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Diaphragmatic Eventration with Acute Gastric , Pancreatic Volvulus and Wandering Spleen Mimicking Features of Gastric Outlet Obstruction in an Young Male Patient Presenting as Acute Abdomen: A Case Report

Authors Kumar Rakesh*1, Kajla R.K2., Dhukiya Ramniwas3, Gothwal Sitaram4, Ali MD Mokaram5 1Resident, Department of , Sardar Patel Medical College Bikaner 2Associate Professor, Department of General Surgery, Sardar Patel Medical College Bikaner 3Senior Resident, Department of General Surgery, Sardar Patel Medical College Bikaner 4Professor and HOD, Department of General Surgery, Sardar Patel Medical College Bikaner 5Resident, Department of General Surgery, Sardar Patel Medical College Bikaner *Corresponding Author Rakesh Kumar Resident, Department of General Surgery, Sardar Patel Medical College Bikaner Email: [email protected]

Abstract Diaphragmatic eventration is a rare abnormality of diaphragm which may be congenital or acquired. It can be completely asymptomatic when it is present as an isolated anomaly. Such patients may also have associated laxity, weakness or absence of intraperitoneal visceral ligaments due to a shared pathogenesis. This predisposes to rotational anomalies of other viscera like gastric volvulus, wandering spleen with or without torsion, pancreatic volvulus contributed by ligament laxity as well as diaphragm weakness. We present a case of a 16 year old male adolescent having rare and unusual combination of diaphragmatic eventration, mesentericoaxialgastric volvulus, wandering spleen and pancreatic volvulus complicated by acute . Key words: Diaphragm eventration; Gastric volvulus; Pancreatic volvulus; Wandering spleen.

Introduction scope of movement due to diaphragmatic The eventration of diaphragm is a rare congenital eventration predisposes to various rotational or acquiredabnormality of diaphragm which is anomalies of gut like gastric volvulus, wandering rarely significant per se.(1)However, it may be spleen with or without torsion/infarction and associated with weakness, absence or laxity of pancreatic volvulus which may lead to intraperitoneal ligaments which hold the viscera in pancreatitis. The following case report presents a position.(2)The combination of above two factors, case of 16 year old male adolescent having rare i.e. ligament laxity accompanied by increased and unusual combination of diaphragmatic

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eventration, mesentericoaxial gastric volvulus, patient underwent another spot radiograph and wandering spleen and pancreatic volvulus Contrast Enhanced CT of abdomen. The complicated by . radiograph confirmed the low position of GE junction and also showed two air fluid levels Case Report within the . A 16 year old male was referred to emergency The CT scan revealed Distended contrast filled department with complaints of epigastric pain of stomach with reversal of relationship between GE acute onset, abdominal distension andsevere junction and characteristic of associated with after 10-15 Mesentericoaxial type gastric volvulus. The minutes of oral intake since 24 hours. There was pylorus was situatedantero-superior to GE no significant past history. General physical junction (Figures 3). Eventration of left dome of examination revealed pallor, dehydration, diaphragm was noted with interposition of splenic tachycardia and tachypnea. Respiratory flexure between dome and gastric fundus. Note examination showed decreased left basal air entry. was also made of superiorly displaced left Abdominal examination revealed asymmetric kidney(fig 4 ). The spleen was enlarged and abdominal distension (more on left side) with atypical in position lying in midline anteriorly tenderness in epigastrium. Fluid resuscitation was suggestive of Wandering spleen due to weak started with a presumptive diagnosis of gastric ligamentous support. However, spleen showed outlet obstruction. Repeated attempts to insert a normal perfusion pattern with no evidence of nasogastric tube failed. On ultrasound the stomach ischaemia/infarction. Unusual orientation of was massively distended filled with fluid pancreatic body and tail, increased bulk, contents,spleen was in atypical position lying peripancreatic fluid pockets and evidence of cystic anteriorly in midline in epigastrium and left fluid collection at the tail suggested pancreatic kidney was pushed superiorly. The left dome of volvulus complicated by pancreatitis which is a diaphragm was abnormally high in position. There rarely associated with gastric volvulus . The distal was mild ascites. part of pancreatic body and tail were twisted and Spot abdominal frontal and left lateral radiographs stuck between spleen and stomach. There was were taken. The AP and left lateral abdominal mild ascites and displacement of jejunal loops radiographs revealed significantly over distended towards left side of abdomen was also noted. stomach with high placed but intact left dome of However, no evidence of any other rotational diaphragm, suggesting event ration. There was anomaly of gut was noted.The patient was another gas shadow between left dome of immediately taken for laparotomy in emergency diaphragm and gastric fundus (which was later operation theatre. Abdomen opened midline and found out to be due to splenic flexure of colon on stomach was rotated around its mesenteric axis CT scan).The distal bowel gases were scanty. with pylorus lying higher and anterior to There was no evidence of . gastroesophageal junction. The stomach walls After another failed attempt for gastric intubation, were edematous, however there was no evidence patient was given oral contrast for delineation of of gangrene. The spleen was lying freely in upper GI tract (Figure 2).The Gastroesophageal epigastium anteriorly due to lack of ligamentous junction was significantly lower in position and attachments. Pancreas shows some necrosed area the duodenal gas was seen at a higher level than at tail due to entrapment in between stomach and the GE junction which suggested high possibility wandering spleen. The stomach was of mesenterico-axial type of gastric volvulus.After decompresssed and the volvulus was reduced, a successful intubation attempt and partial anterior gastropexy and splenopexy were done to drainage of gastric contents (about 1000 mL), prevent recurrence. Eventration of diaphragm

