Anatomical challenge: a rare coexistence of caecal cancer and isolated duodenal malrotation
- Akinori Sekioka ,
- Kunihiko Tsuboi ,
- Yoko Shono and
- Yukito Adachi
- Department of Gastroenterological Surgery, Saiseikai Noe Hospital, Osaka, Japan
- Correspondence to Dr Akinori Sekioka; akinori-sekioka@noe.saiseikai.or.jp
Abstract
Intestinal malrotation is a congenital anomaly, treated mostly during childhood. A small number of cases are incidentally found in adulthood, during operation for other abdominal diseases, such as colon cancer.
Here, we present a case of caecal cancer with isolated duodenal malrotation, a subtype of intestinal malrotation, discovered incidentally during the operation for the cancer. Although the anatomical abnormality made the operation more complicated, laparoscopic resection was safely performed with oncologically adequate lymphadenectomy, owing to intraoperative confirmation of anatomy and careful dissection.
Background
Intestinal malrotation is a congenital anomaly found in 1 to 200–6000 births.1 2 Most of the cases are discovered and treated within 1 year of birth, showing typical symptoms such as vomiting, caused due to volvulus or stricture.2 Some adult cases present with symptoms related to chronic intestinal passage failure or volvulus, while others are discovered incidentally during abdominal surgery for other diseases, often confusing surgeons because of the anatomical abnormality.2 3
In this report, we present a rare combination of caecal cancer with isolated duodenal malrotation, a subtype of intestinal incomplete malrotation. The malrotation was incidentally found during the operation and required careful adhesiolysis and resection because of the anatomical complexity.
Case presentation
A woman in her 80s (150 cm and 40 kg) was referred to the emergency department of our hospital with abdominal pain, which had worsened over the last 2 days. Her general condition was stable, while the abdominal examination revealed mild to moderate lower abdominal pain without panperitoneal irritation. The patient had no previous history of chronic abdominal symptoms.
Investigations
Contrast-enhanced CT revealed stricture of the ileocaecal valve, with ileal dilation (figure 1A,B). Laboratory results showed no inflammation but revealed tumour marker elevation: white cell count 6300/µL, C reactive protein 0.37 mg/dL (reference range 0–0.14), haemoglobin 13.1 g/L (reference range 11.3–14.9), blood urea nitrogen 12.0 mg/dL (reference range 8–20), creatinine 0.78 mg/dL (reference range 0.46–0.79) and carcinoembryonic antigen 10.2 ng/mL (reference range 0–5.0).
CT showing stricture of the ileal end (arrow heads) and the dilated ileum (arrow). (A) Axial CT and (B) coronal CT.
Treatment
After emergent admission, the patient underwent transnasal ileus tube placement for decompression of intestine (figure 2A,B). After efficient decompression, laparoscopic ileocaecal resection was performed. Intraoperative findings showed abnormality of the midgut, duodenum and intestine. The edge of duodenum was found near the root of superior mesenteric artery (SMA), running caudally without passing Treitz ligament (figure 3A–D). Lymphatic resection was started by resecting ileocaecal artery (ICA); however, duodenum or pancreas was not found at the dorsal space of ICA. By revealing superior mesenteric venous (SMV) and a branch of ileocaecal vein, D3 lymphatic resection was completed. At this moment, a loose layer was revealed, which was the fusion fascia between colonic mesentery and intestinal mesentery. Tracing this layer cranially, first and second part of duodenum was identified (figure 4A,B). A lateral approach showed duodenum running dorsally, covered with ascending colon, colonic mesentery and Ladd’s bands (figure 5A,B). The adhesiolysis of this part and resection of the Ladd’s bands enabled identification of the complete duodenum and mobilisation of ileocaecal colon (figure 5C,D). After resecting the ileocaecal part outside the body, side-to-side anastomosis was performed. The operative time was 173 min, and blood loss was 17 mL.
Abdominal X-ray image showing the ileus tube. (A) On the day of the placement of ileus tube, duodenojejunal flexure is unclear (arrow heads). (B) Two days after the placement of ileus tube.
Intraoperative images around the root of SMA. (A) The IVC and aorta can be directly observed, not covered by the jejunum or jejunal mesentery. (B) Schematic image of figure 3A (drawn by Akinori Sekioka). (C) On pulling the intestinal mesentery to the right side of the patient, a part of the duodenum was revealed, attached behind the mesentery. (D) Schematic image of figure 3C (drawn by Akinori Sekioka). Ao, Aorta; Du, duonenum; IVC, inferior vena cava; SMA, superior mesenteric artery.
