Barbara A. Goff and Howard G. Muntz
Total pelvic exenteration is a surgical procedure that involves the en bloc removal of female reproductive organs, rectosigmoid colon, and lower urinary tract. It may include a perineal phase to remove the urethra, vagina, and anus. Modifications can be made depending on tumor location and size.
The first series of pelvic exenterations was published in 1948 by Alexander Brunschwig for the palliative treatment of advanced pelvic malignancies.1 Although the operative mortality in this group of 22 patients was 23%, there were also several long-term survivors, indicating potential benefit beyond palliation. The original operation included implanting both ureters into the colon to produce a wet colostomy; however, this resulted in significant problems with hyperchloremic acidosis, pyelonephritis, and renal failure. In 1956, Bricker2 published a technique of using a closed loop of ileum as a bladder substitution. Over the past 50 years, there have been many advances in perioperative care such as blood products, antibiotics, intensive care support, and surgical techniques such as retractors, cautery, and staplers that now allow for a variety of vaginal reconstructions and urinary conduits that can reduce the impact this procedure has on quality of life.3-10 As a result, pelvic exenteration is now considered a safe and feasible procedure that can cure selected patients for whom there are no other treatment options. In modern series, operative mortality ranges from 0% to 5%, and 5-year survival rates range from 39% to 53% depending on the specific indications for the exenteration.3-7 Patient selection, patient preparation, surgical technique, and postoperative care can have a major impact on the outcome for patients undergoing this operation.
The main indication for pelvic exenteration is the central persistence or recurrence of cervical, vaginal, or vulvar cancer after primary radiation or chemoradiation. Central recurrence is defined as the absence of both pelvic sidewall involvement and distant disease. Some have advocated the use of exenteration for primary treatment of stage IVA cervical cancer; however, with modern chemoradiation, this has become uncommon. Long-term survival after exenterative surgery in women with pelvic failure after surgery and radiation for endometrial cancer or sarcomas has also been reported.11,12 Extensive radiation injury to bladder, vagina, and/or rectum, especially if patients have evidence of significant necrosis with fistula formation, is another potential indication for exenteration.7
Exenterative surgery to resect centrally recurrent pelvic cancer is only rational in the absence of metastatic disease. Para-aortic nodal involvement is generally considered an absolute contraindication for exenteration. If pelvic lymph nodes are found to be the only site of metastatic disease, then patients can still be considered surgical candidates, although long-term cure rates will only be about 15%.13 Palliative exenteration (documented extrapelvic disease with no expectation for cure) should be performed only if there is a high likelihood for significant improvement in quality of life, because 70% of patients will experience major complications.6,8 In general, pelvic side-wall involvement is a contraindication to performing an exenteration, but with laterally extended endopelvic resections14 or intraoperative radiation therapy,15,16 selected patients can also be considered candidates for exenteration. Common selection criteria include the following: (1) a central pelvic malignancy potentially curable by exenteration; (2) absence of local or distant metastases; (3) medical and psychological fitness to withstand exenterative surgery; and (4) ability for postoperative self-care. The likelihood of discovering metastatic disease or an unresectable pelvic tumor at exploratory surgery and abandoning exenterative surgery is 30% to 50%, depending on preoperative selection criteria (eg, tumor size).
Classification of Exenteration
Pelvic exenteration includes 3 basic variations (Figure 32A-1). Total pelvic exenteration consists of en bloc removal of the gynecologic organs, bladder, and rectosigmoid colon. Anterior exenteration is a combination of radical hysterectomy-vaginectomy and cystectomy, and posterior exenteration is a combination of radical hysterectomy-vaginectomy and rectosigmoid colectomy. Pelvic exenteration can also be classified according to the extent of tissue resection and associated anatomical alteration (Figure 32A-2). A type I, or supralevator, exenteration is indicated for lesions confined to the upper pelvis without involvement of the lower one-half of the vagina. A type II, or infralevator, exenteration is the most commonly performed variation and includes visceral resection below the levator ani muscles with limited resection of the levator muscles and urogenital diaphragm. Rarely, a type III exenteration (infralevator with vulvectomy) is required for tumor extension to the vulva or perineum and includes resection of the urogenital diaphragm.