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repaired with prolene no.1 suture. and pancreatic appearance suggestive of site of volvulus. The collection was drained. The surgery was otherwise duodenal gas is higher (yellow arrow). These uneventful. Postoperative period was with fair findings highly suggested mesentericoaxial gastric outcome and patient was discharge on volvulus. postoprtaive day 9.Patient had no complaints in his followup period.

Figure 3 Mesentericoaxial gastric volvulus.Axial series of images craniocaudally show edematous lower osophageal walls (yellow arrow) with nasogastric tube in situ. The is Figure 1 Frontal erect radiograph before extending beyond the pylorus (red arrow) so that successful intubation shows significantly the GE junction (white arrow) is located distended stomach with high placed and thin left posteroinferior to pylorus. dome of diaphragm (down arrow) suggesting eventration, fundal gas (up arrow), duodenal gas (horizontal arrow) and scanty bowel gas (solid arrows). There is another gas shadow between fundus and dome which was due to splenic flexure, later revealed on CT scan.

Figure 4 Coronal section showing a loop of splenic flexure interposed between gastric fundus and left dome (red arrow). The left kidney is also displaced superiorly.

Figure 2 The upper GI contrast study Discussion showingsignificantly lower position of gastro- Eventration of diaphragm is an abnormal elevation esophageal junction (Red arrow) with Bird Beak of part or whole of the intact hemidiaphragm. In

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contrast to diaphragmatic , integrity of abnormally in abdomen, pelvis or even thoracic diaphragm is maintained. It usually occurs as an cavity (when associated with diaphragm-matic isolated entity. Majority of cases are congenital hernia/eventration). This happens when dorsal and rarely acquired.(1) It is most often detected mesogastrium fails to fuse with posterior incidentally on chest radiographs and frequently during fetal development. As a result, asymptomatic.(3) It is due to anatomical weakness there is an increased risk of torsion of splenic or functional deficiency of diaphragmatic pedicle, which in turn leads to splenic musculature which can affect some part or whole and infarction. Splenic torsion is a dreaded life of the musculature. It is more commonly left sided threatening complication of wandering spleen with characteristically marked mediastinal which requires immediate surgical attention. displacement to the right, a feature rarely seen Either splenectomy or splenopexy are performed with paralysis of the diaphragm.(4).Congenital depending upon viability of splenic tissue. eventration is due to failure of proper The term volvulus is derived from the Latin word muscularisation of the diaphragm, wherein a part volvere, meaning to turn or roll. Gastric volvulus of the muscle is replaced by a layer of fibro fatty is a closed loop type of obstruction which occurs connective tissue and few scattered muscle fibres. due to rotation one part of stomach over another The incidence in adults is about 1 in 10000.(5) The for more than 180°. It was first described by Berti incidence is higher than what is encountered in 1866. Three types of volvulus have been clinically because most of the cases are described by Singleton, which include asymptomatic and hence go unnoticed. Acquired organoaxial, mesenteroaxial, and combination- variety is mostly attributed to phrenic nerve injury unclassified (Figure 11).(6) Organoaxial type during birth process.The key radiological finding involves rotation of stomach around the is abnormal elevation of a part of or whole longitudinal axis with the greater curvature most hemidiaphragm with reduced/absent/paradoxical often rotating anteriorly. movement and bulging of abdominal contents into The mesenteroaxial volvulus occurs due to thoracic cavity which can be demonstrated on rotation around shorter transgastric axis, a line plain radiographs, fluoroscopy, ultrasound or CT. joining the middle of lesser and greater curvature. The contour of diaphragm may or may not be The mesenteroaxial volvulus usually occurs preserved. The diaphragm may be thinned, without concomitant , with however, the integrity of diaphragm is always gastrosplenic ligament laxity being the maintained which differentiates it from predisposing factor. The stomach becomes diaphragmatic hernia. Left sided diaphragmatic overdistended. There is reversal in the orientation eventration can be associated with abnormally of pyloroduodenum and GE junction with respect positioned spleen and can be a cause of acute to each other, i.e. the pylorus lies at a higher level abdomen in children.(3)Wandering spleen than GE junction, which is demonstrable with the associated with gastric volvulus has been reported help of upper GI contrast studies or contrast only 4 times earlier in the literature.(3)Wandering enhanced CT of abdomen. The volvulus leads to spleen is a rare condition characterized by the obstruction at GE junction and pylorus, leading to absence or underdevelopment of one or all of the symptoms of gastric outlet obstruction with ligaments that hold the spleen in its normal inability or difficulty to intubate the stomach position in the left upper quadrant of the abdomen. which should strongly suggest the possibility of These ligaments include the pancreaticocolic, gastric volvulus. The classically described splenocolic, gastrosplenic, pancreaticosplenic, Borchardt’s triad of gastric volvulus includes: 1) phrenicocolic, splenorenal, and phrenicosplenic acute severe epigastric pain with distention; 2) ligaments. As a result, spleen is located vomiting followed by violent, intractable,

