Arab Journal of Gastroenterology xxx (2015) xxx–xxx

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Case report

An unusual cause of duodenal obstruction in adults Greetje J. Tack a,⇑, Bas-Jeroen van Kelckhoven b, Johan P. Kuyvenhoven a a b

Department of Gastroenterology and Hepatology, Kennemer Gasthuis, Haarlem, The Netherlands Department of Radiology, Kennemer Gasthuis, Haarlem, The Netherlands

a r t i c l e

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Article history: Received 29 January 2014 Accepted 16 March 2015 Available online xxxx Keywords: Intestinal rotational anomalies Adult Diagnosis

a b s t r a c t Background: Intestinal malrotation refers to a spectrum of anomalies of midgut rotation and fixation at various stages during early embryonic development. In adults, malrotation manifests itself mainly in chronic non-specific abdominal complaints and may therefore be easily misdiagnosed beyond infancy. Case presentation: We present a case of an 82-year-old Caucasian man with vomiting and abdominal pain owing to malrotation complicated by duodenal obstruction and intestinal ischaemia confirmed by radiologic evaluation and autopsy report. Conclusion: Although intestinal malrotation is generally discovered near birth, our case demonstrates that physicians should consider this diagnosis at advanced age as well. In addition, particularly radiologic findings are supportive in diagnosing malrotation. Ó 2015 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved.

Introduction Rotational abnormalities of the intestine have their roots in an arrest of rotation and malfixation of the midgut between the 4th and 12th weeks of gestation. The midgut comprises the second part of the duodenum to the proximal third part of the transverse colon, and from the fifth week of embryonic development a complex rotation around the axis of the superior mesenteric artery (SMA) occurs. First, the proximal loop and distal loop rotate 180° and 90° counterclockwise, with the subsequent position below and left of the SMA, respectively. Second, the bowel shows further rotation ensuring that the proximal loop is situated at the left and the distal loop at the right side of the SMA. Third, displacement of the caecum into the lower right quadrant and shortening of the mesentery with fixation of intestinal segments to the posterior abdominal wall occur [1]. The two most common anomalies of intestinal rotation are non-rotation and malrotation, yet with different clinical significance. A complete lack of rotation in the first period results in non-rotation, which is characterised by localisation of the duodenum right of the SMA and the colon to the left of the midline. Consequently, the duodenojejunal and ileocaecal junctions are situated in the right upper and left lower quadrant, respectively, resulting in a broad mesentery [2]. By contrast, malrotation refers to incomplete rotation that can occur at various ⇑ Corresponding author at: Department of Gastroenterology and Hepatology, Kennemer Gasthuis, Boerhaavelaan 22, 2035 RC Haarlem, The Netherlands. Tel.: +31 235453050. E-mail addresses: [email protected] (G.J. Tack), [email protected] (B.-J. van Kelckhoven), [email protected] (J.P. Kuyvenhoven).

stages. The most frequently observed hallmark is a predominately right-sided proximal small bowel with dislocation of the duodenojejunal junction inferiorly and rightwards, and, in addition, displacement of the ileocaecal junction into epigastrium or right hypochondrium near the ligament of Treitz. In consequence, the narrow mesentery attachment and the non-fixed areas of the intestine predispose to volvulus and strangulation, yet seldom observed in non-rotation. Furthermore, obstruction of the horizontal part of the duodenum owing to extrinsic compression of the bands of Ladd that extend from the caecum across the duodenum to the mesentery can be a dreadful complication of malrotation too [3]. These anomalies are primarily diagnosed in the first year of life [4] and very rare beyond childhood [5,6]. Therefore, physicians usually do not consider this diagnosis in the initial evaluation of abdominal pain after childhood. We present an exceptional case of intestinal obstruction, showing that complications of intestinal malrotation can even be diagnosed in the elderly.

Case presentation An 82-year-old man was admitted to the hospital presenting with nausea, vomiting, acute pain in the upper part of the abdomen, lack of appetite and weight loss. His medical history revealed cardiac ischaemic events, pulmonary embolus and Alzheimer’s disease. There was no clinical evidence for an ileus. Laboratory investigations indicated a total white blood cell count of 149 g/L, CRP < 1 g/L, creatinine 1.57 mg/dL, urea 75 mg/dL and normal liver enzymes and lipase. Upper endoscopy showed gastric retention with stenosis of the duodenal curve, which comprised only a 1.5-

http://dx.doi.org/10.1016/j.ajg.2015.03.002 1687-1979/Ó 2015 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved.

