Three-dimensional reconstruction simulation assisted pancreaticoduodenectomy forpancreatic head carcinoma in a patient with situs inversus totalis: Report of a rare case

Authors

• Xingjian Zhang, Peng Du, Yong Li Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi 330006, China

Abstract

Situs inversus totalis (SIT) is a rare congenital malformation that refers to a complete reversal of the thoracic and abdominal viscera. An extremely small number of SIT patients with pancreatic head cancer have been reported. Surgical procedures in patients with SIT are more complicated and technically difficult due to the rearrangement of anatomical structures. Adequate and meticulous preoperative assessment of tumor regional anatomy, potential cardiovascular anomalies, and aberrant blood vessels by imaging examinations is critical for facilitating the safe and effective performance of the surgery. The current study reports the case of a 69-year-old female with pancreatic head cancer and SIT, and first focuses on the application of 3D reconstruction simulation assisted pancreaticoduodenectomy (PD) in patients with SIT. Based on the data of CT images, we reconstructed a real-time 3D simulation model with Myrian XP to assist with our surgical planning. Then, we performed smoothly PD with complete resection of the tumor. The patient was discharged without any serious complications. Follow-up has been 2 years and the patient is doing well with no recurrences.

I ntroduction

Situs inversus totalis (SIT) is a rare congenital malformation, which manifested as a complete left-to-right reversal of the thoracic and abdominal viscera compared to their usual positions. The reported incidence rate ranges from 1:8000 to 1:25000 . Although no evidence shows this condition does increase the risk of developing cancer, cases of SIT combined with tumors have been reported . The coexistence of SIT and pancreatic head cancer is extremely rare. Reviewing the literature, the first case of pancreatic head carcinomacoexistent with SIT whounderwent pancreatoduodenectomy (PD) was reported by Sakaguchi in 1985 . Up to now, only 7 cases of successful PD in SIT patients with pancreatic head carcinoma have been documented [3-9]. The performance of PD in patients with SIT is more complicated and technically difficultdue to the rearrangement of anatomical structures.Adequate and meticulous preoperative assessment of tumor regional anatomy, potential cardiovascular anomalies, and aberrant blood vessels by imaging examinations is critical for facilitating the safe and effective performance of the surgery.

To the best of our knowledge,diagnostic imaging techniques such as computed tomography (CT), magnetic resonance imaging (MRI), and related three-dimensional (3D) reconstruction were applied to preoperative evaluation in previously documented cases of PD in SIT patients [7-9], but there is no report of 3D reconstruction simulation assisted PD for the pancreatic head carcinoma concurrent with SIT. Regarding the relative positions of tumor,invasion of superior mesenteric vasculature and other adjacent organs, 3D reconstruction has advantages to provide more detailedinformation and intuitive sense compared with two-dimensional (2D) CT, increasing the safety of the operation and the resection rate of tumor [10, 11].

The present study reports a case of SIT patient with pancreatic head carcinoma, and first focuses on the application of the 3D reconstruction simulation assisted PD in patientswith SIT.

CasereportA 69-year-old femalewith known SITadmitted to our hospital withepigastricpain and jaundice for 10days.There were no past medical history and no familyhistory ofSIT and tumors.Investigations revealed cancer of pancreatichead with SIT. Laboratory examination showed alanine transaminase (ALT), aspartate transaminase (AST) and total bilirubin (TBIL) were 106 U/L, 102 U/L and 142.2 μmol/L, respectively, which were all within a normal range. Serum CA19-9 was 191.6 U/mL, which was more than 7 times higher than the normal upper limit. Chest radiography showed mirror-image dextrocardia. Endoscopic retrograde cholangiopancreatogram (ERCP) showed stenosis at the junction of duodenal bulb and descending part of the duodenum, and the duodenoscope failed to pass through this junction.

Figure2. The 3D reconstruction simulation system showed the relationship between tumor and peripheral vessels from different angles (A: anterior angle, B: left angle, C: right angle and D: horizontal angle).

Figure3. The 3D reconstruction simulation system showed the pre tangent plane and the length between pancreatic tumor resection line and SMV (super mesenteric vein) from posterior and anterior angles (A and C are posterior angle, B and D are anterior angle).

Figure4. Intraoperative findings and postoperative pathological results. (A) Anatomy after tumor resection (PV, portal vein; CBD, common bile duct; SPV, splenic vein; SPA, splenic artery).(B) Tumor specimen: green arrows mark the cystic part of the tumor and white arrow marks the solid part of the tumor.(C) The pathology of tumor(H&E staining; magnification, x400). (Dand E)Immunohistochemical examination of tumor：specimens stained weakly positive for CEA (D) and P53 (E) (magnification, x100).

