Acute gastric volvulus: Late-onset ischemic consequences and their management.

We report three infants who presented with acute gastric volvulus and recovered initially after de-torsion, but later presented with sequelae due to ischemia of gastroesophageal junction, stomach and gastroduodenal junction. The first two infants could not be fed orally or by gastrostomy tube because of microgastria and stricture of the lower esophagus and gastroduodenal junction, and were managed on jejunostomy feeds, while the third child was managed on gastrostomy feeds till the gastric substitution surgery. The first case was treated nonsurgically with repeated dilatations, but ultimately succumbed to sepsis and malnutrition. In the second child, attempted dilatation resulted in esophageal perforation and she was reconstructed using ileocecal segment as a substitute for stomach and lower esophagus, and has done well. The third child was managed surgically by the Hunt Lawrence J pouch as stomach substitute and has also done well.

INTRODUCTION

Acute gastric volvulus in infants is known as a rare but life-threatening emergency that requires prompt recognition and treatment.[1] Delay in the diagnosis can result in gastric ischemia and perforation.[2] The aim of this report is to outline the management of late-onset ischemic consequences of acute gastric volvulus, which has so far not been described adequately in the literature.

CASE REPORTS

We describe three infants with acute gastric volvulus who, after initial recovery and discharge from the hospital, were readmitted after about 6 weeks with life-threatening ischemic sequelae in the form of inability to feed orally or via gastrostomy, drooling of saliva, aspiration pneumonia and malnutrition. Investigations performed at this time revealed microgastria with stricture at gastroesophageal and gastroduodenal junction. While the first child died as the parents refused to give consent for further surgery, the other two infants underwent successful substitution of the stomach, lower esophagus and gastroduodenal junction, one with the traditional Hunt Lawrence pouch and the other with an ileocecal segment, with the use of appendix as a feeding stoma.

Case 1

A 1-year-old girl with Crouzon's syndrome presented with acute-onset nonbilious vomiting and incessant cry and after radiological studies was diagnosed as gastric volvulus with eventration of left hemidiaphragm [Figure 1a]. At laparotomy, there was an organoaxial volvulus with partial necrosis of the stomach. De-torsion of stomach, with resection of the necrotic part of the stomach and repair of eventration diaphragm was performed. The child recovered well and was discharged on oral feeds. One month later, the child was reoperated for adhesive intestinal obstruction with gangrene of ileum, and recovered well. Three months later, the child presented with feed intolerance, drooling of saliva and leakage of feeds through gastrostomy. Barium study at this time revealed gastroesophageal junction stenosis with proximal dilatation [Figure 2a]. Because feeding via gastrostomy was not tolerated, a tube was passed through the pylorus into the duodenum for feeding. Multiple esophageal dilatations were carried out [Figure 3a]. Finally, gastric substitution was planned, but the parents refused any further surgery and the child expired due to malnutrition and sepsis.

Figure 1

Plain radiograph abdomen showing gastric volvulus in case 1 (a), case 2 (b) and case 3 (c)

Figure 2

Barium swallow showing distal esophageal stenosis in case 1 (a), case 2 (b) and case 3 (c)

Figure 3

(a) Postesophageal dilatation barium swallow of case 1, (b) Postoperative barium meal follow-through of case 2, showing smooth transit of barium from the esophagus to the small intestine

Case 2

A 1-month-old girl presented with history of nonbilious vomiting and upper abdominal distension and early shock. After resuscitation, radiological studies done revealed gastric volvulus [Figure 1b]. At laparotomy, there was mesentericoaxial type of gastric volvulus with partial necrosis of the stomach and ischemic-looking gastroesophageal junction.

De-tortion with excision of necrotic-looking stomach along with a gastrostomy as well as feeding jejunostomy was performed. As the child was tolerating gastrostomy feeds, the jejunostomy tube was pulled out and she was discharged on gastrostomy feeds. One month later, the child presented with drooling of saliva, feed intolerance and severe aspiration pneumonia. Barium study revealed stenosis of distal esophagus with microgastria and gastroduodenal stenosis [Figure 2b]. Attempted esophageal dilatation resulted in perforation and, hence, the child was managed by jejunostomy feeds via an 8F infant feeding tube passed transduodenally along with frequent esophageal suctioning via a nasogastric tube and was then taken for gastric substitution surgery when adequately stabilized. Laparotomy revealed strictured lower esophagus with very small fibrotic stomach and gastroduodenal stenosis. The strictured lower esophagus and fibrotic stomach was resected and the gastroduodenal region laid open. Ileocecal segment along with appendix was isolated with its mesentery and an ileocecal valve was augmented by intussuscepting the ileum into the cecum. The ileal end was anastomosed to the esophagus and cecum was anastomosed to the laid open gastroduodenal region. The appendix was brought out as feeding stoma, and feeds were started through the stoma. The child was managed on total parenteral nutrition; however, 1 month later, the child developed burst abdomen that was sutured. Although the diaphragmatic eventration was not obvious in the initial radiographs as well as during surgery, it was well delineated in later X-rays and was repaired in another sitting.

