The Journal of Emergency Medicine, Vol. -, No. -, pp. 1–6, 2013 Copyright Ó 2013 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$ - see front matter

http://dx.doi.org/10.1016/j.jemermed.2013.08.046

Clinical Communications: Pediatrics ACUTE GASTRIC VOLVULUS IN A SIX-YEAR-OLD: A CASE REPORT AND REVIEW OF THE LITERATURE Bourke W. Tillman, MD,* Neil H. Merritt, MD,†‡ Heather Emmerton-Coughlin, MD,‡ Shruti Mehrotra, MD,† Terry Zwiep, MD,‡ and Rodrick Lim, MD*† *Department of Medicine, †Department of Paediatrics, and ‡Department of Surgery, Schulich School of Medicine at Western University, Children’s Hospital at London Health Sciences Centre, London, Ontario, Canada Reprint Address: Rodrick Lim, MD, FRCPC, FAAP, Department of Paediatrics, Children’s Hospital at London Health Sciences Centre, 800 Commissioners Road East, London, ON N6C 2V5, Canada

, Abstract—Background: First described in the pediatric population in 1899 by Oltmann, pediatric gastric volvulus is a rare disease, but carries a high mortality rate. Due to vague signs and symptoms it can easily be mistaken for gastroenteritis or appendicitis, but unique radiographic findings can help illuminate the diagnosis. The pathophysiology of gastric volvulus is related to an abnormality in the attachment of at least one of the gastric ligaments, which can occur either primarily or secondarily. The abnormality in these ligaments allows the stomach to freely rotate, eventually causing an obstruction. We describe a unique case occurring in a 6-year-old with no pre-exiting medical conditions as well as the associated radiographic images. Objectives: Our aim is to discuss the presentation and management of a potentially lethal intra-abdominal process that mimics far more benign disease entities and to highlight the pertinent radiographic findings. Case Report: A previously healthy 6-year-old female presented to the emergency department in the middle of the night after sudden onset of vomiting and abdominal pain. On exam her heart rate was mildly elevated but all other vital signs were within normal limits. She was assessed with an abdominal x-ray and given ondansetron. After settling with her parents and having no further bouts of emesis she was sent home. She returned that afternoon febrile with increasing abdominal pain and emesis. Abdominal x-ray revealed a massively distended stomach and left diaphragmatic eventration. She underwent operative intervention and improved over the course of the following week. Conclusion: Acute gastric

volvulus presents a diagnostic challenge. In patients with vague abdominal complaints knowledge of the typical x-ray findings is essential in early identification and treatment. Ó 2013 Elsevier Inc. , Keywords—emergency medicine; pediatrics; radiology; surgery; gastric volvulus

INTRODUCTION Acute gastric volvulus is a rare yet potentially lethal condition. Clinically, it can mimic fairly benign conditions, such as viral gastroenteritis, but it can present with hallmark x-ray findings (1). Even with prompt diagnosis and surgical repair, this condition is estimated to carry a 65% overall mortality rate (2). Although a rare condition (282 acute cases recorded between 1929 and 2007), it is essential that emergency care providers are able to recognize the hallmark x-ray findings and accurately diagnose this condition (2). CASE REPORT A 6-year-old presented to a tertiary care pediatric emergency department (ED) with a 4-h history of vomiting and abdominal pain. The parents denied any prior history of fever or trauma.

RECEIVED: 7 December 2012; FINAL SUBMISSION RECEIVED: 7 July 2013; ACCEPTED: 15 August 2013 1

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B. W. Tillman et al.

Figure 1. Initial abdominal anteroposterior radiograph (arrow showing gastric distension).