FIGURE 32A-1. Variations of exenteration: (A) total pelvic exenteration; (B) anterior pelvic exenteration; and (C) posterior pelvic exenteration.
FIGURE 32A-2. Classification of pelvic exenteration: (A) supralevator exenteration; (B) infralevator exenteration; and (C) infralevator exenteration with vulvectomy.
Expected Clinical Outcomes
Table 32A-1 shows clinical outcomes of the most contemporary series of pelvic exenterations.3-7 Long-term disease-free survival ranges from 30% to 60% depending on nodal and margin status, time from initial radiation to recurrence, size of the lesion, and primary disease site.3-10 Factors that have not been shown to impact survival are age and subsequent treatment following exenteration. Operative mortality is quite low (0%-5%) and is usually a result of sepsis with multiorgan failure, pulmonary embolism, or cardiac events.
Surgical morbidity with pelvic exenteration can be substantial. The median estimated blood loss ranges from 1290 to 2500 mL, and 70% to 85% of patient will require blood transfusions. Median operative time is approximately 8 hours but varies widely depending on the reconstructive procedures that are done following the exenteration. The average length of stay following exenteration is 15 to 28 days. Major morbidity is seen in 30% to 50% of patients. The most common complications seen with exenterations include infection, abscess, anastomotic leaks, wound dehiscence, thromboembolic events, ileus, bowel obstruction, secondary bleeding, and cardiovascular events.3-10
Table 32A-1 Clinical Outcomes of Pelvic Exenteration
Box 32A-1 Master Surgeon’s Corner
If a perineal phase is needed, have 2 surgical teams.
Ligation of the anterior division of the internal iliac artery, once resectability has been determined, can reduce blood loss.
In patients who are heavily irradiated, the use of flaps can significantly reduce the risk of infection and fistula formation.
Patient Evaluation and Work-up
A thorough preoperative assessment is mandatory. Assuming only physiologically fit and psychologically sound patients are being evaluated, the key factors for consideration include histologic confirmation of recurrent disease, resectability of the recurrent tumor, and absence of metastatic disease.
Because radiation necrosis can mimic the appearance of recurrent cancer (and vice versa), an examination under anesthesia should be performed with multiple biopsies, including deep Tru-cut biopsies. Directed biopsies obtained with interventional radiology guidance may be required.
Resectability of a centrally recurrent pelvic tumor is not always predictable. The triad of ipsilateral leg edema, sciatic pain, and ureteral obstruction is almost always associated with sidewall extension and unresectability. Evaluation of disease resectability can be aided by pelvic magnetic resonance imaging and positron emission tomography/computed tomography. However, in the setting of fibrosis, infection, and fistula formation, all of these modalities can have significant false-positive rates.17-20 Examination under anesthesia is often the best way for a surgeon to determine sidewall involvement. If there is any question about resectability because of sidewall involvement, patients should be offered surgical exploration and possible exenteration.
Even after thorough preoperative assessment, there is still a 30% chance that at the time of exploration for exenteration, the procedure will be abandoned due to unresectable pelvic disease or small-volume peritoneal metastases.20
Patients should understand there is a 30% chance that the operation will not be completed,20 and even if completed, cure rates vary widely (30%-60%).3-10 Patients need to be counseled about the loss or severe alteration of bladder, rectal, and sexual function, as well as potential emotional distress.21,22
Nutritional status and pulmonary function should be optimized prior to exenteration. Medical comorbidi-ties, such as diabetes, hypertension, and heart disease, should be under good control. Consultation with a stoma therapist to mark location(s) and review function is recommended. Psychological evaluation and consultation with a surviving patient having previously undergone a similar exenterative procedure may be helpful in assessing the patient’s emotional readiness for surgery. Patients should undergo a mechanical bowel preparation the day before surgery and receive appropriate antibiotic and thromboembolic prophylaxis. Placement of a central line and having adequate blood product availability are also advised.
