Minimally Invasive Esophagectomy: Feasibility in Esophageal Carcinoma With Inferior Vena Cava Anomaly

Inferior vena cava (IVC) and azygos vein anomaly is very rare. Available case studies report difficult esophagectomy in patients with esophageal carcinoma with IVC anomaly. Minimally invasive esophagectomy with preservation of the azygos vein in such patients is technically challenging. We report a case of a 44-year-old woman diagnosed with middle thoracic esophageal carcinoma with double IVC and dilated azygos vein. Thus minimally invasive surgery is feasible in such patients but requires high technical skills and ample experience to carry out this kind of surgery, and it should only be attempted by a multidisciplinary team. Inferior vena cava (IVC) and azygos vein anomaly is very rare. Available case studies report difficult esophagectomy in patients with esophageal carcinoma with IVC anomaly. Minimally invasive esophagectomy with preservation of the azygos vein in such patients is technically challenging. We report a case of a 44-year-old woman diagnosed with middle thoracic esophageal carcinoma with double IVC and dilated azygos vein. Thus minimally invasive surgery is feasible in such patients but requires high technical skills and ample experience to carry out this kind of surgery, and it should only be attempted by a multidisciplinary team. Azygos vein enlargement with an inferior vena cava (IVC) anomaly is a rare condition, and duplicated IVC has been estimated to occur in 0.2% to 3% of the population.1Phillips E. Embryology, normal anatomy, and anomalies.in: Ferris E.J. Hipona F.A. Kahn P.C. Phillips E. Shapiro J.H. Venography of the Inferior Vena Cava and Its Branches. Williams & Wilkins, Baltimore, MD1969: 1-32Google Scholar Of the many types of IVC anatomic anomalies, 1 of the most frequently reported is interruption of the IVC with azygos continuation with an estimated prevalence of 0.15%.2Koc Z. Oguzkurt L. Interruption or congenital stenosis of the inferior vena cava: prevalence, imaging, and clinical findings.Eur J Radiol. 2007; 62: 257-266Abstract Full Text Full Text PDF PubMed Scopus (116) Google Scholar Although these anomalies are generally asymptomatic, they can have important clinical implications in decision-making and management in certain settings, especially requiring surgical procedures. Given the low frequency of this disorder, little is known about the effects of surgery on the patient’s hemodynamics in the event that this vein is sectioned during a particular surgical procedure.3Mayo J. Gray R. St Louis E. et al.Anomalies of the inferior vena cava.Am J Roentgenol. 1983; 140: 639-652Crossref PubMed Scopus (191) Google Scholar,4Chuang V.P. Mena C.E. Hoskins P.A. Congenital anomalies of the inferior vena cava. Review of embryogenesis and presentation of a simplified classification.Br J Radiol. 1974; 47: 206-213Crossref PubMed Scopus (304) Google Scholar Therefore, preoperative evaluation of IVC anomaly is necessary for patients undergoing esophagectomy because it is extremely important to maintain the integrity of the azygos vein in such patients. Only 4 case reports are available for difficult esophagectomy with preservation of azygos vein in patients of esophageal carcinoma with this rare IVC anomaly.5Haraguchi S. Hioki M. Hisayoshi T. et al.Enucleation of esophageal leiomyoma with azygos continuation of the inferior vena cava: report of a case.Surg Today. 2006; 36: 722-726Crossref PubMed Scopus (5) Google Scholar, 6Martín-Malagón A. Bravo A. Arteaga I. Rodríguez L. Estévez F. Alarcó A. Ivor Lewis esophagectomy in a patient with enlarged azygos vein: a lesson to learn.Ann Thorac Surg. 2008; 85: 326-328Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar, 7Shintakuya R. Mukaida H. Mimura T. et al.A case of thoracic esophageal cancer with an unusual type of duplicated inferior vena cava.Gen Thorac Cardiovasc Surg. 2014; 62: 327-330Crossref PubMed Scopus (3) Google Scholar, 8Yi Shen Xiang Zhuang Ping Xiao Wei Dai Qiang Li Esophagectomy in a patient with azygos vein continuation of the inferior vena cava: report of a case.World J Surg Oncol. 2015; 13: 242Crossref PubMed Scopus (5) Google Scholar Preserving the azygos vein is crucial in these cases and is technical demanding. Establishment of a prior venovenous bypass and protection of the azygos arch are also reported.8Yi Shen Xiang Zhuang Ping Xiao Wei Dai Qiang Li Esophagectomy in a patient with azygos vein continuation of the inferior vena cava: report of a case.World J Surg Oncol. 