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nonproductive retching; and 3) difficulty or be vigilant about other associated abnormalities inability to pass a nasogastric tube into the such as wandering spleen so that all can be fixed stomach.(7) during a single laparotomy to prevent Gastric volvulus is an acute surgical emergency occurrence/recurrence in future. Prophylactic which can lead to gastric strangulation, infarction splenopexy in a patient with gastric volvulus and or perforation and nonoperative mortality rate as vice versa is desirable. high as 80%.(8)Open laparotomy with derotation and anterior gastropexy is the treatment of choice. References Pancreatic volvulus is a rare entity, with only a 1. Lawler RH, West J, Brosnan J.Eventration few isolated cases described in conjunction with of diaphragm. Am J Surg.1948;75(4):624- wandering spleen.[2]It occurs because of 7. entrapment of the tail in the twisted splenorenal 2. Gorsi U,Bhatia A,Gupta R, Bharathi S, ligament. On imaging, the pancreatic tail appears Khandelwal N. Pancreatic Volvulus with entrapped between wandered spleen and stomach. Wandering Spleen and Gastric Volvulus: Pancreatitis can occur as a complication, most An Unusual Triad for Acute Abdomen in a likely due to ischemia caused by stretching of its Surgical Emergency. Saudi J vascular pedicle.(2) In our case, the severe Gastroenterol. 2014;20(3):195–8. epigastric pain was in part contributed by 3. Kulkarni ML, Sneharoopa B, Vani HN, pancreatitis. Nawaz S, Kannan B, Kulkarni So, after a brief review of each entity, we see that PM.Eventration of diaphragm and its each disease process by itself is rare, and the associations. Indian J Pediatr. occurrence of all four simultaneously makes this 2007;74(2):202-5. case unique. Although two or three of the above 4. David Sutton, ed. Textbook of Radiology entities occurring simultaneously in a patient have and Imaging, 7th edition, Churchill been described and reported in literature, this is Livingstone, London 2003; 52. probably the first reported case where all four 5. Irene MI, Booker PD. Congenital occurred simultaneously. Though very unlikely, it Diaphragmatic hernia and eventration of is possible due to a common shared pathological the diaphragm. In Lister J. Irene MI, eds. process which leads to diaphragmatic eventration Neonatal Surgery, 3rd edition. London; as well as weakening, laxity or absence of Butterworth. 1990; 214. intraperitoneal supportive visceral ligaments 6. Singleton AC. Chronic gastric volvulus. Radiology. 1940;34:53-61. Conclusion 7. Sevcik WE, Steiner IP.Acute gastric Gastric volvulus should always be suspected in a volvulus: case report and review of the patient with symptoms of acute gastric outlet literature.CJEM. 1999;1(3):200-3. obstruction, especially when there is difficulty in 8. Palanivelu C, Rangarajan M, Shetty AR, negotiating the nasogastric tube. The prompt Senthilkumar R. Laparoscopic suture diagnosis can be life saving for the patient. The gastropexy for gastric volvulus: a report of diagnosis is not very difficult on imaging if there 14 cases. SurgEndosc. 2007;21(6):863-6. is strong clinical suspicion. Diaphragmatic hernia or eventration give an important clue. Most helpful investigations are upper GI contrast studies and contrast enhanced CT scan of abdomen. The radiologist and surgeon should also

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