Instrumentation for exenteration varies according to surgeon preference but should include a self-retaining retractor such as a Bookwalter, bowel staplers, ureteral stents, vaginal stents, and stoma appliances. Electrothermal bipolar coagulation instruments, such as the vessel-sealing and cutting device LigaSure, can decrease the need for suturing, decrease surgical time, and reduce intraoperative blood loss.7
Anesthesia and Positioning
General anesthesia is mandatory; an epidural catheter can be placed for postoperative pain management. Patients are placed in a low dorsal lithotomy position in Allen or Yellow Fin stirrups, and the surgical preparation should extend from the nipple line to the knees to ensure adequate access for use of abdominal or lower extremity–based myocutaneous flaps for pelvic reconstruction.
Surgical Exploration and Evaluation of Para-aortic Lymph Nodes
A generous midline incision is made, taking into account the potential need for abdominal flap creation and stoma placement, and normal anatomy restored. A thorough exploration is performed to evaluate for intraperitoneal malignancy or other distant metastasis, and suspicious lesions are sent for frozen-section analysis. A self-retaining retractor is placed, para-aortic lymph node basins are explored (see Chapter 28) and dissected, and specimens are sent for frozen-section analysis while attention is directed toward developing the pelvic phase of the operation. Involvement of para-aortic lymph nodes would be a contraindication to proceeding.
Opening the Pelvic Sidewall and Evaluation of Pelvic Lymph Nodes
The pelvic sidewalls are opened by incising the round ligaments and mobilizing the broad ligament medially and developing the pararectal and paravesical spaces. The external iliac and obturator lymph nodes are dissected (see Chapter 28), and any suspicious nodes are sent for frozen-section analysis. With the lymph nodes removed and the paravesical and pararectal spaces developed, the pelvic sidewalls can now be directly assessed for involvement. To verify the absence of side-wall extension, a finger is inserted in the paravesical space, and a finger is inserted into the pararectal space, and the intervening tissue of the cardinal ligament is palpated down to the pelvic floor to evaluate the proximity of tumor to the pelvic wall.
Bladder Mobilization and Development of Retropubic Space
The peritoneal incision is extended along the posterior border of the symphysis pubis, and the retropubic space of Retzius is developed using a combination of blunt and sharp dissection down to the pelvic floor. The anterior pelvic dissection proceeds laterally, resulting in unification of the retropubic space and the bilateral paravesical spaces.
Rectum Mobilization and Development of Presacral Space
Following the anterior pelvic dissection, the ureters are fully mobilized down the cardinal ligament and held with Vessi-loops for traction. The peritoneal incision is extended medially into the posterior pelvis toward the sigmoid mesentery. The pararectal spaces are extended posteriorly and medially underneath the sigmoid mesentery to develop the presacral (retrorectal) space, working in the avascular plane anterior to the sacrum. The absence of sacral involvement should be verified by confirming the ability to lift the rectosigmoid colon out of the sacral hollow. This is the last decision point to abandon the exenteration before dividing the bowel and ureters.
Division of Rectosigmoid Colon and Ureters; Unification of Pelvic Spaces
The rectosigmoid colon is divided using a linear stapling device (eg, gastrointestinal anastomotic), and the sigmoid mesentery is taken down between clamps or using a vessel-sealing and cutting device (eg, LigaSure); the inferior mesenteric vascular pedicle is clamped, divided, and secured with suture ligatures (see Chapter 30). The presacral space is then developed down to the pelvic floor. The ureters are ligated and divided at least 2 cm from the central pelvic tumor mass. The proximal ureters and sigmoid colon can be packed out of the operative field until the reconstructive phase. At this point, the pelvic spaces are unified with the exception of the cardinal ligaments and anterior division of the internal iliac vessels (Figure 32A-3).
FIGURE 32A-3. Initial phase of total pelvic exenteration. The retropubic space is extended laterally to reach continuity with the paravesical spaces, and the presacral space is extended laterally to reach continuity with the pararectal spaces.
