Peutz-Jeghers syndrome in a woman presenting as intussusception: A case report

IntroductionPeutz-Jeghers syndrome (PJS) is an uncommon autosomal dominant syndrome with a variable to high penetrance that leads to the development of polyps within the gastrointestinal mucosa. Here we report a case of an adult female suffering jejunoileal intussusception due to PJS.Presentation of caseA 30-year-old woman came to an emergency department with a small bowel obstruction caused by intussusception. The patient underwent an emergency exploratory laparotomy. An intussusception at the level of 60 cm from the ligamentum treitz was revealed, and the intussusception small bowel segment was not viable; we decided to perform segmental jejunoileal resection with the Bishop-Koop procedure, and the specimen histopathology of the segmental jejunoileal resection showed a typical hamartomatous polyp features. Two month later, diagnostic endoscopy showed multiple polyps (between 5 and 15 mm) in the large bowel. The polyps were removed with endoscopic polypectomy and examined histopathologically, showing characteristics of PJS. Further detailed family history was obtained, and similar skin lesions were detected on our patient’s child (since birth). Although endoscopy screening identified multiple polyps in the child’s ileum and large bowel, he was not suffering from abdominal symptoms.ConclusionIn patients with intussusception at a young age, PJS can be caused by the presence of a hamartoma polyp as a trigger for intussusception. If there are multiple polyps found in the gastrointestinal mucosa and other pathognomonic signs are found, such as hyperdense macular lesions on the lip and buccal mucosa, such cases should be confirmed as PJS.     

Laparoscopic management of small-bowel intussusception in a 64-year-old female with ileoal lipomas

A 64-year-old female patient presented with upper ab-dominal pain and vomiting. Ultrasonography showed a hyperechoic mass in the right lower abdomen and computed tomography showed a low-density mass with intestinal invagination. An emergency laparo-scopic right-hemicolectomy was performed, and the resected specimen was found to contain three tumors, which were identified histopathologically as intestinal lipomas. Adult intussusception is relatively rare and difficult to be diagnosed, since most symptoms of adult intussusception are nonspecific. We report our clinical experience of the diagnosis and emergent laparoscopic surgery for an adult patient with intussusception.     

Peutz-Jeghers综合征致儿童反复性肠套叠临床分析

目的 探讨黑斑息肉病(PJs)导致小儿反复性肠套叠的诊治.方法 回顾性分析5例PJS继发反复性肠套叠病例资料.结果 5例反复性肠套叠均经手术和病理组织学证实为PJS所致,术后恢复良好,随访无复发.结论 PJS导致小儿肠套叠易复发,术中处理肠套叠同时切除息肉是有效的治疗方法,术后仍需长期监测、定期复查.     

A Solitary Gastric Peutz-Jeghers Type Polyp: Report of a Case

Peutz-Jeghers type polyps of the stomach are rare and almost always associated with intestinal polyposis and mucocutaneous pigmentation; a condition known as Peutz-Jeghers syndrome. The case presented in this report refers to a woman found to have a large solitary Peutz-Jeghers type polyp of the stomach, with a maximal diameter of 7 cm. Extended investigation did not reveal intestinal polyposis or any other sign of Peutz-Jeghers syndrome. Because of the size of the polyp, a partial gastrectomy was performed. To the best of our knowledge, there are only three other reports in the literature of a solitary Peutz-Jeghers type gastric polyp occurring in the absence of Peutz-Jeghers syndrome. This patient is scheduled to undergo a follow-up examination every 2 years to detect any sign of the development of Peutz-Jeghers syndrome or malignancies commonly associated with it. Received: February 29, 2000 / Accepted: November 20, 2000     

黑斑息肉综合征的研究进展

目的介绍黑斑息肉综合征(PJS)的研究和临床诊治进展。方法对。PJS近几年国内、外的研究和临床诊治进展的文献作一概述。结果PJS是一种常染色体显性遗传病，以口周皮肤、唇、颊黏膜和指趾末端存在黑色素沉着和消化道存在多发性息肉为特征，其肠内、肠外组织和器官易发生癌症，是一种肿瘤易感综合征，其致病基因为19p上的STK11基因。结论PJS致病基因与肿瘤的发生和发展密切相关。PJS患者消化道内和消化道外组织和器官都容易发生恶变。     

The role of laparoscopy in the management of childhood intussusception

apd: 6 February 2001     

Laparoscopic resolution of intussusception after Roux-en-Y gastric bypass

Introduction

Roux-en-Y gastric bypass (RYGB) has been the most common surgical operation used to treat obesity and its inherent co-morbidities. Intussusception with bowel obstruction after RYGB is a rare complication and its physiopathology remains unclear. The diagnosis is generally based on typical image of computed tomography (CT) scan and a surgical exploration is generally recommended.

