J Gastrointest Surg DOI 10.1007/s11605-015-2827-y
Duodeno-jejunal Intussusception with Acute Pancreatitis in Peutz-Jeghers Syndrome: The First Case Presentation in Childhood Jiro Kimura 1,2 & Kiyoshi Sasaki 1 & Takehiro Okabayashi 2 & Yasuo Shima 2 & Jun Iwata 3 & Sojiro Morita 4
Received: 30 March 2015 / Accepted: 9 April 2015 # 2015 The Society for Surgery of the Alimentary Tract
Keywords Peutz-Jeghers syndrome . Duodeno-jejunal intussusception . Acute pancreatitis
Case Presentation Peutz-Jeghers syndrome (PJS) is a rare condition characterized by autosomal dominant inheritance, mucocutaneous pigmentation, and gastrointestinal hamartomatous polyps, and is a subtype of hereditary gastrointestinal polyposis.1 Among PJS, there is no difference of incidence among race and sex, and the incidence has been estimated to be 1 in 120,000–150, 000 births.1 The cause of PJS is a germline mutation of STK11/LKB1 (serine/threonine kinase 11) tumor suppressor gene in most patients (70–80 %).2 A 13-year-old girl suffered from left upper quadrant pain for 4 days and admitted into our hospital. There were no previous symptoms but she had family history of PJS. On physical examination, her lip was markedly pigment, but no abdominal tenderness was found. Laboratory parameters investigated that
* Takehiro Okabayashi [email protected]
Department of Pediatric Surgery, Kochi Health Sciences Center, Kochi, Japan
Department of Gastroenterological Surgery, Kochi Health Sciences Center, 2125-1 Ike, Kochi City, Kochi 781-8555, Japan
Department of Diagnostic Pathology, Kochi Health Sciences Center, Kochi, Japan
Department of Radiology, Kochi Health Sciences Center, Kochi, Japan
white blood cell count was 12,660/μL (normal range, 3800 to 10,100/μL) and serum amylase level was 615 IU/L (normal range, 60 to 200 IU/L). Other laboratory tests, including hematology, tumor markers (carcinoembryonic antigen and carbohydrate 19–9), and viral infection including hepatitis B status and C status, were normal. Computed tomography (CT) revealed a well-enhanced intraluminal mass with a maximum diameter of 4.0 cm in the second portion of the duodenum and was invaginated into the proximal jejunum (Fig. 1a). Furthermore, enhanced-contrast CT demonstrated that peri-pancreatic inflammation around the pancreatic tail results in hazy and/or reticular stranding of the surrounding fat, although a part of the pancreatic head and body enhanced (Fig. 1b). At upper gastrointestinal (GI) endoscopy, brittle mass where the duodenal lumen was occupied was noted in the second portion of the duodenum, and it was not possible to observe the papilla of the Vater (Fig. 1c); therefore, it was difficult to remove the polyp endoscopically. Upper GI series indicated that the polyp was located through the duodenal bulb to descending portion and confirmed that the large solitary mass was invaginated (Fig. 1d). Oral intake of even low fat meal induced high fever, vomiting, and abdominal pain several times. Clinical symptoms also presented that both duodenal obstruction and acute pancreatitis due to the large polyp of the duodenum were repeated. Since the onset of acute pancreatitis, 3 months had past. We chose a surgical treatment for duodeno-jejunal intussusception with acute pancreatitis in Peutz-Jeghers syndrome child. On a gloss appearance at the operation, the delle on the anterior wall of the duodenal bulb was recognized, and at first Hutchinson’s maneuver was performed (Fig. 2a). We intraoperatively judged that the polyp might be removed without hurting the papilla of the Vater (Fig. 2b). The histopathological examination revealed tree branching pattern caused by the
J Gastrointest Surg Fig. 1 Abdominal contrastenhanced CT showed that a duodenal mass (arrows) was invaginated (arrow head) into the proximal jejunum (a). Rise in fat density was shown around the body and tail of the pancreas (b). At upper GI endoscopy, brittle mass where the duodenal lumen was occupied was noted in the second portion of the duodenum, and it was not possible to observe the papilla of the Vater (c). Upper GI series were performed to reconfirm location of the polyp. It showed that the polyp was located on the duodenal bulb or descending portion (arrow) (d)
smooth muscles and ductal hyperplasia (Fig. 2c). It showed a hamartomatous polyp without evidence of any malignancy. These features were consistent with the diagnosis of a PeutzJeghers-type hamartomatous polyp. The patient showed a favorable clinical course and discharged from our hospital on Fig. 2 On a gloss appearance at the operation, the delle on the anterior wall of the duodenal bulb was recognized, and at first Hutchinson’s maneuver was performed (a). The polyp was located in the duodenal bulb without involving the papilla of the Vater, so duodenotomy was performed (b). Histopathological examination revealed tree branching pattern caused by the smooth muscles (black arrow) and ductal hyperplasia (red arrow) (c). It showed a hamartomatous polyp without evidence of any malignancy (H&E stain, ×10)
the 14th day after surgery, and no sign of recurrence of gastrointestinal obstruction and acute pancreatitis was observed 6 months after surgical management. Duodeno-jejuno (DJ) intussusception in PJS patients is very rare because the duodenum is fixed in the retroperitoneum and
J Gastrointest Surg
the Peutz-Jeghers polyps are less commonly found in the duodenum.3 To our knowledge, this is the first presentation of a patient with duodeno-jejunal intussusception with acute pancreatitis in Peutz-Jeghers syndrome in childhood. Oncologically, the polyp is usually hamartoma without dysplasia of glandular cell. Histological evidence of carcinogenesis in PJS, however, follows the pathway of a hamartoma developing into an adenoma, which eventually becomes a carcinoma. It is important to diagnose as PJS early, especially in patients with family history of PJS, because the relative risk of developing gastrointestinal carcinoma increases 18-fold compared to the general population. So upper GI endoscopy and colonoscopy (or capsule endoscopy) are recommended every 2–3 years.4 In therapeutic management, Hutchinson’s maneuver was commonly performed in the intussusception with PJS patients, because the intussusception in PJS is mild and usually selfreducing. In this case, the surgical treatment was suggested to be the most appropriate, since the release of the DJ intussusception and the resection of the polyp of the duodenum by endoscope were unsuccessful for this PJS child with acute pancreatitis. Fortunately, minimal invasive surgery would be performed according to the careful surgical procedure, because the polyp could be resected without pancreaticoduodenectomy. Because surgical resection of the pancreas would have a significant negative impact on the patient’s life and preservation of intact organ is important for the patients’ quality of life,
especially in the case of pancreaticoduodenectomy, minimal invasive surgery for the DJ benign polyp should be performed if possible. In conclusion, we described the first case presentation of DJ intussusception with acute pancreatitis in PJS in childhood which was treated surgically. Since the radical lymph node dissection is not mandatory, preservation of intact organs should be considered in the treatment of DJ intussusception with acute pancreatitis from PJS, especially in childhood.
Financial Support Financial support for this article was given by the Kochi organization for medical reformation and renewal grants.
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