Division of Cardinal Ligaments
The central pelvic tumor specimen is placed on contralateral traction with the surgeon’s fingers or straightened Heaney retractors used to expose the pelvic sidewall. The internal iliac vessels are often indistinguishable as separate structures in a previously radiated field but lie within the tissue of the cardinal ligament. Working from the pelvic brim toward the pelvic floor, the cardinal ligament tissue is serially clamped at the pelvic sidewall, divided, and secured with suture ligatures (Figure 32A-4). If the internal iliac artery and vein are identifiable, they can either be preserved and the uterine vascular pedicle divided at its origin, or resected en bloc. If sacrificed, the internal iliac vessels should be individually secured with vascular clamps and divided a short distance from the pelvic wall to allow for an adequate pedicle in the event of unexpected hemorrhage. Division of the internal iliac arteries may limit neovagina reconstructive options (see Chapter 32C). Posteriorly, the mesorectum and rectal pillars are divided between clamps or taken down to the pelvic floor using a vessel-sealing and cutting device (eg, LigaSure). The exposure and dissection are duplicated on the contralateral side.
FIGURE 32A-4. Total pelvic exenteration. The cardinal ligaments (with or without the internal iliac vessels) are resected at the level of the pelvic wall down to the levator muscles of the pelvic floor for a supralevator exenteration; the levator muscles are included in the resection for infralevator exenteration.
Supralevator Exenteration: Final Extirpative Steps
The final extirpative phase of a supralevator exenteration begins by placing posterior traction on the central specimen exposing the anterior pelvis. The Foley catheter can be palpable within the urethra. The surrounding paravesical tissue is taken down to the pelvic floor, and the urethra is divided using the electrosurgical blade. The vagina can be cross-clamped and divided or circumscribed using the electrosurgical blade at the level of the pelvic floor. The distal rectum is divided using a linear stapling (eg, transverse anastomosis) device (see Chapter 30), and the specimen is removed (Figure 32A-5). A laparotomy pack is placed in the pelvis to tamponade any small bleeding sites while the specimen is inspected to ensure grossly negative resection margins.
FIGURE 32A-5. Total supralevator pelvic exenteration: extirpation of the specimen with resulting pelvic defect.
Infralevator Exenteration: Resection of Levator Muscles
For an infralevator exenteration, a second surgical team begins the perineal phase when the abdominal dissection reaches the level of the levator muscles. The central pelvic tumor specimen is placed on counter-traction, and the electrosurgical blade is used to incise the levator muscle plate circumferentially at least 2 cm lateral to the area of tumor extension (Figure 32A-6).
FIGURE 32A-6. Total infralevator pelvic exenteration: resection of levator muscle plate.
Infralevator Exenteration: Perineal Phase and Specimen Removal
The second surgical team outlines the planned peri-neal resection to encompass a variable extent of vulvectomy tailored to the extent of tumor involvement (Figure 32A-7). The subcutaneous dissection is developed in the paravesical and pararectal spaces cephalad, using a combination of clamps with suture ligatures and the electrosurgical blade. The abdominal surgeon can place a hand in the pelvis to help guide the peri-neal dissection. After the perineal phase has reached the fascial plane of the pelvic floor, 4 potential spaces are developed: the suprapubic and presacral spaces and the right and left paravaginal spaces (Figure 32A-8). These potential spaces are separated by 5 pedicles: 2 pubourethral, 2 rectal pillar, and the posterior anococcygeal. These pedicles are clamped, divided, and secured with suture ligatures. Circumferential dissection results in complete detachment of the specimen, which can be removed abdominally or vaginally (Figure 32A-9).
FIGURE 32A-7. Total infralevator exenteration. The extent of the perineal resection is tailored to the degree of lower vaginal or vulvar involvement with tumor.
FIGURE 32A-8. Total infralevator exenteration. The suprapubic, paravaginal, and presacral spaces are developed to define the pubourethral, rectal pillar, and anococcygeal pedicles.
FIGURE 32A-9. Total infralevator exenteration: pelvic defect.
Prior to closing the abdomen, the operative site should be copiously irrigated and hemostasis confirmed. The abdomen and pelvis should be adequately drained to promote wound healing. There will be extensive third spacing and small urinary leaks from the conduit, so adequate drainage is essential. The wound should be closed in a mass closure with delayed absorbable or permanent suture.
Closure and Final Steps
The simplest and most expedient way to close the perineum is for the second surgical team to perform a layered closure of the deep pelvic and perineal tissues (Figure 32A-10). Reconstruction of the urinary tract should be performed next according to the patient’s desires and available surgical options (see Chapter 32B). Due to the large amount of dead space left after an exenteration, a neovagina, a rectus flap, or an omental flap is needed to fill the pelvis and bring in tissue with a good blood supply. Our recommendation, even in women who do not want a neovagina, is that a rectus flap be placed into the pelvis and an omental J-flap placed on top of it (see Chapter 32C).
