A 5-year-old boy presented with complaints of abdominal pain and vomiting for 1 week.
The child was initially evaluated with Ultrasound, which showed multiple segments of ileoileal intussusception predominantly in the left lumbar region, showing the ‘pseudokidney’ sign.
Circumferential bowel wall thickening with luminal narrowing involving multiple segments of small bowel were noted, with the thickened bowel wall showing loss of mural stratification and significant internal vascularity on Colour Doppler. Few enlarged mesenteric nodes were also seen.
A large heteroechoic mass was also noted in the right suprarenal region, abutting the inferior surface of the liver and the right kidney.
A contrast enhanced CT of the thorax and abdomen was done, which revealed multiple long segment ileoileal intussusceptions with thickened small bowel loops, and enlarged mesenteric lymph nodes. The right suprarenal lesion seen on ultrasound was also demonstrated on CT, and showed heterogeneous enhancement with central necrotic areas. Sections through the thorax revealed a left supraclavicular nodal mass; an enhancing right pleural deposit, and mild right pleural effusion.
Intussusception is when a proximal segment of bowel telescopes into the distal segment . The donor loop is called the intussusceptum and the receiving loop the intussuscipiens . It is classified according to site - small bowel, large bowel or both . Intussusception in children is mostly idiopathic, although a causative factor or ‘lead point’ can be identified in some cases, such as lymphoid hyperplasia, Meckel’s diverticulum, benign neoplasms (lipoma, leiomyoma, polyps), and malignant causes such as lymphoma [1.]. Multiple simultaneous intussusceptions are rare - few causes include Peutz Jegher syndrome, intestinal duplication, and Meckel’s diverticulum [1,3].
In this case, findings of thickened bowel loops with increased vascularity, mesenteric lymphadenopathy, suprarenal mass, pleural and thoracic nodal involvement raised the suspicion of Burkitt’s lymphoma.
Burkitt’s lymphoma is said to be the most common cause of intussusception in children over the age of 4 , although rarely reported as a cause of multiple simultaneous intussusception.
It is the most common Non-Hodgkin’s lymphoma affecting children and has 3 forms- sporadic, endemic and immune deficiency associated [5,6]. The abdomen is commonly involved in the sporadic form .
Ultrasound features of intussusception include the ‘target sign’ and ‘pseudokidney sign’ with a hypoechoic outer ring and hyperechoic center [2.]
CT features include an oblong sausage shaped or target shaped mass, containing mesenteric fat and vessels within it .
In Burkitt’s lymphoma, the ileocaecal region is commonly involved due to its rich lymphatic network [5,6]. Imaging findings include focal masses and bowel wall thickening with loss of mural stratification on Ultrasound [4,5,6]. Bowel obstruction usually occurs due to intussusception [4,5].
Other abdominal manifestations include retroperitoneal and mesenteric nodal masses, solid organ involvement (kidneys most commonly) and peritoneal seeding [5,6,7].
Head and neck manifestations include Waldeyer’s ring lymph nodal enlargement and facial bone involvement [5,6,7].
Chest findings include mediastinal adenopathy, pleural and lung parenchymal involvement .
Multiple intussusceptions often do not reduce with hydrostatic enema and require surgery .
Despite the aggressive nature of Burkitt’s lymphoma, it is sensitive to chemotherapy. Hence, early diagnosis and treatment is important to prevent complications .
Intussusception is a common pediatric emergency and can be easily recognized on Ultrasound by the target and pseudokidney signs, and on CT by its characteristic appearance.
A lead point should be sought in cases of multiple intussusceptions.
The presence of bowel wall thickening, lymphadenopathy and intussusception in a child should raise the suspicion of Burkitt’s lymphoma.
Written informed patient consent for publication has been obtained.
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