Laparoscopic reduction of appendicocecal intussusception due to mucinous cystadenoma in an adult.

OBJECTIVE: Laparoscopic reduction of appendicocecal intussusception due to mucinous cystadenoma is herein described. METHODS: A 32-year-old female had intermittent abdominal pain and nonbloody diarrhea. The preoperative diagnosis was intussusception of the colon with a benign tumor by CT-scan findings. RESULTS: Laparoscopic reduction of intussusception and ileocecal resection were successfully performed. Gauze sponge sticks were used to provide compression from the distal colon. This maneuver was very effective to reduce the intussusception. CONCLUSION: Laparoscopic reduction of intussusception due to a benign tumor is feasible and safe even in adults. Mucinous cystadenoma is one of the causes of intussusception in adults and a good indication for the laparoscopic approach.     

Laparoscopic versus open reduction of intussusception in children: a single-institution comparative experience

Background

The role of laparoscopic surgery in treating intussusception has been controversial. This study reviews our institution's experience with the laparoscopic approach (LAP) compared to the open surgical approach (OPEN).

Methods

Retrospective analysis of all patients undergoing surgery for intussusception at our center from January 2002 to February 2006. Statistical assessment included Student's t test and χ² analysis.

Results

A total of 41 patients required operation for intussusception (18 LAP, 23 OPEN). Mean age was 22 months for LAP and 11 months for OPEN (P = .17). In the LAP group, 28% (5/18) were converted to an open procedure. Operative times and complications were not significantly different. Pathologic lead points were found in 33% (6/18) LAP and 35% (8/23) OPEN patients (P = 1.0). Time to full feeds was significantly shorter (LAP vs OPEN: 3.4 ± 2.7 vs 5.6 ± 3.4 days, P = .02). Length of stay was shorter (LAP vs OPEN: 4.8 ± 3.5 vs 9.1 ± 7.5 days, P = .03).

Conclusions

Intussusception can be treated safely and effectively using a LAP with a significant decrease in time to full feeds and length of stay. The LAP should be considered as the initial approach for stable patients with intussusception requiring operative intervention.     

Peutz-Jeghers综合征29例诊治分析

目的 探讨Peutz-Jeghers综合征的临床特点及治疗方法。 方法 回顾性分析北京协和医院1984-2008年收治的29例Peutz-Jeghers综合征临床资料。 结果 29例病人均有胃肠道息肉,27例存在黑色素斑点沉着,10例（34%）有明确家族史。临床表现以腹痛、便血为主。并发肠套叠12例（41%）,肠梗阻11例（38%）。行手术治疗19例,内镜切除6例,保守治疗4例。病理结果：错构瘤26例,伴不典型增生3例。 结论 Peutz-Jeghers综合征具有较为典型的临床表现,易合并肠梗阻、肠套叠、消化道出血等并发症,治疗上可采用手术治疗与内镜下息肉切除相结合的方式。Peutz-Jeghers综合征病人为恶性肿瘤高风险人群,必须坚持常年随访。     

Combined endoscopic and surgical treatment for the polyposis of Peutz-Jeghers syndrome

Repeated laparotomy with extensive small bowel resectioning and eventual short-bowel syndrome is a major problem in Peutz-Jeghers syndrome (PJS) patients. This problem is caused by gastrointestinal polyposis with intussusception. A combined surgical and endoscopic approach can assess the extent of the polyposis, and small polyps can be removed by snare polypectomy. This can avert multiple enterotomies and decrease bowel resection segments. We applied an intraoperative colonscope via the enterotomy route in an 20-year-old PJS woman, and successfully removed the other 10 polyps distributed in the whole small bowel. As part of an aggressive approach to the management of polyposis in PJS, complete polypectomy can provide a longer symptom-free interval and remove potentially premaligment polyps. Received: 9 September 1999/Accepted: 9 November 1999/Online publication: 22 August 2000     