Intraoperative image after resecting the ICV and ICA, manoeuvring the right mesocolon from the tissue behind it. (A) Blunt dissection by laparoscopic forceps between the right mesocolon and duodenum. (B) Schematic image of figure 4A (drawn by Akinori Sekioka). The area of two-headed arrow is the jejunal mesentery. Du, duodenum; ICA, ileocaecal artery; ICV, ileocaecal vein.
Intraoperative image showing lateral approach. (A) The intestine from the duodenum and jejunum was covered with the Ladd’s bands. (B) Schematic image of figure 5A (drawn by Akinori Sekioka). The area of two-headed arrow is the Ladd’s bands. (C) After resecting the Ladd’s bands. (D) Schematic image of figure 5D (drawn by Akinori Sekioka). The intestine from duodenum to jejunum was revealed and straightened.
Outcome and follow-up
The postoperative X-ray showed a straightened duodenum (figure 6). The ileus tube was removed on postoperative day (POD) 2, and oral intake was started on POD 3. The postoperative course was uneventful, and the patient was discharged on POD 9. In the pathological findings, there was a stricture of the ileocaecal valve with T3 adenocarcinoma, moderately differentiated, N1 (metastatic paracolonic lymph node); stage IIIB (the UICC TNM staging, eighth edition).
Abdominal X-ray image after the operation. Transition area from duodenum to jejunum passed caudally without kinking (arrow heads).
Capecitabine was administrated as adjuvant chemotherapy. After 6 months of surgery, there were no abdominal symptoms or cancer recurrence.
Discussion
Intestinal malrotation is mostly diagnosed and treated in childhood, with 30%–90% of the cases being symptomatic during infancy.1 3 4 It is rarely diagnosed in adulthood, presenting as intermittent or chronic abdominal pain due to stricture or volvulus. Very rarely, it is revealed during operation for other abdominal diseases.2–4
There are four major types of intestinal malrotation: type 1—non rotation, type 2—incomplete malrotation, type 3—reverse rotation and type 4—anomalous fixation of the mesentery.1 5 6 Type 1 is the most common, found in 2 per 1000 cases of upper gastrointestinal contrast studies.7 Previous studies have reported a lower incidence of type 2 malrotation than the other types, probably because it is unfamiliar to clinicians, includes several subtypes and is less apparent on radiological and intraoperative findings than type 1 or type 3.2 5 8 When found along with colonic cancer, studies have reported either type 1 or type 3 malrotation.9 10
In the present case (figure 7), intestinal malrotation was classified as type 2; more specifically as isolated duodenal malrotation.2 Isolated duodenal malrotation has been rarely reported, although without any detailed clinical description.11 We could not identify the isolated duodenal malrotation preoperatively, as the caecum and ascending colon were situated in the right abdomen, and the dislocation of SMA and SMV was not confirmed. Thus, type 2 malrotation would more commonly be found incidentally during operation for other diseases, thereby confusing the surgeon.
Schematic image of the position of the gut in the patient (drawn by Akinori Sekioka).
Anatomically, cases of intestinal malrotation with malignant diseases can be surgically challenging.12 13 The anomaly of the vessels or mesentery can lead to damage of the mesenteric vasculature during tumour resection or lymphadenectomy. In the present case, a part of the duodenum and jejunum was craniocaudally sandwiched by the colonic mesentery and the retroperitoneal plane, which could have been damaged by inadequate lymphatic resection. Careful adhesion could clarify adequate fusion fascia so that safe and oncologically reasonable resection was completed.
To the best of our knowledge, this is the first report of isolated duodenal malrotation combined with caecal cancer. Although the patient did not have symptoms of malrotation, treatment for caecal cancer required the manoeuvre of ileocaecal region, including resection of the Ladd’s bands. Also, isolated duodenal malrotation is a risk factor for right paraduodenal hernia; therefore, resection of the Ladd’s bands is valid in this case.11 14
Learning points
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Intestinal incomplete malrotation is radiologically less apparent than non-rotation or reverse rotation.
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A combination of caecal cancer and isolated duodenal malrotation can be an anatomical challenge for cancer resection.
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In the present case, a part of the duodenum, jejunum and mesentery was sandwiched between the right-sided colonic mesentery and the retroperitoneal plane.
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If intestinal malrotation is discovered during operation for another disease, intraoperative confirmation of the anatomical abnormality and careful dissection are crucial for a successful outcome.
Ethics statements
Patient consent for publication
Footnotes
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Contributors AS: written the abstract, case details and discussion. YS: made substantial contributions to acquisition of data, conception and design. KT: participated in drafting the article and revised it critically. YA: framed the manuscript and helped in writing the discussion.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
References
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