2015; 13: 242Crossref PubMed Scopus (5) Google Scholar Minimally invasive esophagectomy (MIE) is becoming the standard of care for mid- or lower esophageal carcinoma, but preserving the dilated azygos vein during MIE makes it extremely challenging. We report a case of esophageal carcinoma with double IVC and dilated azygos vein managed with MIE. A 44-year-old woman without any known comorbidity presented with complaints of painless progressive dysphagia associated with anorexia and weight loss for 1½ years. She was evaluated and diagnosed with a moderately differentiated squamous cell carcinoma of the middle thoracic esophagus by endoscopy and biopsy. Computed tomography (CT) was suggestive of a circumferential mural wall thickening in an ∼7.7-cm segment in the thoracic esophagus. An indentation on the azygos vein was posteriorly present (Figure 1), and double IVCs were present on each side of the aorta. The left renal vein was draining into the left IVC, which continued into the azygos vein in the thorax and drained into the superior vena cava. The azygos vein was dilated to a 1.1-cm diameter (Figure 2), potentially causing catastrophic injury to the azygous vein and resulting in impaired drainage of the left kidney.Figure 2Variation in the inferior vena cava (IVC) in our case. There is a double IVC with azygous continuation of the left IVC and an interconnecting vein between both common iliac veins.View Large Image Figure ViewerDownload Hi-res image Download (PPT) The patient received 3 cycles of carboplatin-paclitaxel–based neoadjuvant chemotherapy. Repeat CT showed a significant reduction in tumor bulk. She was planned for MIE with gastric pullup with preservation of the azygos vein. We usually perform MIE by dividing the azygos vein, which allows better access to the tumor especially located in the middle thoracic esophagus, for an easy anastomotic reconstruction and to facilitate optimal oncologic resection. In this case however preservation of the azygos vein was very crucial. The patient was placed in the prone position, and thoracoscopy was done from the right side. Our aim was preservation of the azygous vein with esophagectomy without compromising the oncologic outcome. We proceeded cautiously without prior venovenous bypass. Intraoperatively the no-touch technique was used for the azygous vein because it was remarkably dilated (diameter of 1.1 cm) and tense. To reduce the pressure in the azygous vein we restricted intravenous fluid intraoperatively. Gentle traction on the esophagus was used, facilitating the dissecting between the esophagus and aorta, and careful dissection of the esophagus was done. En bloc thoracoscopic esophagus mobilization was performed, safeguarding the azygos vein. A gastric conduit was made laparoscopically, and the gastric tube was pull up through the naive route behind the azygos vein and was performed successfully (Figure 3). On postoperative day 2 the patient was diagnosed with chylothorax. Due to a suspicion of thoracic duct injury she underwent conventional lymphagiography, which showed a chyle leak from the left posterior segment of the midthoracic duct with collaterals on both sides likely near the azygos vein. No cisterna chyli was visible in the abdomen or multiple collaterals present in the abdomen. Embolization was deferred because of nonvisualization of cisterna chyli. The patient was initially managed conservatively. On postoperative day 6 she again had complaints of difficulty breathing and neck pain. The clinical examination suggestive of collection, and the patient had respiratory distress with inspiratory stridor for which she was intubated. CT showed chylothorax with a loculated collection between the conduit and trachea, causing compression over the trachea. In view of both a failed embolization trial and conservative treatment she underwent thoracoscopic drainage of the collection and thoracic duct ligation on postoperative day 7. Reexploration with thoracoscopy, as in this case, is even more difficult than primary surgery because the tissue was inflamed and care must be taken when working between the conduit and aorta, taking care also of the conduit. Intraoperatively adhesions were present between the lung, conduit, and pleura. A loculated collection was present in the right upper thorax and between the conduit and vertebra. The thoracic duct was identified and chyle leak noted just below the carina between the aorta and azygos vein on the left side of the vertebral body. The thoracic duct was clipped. The postoperative period after the second surgery was uneventful, and the patient was discharged on postoperative day 15 from the first surgery. Pathology of the resected specimen showed a moderately differentiated squamous cell carcinoma, ypT2 N0 (0/17 lymph nodes). She did not received adjuvant treatment, and a CT was suggestive of no recurrence at the 6-month follow-up. MIE is feasible in cases of middle thoracic esophageal carcinoma with double IVC and dilated azygos vein. A preoperative assessment and multidisciplinary approach are crucial for patient outcomes. High technical skills and great experience are necessary to carry out this kind of surgery. Our case is different in various aspects because we did not performed the venovenous bypass before the esophagectomy, so bypass is not absolutely necessary in this condition. However, we have to be more cautious during surgery. We did minimally invasive surgery and even managed its complications using the thoracoscopic technique, which is more technically challenging.