The parents denied any significant past medical or surgical history. The patient was on no medication and had met all of her developmental milestones. On examination she had a mildly tender abdomen with a distended stomach. She was treated with 4 mg of oral ondansetron and underwent an abdominal series. The x-ray study showed a normal gas pattern with a distended gastric bubble (Figure 1). The patient promptly fell asleep after administration of the antiemetic and rested comfortably for the entire 4 h of observation in the department. She was discharged home to undergo an oral fluid challenge with parental supervision. Approximately 8½ h later, the patient returned to the ED. The parents stated that she was still unable to tolerate any oral intake. Furthermore, her triage vitals showed a heart rate of 194 beats/min and a temperature of 38.5 C. On abdominal examination, the patient was noted to be guarding, and a distended stomach was again palpated. After initiation of intravenous resuscitation, an ultrasound was ordered to rule out appendicitis. The staff radiologist reported that they were unable to visualize the appendix but were concerned that there may be free air. As such, Pediatric Surgery was consulted and a repeat abdominal x-ray study was ordered. The repeat x-ray study demonstrated a severely distended stomach and

Figure 2. Repeat abdominal anteroposterior radiograph showing severe gastric distension & left diaphragmatic eventration (arrow showing diaphragmatic eventration).

left diaphragmatic eventration with no free air (Figure 2). At this point, the patient was taken to the operating room for an emergent laparotomy. In the operating room, a nasogastric tube was placed to decompress the stomach, yielding dark bloody fluid. Immediately upon entering the abdominal cavity, bloody ascitic fluid, a foul odor, and a grossly distended stomach were encountered. The stomach was torted about the pylorus and the patient was recognized to have an organoaxial gastric volvulus. The stomach was grossly distended, and appeared densely ischemic. The fundus appeared dark, almost black, and there was extensive subserosal hemorrhage (Figure 3). There was no free air or perforation apparent. The stomach was hypermobile, with absence of the short gastric vessels to affix the fundus and greater curve up to the left upper quadrant, as well as deficient gastrocolic and gastrohepatic attachments. The volvulus was reduced and almost immediately the stomach began to pink up. Throughout the procedure the patient was remarkably metabolically and hemodynamically stable. This permitted a period of observation in the operating room to assess the viability of the stomach. After 30 min the

Acute Gastric Volvulus in a 6-Year-Old

Figure 3. Initial visual appearance of stomach.

stomach continued to improve in condition, with no part of the stomach appearing to have suffered full-thickness necrosis (Figure 4). This allowed the stomach to be preserved. The abdomen was copiously irrigated and all other abdominal viscera were inspected and appeared normal. The distal esophagus, pylorus, and proximal duodenum appeared completely normal. The stomach was pexed to the abdominal wall and the abdomen closed. Postoperatively, the patient was transferred to the pediatric critical care unit for observation. Nine days after surgery the patient was discharged home without further complication.

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tween 1929 and 2007, there have only been 581 recorded cases of pediatric gastric volvulus, with 252 being an acute presentation. Fifty-eight percent of these acute cases occurred in the first year of life and only 10% presented between the ages of 6 and 12 years (2). Although rare, this is a potentially fatal condition with an overall mortality rate of 65% (2). Gastric volvulus is defined as torsion of more than 180 of the stomach (4,5). This can occur in either the longitudinal axis, the mesenteroaxial axis, or around both axes (Figures 5–7) (1). In the normal abdomen, the stomach is able to expand and contract, yet is kept under adequate tension by the four gastric ligaments. If there is an abnormality in the length of one of these ligaments, or one of these ligaments is not present, the stomach is able to tort during its normal function or during times of distention (6). This leads to a gastric volvulus. Gastric volvuluses can be defined as primary or secondary as well as acute or chronic (Table 1). It is important to realize that when describing a volvulus as either primary or secondary, we are referring to the underlying pathophysiology, whereas the terms acute and chronic refer to the specific clinical presentation. Primary gastric volvuluses are related to an abnormality in the attachment of one of the four gastric ligaments, whereas secondary gastric volvuluses can be related to a preexisting condition, which interferes with either the attachments of the gastric ligaments, or normal gastric function (7). Common secondary causes include congenital

DISCUSSION First described in the pediatric population in 1899 by Oltmann, pediatric gastric volvulus is a rare disease (3). Be-

Figure 4. Appearance of stomach 1 h post reduction.

Figure 5. The stomach can rotate around the longitudinal axis, the mesenteroaxial axis, or around both axes.