Please cite this article in press as: Tack GJ et al. An unusual cause of duodenal obstruction in adults. Arab J Gastroenterol (2015), http://dx.doi.org/10.1016/ j.ajg.2015.03.002

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G.J. Tack et al. / Arab Journal of Gastroenterology xxx (2015) xxx–xxx

Fig. 1. Axial contrast-enhanced computed tomography reveals the typical appearance of a right-sided duodenojejunal junction and displacement of the ileocaecal junction into epigastrium. The black arrow points a duodenal tube. Furthermore, a ‘whirlpool sign’ and inverted position of the SMA (black arrowhead) and the SMV (white arrowhead) are shown.

cm hole that could be passed by the scope. Histological analysis of duodenal biopsies revealed normal villi and no signs of inflammation or malignancy. Axial computed tomography (Fig. 1) with intravenous contrast and contrast by a duodenal tube (black arrow) showed the classic finding of malrotation as non-progression of the duodenum and jejunal loops across the midline resulting in localisation at the right side of the abdomen. In addition, the small bowel twists around the SMA and creates a so-called ‘whirlpool’ sign. Furthermore, an abnormal position of the superior mesenteric vein (SMV) and artery was observed, consisting of a vertical relationship and a left–right inversion with the vein located to the left. There were no abnormalities of the uncinate process of the pancreas. Although surgical intervention was a prerequisite for prolonged survival in this case, in consultation with patient and patient’s next of kin, it was decided not to perform surgery considering co-morbidity. The patient died within 1 week. The autopsy report confirmed the diagnosis of malrotation with subsequent ischaemia of the proximal small intestinal mucosa.

Discussion Intestinal malrotation refers to incomplete midgut rotation and fixation in early foetal life. The precise aetiology is unknown and so far no clear genomic association has been observed; however, malrotation is more frequently observed within families [7]. Although generally discovered near birth, our case demonstrates that malrotation may manifest itself in the elderly as well. In agreement, newly diagnosed malrotation in adulthood is a very rare phenomenon with an estimated incidence of 0.2% [8]. As incomplete midgut rotation and malfixation can arise at a wide range of locations, variability in clinical course and radiologic findings can be observed. Our patient had symptoms of acute abdominal pain, bilious vomiting and weight loss consistent with a volvulus and duodenal obstruction, two dreaded complications of malrotation. Acute symptoms of malrotation, however, are mainly displayed in the first months of life, whereas adulthood malrotation generally is characterised by chronic non-specific abdominal complaints, including recurrent abdominal pain,

