Minimally invasive Mckeown esophagectomy with modified three-field lymphadenectomy in case of situs inversus totalis with carcinoma mid esophagus.

Performing complex surgical procedures in patients with situs inversus totalis is a challenge because of the need to orient to the completely mirror transposed abdominal and thoracic viscera. We report our experience in performing a three phase minimally invasive (thoracoscopic and laparoscopic) esophagectomy for carcinoma of the mid esophagus in a patient with situs inversus totalis. We believe that this is the first reported case of this kind.

INTRODUCTION

Situs inversus totalis (SIT) is rare congenital anatomic anomaly that is characterized by complete mirror transposition of abdominal and thoracic viscera. From a surgeon's eye, this represents a challenge to orient and perform complex surgical procedures. This is further accentuated in minimally invasive surgery. We share our experience in performing a three phase minimally invasive (thoracoscopic and laparoscopic) esophagectomy for carcinoma of the mid esophagus in the prone position. We believe that this is the first reported case of complete minimal invasive esophagectomy (MIO) in the prone position technique.

CASE REPORT

A 62-year-old gentleman with squamous cell carcinoma of the mid oesophagus (Stage T4aN1M0) was evaluated for MIO following neoadjuvant chemotherapy that had resulted in a good response. It is the institutional protocol to perform a minimally invasive Mckeown procedure with a modifed three-field lymphadenectomy.

Planning

The computed tomography findings of the anatomical variation had revealed a complete mirror image pattern of the viscera and vasculature in the abdomen and thorax [Figure 1]. It was hence decided that the patient would initially undergo a left thoracoscopic mobilization of the esophagus (Phase 1) in the prone position followed by laparoscopic gastric mobilization of the stomach (Phase 2) and finally a neck anastomosis (Phase 3).

Figure 1

Computed tomography images

Operative Technique

Patient position and set up

Patient was placed in the prone position on double-lung ventilation using a single lumen endotracheal tube was the standard approach. Operating surgeon, camera and surgical assistants stood on the left side of the patient with monitor on the right side. Abdominal part of the procedure was done in the supine position with split legs apart.

Thoracoscopic mobilisation of the esophagus (Phase 1)

An initial optical trocar (10 mm) was placed, and thoracoscopic inspection of the anatomy was done. A left sided azygos arch was initially noticed arching above the left main bronchus. Mediastinal pleura were opened using monopolor hook in a U and inverted U fashion above and below the azygos vein respectively. The azygos vein was isolated skeletonised and divided in between sutures [Figure 2]. A bulky mid esophageal tumor was noted.

Figure 2

Phase 1 of mobilization of esophagus with lymph nodes clearance

Infracarinal dissection [Figure 2] was commenced with en bloc clearance of the carinal group of lymph node and further dissection in the plane just posterior to the pericardium until the OG junction. The supracarinal dissection was followed by identifying the left vagus and following the same proximally until the left recurrent laryngeal nerve arching underneath the left subclavian artery. Lymph nodes in the left parathracheal and along the lateral reticular nucleus were cleared [Figure 2]. The aortopulmonary window was cleared of the lymph nodes and inferior dissection along the right side of the esophagus continued clearing the paraesophageal group of nodes on the right until the OG junction [Figure 2].

Laparoscopic gastric mobilization and conduit formation (Phase 2)

The patient was turned over to the supine position and ports were placed in an appropriate comparative configuration to the ports placed in the patient without situs inversus and were found to have mirror image of the normal anatomy [Figure 3]. After the division of gastrocolic omentum, trifurcation lymphadenectomy was done. The left gastric artery was clipped and divided and an en bloc lymph nodal tissue mass is left with the lesser curve. The tissue in the posterior aspect of the stomach up to the crus is cleared with a completely mobilized stomach from the hiatus to the pylorus [Figure 3]. The gastric conduit formed using Endo-GIA staplers that would form the neo esophagus was not completely detached from the remnant specimen to enable extraction through the cervical wound.

Figure 3

Phase 2 of gastric mobilization and conduit formation

Cervical part of the procedure (Phase 3)

The cervical part of esophagus was exposed via left collar incision with neck slightly extended and turned toward right in the supine position. Mobilized part of esophagus along with gastric tube retrieved in continuity. A side to side linear stapled esophagogastrostomy fashioned using a double stapling technique and the remainder of the specimen detached in the process.

Postoperative period and follow-up

The recovery period was uneventful, and enteral feed was started on 7th postoperative day and discharged on 11th day. The total operative time was 286 min. Histopathological report was suggestive of moderately differentiated squamous cell carcinoma (pT3 N1). Follow-up after 18 months remains uneventful.

DISCUSSION

Situs inversus totalis is a rare autosomal recessive condition that occurs in 1:5000-10,000 adults[1] which was first described in 1600 by Fabricius.[2] No definitive etiology has been established, and SIT itself has no pathophysiological significance.[3] Typically, persons with SIT have normal life expectancy, and the great majority of them are unaware of their unusual anatomy until they seek medical attention for an unrelated condition.

A surgeon may encounter a case of SIT needing surgery once or twice in a career because of its extremely low prevalence. Therefore, it is imperative that prior planning is made when considering the surgical technique and associated challenges anticipated. However, these anatomical abnormalities result in both diagnostic and surgical difficulties due to the resulting organ inversion and frequent concurrence of vascular and gastrointestinal anomalies. Preoperative radiological imaging becomes more important in such cases to determine the various arterial and venous anomalies.

The role of laparoscopic surgery in SIT for laparoscopic cholecystectomy was first reported in literature. Thereafter, reports and outcome have been reported in small number of cases especially advanced laparoscopic surgery. Few cases of laparoscopic gastrectomies and colectomies[4] are reported probably due to very low incidence and hesitancy to perform laparoscopic resections. Review of the world literature revealed that only three cases with SIT had undergone treatment for esophageal cancer. Mimae et al. reported a case of open esophagectomy,[5] Yoshida et al. and Yagi et al. presented a successful case of video-assisted thoracoscopic surgery esophagectomy with hand-assisted laparoscopic splenectomy gastric mobilization.[67]

The role of MIO is well established in various studies.[8] The technique of prone position MIO and its outcome is already well described by the author and co-authors in their earlier series.[9] MIO when compared to traditional open surgery scores significantly in decreasing pulmonary complications and results of mediastinal dissection and lymph node clearance is comparable to traditional open surgery.

Minimal invasive esophagectomy in the settings of SIT is particularly challenging due to difficult orientation and inherent morbidities of procedure itself affecting the outcome of patient. To our knowledge, our report would be the first patient with SIT and carcinoma oesophagus undergoing a complete MIO (thoracoscopic and laparoscopic approach) in the prone position with dual lung ventilation.

We did not have any significant intraoperative problems although operative time was 30-40 min longer than usual MIO that can be attributed to unaccustomed orientation of anatomy. Though there was no significant anatomic alteration except the mirrored visceral position in above case. With appropriate preoperative assessment and planning surgical teams with adequate experience in minimal access surgery at high-volume centers can safely offer advanced laparoscopic procedures with successful outcome in patients with SIT.

CONCLUSION

Malignancies in patients of SIT are rare cases to encounter. Minimal access surgery can be performed in these patients safely with successful outcome in high-volume centers with appropriate surgical expertise.