Surgery

Associated conditions

Management

Conservative management; prokinetics, antisecretories, prone position

Depends on clinical presentation; acute vs. chronic

Congenital diaphragmatic hernia, paraesophageal hernia, wandering spleen, Down Syndrome Depends on clinical presentation; acute vs. chronic None

Can be either acute or chronic Usually organoaxial

Recurrent abdominal pain, chronic vomiting, gastric distension, failure to thrive, recurrent chest infections Depends on underlying cause

Chronic Acute

Nonbilious emesis, epigastric pain, abdominal distension, respiratory distress, cardiovascular collapse Depends on underlying cause Presentation

Figure 7. Mesenteroaxial gastric volvulus.

Table 1. Comparison of Acute vs. Chronic and Primary vs. Secondary Gastric Volvulus

diaphragmatic hernia, paraesophageal hernia, and wandering spleen (4). The axis of the gastric volvulus seems linked to the underlying pathology, with most mesenteroaxial volvuluses occurring in secondary gastric volvuluses, whereas organoaxial volvuluses (as in our patient) are more common in primary cases (4). The differentiation between acute and chronic gastric volvulus is important, as the presentation, implications, and management are quite often different. In acute presentations, gastric volvulus is life threatening and requires emergent surgical intervention, whereas chronic gastric volvulus may be amenable to conservative management (2,6,7). In the case of chronic gastric volvulus, patients tend to present with recurrent nonspecific symptoms including recurrent abdominal pain, vomiting, gastric distension, failure to thrive, and recurrent chest infections (4,5). These symptoms eventually lead to upper gastrointestinal imaging, which in turn help reveal the diagnosis of chronic gastric volvulus.

Primary

Figure 6. Organoaxial gastric volvulus or ‘‘upside-down’’ stomach.

Can be either acute or chronic Usually mesenteroaxial

B. W. Tillman et al.

Secondary

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Acute Gastric Volvulus in a 6-Year-Old

The presentation of an acute gastric volvulus is related to the degree of rotation, the subsequent gastric obstruction, and the ability of the stomach to spontaneously detort. Symptoms include sudden-onset persistent, nonbilious emesis, epigastric pain, and abdominal distension. Borchardt’s triad—epigastric swelling, retching, and inability to pass a nasogastric tube—is commonly referred to when considering the diagnosis of gastric volvulus, yet one case series reported that this triad was present in only 50% of patients with an acute gastric volvulus (8). Other accompanying symptoms include respiratory distress and cardiac collapse secondary to increased intrathoracic pressure (7–9). Due to the variable and nonspecific presentation of gastric volvulus, a history of conditions with diaphragmatic abnormality should raise suspicion as well as a history of Down syndrome due to the associated increased incidence of diaphragmatic abnormalities. Not only do these conditions increase the likelihood of a secondary gastric volvulus, they can also delay diagnosis if gastric volvulus is not considered (4,7). Some studies have also reported a link between the degree of illness and the direction of rotation (6). It has been proposed that, due to the fact that in mesenteroaxial volvuluses there is close approximation of the gastroesophageal junction and the pylorus, the stomach is only fixed on a narrow pedicle that leads to an increased risk of obstruction and ischemia (Figure 7) (6). Conversely, in longitudinal volvulus, the gastroesophageal junction and pylorus maintain their normal anatomical location, decreasing the risk of ischemia (Figure 6) (6). Although this makes physiological sense, there have been reported cases of ischemia in longitudinal volvuluses, including our own case, and definitive management should not be delayed to delineate the axis of torsion. Gastric volvuluses can have distinct radiographic features, making plain radiographs of the chest and abdomen very useful. Common findings highly suggestive of a gastric volvulus include a distended stomach, eventrated left diaphragm, and a paucity of distal bowel (6,9). The cause of these distinct radiographic findings is related to the direction in which the gastric volvulus has rotated. In a longitudinal volvulus, also known as an organoaxial volvulus, the greater curvature of the stomach rests above the lesser curvature (Figure 6). This results in the appearance of a left diaphragmatic eventration on plain radiograph and an upside down stomach, although the upside down stomach is more easily seen during a barium swallow (2). In a mesenteroaxial volvulus, the antrum and pylorus of the stomach rotate anteriorly and superior to the gastroesophageal junction (Figure 7). This again results in an eventration of the left hemidiaphragm without the upside-down stomach appearance (1).

