Loo Jerry,  Iskander Paul,  Chen Johnathan,  Hall Theodore,  Boechat Ines,  Ghahremani Shahnaz

Final Pr. ID: Poster #: EDU-010

Intestinal failure is a rare but devastating condition in pediatric patients that is often caused by short bowel syndrome (SBS) secondary to necrotizing enterocolitis, volvulus, or intestinal atresia. Patients require the use of total parenteral nutrition (TPN) to survive, which can lead to cholestasis and end stage liver disease (ESLD). Treatment of intestinal failure in pediatric patients who have developed subsequent liver failure from TPN with multivisceral (MVTx) transplantation has progressed dramatically in the past two decades as a result of improvements in immunosuppression, surgical technique, and post-transplant care. The number of transplantations has always been relatively low due to a lack of appropriate donors and the rarity of intestinal failure. However, given the high acuity of patients that do ultimately undergo these procedures, recognition of post-operative complications is critical for graft survival and improved outcomes. This educational exhibit will review the indications and relevant anatomy for these types of transplantations. Magnetic resonance (MR) findings related to post-transplantation complications will be presented with imaging correlates from other modalities (CT, PET/CT, US) as needed. Read More

Authors:  Loo Jerry , Iskander Paul , Chen Johnathan , Hall Theodore , Boechat Ines , Ghahremani Shahnaz

Keywords:  intestinal, liver, magnetic resonance

Maria Anthony Rayer Dhilip Andrew,  Snyder Elizabeth,  Singh Sudha,  Johnstone Lindsey,  Sarma Asha,  Krishnasarma Rekha

Final Pr. ID: Poster #: EDU-015

Megacystis microcolon hypoperistalsis syndrome (MMIHS) also known as Berdon syndrome is a rare genetic disorder with a poor prognosis characterized by hypoperistalsis of the bladder and bowel. When expediently diagnosed and managed, survival can be extended. The radiologist plays a role in the initial diagnosis and recommending further imaging based on the known constellation of findings in collaboration with the pediatric surgeon and pediatric urologist. The radiologist could be the first to appreciate characteristic findings and suggest the possibility of this syndrome.

The purpose of this educational exhibit is to describe the imaging characteristics of Berdon syndrome from the fetal stage to early childhood with an emphasis on 1) main radiologic findings, 2) helpful imaging features to differentiate Berdon syndrome from other similar conditions, 3) appropriate imaging studies to assist in supporting the diagnosis, and 4) clinical findings, management, and outcome. We will review 9 cases and their corresponding imaging.

Sources:

Wymer KM, Anderson BB, Wilkens AA, Gundeti MS. Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management. J Pediatr Surg. 2016;51(9):1565-1573.

Puri P, Shinkai M. Megacystis microcolon intestinal hypoperistalsis syndrome. Seminars in Pediatric Surgery. 2005;14(1):58-63.

Rolle U, O’Briain S, Pearl RH, Puri P. Megacystis-microcolon-intestinal hypoperistalsis syndrome: evidence of intestinal myopathy. Pediatr Surg Int. 2002;18(1):2-5. Read More

Authors:  Maria Anthony Rayer Dhilip Andrew , Snyder Elizabeth , Singh Sudha , Johnstone Lindsey , Sarma Asha , Krishnasarma Rekha

Keywords:  Berdon, intestinal hypoperistalsis, malrotation

Teo Yi-ming

Final Pr. ID: Poster #: CR-006

Intestinal lymphangiectasia is a rare disease characterised by dilated intestinal lacteals causing loss of lymph into the small bowel lumen and resultant hypoproteinemia, hypogammaglobulinemia, hypoalbuminemia and lymphopenia. The disease may occur as a primary / congenital form (primary idiopathic intestinal lymphangectasia / Waldmann disease) or as a secondary form resulting from causes of lymphatic obstruction, such as tumor or fibrosis.

Our case report describes the workup of an adolescent girl with known Primary Intestinal Lymphangiectasia (Waldmann disease) presenting with an acute history of abdominal pain and vomiting. We present her prior imaging leading to her initial diagnosis of Waldmann disease, as well as at the time of acute presentation, with imaging features raising suspicion for, and eventually leading to histo-pathologic confirmation of small bowel B-cell lymphoma.

Via this case report poster, we hope to create awareness of this rare disease, as well as its feared association of lymphoma. Typical imaging features are presented and discussed.
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Authors:  Teo Yi-ming

Keywords:  Intestinal Lymphangiectasia, Lymphoma, Waldmann Disease