Abstract: contribution to the literature. Case presentation: A

Abstract: Peutz-Jegherssyndrome is an autosomal dominant disordercharacterized by hamartomatous polyps and mucocutanous pigmentation. Mostof polyps are localized in the small intestine cause jejuno-jejunal andjejuno-ileal intussusceptions frequently in children. Endoscopic or surgicaltreatment is necessary for large polyps while spontaneous reduction ofinvagination caused by small polyps.

 In this report wepresent a 25-year-old female patient who was admitted to our hospital withsymptoms of ileus for 8 days. Physical and endoscopic  findings revealedthe diagnosis of Peutz- Jeghers syndrome. Her complaints persisted despiteendoscopic polypectomy and she was underwent laporoscopic and surgicaltreatment.Introduction: Peutz-Jegherssyndrome (PJS) is a rare autosomal dominant herited disorder characterized byhamartomatous polyps capable of holding the entire gastrointesinal tractus.Most of the polyps are localized to the small bowel cause gastrointestinalbleeding and iron defficiency anemia.

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Polyps are detected about 5-15% in adults as a rare cause of ileus. Thisunusual presentation of a rare condition as multisegmentary and long segment involvementin the patient may be a noteworthy contribution to the literature.  Case presentation: A 25-year-female patient was admitted to our emergencyservice with nause-vomiting, widespread colicky abdominal pain over one week.She had a history of intermittent abdominal pain for the last eight years.

On physicalexamination she was afebrile and blood pressure of 110/70 mm Hg. There wastenderness and guarding in the right iliac fossa and left-upper quadrant. Approximately5 cm- diameter palpable mass was detected at right lower quadrant.

Bowel soundswere increased.Blood investigations showed no anemia ( Hb: 12.3 gm/dl).

Herleukocyte count was increased 13.3×1000 cells/L. Except white spheres, allother laboratory tests were normal. Intravenous fluids and antibiotics werestarted to the patient.Our patient had been previously diagnosed with PJS 8 yearsbefore; endoscopically detected multiple polyps in stomach and mucocutanous hyperpigmentation.

She had the history of hospitalization for 5 times due to ileus but no surgicaltreatment was performed. She had been hospitalized, kept under observation andinvaginated segment reducted spontaneously. In this hospitalization; there wasno specific feature on her abdominal plain radiograph (figure-1). Abdominalultrasonography revealed two target signs of invagination at left-upper andright-lower quadrant. Intravenous and oral-rectal contrast enhanced CT exam wasperformed. Long segment jejuno-jejunal and ileo-caecal  invagination with centralized mesenteric fattytissue and multipl lymphadenopathies  wasdetected causing pre-obstructive dilatation with air-fluid levels in bowellumen (figure-2,3,4). A solid lesion 3 cm-diameter with peduncle not showingcontrast staining with supposed to be the swelling lesion at the level of theleft upper quadrant was detected on CT. Colonoscopy was performed and found multiplemilimetric sessile polyps in the distal part of ileum and whole colonicsegments.

Altough polyps were removed endoscopically, her complaints continuedafter colonoscopic polypectomy. Surgeons decided to laparoscopic surgery. The ileo-ilealinvaginated segment that 40 cm proximal of caecum was retracted by pullingproximal and distal parts of the bowel by laparoscopically. Intra-operatively afew pedunculated polyps were detected in the jejuno-jejunal invaginated segmentlocalized in the left upper quadrant. It was turned to the open surgery to makeresection and end to end anostomosis. Gross pathologic findings revealed a 30x20x15mm, a 15x10x10 cm and two milimetric polypoid lesions.

The resected segment was sent for histopathologicexam. Multiple sections was studied from the polyps. Histopathologicalexamination revealed the hamartomatous polyps with adenomatous changes. Therewas no evidence of any dysplasia and malignancy. (Figure 5). There was no problem in her post-operative process and shewas discharged home on post-op.

4. day. Discussion:  Peutz-Jeghers syndrome is a rare autosomal dominant disordercharacterized by mucocutaneous hyperpigmentation, hamartomatous polyps ofgastrointestinal tract and multiple neoplasms.

The incidence of the disease isas one in 120,000 births. Fewer than 5% of patients with PJS lack the abnormalmucocutaneous melanotic pigmentation, and fewer than 5% of patients with thepigmentation have no PJ polyps 6. Our case is difficult to diagnose with PJSdue to lack of any pigmentation. Family history of PJS and colonoscopicfindings suggested the etiology.Patients withPJS commonly present with recurrent abdominal colicky pain caused byinvagination.

This clinical presentation has been observed in 47% to 69%of adult patients with PJS and most of them were due to polyps located in thesmall intestine 7. The most of the cases reported in literature hadjejunal-ileal invagination while colonic is very rare (8,9,10,11). Our case hadcolo-colonic intussusception with two other else small bowel segmentinvagination. Abdominal colicky pain, tenderness is the most seen findings.

Ctimaging is very helpful for the detection of obstruction level and guiding forthe endoscopic or laparoscopic-surgical treatment. Endoscopy has an important role in the diagnosis andtreatment phase of the disesase, reduces the need for surgery and small bowelsyndrome in the cases of multiple intussusception. Endoscopic removal is theideal method of treating a pedunculated polyp; however, when this is notpossible, laparoscopy can be a safe and effective alternative method forreduction of the intussusception and bowel resection 12,13. In our case,endoscopic polypectomy was insufficient cause of multisegmenter involvement sothat patient was underwent laparoscopic procedure. It could not be reached allthe invaginated segments by laparoscopically so that the operation was turnedto the open surgery. Partial jejunal- ileal resection and end to endanostomosis was performed.

 Conclusion: Multisegmenter andcolonic intussusception in PJS is a different situation because of itsinfrequency. Patients usually have recurrent, chronic and nonspesific symptoms.Diagnosis is easy with the clinical and imaging findings. Endoscopic treatmentis the first choice for the young patients avoid of small bowel syndrome andmultiple surgeric procedures. It should be kept inmind for PJS in patients with no mucocutanous pigmentation presented withinvagination.  Table 1. The characteristics of intussusception in adultsand children Adult intussusception Intussusception in children Frequency 5% ofall cases 95% of all cases Part of the bowel involved Commonly involves smallbowel Usually involved colon Symptoms Classical symptoms of intussusception arenot always present Classical symptoms are present in many cases Cause Caused bymalignancy (50%) or polyp Idiopathic due to lymphoid hyperplasia in Peyer’spatches Treatment Treatment is usually resection of intussuscepted segmentwithout reduction (to prevent seedling of malignant cells) Treatment ishydrostatic reduction and if needed manual reduction by lapar (8)) 

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