Discussion

The first case of SIT was reported by Fabricus in 1600 . Although this anomaly does not affect normal health or longevity and often diagnosed incidentally during check-up examinations, SIT can be accompanied by cardiopulmonary malformations, digestive system abnormalities, and a variety of respiratory and urologic anomalies [12]. Typically, the risk of cardiovascular anomalies of patients with SIT is 10 times greater than normal individuals . Moreover, approximately20-25% of individuals with SIT have concurrent chronicsinusitis and bronchiectasis, termed as Kartagener syndrome . There was neither Kartagener syndrome nor obvious anatomic abnormalities except forSIT in our case.The exact etiology of SIT during embryonic development remains unclear. Some studies suggested that chromosomal abnormalities might lead to a reversal of right-left polarity during early embryonic development [14, 15]. However, other researches showed that the role of mechanical nodal fluid flow may be associated with left-right patterning [16, 17]. Furthermore, some studies suggested that the KIF3 complex, cell-adhesion factor N-cadherin and beta-catenin may be high-risk factors for tumors in subgroup of SIT, such as lung cancer or gastric cancer [18, 19], but pancreatic cancer concomitant with SIT was not reported before. The coexistence of SIT and pancreatic head cancer is extremely rare. To date, only seven cases of pancreatic head cancer with SIT have been reported, and the present report emphasizes the eighth case, as shown in Table 1.

The PD is still the curative treatment for pancreatic head cancer since it was performed by Whipple in 1935. With the development of surgical techniques and perioperative care, the mortality rate of PD has dropped to less than 5% in some centers [21, 22],however, the postoperative morbidity of PD remains as high as up to 50% and thereby is considered to be the main factor for long postoperative hospital stay and high cost for PD. Due to its high complexity of the surgical operation and postoperative morbidity, PD for the pancreatic head cancer can be very challenging for surgeons, especially unusual surgical field of vision with mirror-image anatomy in patients with SIT. We summarized an application of 3D reconstruction simulation for preoperative assessment assisted PD for the pancreatic head cancer patient concurrent with SIT.

Several factors affect the complete resection of pancreatic head tumor during PD, including invasion of superior mesenteric vein or portal vein, invasion of abdominal aorta or inferior vena cava, or other important adjacent organs. Some studies suggested that 3D reconstruction is an optimal method for preoperative assessment to reveal the tumor, vascular variance and tumor-induced vascular changes, which can improve the surgical quality and reduce surgical risk [10, 11, 24]. Multi-slice spiral CT has also 3D reconstruction function, which can be used for showing the pancreatic head tumor and peripancreatic vessels. But 3D reconstruction of CT can not clearly show the relationship between tumor and its adjacent vessels, and not provide the 3D, intuitive, multi-angle observation when the vessels are squeezed, oppressed or infiltrated by tumor or partially overlapped by tumor and surrounding organs, or anatomical abnormalities. Therefore, the assessments by the reconstruction of CT on whether pancreatic tumor can be resected are often unsatisfactory. A meta-analysis showed that the sensitivity and specificity of multi-slice spiral CT in the evaluation of the pancreatic head tumor resectability were generally 68% and 89%, respectively, and the sensitivity for the evaluation of vessel involvement was 68% . 3D reconstruction simulation system can reconstruct a model of tumor and its surrounding organs to reveal their exact relationship, which can improve the resection rate of tumor and reduce tissue injury.

As far as we know, there is still no literature reporting on the application of 3D reconstruction simulation in pancreatic head cancer concurrent with SIT. In the present study, we reprocessed the CT data of the pancreatic head cancer patient

with SIT by using the 3D reconstruction simulation system (Myrian XP).The pancreatic head tumor, pancreatic tissue, and peripancreatic vessels are marked in different colors, and the relationship between tumor and adjacent vessels is observed by 3D graphics zooming, rotation and transparency, which overcomes the limits of CT image. After 3D reconstruction, it showed clearly the location of tumor and its relationship with the vessels and adjacent organs from different angles in the SIT patient, which facilitates the formulation and implementation of surgical treatment. In our study, we amputated the tumor according to the pancreatic tumor resection line and the length between pancreatic tumor resection line and SMV, which was preoperative set by 3D reconstruction simulation during operation. we successfully removed the tumor with no tumor residue in the pancreatic stump. Therefore, we suggested that the method for the pancreatic tumor resection line is convenient and helpful to improve tumor resection rate and worthy of promotion, but the optimal length through which we can preserve normal pancreatic tissue as much as possible remains unknown.In conclusion, the assistance of preoperative assessment by 3D reconstruction of the imaging results is very important in facilitating the surgical treatment for SIT patient with pancreatic head cancer, which can improve the resection rate of tumor and reduce the risk of abdominal viscera damage, and should beroutinely performed when available.

Acknowledgment:

We would like to thank YeYuan Chen (Department of Radiology, The First Affiliated Hospital of Nanchang University) for the imaging data and description and Zhendong Zhang (Department of Pathology, The First Affiliated Hospital of Nanchang University) for pathological examination and immunohistochemical staining of surgical specimens

Authors' contributions

Peng Du, Xinjiang Zhang and Yong Liparticipated in the treatment of this patient and helped to draft the manuscript. Peng Du performed the data analyses and wrote the manuscript. Yong Li gave the final approval of the version to be submitted. All authors read and approved the final manuscript.Funding This study was supported by Natural Science Foundation for Youth of JiangXi Province, Grant Award Number: 20192BAB215012

Competing interests

There are no competing interests

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