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Plain radiography is not the only method of diagnosis, and in fact, upper gastrointestinal contrast study is considered the diagnostic study of choice, as well as the primary method of detection of volvulus below the diaphragm (5,6). In one study it was able to demonstrate the type of volvulus in all patients in whom it was performed, as well as confirm the presence of gastric outlet obstruction (6). Of course, due to the need for urgent intervention in acute gastric volvulus, obtaining an upper gastrointestinal contrast study may not always be practical (2). Current treatment for acute gastric volvulus is immediate surgical consultation and intervention (5). This includes decompression of the stomach, restoration of normal orientation, treatment of any predisposing factors, and anterior and fundal gastropexy with or without gastrostomy (5,8). In contrast, chronic gastric volvulus is treated with prokinetics and antisecretory medication as well as other conservative measures, including sleeping in the prone position with the head up, and surgery is reserved only for the more persistent or severe cases of chronic gastric volvulus (5). CONCLUSION Our patient presented with sudden-onset epigastric pain and vomiting, yet was settled with ondansetron, was above the age of 1 year, and had no previous history of diaphragmatic abnormalities. As such, the diagnosis of acute primary gastric volvulus was quite difficult to make on initial presentation. This only serves to highlight the diagnostic challenge posed by gastric volvulus, as well as the necessity in familiarizing one’s self with classic radiographic findings. In summary, acute primary gastric volvulus is a rare, yet life-threatening diagnosis that can mimic viral gastric illness, as such, it is important to maintain a high degree of suspicion. It most commonly occurs in children under the age of 1 year, but can occur at any age. Although Borchardt’s triad is described as the classic presentation, only about 50% of pediatric patients present with all three symptoms. Therefore, when the classic signs of gastric volvulus are present on abdominal radiography, gastric distention with or without left diaphragmatic eventration, and the appearance of an upside-down stomach, early surgical consultation is essential. Acknowledgment—The authors extend a special thank you to Benjamin Tillmann for the illustration of Figures 5–7.

REFERENCES 1. Cole BC, Dickinson SJ. Acute volvulus of the stomach in infants and children. Surgery 1971;70:707–17.

6 2. Cribbs RK, Gow KW, Wulkan ML. Gastric volvulus in infants and children. Pediatrics 2008;122:e752–62. 3. Manikoth P, Nair P, Zachariah N, Sajwani M. Neonatal acute gastric volvulus. Arch Dis Child Fetal Neonatal Ed 2004;89:F388–9. 4. Darani A, Mendoza-Sagaon M, Reinberg O. Gastric volvulus in children. J Pediatr Surg 2005;40:855–8. 5. Porcaro F, Mattioli G, Romano C. Pediatric gastric volvulus: diagnostic and clinical approach. Case Rep Gastroenterol 2013; 7:63–8.

B. W. Tillman et al. 6. Oh SK, Han BK, Levin TL, Murphy R, Blitman NM, Ramos C. Gastric volvulus in children: the twists and turns of an unusual entity. Pediatr Radiol 2007;38:297–304. 7. Gerstle JT, Chiu P, Emil S. Gastric volvulus in children: lessons learned from delayed diagnosis. Semin Pediatr Surg 2009;18:98–103. 8. Miraz B, Ijaz L, Sheikh A. Gastric volvulus in children: our experience. Indian J Gastroenterol 2012;31:258–62. 9. Chattopadyhyay A. Neonatal gastric volvulus: another case of ‘‘mucousy baby’’ with gasless abdomen. Ind J Pediatr 2010;77:691–2.

Acute gastric volvulus in a six-year-old: a case report and review of the literature.

First described in the pediatric population in 1899 by Oltmann, pediatric gastric volvulus is a rare disease, but carries a high mortality rate. Due t...
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