Situs inversus totalis patients with gastric cancer: Robotic surgery the standard of treatment?-A case report.

INTRODUCTION AND IMPORTANCE: Situs inversus totalis (SIT) is a very rare congenital condition. Situs inversus totalis (SIT) patients who present with gastric cancer have been reported in Japan, China, the United States, and other countries. China has a high incidence of gastric cancer, accounting for 40% of the global annual incidence. Surgical treatment options for situs inversus totalis (SIT) gastric cancer patients are of great concern due to the rare nature of the condition and the anatomical variations. This case aims to demonstrate the utility of robotic surgery in treating situs inversus totalis patients with gastric cancer. CASE
PRESENTATION: We report a 69-year-old male situs inversus totalis (SIT) gastric cancer patient who successfully underwent a DaVinci robotic-assisted distal gastrectomy with Roux-en-Y reconstruction. The patient had no complications after the operation and was discharged postoperative day 15. CLINICAL DISCUSSION: Gastric cancer is an aggressive disease that requires timely diagnosis and appropriate intervention. Unfortunately, many patients present late with gastric cancer and do not benefit from surgical or other appropriate interventions. Patients who are eligible for surgery however still need a clean marginal resection to maximize prognosis, which is not always possible due to complex anatomy or variations as seen in situs inversus totalis. DaVinci robotic surgery system is a new generation of minimally invasive operating systems after conventional laparoscopy, and its visual field clarity, operating flexibility, and instrument stability have obvious advantages over conventional laparoscopic surgery and traditional open surgery.
CONCLUSION: Robotic surgery for situs inversus totalis (SIT) patients is more advantageous than laparoscopic and traditional surgeries as it offers a broader view of the variant anatomy and allows optimum dexterity and clarity.

Introduction

Gastric cancer is the fifth most common cancer and the third most common cancer-related mortality worldwide [1]. About 700,349 patients died of gastric cancer out of an approximation of 933,937 men and women who were diagnosed with gastric cancer in 2002 [2]. China has a high incidence of gastric cancer, accounting for about 40% of the global annual incidence. Endoscopic resection is the main treatment of early gastric cancer. Surgery with D2 lymphadenectomy is the suggested curative-intent treatment for all patients with operable disease [1,3].

Situs Inversus totalis (SIT) is a very rare congenital anomaly that presents as a complete mirror image transposition of the thoracic and abdominal viscera.

The etiology of SIT is not yet understood but a few theories have been proposed:

It is related to a genetic anomaly that occurs in the second week of embryological development [4]. It may be related to the mutation of coiled-coil domains containing 1(CCDC11) [5]. It is transmitted as an autosomal recessive trait. The immobility of the nodal cilia inhibits the flow of extraembryonic fluid during embryogenesis leading to the formation of SIT [6].

No direct relationship between SIT and gastric cancer has been established but there have been few reported cases of SIT with gastric cancers worldwide [7]. We report an SIT patient with T3N3M0 gastric carcinoma. A robotic-assisted D2 distal gastrectomy was performed. This work has been reported in line with the SCARE criteria [8].

Case description

A 69-year-old male presented with a 3-day history of an abdominal space-occupying mass accompanied by melena for 5 days. The patient’s symptoms worsened, with accompanying bouts of dizzy spells, which made him seek medical treatment at his county hospital. The patient had a 40 pack-year smoking history. The patient had been diagnosed as situs inversus totalis (SIT) with no other significant past medical history. The patient had no significant drug history and family history. Physical examinations were unremarkable except for a positive murphy’s sign and a weight loss of about 6 kg since the onset of the disease. The patient received symptomatic treatment which is not very clear and a gastroscopy at the local county hospital. A gastroscopy report from the local hospital showed:

Nature of gastric ulcer to be pathological

Chronic superficial gastritis.

Endoscopy biopsy report ;(gastric antrum), moderately to well-differentiated adenocarcinoma.

