Rectal cancer with situs inversus totalis and previous malignant middle cerebral artery infarction: a case report and review of the literature

Abstract

Background

Situs inversus totalis is a rare congenital condition where there is complete inversion of thoracic and abdominal organs. There is limited information on the laparoscopic treatment of rectal cancer in patients with situs inversus totalis accompanied by other underlying conditions.

Case presentation

We report the case of a 61-year-old Chinese male with rectal cancer accompanied by situs inversus totalis, malignant middle cerebral artery infarction, stenosis of the right internal carotid artery and anterior cerebral artery, and diabetes who underwent a successful laparoscopic radical resection of rectal cancer. We discuss the diagnosis, treatment, and considerations for patients with situs inversus totalis accompanied by other underlying conditions. We performed relevant inspections, organized multidisciplinary team discussions, and made sufficient perioperative preparations. The patient had no intraoperative complications and was successfully discharged from the hospital. Pathological stage was T3N0M0, and the patient did not develop tumor recurrence or metastasis in the 24-month follow-up period.

Conclusions

We conclude that patients with rectal cancer with situs inversus totalis, even when accompanied by underlying diseases, can undergo surgical treatment with adequate preparation, and surgical treatment is the most effective treatment method.

Background

Situs inversus totalis (SIT), also known as complete inversion of thoracic and abdominal organs, indicates that all internal organs in the thoracic and abdominal cavity are in the opposite position compared with those in normal individuals. Resembling a mirror image, it is also referred to as “mirror face person.” This congenital disease is relatively rare. While it generally does not cause organ function defects or affect daily life, it can significantly impact the diagnosis and surgical treatment of visceral diseases [1]. Recently, there has been an increase in the number of younger patients suffering from neck and cerebral vascular stenosis, cerebral blood supply insufficiency, cerebral infarction, and hemiplegia [2].

Malignant middle cerebral artery (MCA) infarction is a severe and devastating ischemic stroke. It is characterized by an ischemic lesion area that is greater than 50% of the MCA area and typically invades the adjacent vascular region [3]. The early mortality rate for malignant MCA infarction is very high, with the main factor being cytotoxic edema, which has a devastating impact on the brain’s midline structure [4, 5]. The mortality rate is between 1/4 and 4/5, and surviving patients often experience severe neurological sequelae [6, 7].

Currently, patients with SIT and underlying diseases undergoing surgical treatment are scarce [8]. To date, no reports of patients with SIT accompanied by fundamental diseases undergoing laparoscopic rectal cancer surgery and successful discharges are available domestically or internationally. Here, we present a case of rectal cancer with SIT, accompanied by malignant MCA infarction, neck artery stenosis, and diabetes. Laparoscopic radical resection of rectal cancer was performed in November 2020, and as far as we know, this is the first case of rectal cancer related to SIT in China with underlying diseases. We also conducted a review and analysis of relevant literature and discuss the diagnosis, treatment, and considerations for this patient.

Case presentation

A 61-year-old Chinese male was admitted to the hospital for 1 week owing to abdominal distension, mucous pus, and blood.

Past history: The patient had a massive cerebral infarction in 2018, which resulted in left leg paralysis and urinary incontinence. The patient has been on a long-term medication regimen, including aspirin and atorvastatin. He also had a history of diabetes, managed with oral metformin for 2 years.

Physical examination: The apical pulsation was located in the fifth intercostal space at the center of the right clavicle. The muscle strength of the left lower limb was level 0. There was abdominal distension. The anal examination showed no abnormal mass, and there was blood stains on the gloves, showing a dark-red color.

Laboratory examination: moderate anemia (hemoglobin 72 g/L); platelets: 470 × 10⁹/L; blood glucose: 6.52 mmol/L; Cancino-embryonic antigen (CEA): 3.87 ng/mL; Carbohydrate Antigen 199 (CA19-9): 21.95u/ml.

CT scans of head, chest, and abdomen: Multiple cerebral infarctions, left–right inversion of chest and abdominal organs, abnormal spleen morphology, and rectal space occupation were observed (Fig. 1A–D). CT angiography (CTA) of the head and neck revealed multiple sclerosis signs of carotid artery and cerebral artery, multiple stenosis of different degrees, severe stenosis of the right internal carotid artery, and right anterior and middle cerebral artery (Fig. 1E).

