Variation of Polysplenia Syndrome with Intestinal Malrotation

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Variation of Polysplenia Syndrome with Intestinal Malrotation Hannah Chang, Ph.D., HMS III Gillian Lieberman, M.D. Beth Israel Deaconess Medical Center Harvard Medical School March 20, 2009

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Talk Outline Introduction : clinical case Background : gastrointestinal malrotation Re-examination : optional radiographic discoveries Potential conclusion : variation of polysplenia disorder with malratotion Take home focuses

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Our patient MF: Clinical Presentation MF is a 28-year-old lady with a few month history of dubious discontinuous stomach torment, with queasiness and bloating. Her indications were not connected with nourishment admission. She has had ordinary solid discharges and it generally sound. After various treks to her essential care doctor without determination of manifestations, she introduced to our healing facility for preclude of a ruptured appendix. As a major aspect of her workup, an Abdominal/Pelvic CT filter , and an Upper GI ponder with barium and little inside complete (UGI with SBFT) were acquired.

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Our patient MF had an Abdominal/Pelvic CT check and an Upper GI (UGI) ponder with barium and little entrail finish (SBFT) as a feature of her workup.

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Our patient MF: Abdominal CT Scan PACS, BIDMC

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Our patient MF: Abdominal CT Findings Small entrail Colon PACS, BIDMC C+ CT (coronal reproduction)

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Our patient MF: UGI + SBFT Duodenal Finding PACS, BIDMC UGI with barium and air + SBFT Incomplete compass of the 4 th part of the duodenal PACS, BIDMC UGI with barium and air + SBFT

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Our patient MF: UGI + SBFT Ileocecal Finding PACS, BIDMC UGI with barium and air + SBFT PACS, BIDMC Normal area: ileocecal intersection UGI with barium and air + SBFT

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Let's spend a minute to audit the procedure of embyronic midgut pivot.

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Stage 1: Midgut Exits the Abdomen Moore KL & Dalley AF (1999) At 6 weeks incubation, the midgut circle is compelled to leave the stomach depression because of the vast size of the beginning liver and kidneys. Around 10 weeks, the midgut starts to return. In any case, initial, a progression of turns around the unrivaled mesenteric supply route happens.

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Stage 2: Counterclock-wise Midgut Rotation Moore KL & Dalley AF (1999) With the predominant mesenteric course (SMA) as a pivot, the cranial and caudal appendages of the midgut circle turn counterclock-wise while coming back to the stomach depression in the meantime.

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Stage 3: Cecal Descent and Colonic Tacking Moore KL & Dalley AF (1999) After an aggregate of 270 degrees of counterclock-revolution, the duodenum, little insides, and plunging colons are set up. Next, the cecum drops, carrying with it the rising colon. At long last, the mesentary of the rising and diving colons intertwine with the peritoneum of the stomach dividers.

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Any part of the procedure of midgut revolution can go astray. What are the most well-known formative entanglements?

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Developmental Complications of Midgut Rotation Omphalocele : disappointment of midgut to come back to the stomach cavity. 1:5000 live births. Rotational variations from the norm : most generally, non-revolution, or capture of cecal drop and colonic attaching. 1:500 by a few appraisals. Midgut volvulus : trade off of vascular supply from volvulus around slender mesenteric pedicle. Surgical crisis. 1:5000 live births.

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Let's presently take a gander at some correlation cases for exemplary radiographic discoveries for intestinal malrotation.

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Comparison case #1: Ladd Band Hill, M. UNSW Embryology. http://embryology.med.unsw.edu.au/Ladd band Midgut volvulus UGI with barium and air + SBFT Burk MS, et al. Am J Surg (2008)

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Comparison case #2: Inversion of SMA/SMV SMA (A) SMV (B) C+ pivotal CT Gamblin TC, et al. Current Surgery (2003)

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Comparison case #3: Mesenteric Rotation Around Narrow Pedicle ("Whirlpool Sign") C+ pivotal CT Matzke GM, et al. Surg Endosc (2005)

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Let's currently come back to our patient MF. Her stomach discoveries proposed it was not an "exemplary malrotation" with RUQ cecum and Ladd band. Truth be told, her privilege sided colon and left-sided little entrails were precisely inverse to that normal for malrotation from Stage 3 capture. To make a last conclusion and perhaps give treatment, she was taken to the OR for laparoscopic investigation of her belly.

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Our patient MF: Surgical Treatment PACS, BIDMC Appendectomy Removal of band between rising/dropping colon C+ CT (coronal reproduction)

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Our patient MF: Clinical Course Patient MF endured the surgery well and had insignificant draining intra-operatively. She had a smooth post-agent course and was released 1 day after surgery.

