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Jejunal Intussusception in Peutz-Jeghers Syndrome Mimicking Acute Appendicitis.

DISCUSSION Peutz-Jeghers syndrome is an autosomal dominant disorder with an incidence from 1 in 8300 to 1 in 120,000 births. Mutations in the STK11 gene have been identified as a cause of PJS. Peutz-Jeghers syndrome is a hereditary polyposis syndrome characterized by mucocutaneous hyperpigmentation and hamartomatous polyps. Mucocutaneous pigmentation occurs commonly around the lips, buccal mucosa, eyes, and fingers. Hamartomatous polyps can occur anywhere in the gastrointestinal tract but are most common in the jejunum followed by the ileum and duodenum. Hamartomatous polyps usually present as surgical emergencies in the first decade of life, usually secondary to intussusception, bowel obstruction, and bleeding. Intussusception is the telescoping of a segment of bowel and surroundingmesentery into another loop of bowel. It usually presents with abdominal pain and symptoms of intestinal obstruction. Because the mesentery and the vessels it contains are incorporated into the intussusception, venous return is initially impaired leading to edema. Ultimately, arterial supply to the bowel is affected followed by bowel ischemia and necrosis. Ultrasound is very valuable in the evaluation of intussusception. The transverse gray-scale sonographic appearance of intussusception is usually a mass with multiple concentric rings; this is referred to as the ‘‘target sign.’’ On the longitudinal images, the intussusception bears a semblance to the kidney, and this is referred to as the ‘‘pseudokidney sign.’’ Figure 1 is a longitudinal gray-scale ultrasound image showing the ‘‘pseudokidney’’ sign. Figure 2 is a transverse gray-scale ultrasound image showing concentric rings. Computed tomography scan may show a ‘‘bowel-in-bowel’’ appearance as well as invaginating mesenteric fat. Figure 3 is an axial computed tomography image demonstrating the ‘‘bowel-inbowel’’ appearance. The arrowpoints to invaginatedmesenteric fat. Most children with acute appendicitis experience generalized or periumbilical abdominal pain, which subsequently migrates to the right lower quadrant; most patients with acute appendicitis will also have an elevated white blood cell count. Our patient’s clinical presentation was therefore suggestive of acute appendicitis to the clinicians. Ileocolic intussusception has been previously described in the literature as a differential diagnosis for appendicitis, but to our knowledge, there is no report of jejunal intussusception mimicking acute appendicitis. The management of intussusception can be nonsurgical or surgical. Nonsurgical reduction is an important primary treatment for pediatric intussusception and has a high success rate and low incidence of complication. A reduction technique usually has 2 components; the first is the guidance method, such as ultrasound or fluoroscopy. The other is the contrast medium, which can be air, barium, or saline. Nonsurgical reduction is usually reserved for uncomplicated cases, whereas surgery is indicated if ischemia is present.