Fig. 1

Images of CT examination, enteroscopy, surgery, and histopathological examination. A CT showed that the heart was located on the right side; B CT showed inversion of abdominal viscera and spleen malformation; C CT showed cerebral infarction; D CT showed rectal tumors; E CT angiography showed severe stenosis of the carotid artery on angiography; F Enteroscopy revealed a rectal tumor; G Colonoscopy and histopathological examination; H The appendix seen during the operation is located on the left side; I: Colorectal blood vessels seen during surgery; J Surgical stoma and postoperative recovery; K Postoperative pathological examination

Enteroscopy: Irregular, brittle, and hard masses at a distance of 15 cm from the edge of the anus were observed, causing intestinal stenosis. Colonoscopy could not be thoroughly examined. Pathological diagnosis: adenocarcinoma (Fig. 1F, G).

Color Doppler ultrasonic examination: Mirror dextrocardium, with a small amount of tricuspid regurgitation.

Preoperative diagnosis: rectal cancer with incomplete intestinal obstruction (cT3N0M0), cerebral infarction, anemia, hemiplegia, severe carotid artery stenosis, severe cerebral artery stenosis, diabetes, and SIT.

Treatment

The multidisciplinary team (MDT) discussed the patient’s case prior to the surgery. As the patient had rectal cancer and incomplete intestinal obstruction, it was decided to perform surgical treatment. The surgical approach chosen was laparoscopic radical resection of rectal cancer (Hartermann). The patient’s anemia was corrected through blood transfusion treatment before surgery. Low-molecular-weight heparin calcium (IH Q12H) was used instead of aspirin, and was discontinued 24 h before surgery.

Before anesthesia, the internal jugular vein puncture and radial artery puncture were conducted to monitor the patient’s dynamic blood pressure. A rapid infusion of 300 ml of liquid was administered before anesthesia induction. During the operation, the infusion rate was adjusted on the basis of the patient’s blood loss, blood pressure levels, and central venous pressure. Vasoactive drugs were administered if necessary to maintain blood pressure at 110/70 mmHg or above.

The surgeon and assistant were positioned in the left–right mirror image of the normal position. During the laparoscopic exploration, the liver, stomach, spleen, and colon were in a mirror-image position of their normal anatomy. Further exploration revealed that all of the abdominal organs were inverted, with the appendix located in the left iliac fossa (Fig. 1H). The sigmoid colon was connected to the intrapelvic rectum in the right lower abdomen, and the tumor was identified at the junction of the upper rectal segment and the sigmoid colon, measuring approximately 6 × 4 cm² and invading the serous layer.

Distal sigmoid colon and proximal rectal lumen were dilated, the intestinal wall was edematous, and distal rectal lumen was empty. The inferior mesenteric artery was exposed, and 253 groups of lymph nodes were dissected. No descending colon artery was found during exploration, and the first sigmoid colon artery was preserved. The superior rectal artery was ligated and cut off, and the other sigmoid arteries were ligated and cut off in turn (Fig. 1I).

The rectum was removed 5 cm away from the distal end of the tumor. A small incision, approximately 5 cm long, was made in the right lower abdomen. The proximal rectum and sigmoid colon were then pulled out, with the sigmoid colon being removed 10 cm away from the upper edge of the tumor and sigmoidostomy performed (Fig. 1J). The specimen was sent to the pathology department.

During the whole operation, the patient’s blood pressure was maintained (115–125/70–80 mmHg), their heart rate was 55–65 beats per minute, and there was 40 ml of intraoperative bleeding. Following the operation, the patient was observed in the intensive care department and started using low-molecular-weight heparin calcium (ihq12h) on the first day post operation, They were then transferred to the general ward 2 days later. The patient’s recovery was similar to that of ordinary patients, and he was discharged smoothly. Postoperative pathological diagnosis was moderately differentiated adenocarcinoma of rectum (T3N0M0) (Fig. 1K). After discharge, we followed up the patients for 24 months and found no tumor recurrence or metastasis.

Discussion

We entered relevant terms such as situs inversus totalis and colorectal cancer into search engines such as CNKI, Wanfang Data, PLOS Public Science Library, and PubMed. From January 2011 to December 2022, a total of 23 articles were found about colorectal cancer with SIT, including 3 in Chinese and 20 in English. In these literature reports, only one report was associated with an underlying disease (chronic obstructive pulmonary disease), where the patient died of pneumonia after operation [8]. Another article found postoperative complications: slight lymphatic leakage [9] (Table 1).

Table 1 Summary of cases with complete transposition of viscera combined with colorectal cancer

According Wood and Blaclock, organ reversal was first discovered and proposed by Fabricius in 1600 [10]. SIT belongs to a type of organ displacement where the anatomical position of all internal organs in the chest and abdominal cavity is completely opposite to that of normal people. Like the image in the mirror, SIT is also called a “mirror face person”, and it is a rare congenital malformation. There is no accurate figure for the incidence of SIT, but according to literature reports, it is about 1:25,000 to 1:6500 [11]. There is no complete scientific basis for the cause of SIT, but some experts believe that it is related to a mutation in a parent gene [12]. SIT exhibits recessive inheritance, with a genetic probability of only 1 in 100,000, with the proportion of males being relatively high, at about 1.5:1 [13].

