|Year : 2014 | Volume
| Issue : 1 | Page : 35-37
Recurrent jejunal perforation in a patient with Zollinger Ellison syndrome
Jagadamba Sharan1, Vijai Prakash Srivastava1, Deepika Verma2, Osman Musa3
1 Department of Surgery, Rohilkhand Medical College and Hospital, Bareilly, Uttar Pradesh, India
2 Department of Microbiology, Rohilkhand Medical College and Hospital, Bareilly, Uttar Pradesh, India
3 Department of Surgery, Era's Lucknow Medical College and Hospital, Lucknow, Uttar Pradesh, India
|Date of Web Publication||10-Mar-2014|
Department of Surgery, Rohilkhand Medical College and Hospital, Pilibhit by Pass, Bareilly, Uttar Pradesh - 243 006
Source of Support: None, Conflict of Interest: None
Zollinger Ellison syndrome (ZES) presenting with jejunal perforation is a very rare presentation with very few cases reported worldwide and none with recurrent jejunal perforation. A 40-year-old male came with perforation peritonitis with history of abdomen pain off and on, confirmed radiologically and exploratory laparotomy done. A small jejunal perforation (3 mm) was noted and primary repair was done. Oral pantoprazole was given and patient was discharged after 10 days. He was asked to come after 1 week for follow-up but he didn't turn up. After 6 weeks, the patient again presented with perforation peritonitis and profound shock. Resuscitation was done and on exploration, jejunal perforation was noted with no evidence of any tumor in the duodenum or, stomach wall or, the pancreas. There was no specific swelling in any of the lymph nodes. Biopsy was taken and primary repair was done. Retrospective analysis for this rare presentation was done which suggested ZES.
Keywords: Perforation peritonitis, Recurrent jejunal perforation, Zollinger Ellison Syndrome
|How to cite this article:|
Sharan J, Srivastava VP, Verma D, Musa O. Recurrent jejunal perforation in a patient with Zollinger Ellison syndrome. J NTR Univ Health Sci 2014;3:35-7
|How to cite this URL:|
Sharan J, Srivastava VP, Verma D, Musa O. Recurrent jejunal perforation in a patient with Zollinger Ellison syndrome. J NTR Univ Health Sci [serial online] 2014 [cited 2020 Apr 4];3:35-7. Available from: http://www.jdrntruhs.org/text.asp?2014/3/1/35/128428
| Introduction|| |
Perforation of the jejunum is uncommon. The etiology is often unknown; the possible causes leading to jejunal perforation are - trauma, foreign body, jejunal diverticulitis, steroid therapy, collagen disease associated necrotizing vasculitis, superior mesenteric arterial occlusion, and rarely malignancy.  Factors promoting this rare condition are - advanced age, arteriosclerosis, prolonged oral administration of chlorothiazide, oral potassium therapy.  Zollinger Ellison syndrome (ZES) presenting with jejunal perforation is a very rare presentation with very few cases reported worldwide and none with recurrent jejunal perforation.