Subsequent barium study revealed free flow of barium into the small bowel [Figure 3b], and the child was started on oral feeds. The child slowly gained weight from 2.9 kg at first surgery to 4.3 kg, and was discharged after a total hospital stay of 6 months. Currently, the child is 7.2 kg weight at 15 months of age, on complete oral diet with vitamin B12 supplements and without any dysphagia or reflux. The appendicular stoma is asymptomatic, has no discharge of feeds through it and is due for closure shortly.

Case 3

This was a 3-month-old girl whose parents refused surgery for left diaphragmatic hernia at an earlier age, and who was subsequently brought for sudden-onset abdominal distension and vomiting [Figure 1c]. At laparotomy, mesenterico axial type of gastric volvulus was corrected and Bochdalek's hernia was repaired. The stomach initially appeared dusky, but regained color and was hence repositioned and the child was discharged on oral feeds. The child developed progressive dysphagia and intolerance of oral feeds. Barium contrast study revealed distended lower esophagus with stricture and no contrast entering the stomach [Figure 2c]. An endoscopy performed did not allow even a guide wire. Laparotomy revealed dense adhesions and small receptacle of stomach, opening into the duodenum, and feeding gastrostomy was carried out. Definitive repair was done after 8 months (weight – 7 kg), and it revealed a blind-ending esophagus and a distal remnant of stomach, with no communication between them. A Hunt Lawrence jejunal pouch was created and anastomosed to the lower esophagus. Contrast study revealed good flow of contrast into the jejunal pouch with no leak or reflux. The child was discharged on oral feeds and had a weight gain of 600 g in 2 months. The child is tolerating the oral diet and does not have any dysphagia or reflux.

DISCUSSION

Gastric volvulus in infants, children and adolescents is a rare event.[34] All three infants in our series also had partial gangrene of the stomach, indicating initial severe ischemic insult, as noted in the earlier literature also.[56] Late-onset ischemic consequences following acute gastric volvulus is not sufficiently described in the literature. Only one case of successful treatment of acute esophageal necrosis caused by gastric volvulus in a 79-year-old woman has been described so far.[7] In the pediatric age group, only one case of early-onset gastric outlet obstruction requiring gastroduodenostomy is described[3] and another case of gangrene of whole stomach requiring gastrectomy is reported.[8]

All the three infants in our series initially presented with acute gastric volvulus with severe ischemia of stomach, but the decision to remove the entire stomach was not taken as it was thought to be viable; hence, only the necrotic part was resected. Although the initial recovery was satisfactory, indicating that the stomach had enough blood supply to survive, all the infants presented after about 6 weeks, with feed intolerance via gastrostomy, drooling of saliva and aspiration pneumonia and failure to thrive. This happens due to severe ischemic insult during the initial acute volvulus phase, which later forms stenosis or stricture of the lower esophagus, stomach and the gastroduodenal junction. Consideration of jejunostomy during the initial de-tortion surgery is a good option, as maintaining adequate nutrition via gastrostomy becomes difficult due to the late-onset ischemic consequences causing gastroesophageal and gastroduodenal stenosis. After adequate stabilization of these children on jejunostomy feeds, they should be considered for definitive surgery in the form of substitution of lower esophagus and stomach.

The advantages of using ileocecal segment for gastroesophageal substitution in infants are – it can be easily isolated with its mesentery, the caliber of ileum matches with the lower esophagus and cecum forms a good gastric reservoir, ileocecal valve prevents regurgitation of bile into the lower esophagus and appendix can be used as a temporary feeding stoma.

In conclusion, we highlight the occurrence of late-onset esophago-gastro-duodenal stricture in gastric volvulus, even though the initial recovery was seen after de-torsion and necrosectomy. While feeding jejunostomy can be a very useful temporizing procedure, given the high morbidity and mortality of this uncommon disorder, and based on the outcome of our cases, we believe that gastric substitution using either the ileocecal segment or jejunal pouch is the definitive surgery.