After preoperative examinations, he was diagnosed with gastric cancer. His CT scan (Fig. 1) showed; dextrocardia, visceral inversion, transposition of the great blood vessels, small nodules in the lower left lung, mild chronic inflammation of the right lung, hepatic cyst, small cysts on both kidneys, and a thickened wall of the gastric antrum. A perioperative plan to undergo a robotic gastrectomy and adjuvant chemotherapy was made. The patient underwent a robotic-assisted distal gastrectomy on the 10th day of hospital stay by the professor of our center, assisted by an attending and a postgraduate student. The patient was put under general anaesthesia in a supine position. We then performed routine disinfection, draping, and established a pneumoperitoneum. Diagnostic laparoscopy confirmed a mirror transposition of thoracic and abdominal organs hence confirming SIT and also revealed the tumor was located near the lesser curvature of the posterior wall of the gastric antrum, with a size of about 3 cm × 3 cm. The robot arms were placed in their routine distal gastrectomy positions (Fig. 2). Considering the preoperative assessments and intraoperative exploration, a robotic-assisted radical gastrectomy (distal gastrectomy) and intestinal adhesiolysis was performed. Major vessels along with thoracic and abdominal organs were transposed as mirror images of the normal anatomy. A 7 cm long median incision on the upper abdomen (Fig. 3) was made to collect the specimen and complete the Roux-en-Y reconstruction after the robotic instruments were removed. The position of the lead surgeon and the first assistant were maintained just like any other routine distal gastrectomy procedure after undocking the robot. A drainage tube (Fig. 3) was placed in the lower hepatic line and duodenum stump near the anastomotic stoma. The duration of the entire operation was 3 h and 25 min, and intraoperative blood loss was 20 mL. There was no intraoperative complications. Postoperative fluoroscopy (Fig. 4) was normal and recovery days were uneventful, the patient passed gas and stool on the postoperative 13th and 14th days respectively. The patient had a total hospital stay of 26 days; 15 days postoperative hospital stay, and was discharged without any complications. The patient was seen 3 months postoperative and is doing fairly well without any complications. The patient is on a single drug post-operative chemotherapy regimen.

Fig. 1

Preoperative CT confirming situs inversus totalis with a slightly thickened wall of the gastric antrum. Left(non-contrast), right (with contrast).

Fig. 2

Scheme of trocars, robotic arms and accessory trocar for the assistant.

Fig. 3

Surgical sites and drainage site on post-op day 6.

Fig. 4

Left- image is postoperative fluoroscopy confirming smooth passage of contrast in the oesophagus with no filling defect. Right- image shows the remnant stomach is well filled with no obstruction in the cardia, no abnormality in the small intestinal anastomosis, no stenosis or fistula formed.

Post-operative pathology report (Fig. 5Fig. 5) indicated:

Fig. 5

Postoperative specimen and histology report.

Tumor site; distal stomach.

Pathological stage; T3N3aM0.

General type; ulcer type.

Tumor size; 2.5 × 2.0 × 0.6cm

Histological type; adenocarcinoma.

Histological grade; grade II-III.

Infiltration depth; subserosal layer.

Lauren classification; intestinal type.

Surgical margin invasion; no cancer involvement found in the surgical margin.

Regional lymph node involvement; metastatic cancer was found in lymph nodes (7/34),

Vascular and nerve invasion present.

Immunochemistry;HER-2(3+), P53(−), MLH1(+), PMS2(+), MSH2(+), MSH6(+), Ki-67(+about 80%).

Discussion

SIT patients with various cancers have been reported [9], however the first SIT patient with gastric cancer was reported by Allen [3] in 1936, and in 2012 the first of such patient received treatment [10]. While Kim et al. suggest a theory of no direct link between SIT and gastric cancer, there have relatively been quite a significant number of cases of SIT with gastric cancers [7]. Cao et al. postulate that SIT patients may develop cancer due to malfunction of the kinesin superfamily protein (KiF3) complex [9]. Approximately 60% of patients with SIT have other congenital anomalies of the gastrointestinal tract including biliary atresia, rotational anomalies, colon aganglionosis, splenic agenesis, small bowel atresia, and duplication [11].

Several papers have since then reported difficulties in offering surgical treatment to patients with SIT due to difficulty reaching structures due to the surgeon's position and the mirror transposition of organs [12], difficulties in the anatomical orientation hence causing confusion intraoperatively [13] and difficulty in confirming surgical anatomy as the surgical field is hard to see [14].

Even with the anatomical variations in SIT patients with gastric cancer, safe operative treatment can be offered with a more careful approach and paying much attention to the inverted anatomical structures intraoperatively [15]. Yinghao et al. [9] report that out of 15 SIT patients who underwent various surgical procedures, 9 had their surgeries performed with the surgeons taking their usual surgeon positions; with only 1 receiving robotic-assisted distal gastrectomy out of 15 gastrectomies.