| Case Report|| |
A 40-year-old male presented with abdomen pain, vomiting, abdominal distension, and not passing feces and flatus for 1 day with past history of abdomen pain off and on and no history of frequent or chronic diarrhea. Family history was not significant. Perforation peritonitis was suspected, confirmed radiologically and exploratory laparotomy was done. A small perforation (3 mm) was noted in the upper jejunum. There was no specific swelling in any of the lymph nodes. Primary repair of the perforation was done. Post operative period was uneventful, except for epigastric discomfort and heartburn. Oral pantoprazole was given and the patient discharged after 10 days and asked to come after 1 week for follow-up and further investigations but he didn't turn up. After 6 weeks, the patient again presented to the casualty with abdomen pain, vomiting, and not passing feces and flatus for 1 day. On examination, patient was found to be in profound shock and resuscitation was done. Intestinal perforation was suspected clinically, confirmed radiologically and exploratory laparotomy was done. A perforation was noted in the upper jejunum (5 mm) [Figure 1]. Entire bowel was found to be congested with no evidence of any other pathology. There was no specific swelling in any of the lymph nodes. No evidence of any tumor was found in the duodenum, stomach wall, or the pancreas. Biopsy was taken from the perforation site. Primary repair of the perforation was done as the patient was not fit for definitive surgery [Figure 2]. A retrospective analysis of the causes for this rare presentation was done. Fasting serum gastrin level was found to be very high-740 ng/L (normal <150 ng/L).  Basal acid output measurement was done keeping the patient off all acid secretory inhibitors for 3 days which was also very high-18 meq/hr (normal 2-5 meq/hr).  Blood sugar, liver function tests, renal function tests, serum calcium, serum parathyroid hormone (PTH), and prolactin were normal. No abnormality was detected in ultrasonography (USG) of whole abdomen. Upper gastrointestinal (GI) endoscopy showed antral gastritis with multiple small superficial ulcers in the duodenum. Magnetic resonance imaging (MRI) of whole abdomen detected no abnormality in the duodenum, stomach, pancreas, lymph nodes, or liver. Histopathological examination of the biopsy tissue suggested nonspecific inflammatory changes. Based on the above findings, a diagnosis of ZES was made. High dose of oral pantoprazole (80 mg/day)  was given. Patient was asked to come for follow-up at regular intervals. There was no recurrence of symptoms during the follow-up period of 3 years.
| Discussion|| |
ZES is a rare disease caused by a gastrin producing tumor (Gastrinoma). Gastrinoma is the second most common islet cell tumor.  Incidence is 1-3 per million.  Mean age is 43 years.  Men to women ratio is 1.3:1.  Multiple endocrine neoplasia type I (MEN I) syndrome is present in 20-25% of ZES patients.  The common site of the tumor is duodenum (50-70%), followed by (20-40%).  About 90% tumors are located in the Gastrinoma Triangle.  Rarely, mesentery, biliary tract, stomach, and ovary are involved. 
60-90% of gastrinomas are malignant with metastatic spread to lymph nodes and liver.  Clinical presentation of ZES includes abdominal pain (75%), diarrhea (73%), heartburn (44%), nausea (33%), vomiting (25%), and weight loss (17%).  Very rarely ZES presents as perforation prior to diagnosis. Markedly, elevated fasting serum gastrin level combined with gastric acid hypersecretion suggests ZES. ,, The secretin provocative test is done when the fasting serum gastrin level is moderately elevated and the diagnosis is in doubt.  The visualization rate for localization of gastrinomas in abdominal USG, computed tomography (CT), MRI, varies between 20-60%.  However, these are useful for detection of hepatic metastases and gastrinoma larger than 2 cm in diameter.  Besides these, upper GI endoscopy and endoscopic USG lead to better detection rates.  Newer tests such as selective arterial secretagogue injection test (SASI test) and somatostatin receptor scintigraphy (SRS) provide better localization of the tumor. , Intraoperative procedures such as intraoperative US (IOUS) for small pancreatic gastrinomas and intraoperative duodenoscopy for duodenal gastrinoma are also done. 
Irving Waxman et al., examined the characteristics of patients with ZES who developed a perforation prior to diagnosis.  Of 160 patients, perforation occurred prior to the diagnosis in 11 (7%). At surgery, perforations were found in the duodenum in six cases and in the jejunum in five. In none of the cases, tumor was identified at emergency surgery. The diagnosis of ZES was made only in the postoperative period. Features of perforation in ZES were not specific. Jejunal perforation or perforation associated with a history of diarrhea is suggestive of the diagnosis. Uesugi et al., reported ZES in a 42-year-old male who underwent emergency laparotomy due to a perforated jejunal ulcer and was found to have multiple perforations, jejunum was partially resected.  Patient required re-exploration after 2 days due to intra-abdominal hemorrhage, sub mucosal duodenal gastrinoma was suspected, and pancreatoduodenectomy was done. The patient could not survive. Histopathology confirmed malignant gastrinoma.
| Conclusions|| |
Jejunal perforation is rare. Recurrent jejunal perforation can be a very rare presentation of ZES, and shall be suspected in such cases. These patients shall be evaluated and investigated properly for ZES and managed accordingly.
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[Figure 1], [Figure 2]