Total pelvic exenteration (TPE) may be the only procedure that can cure T4 rectal cancer that directly invades the urinary bladder or prostate. Here, we describe our experience of laparoscopic TPE with en bloc lateral lymph node dissection for advanced primary rectal cancer. A 62-year-old man diagnosed with advanced lower rectal cancer (T4bN0M0) underwent laparoscopic TPE with en bloc lateral lymph node dissection after neoadjuvant chemoradiotherapy. Ligation of the dorsal vein complex was performed under direct visualization through the perineal approach, and the large perineal defect was reconstructed using bilateral V-Y advancement of the gluteus maximus musculocutaneous flaps. The ileal conduit was constructed extracorporeally through an extended umbilical port that was extended to 4 cm. The total operative time was 831 min and estimated blood loss was 600 mL. Laparoscopic TPE appears to be safe and feasible in selected patients.
In the era of neoadjuvant chemoradiotherapy/radiotherapy and total mesorectal excision, overall oncological outcomes after curative resection of rectal cancer are excellent, with local recurrence rates as low as 5–10%. However, lateral nodal disease is a major cause of local recurrence after neoadjuvant chemoradiotherapy/radiotherapy and total mesorectal excision. Patients with lateral nodal disease have a local recurrence rate of up to 30%. The oncological benefits of lateral pelvic lymph node dissection (LPLND) in reducing local recurrence, particularly in the lateral compartment, have been demonstrated. Although LPLND is not standard in Western countries, technical improvements in minimally invasive surgery have resulted in rapid technical standardization of this complicated procedure. The feasibility and short- and long-term outcomes of laparoscopic and robotic LPLND have been reported widely. A minimally invasive approach has the advantages of less bleeding and providing a better surgical view of the deep pelvic anatomy than an open approach. With precise autonomic nerve preservation, postoperative genitourinary dysfunction has been reported to be minimal. We review recent evidence on the management of lateral nodal disease in rectal cancer and technical improvements of LPLND, focusing on laparoscopic and robotic LPLND.
The vascular type of Ehlers-Danlos syndrome (vEDS) is a rare inherited disease of the connective tissues, and is caused by abnormal type III collagen resulting from heterogeneous mutations of the type III collagen COL3A1 gene. We herein report the case of a vEDS patient who developed a sigmoid colon perforation and was given a definitive diagnosis by a genetic and biomolecular assay. The patient demonstrated clinical manifestations caused by tissue weakness such as frequent pneumothorax events and a detached retina. During the operation, we noticed easy bruising and thin skin with visible veins on the patient's abdominal wall. Finally, a diagnosis was confirmed by the reduction of type III collagen synthesis and by the identification of a mutation in the gene for type III collagen. We conclude that it is difficult to diagnose a vEDS patient without clinical experiences and specialized genetic methods. Furthermore, all organs must be treated gently during therapy, because the tissues of vEDS patients are extremely fragile.
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