoaxial gastric volvulus complicating a congenital left diaphragmatic hernia