While the internal organs of patients with SIT have the opposite anatomical position compared with normal people, their function is unaffected, so simple SIT does not require special treatment. However, the variation of blood vessels and organ malformations in SIT patients are several times higher than those in normal people. When a patient with SIT requires surgical treatment for a disease, their inherent organ anatomy abnormality will bring many difficulties regarding diagnosis and the treatment process [1]. This patient is a special case of rectal cancer with SIT, absence of the descending colon artery, spleen malformation, cerebrovascular disease, and malignant MCA infarction.

With the change of people’s living habits and diet structure, more and more patients have atherosclerosis, plaque formation, and stenosis. The number of patients with severe cerebral ischemia and even cerebral infarction and hemiplegia is increasing, and they are becoming younger and younger. There is also a significant increase in patients with perioperative cerebral infarction. The incidence of cerebral infarction during colorectal surgery is approximately 0.32% [14, 15]. Patients with cerebrovascular diseases and cerebral infarction during surgery often experience difficulties due to abnormal coagulation function after long-term use of anticoagulants.

Patients with malignant tumors have malignant tumor-related hypercoagulability, and some patients will have Trousseau syndrome, which may further cause recurrence and recurrence of malignant MCA infarction [16].

It is estimated that 15% of cancer patients will have thromboembolic events during the course of the disease, and up to 50% of patients have evidence of venous thromboembolism at autopsy [17].

Returning to this case, the patient was hospitalized owing to massive cerebral infarction in 2018, and developed left limb paralysis and incontinence. The patient was considered to have malignant MCA infarction after CT and head CTA examination in this hospitalization. Although the patient had SIT, no chronic sinusitis and bronchiectasis were found, so it did not conform to the characteristic clinical triad of Kartagener syndrome. This is the first report of a patient with SIT and malignant MCA infarction undergoing laparoscopic radical resection for rectal cancer. This patient with rectal tumor presented with incomplete intestinal obstruction and anemia, and there is an absolute indication for surgery. Although this patient was accompanied by whole organ inversion and malignant MCA infarction, it was not an absolute contraindication to surgery.

The congenital visceral malformation of SIT will bring new challenges to the operation. Patients with SIT are prone to anatomical abnormalities such as ectopic blood vessels and organ malformations. The patient had rectal cancer with malignant MCA infarction and SIT. The patient underwent enhanced CT examination before operation with confirmed spleen deformity and absence of descending colon artery during operation. The operator is greatly affected by the abnormal anatomy, and one needs to handle the blood vessels carefully. Therefore, to avoid sigmoid ischemia, we reserved the first sigmoid artery from the inferior mesenteric artery. In addition, the standing position of surgeons is affected by total organ inversion in the patient. Under normal circumstances, the operator is on the right side of the patient, which is suitable for surgical operation; Patients with total organ inversion deformity are more suitable for doctors with left-hand dominance. Although the surgeon is located on the left side of the patient after adjusting the position, which is helpful for the surgeon, there are still some difficulties. Especially for this patient, there is a risk of recurrent malignant MCA infarction.

Patients who need surgery, especially those with a complicated medical history, require adequate preoperative examination, evaluation, and preparation. For this patient with rectal cancer, SIT, diabetes, and severe cerebrovascular disease taking aspirin anticoagulation therapy, monitoring vital signs during operation, maintaining hemodynamic stability, and ensuring cerebral perfusion are crucial to minimize the risk of cerebral infarction. To achieve this, effective communication between the perioperative surgeon and anesthesiologist is essential, as well as establishing a comprehensive detection system, and fluid infusion channel for the patient. Insulating the head and neck to avoid vasospasm due to low temperature and providing rehydration before anesthesia to supplement blood volume can prevent complications. Moreover, vasoactive drugs such as dopamine and norepinephrine can correct blood pressure fluctuations, and blood transfusion and fluid replacement can be given at any time according to intraoperative bleeding, exudation, blood pressure, and central venous pressure. Reducing trauma, pollution, and bleeding is also important, and the systolic blood pressure of the patient must be maintained during the whole operation.

Conclusion

Although this case of rectal cancer was accompanied by underlying diseases and total organ inversion malformation, it is also enlightening. It raises awareness that surgical procedures for patients with special circumstances require thorough preparation. Consequently, a patient with rectal cancer with SIT, even when accompanied by underlying diseases, can undergo surgical treatment with adequate preparation, and surgical treatment is the most effective treatment method.