Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy.

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The most important branches of abdominal aorta are the celiac trunk and the superior mesenteric artery, which represent almost the entire vascularization of the visceral territory of gastrointestinal tract [1]. They usually originate respectively from the aorta to the level of T12 and L1. In literature, this conformation is reported only in 87% of cases, so there are reported many variants [2]. In this work we describe a case of a child affected by Kabuki’s syndrome with an intestinal malrotation, carrier of an abnormal conformation of these arterial vessels, in association with a bilateral pelvic ectopia of kidneys.

A 6 year old girl, affected by a Kabuki’s syndrome, was admitted to our clinic for abdominal pain and bilious vomiting. Abdominal X-ray and ultrasound were performed and an intestinal occlusion was excluded. A nasogastric tube was placed. An amount of 500 cc of biliary secretion was evacuated in the first 24 hours, with improvement of clinical situation.  MRI and CT scan were performed in view of child having repeated similar episodes since birth, with the diagnostic suspicion of an aorto-mesenteric compass. Radiological images were processed with our V-render software and it showed the presence of a common origin of the celiac trunk and superior mesenteric artery. This is known as an anatomic variation called celiaco-mesenteric trunk. The mesenteric angle was smaller than normal, and it strengthened our suspicion. In addition, images revealed also the presence of a bilateral pelvic ectopia of kidneys. Renal arteries took origin from abdominal aorta at the level of the iliac bifurcation [Fig.1]. Two months later we performed a laparotomy, during which it was discovered an intestinal malrotation, instead of an aorto-mesenteric compass. Postoperative course was uneventful, feeding was started on fourth postoperative day, patient was discharged on 7th postoperative day.

The anatomy of the celiac trunk was firstly described by Haller in 1756. However, it is known that the classical conformation is not always present [3]. Many anatomical variants have been reported by Lipshutz in 1917, who firstly described a celiaco-mesenteric trunk (CMT) [4]. Among variants of celiac trunk, the CMT is the least frequent with an incidence of 1-2.8% [6,7]. In literature, this vascular malformation is reported in association with other anomalies affecting the common hepatic, the left gastric and the splenic arteries [8,9]. Most of papers about CMT, however, are related to series of autoptic and radiological studies in adults [10] because it is usually an asymptomatic anomaly. The association of CMT with a malrotation is to date not described in literature. However this relationship is important because it exposes patient to a risk of a massive volvulus. 

The involvement of celiac-mesenteric artery (CMA), indeed, results in necrosis of majority of bowel and of districts of celiac trunk that can have a dramatic impact on a child’s life and many long term catastrophic consequences. The aim of this paper is to describe an unique case of CMA associated with abnormal onset of renal arteries that have been well documented by imaging. However, it is important to underline that we made diagnosis of this anatomical variant only for the presence of malrotation. 

An adequate evaluation of radiologic images and the use of 3D reconstruction permits to diagnose anatomic variant. 

Contributors: EB, FM: manuscript writing, case management; RA: manuscript editing, case management; MA, MM, MM: critical inputs into the manuscript and literature search. EB will act as guarantor. All authors approved the final version of the manuscript.

Funding: None; Competing interests: None stated.