Intussusception of the small bowel associated with nephrotic syndrome

We report the case of a 2-year-old boy who developed a small bowel intussusception during treatment failure of his first episode of nephrotic syndrome. Despite the absence of typical symptoms other than abdominal pain, the intussusception was diagnosed by ultrasonography and computed tomography and successfully reduced by air enema. No pathological lead point was discovered, and no symptoms of Henoch-Schönlein purpura developed later. Intussusception should be considered in the differential diagnosis of abdominal pain in patients with nephrotic syndrome, especially in patients exhibiting prolonged edema. Ultrasonography or computed tomography should be performed, even in the absence of other typical symptoms suggestive of intussusception. We should also bear in mind that the intussusception associated with nephrotic syndrome might occur at regions other than the typical ileocolic region, such as within the small intestine.     

腹腔镜二孔法小儿肠套叠整复术

【摘要】 目的 探讨腹腔镜二孔法小儿肠套叠整复术的可行性及手术经验。方法〓2009年6月至2014年6月期间,32例空气灌肠复位失败或复位成功后复发的肠套叠患儿行腹腔镜二孔法肠套叠整复术,回顾患儿临床资料,对比二孔法复位成功和中转开腹复位两组患儿的年龄、起病时间、套叠类型、套头长度、合并病变等资料,分析影响腹腔镜下复位成功率的因素。结果〓32例患儿中24例应用腹腔镜二孔法肠套叠整复术复位成功,成功率75%,包括3例复发性肠套叠患儿。腹腔镜探查发现其中2例合并梅克尔憩室。其余8例需扩大脐部伤口中转开腹复位,中转手术原因包括:肠管穿孔或坏死后套叠肠管粘连4例、回肠息肉套头内嵌顿1例、套头过长3例。中转患儿的套头长度和合并病变占比明显长于腔镜复位组,其差异有统计学意义,而患儿年龄、起病时间及术后并发症两组间的差异无统计学意义。结论〓腹腔镜二孔法肠套叠整复术具有手术微创、成功率高的优点,掌握好适应症后可成为空气灌肠复位失败的非重症患儿和复发性肠套叠患儿优先选择的手术方法;套头长度和合并病变可能是影响二孔法复位成功率的因素。     

Small bowel intussusception in adults

Intussusception is the telescoping of a proximal segment of the gastrointestinal tract into an adjacent distal segment. This rare form of bowel obstruction occurs infrequently in adults. We report a case of small bowel intussusception in an adult male patient. We have also performed a literature review of this rare condition.     

Intussusception of the small intestine due to Peutz-Jeghers syndrome: a case report and review of the literature

One case of small bowel's intussuception has been found in one patient with abdominal pains. Since 13 years this patient has a Peutz-Jeghers syndrome. A resection of the small bowel has been performed followed by total intraoperative enteroscopy. Besides small bowel, Peutz-Jeghers syndrome can affect many organs with an increased risk for cancer for patients affected by this genetic disease.     

Juvenile polyps in the small intestine presenting as jejunojejunal intussusception in a 10-year-old child: report of a case

We report herein the case of a 10-year-old child with small bowel obstruction caused by jejunojejunal intussusception of a juvenile polyp. To the best of our knowledge, this is only the third case reported of a juvenile polyp in the small intestine and the first case to be documented in the English literature. Received: May 7, 2001 / Accepted: September 11, 2001     

Laparoendoscopically assisted endoscopic small bowel polypectomy in a patient with Peutz-Jeghers syndrome

Peutz-Jeghers syndrome is a rare condition, requiring regular endoscopic or radiological diagnostic studies. Polyps are a potential source of dysplasia and malignancy. We present a case in which we used a new diagnostic and therapeutic approach to this pathology by performing a laparoscopically assisted endoscopic polypectomy. This approach is minimally invasive and can be repeated if necessary. It might also be adopted for diagnosis and treatment of other intestinal pathologies, such as vascular malformations in the small bowel.     

Small bowel intussusception after blunt abdominal trauma in a 6-year-old boy: case report and review of 6 cases reported in the literature

Although intussusception is a well-known cause of acute abdomen in the pediatric population, traumatic intussusception is exceedingly rare and has been reported previously only 6 times in a preadolescent child. We present a case of ileoileal intussusception in a previously healthy 6-year-old boy after blunt trauma to the abdomen and review the English language literature on the subject.