In our case, we offered a robotic assisted D2 distal gastrectomy and hand sewn anastomoses with Roux-en-Y reconstruction. Roux-en-Y reconstruction was chosen because of its added advantages of improving postoperative quality of life due to less incidence of remnant gastritis, reflux esophagitis, dumping symptoms, and reflux symptoms. Roux-en-Y reconstruction also appears to be a better choice following distal stomach resection considering the long-term postoperative outcomes [16,17].

The pre-operative evaluation indicated that the tumor did not extend beyond the serosal membrane, and there was no local invasion or abdominal metastasis. CT confirmed the transposition of internal organs, which would most likely make the surgical procedure different from the normal routine. In a laparoscopic surgery setting, the first assistant needs to cooperate with the lead surgeon and they both depend on the second assistant who handles the laparoscope to visualize the surgical field which increases the error rate under unconventional conditions such as in our SIT patient with vessel anomaly. Robotic surgery can reduce the errors caused by the cooperation of multiple operators in laparoscopic surgery therefore, we chose Da Vinci robotic surgery.

There is a significant number of existing literature that establishes the safety of robotic surgery in the treatment of several surgical conditions including but not limited to gastric cancer even with variant anatomy, such as in situs inversus totalis.

Kim et al. concluded that robotic pancreticoduodenectomy has no negative impact on surgical and oncological outcomes in patients with totally replaced right hepatic artery [18].

Longer operation time, less intraoperative blood loss, significant increased mean number of retrieved lymph nodes in terms of oncological outcomes and a lower rate of surgical complication have been associated with robotic surgery [19]. Zong et al. in a meta analysis concluded that robotic gastrectomy may be a technically feasible alternative for gastric cancer because of its affirmative role in both subtotal and total gastrectomies compared with laparoscopic and open resections [20].

A variant anatomy will inevitably conflict with surgical habits and bring difficulties if extra care is not taken. It has been well established that encountering complex vascular anatomy increases the difficulty for surgeons and failure to identify variations during surgical procedures can result in unwanted bleeding [21]. Organ damage, particularly vascular damage has also been associated with variant anatomy [22]. In our case, a very careful and thorough observation was made to identify the organ, vessels, and tissue variations.

Knowledge in detail about normal anatomy and variation in the branching pattern of the celiac trunk is important in surgical, oncological, and radiological interventional procedures and must be taken into account to avoid possible complications [23].

The first assistant said, although this was his first case of SIT gastrectomy, no problems were encountered during the procedure. Although the duration of the procedure was longer than usual, the difference was not very significant. Surgical dissection of gastric cancer is a complex procedure with many vessels to be careful of. DaVinci robotic surgery system is a new generation of minimally invasive operating systems after conventional laparoscopy, and its visual field clarity, operating flexibility, and instrument stability have obvious advantages over conventional laparoscopic surgery.

Conclusion

Gastric cancer patients with SIT are very rare, therefore not much has been established as the ideal surgical procedure.

Robotic-assisted gastrectomy for SIT patients is very safe as it allows a wide range of dexterity and a broad view of the anatomical variations. With robotic gastrectomy, surgeon position is not a thing of concern and therefore should be encouraged in gastric cancer patients with difficult anatomy as seen in SIT.

Declaration of Competing Interest

The authors report no declarations of interest.

Sources of funding

The . Grant number 81871995.

Sponsors had no involvement in the collection, analysis and interpretation of data; in the writing of the manuscript; and in the decision to submit the manuscript for publication.

Ethical approval

The study is exempted from ethical approval.

Consent

Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Author contribution

Eugene Abbey: assisted in the procedure, literature review, corresponding author, writing-original draft.

Prof. Fu Yang: performed the procedure, supervision, project administration, guarantor, case description and discussion.

Liu Qi: assisted in the procedure, literature review, case description and discussion, edited the video of the procedure.

Jiang Jian Wu: literature review, case description and discussion.

Li Tong: literature review.

Zhang Zhen: literature review.

All authors read and approved the final manuscript.

Registration of research studies

Not applicable.

Guarantor

Prof. Fu Yang.

Provenance and peer review

Not commissioned, externally peer-reviewed.