bloating, intermittent nausea and early satiety, malabsorption syndrome and alternately constipation and diarrhoea [3,10–12]. A number of patients do not even have any symptoms and malrotation is an incidental finding on imaging or during surgery. In line with the present case, physical examination and laboratory findings do not contribute to diagnosis; however, in some cases it may reveal features consistent with sepsis, necrotising enterocolitis or bleeding. Distinctive features of midgut malrotation are primarily established by radiological investigations. Upper gastrointestinal contrast series remain the reference to diagnosis in infants given an accuracy of 80% and the increased radiation risk associated with computed tomography [13,14], whereas findings on a plain radiography are often non-specific [15]. As demonstrated in our patient, in adults however computed tomography is very valuable in diagnosis with a diagnostic accuracy of approximately 80% [13]. In addition to the typical appearance of the right-sided duodenal–jejunal junction and displacement of the ileocaecal junction into epigastrium, a twist of the intestine around the SMA resulting in a so-called ‘whirlpool’ appearance can be an important diagnostic clue as shown in the current case (Fig. 1) [16]. Furthermore, abdominal computed tomography with contrast medium may reveal an abnormal position of the SMV and SMA consisting of a vertical relationship and a left– right inversion, with the vein located abnormally to the left of the artery (Fig. 1) [17]. However, the latter is not specific for malrotation and may be a normal anatomical variation or lacking in malrotation [18]. The uncinate process of the pancreas had a normal appearance in our patient, yet might be absent or underdeveloped in malrotation too [9]. The same accounts for the absence of a retroperitoneal location of the third portion of the duodenum on computed tomography [19]. Although surgical treatment was warranted in our patient because of a symptomatic volvulus and duodenal obstruction, in consultation with patient and patient’s next of kin it was decided not to perform surgery considering his co-morbidity. The Ladd procedure, first described in 1936 by Ladd [20], is the cornerstone of treatment today, consisting of division of Ladd’s bands, correction of the intestinal twist, inspection of and cutting the small bowel mesentery. An additional appendectomy during the Ladd procedure is indicated as an increased risk of damage to the appendiceal vessels during the procedure is observed and the abnormal position of the appendix might cause a delay in diagnosing appendicitis in the future. As most symptoms associated with midgut malrotation occur in childhood, surgical intervention in adults is rarely studied, yet the Ladd procedure has a favourable long-term complication and recurrence rate in children [21]. A laparoscopic technique has a comparable safety and complication profile as compared to open surgery [22], but less suitable in case of volvulus and commonly performed in malrotation-associated duodenal obstruction [6]. In absence of intestinal ischaemia, detorsion might be an alternative treatment option; however, there is a risk of recurrence. Also, a non-surgical intervention with nasoenteric tube placement has been suggested as a successful treatment option in case of duodenal obstruction with volvulus in pregnancy to prevent recurrent rotation of the gut [23]. In contrast to symptomatic patients, a prophylactic Ladd’s procedure in asymptomatic malrotation is still under debate. Some study groups do not recommend prophylactic surgery considering the inappreciable low risk of intestinal volvulus or only in children as the beneficial effect does not balance the risk of postoperative complications in adults. On the contrary, others advocate surgical intervention in all cases due to the potentially life-threatening complications of malrotation that can occur at any age [24,25]. In conclusion, midgut malrotation is very rare in adults and mainly presents with chronic non-specific abdominal complaints. Therefore, it may easily be misdiagnosed beyond infancy with

Please cite this article in press as: Tack GJ et al. An unusual cause of duodenal obstruction in adults. Arab J Gastroenterol (2015), http://dx.doi.org/10.1016/ j.ajg.2015.03.002

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subsequent delay in diagnosis. Early recognition and treatment are of utmost importance and can be life saving. Consent Written informed consent was obtained from a relative (daughter) of the patient for publication of this case report and any accompanying images. List of abbreviations SMA, superior mesenteric artery; SMV, superior mesenteric vein. Authors’ contributions G.J.T. drafted the manuscript. J.P.K. and B.J.K. critically read the manuscript from a gastroenterologist’s and radiologist’s point of view, respectively. All authors read and approved the final manuscript. Competing interests The authors declare that they have no competing interests. Acknowledgements None. References [1] Lohman AHM, Ten Donkelaar HJ. De buik. In: Ten Donkelaar HJ, Lohman AHM, editors. Klinische anatomie en embryologie. Maarssen: Elsevier; 2013. p. 336–42. [2] Millar AJ, Rode H, Cywes S. Malrotation and volvulus in infancy and childhood. Semin Pediatr Surg 2003;12(4):229–36. [3] Dietz DW, Walsh RM, Grundfest-Broniatowski S, Lavery IC, Fazio VW, Vogt DP. Intestinal malrotation: a rare but important cause of bowel obstruction in adults. Dis Colon Rectum 2002;45(10):1381–6. [4] Torres AM, Ziegler MM. Malrotation of the intestine. World J Surg 1993;17(3):326–31. [5] Gamblin TC, Stephens Jr RE, Johnson RK, Rothwell M. Adult malrotation: a case report and review of the literature. Curr Surg 2003;60(5):517–20.

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Please cite this article in press as: Tack GJ et al. An unusual cause of duodenal obstruction in adults. Arab J Gastroenterol (2015), http://dx.doi.org/10.1016/ j.ajg.2015.03.002

An unusual cause of duodenal obstruction in adults.

Intestinal malrotation refers to a spectrum of anomalies of midgut rotation and fixation at various stages during early embryonic development. In adul...
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