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Let's presently come back to MF's stomach CT discoveries and call attention to some fascinating coincidental discoveries.

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Our patient MF: Incidental CT Finding - Polysplenia PACS, BIDMC PACS, BIDMC C+ hub CT Multiple splenules C+ CT (coronal reproduction)

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A B Our patient MF: Incidental CT Finding – Duplicated Inferior Vena Cava (IVC) PACS, BIDMC A PACS, BIDMC C+ hub CT PACS, BIDMC B C+ hub CT C+ CT (coronal remaking)

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Let's talk about one conceivable binding together conclusion to clarify all of patient MF's radiographic discoveries.

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Clinical Presentation of Polysplenia Syndrome Abdominal torment Polysplenia Heterotaxy (stomach, liver, heart) Short pancreas Intestinal malrotation IVC variations from the norm Azygos/hemizygos continuation Preduodenal entryway vein Situs uncertain/inversus = Patient MF Gayer G, et al. Abdom Imaging (1999)

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Comparison case #4: Radiographic Findings for Polysplenia Syndrome Polysplenia Dilated azygos vein C-hub CT Gayer G, et al. Abdom Imaging (1999)

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Comparison case #5: Heterotaxy in Polysplenia Syndrome Liver Heart Stomach C+ pivotal CT Gayer G, et al. Abdom Imaging (1999)

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Our patient MF: Clinical Outcome Since release, persistent MF has introduced to our healing facility two more times for ambiguous stomach torment. Urinary tract contamination and gynecologic etiologies were precluded. It stays to be demonstrated whether her surprising stomach life structures might bring about reversible, transient mesenteric vascular trade off, which thusly, prompts to her stomach torment.

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Finally, we should examine a couple bring home focuses picked up from our patient MF.

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Take Home Points Intestinal malrotation ought to be considered in grown-ups with unclear stomach side effects Accurate radiographic analysis of intestinal malrotation can anticipate superfluous confusions and additionally surgeries Polysplenia, IVC variation from the norm, intestinal malrotation, and cardiovascular irregularities can be syndromic in asymptomatic patients. These discoveries may have clinical criticalness later on.

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Acknowledgments Gillian Lieberman, M.D. Maria Levantakis Brian Callahan, M.D. Dan Jones, M.D. Robert Lim, M.D.

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References Gayer G, Apter S, Jonas T, Amitai M, Zissin R, Sella T, Weiss P, Hertz M. "Polysplenia disorder identified in adulthood: report of eight cases and audit of the writing". Abdom Imaging. 1999. 24(2): 178-84. Zissin R, Rathaus V, Oscadchy A, Kots E, Gayer G, Shapiro-Feinberg M. "Intestinal malroataion as a coincidental finding on CT in grown-ups". Abdom Imaging. 1999. 24(6): 550-5. Matzke GM, Dozois EJ, Larson DW, Moir CR. "Surgical administration of intestinal malrotation in grown-ups: near results for open and laparoscopic Ladd methodology". Surg Endosc. 2005. 19(10):1416-9. Gamblin TC, Stephens RE Jr, Johnson RK, Rothwell M. "Grown-up malrotation: a case report and survey of the writing". Curr Surg. 2003. 60(5): 517-20. Nonaka S, Shiratori H, Saijoh Y, Hamada H. "Assurance of left-right designing of the mouse developing life by counterfeit nodal stream". Nature. 2002. 418 (6893): 96-99. Proceeded…

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References 6. Taylor HO, Barish M, Soybel D. "Unwinding intestinal malrotation with 3-imensional PC tomography". Clin Gastroenterol Hepatol. 2006. 4(8): xxix. 7. Lin CJ, Tiu CM, Chou YH, Chen JD, Liang WY, Chang CY. "CT presentation of cracked a ruptured appendix in a grown-up with fragmented intestinal malrotation". Emerg Radiol. 2004. 10(4): 210-2. 8. Tsuda Y, Nishimura K, Kawakami S, Kimura I, Nakano Y, Konishi J. "Preduodenal entrance vein and atypical continuation of second rate vena cava: CT discoveries". Diary of Computer Assisted Tomography. 1991. 15(4): 585-588. 9. Pickhardt PJ and Bhalla S. "Intestinal malrotation in youths and grown-ups: range of clinical an imaging highlights." AJR. 2002. 179: 1429-1435. 9. Moore KL & Dalley AF. Clinical Oriented Anatomy. fourth Edition. 1999. 10. Slope, M. The University of North South Whales. Embryology Project. (http://embryology.med.unsw.edu.au)

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