FIGURE 32A-10. Total infralevator exenteration: simple closure of the pelvic floor.
Once the vaginal, pelvic, and urinary reconstructions are performed, attention is directed to the intestinal tract. Re-establishing intestinal continuity should be undertaken with caution, because the risk of anastomotic dehiscence or leak is as high as 50% after radiation. After tolerance-dose pelvic radiation, our preference is to perform end colostomy at the time of total or posterior pelvic exenteration. The colostomy is usually the final procedure prior to closing. A 2- to 3-cm circular piece of skin is removed from the site of the stoma (see Chapter 30). The subcutaneous tissue is dissected to the fascia, and a cruciate incision is made in the anterior abdominal wall fascia so that the rectosigmoid can easily be brought through the anterior abdominal wall. Retraction of the colon back into the abdomen is prevented by carefully placing sutures between the fascia and proximal bowel. Additional sutures can also be placed on the peritoneal side. The stoma should be matured to the skin in a rosebud fashion (skin to proximal bowel to distal bowel edge) once the abdomen is closed to prevent any fecal contamination.
Box 32A-2 Caution Points
During the perineal phase, have the abdominal surgical team guide the dissection to ensure the lateral margins are adequate.
Do not disrupt presacral veins when dissecting the rectum off the sacral promontory and sacral hollow.
Dissect ureters as low in the pelvis as possible so there is adequate length for anastomosis.
If only the bladder, urethra, uterus, cervix, and vagina need to be removed and the rectum can be spared, then the posterior part of the dissection is modified. For an anterior exenteration, the peritoneum between the rectum and vagina is incised and the rectovaginal septum developed. The entire vaginal tube can be mobilized in a combined abdominal and perineal approach if the entire vagina needs to be taken, or a portion of the posterior vagina can be left in place if margins are adequate.
With a posterior exenteration, the anterior part of the exenteration is modified. The ureters are dissected to their entry into the bladder, similar to a radical hysterectomy. The dissection between the vagina and bladder is taken down to the perineal dissection or until there are adequate margins around the cancer. The entire vaginal tube can be taken if needed, or a portion of the anterior vagina can be left if the margins are adequate. Posterior exenterations for central recurrence following radiation are not often performed because of the high likelihood of bladder dysfunction and urinary fistulization when the rectum, vagina, and cervix are removed. In addition, because of the damage to the hypogastric plexus that enervates the bladder, many patients will require some type of catheter drainage to facilitate bladder emptying.
It is common for many patients to require an intensive care unit stay for 24 to 48 hours after surgery to manage large blood loss, transfusion, and fluid shifts. Postoperative pain is best managed with patient-controlled epidural analgesia. Total parenteral nutrition can be started to help facilitate adequate nutrition and wound healing. Early feeding can be initiated, although prolonged ileus may prevent this. Venous thromboembolism prophylaxis should be continued during the entire hospitalization; some authors recommend a total of 4 weeks of medical prophylaxis. Ambulation is usually begun by day 2 to 3.
Drains should remain in place for approximately 7 to 10 days and may be discontinued when the output is less than 100 mL in a 24-hour period. If there is concern about ureteral leakage, the fluid in the drain can be sent for creatinine. A creatinine from drain fluid that is significantly higher than the serum value is indicative of a leak. If there is a leak, with adequate drainage and ureteral stenting, the leak will usually heal.
Complications following an exenteration will occur in approximately 50% of patients. Wound infections, pelvic abscess requiring drainage, anastomotic leaks, bowel obstruction, fistula, venous thrombosis, and other medical complications are the most common.
Box 32A-3 Complications and Morbidity
Major bleeding is the most common intraoperative complication. Adequate blood should be available. Be prepared to give platelets and fresh frozen plasma if bleeding is significant.
Thromboembolic complications are common in the perioperative period. Both mechanical and medical prophylaxis should be used.
Infectious complications are very common. Patients need redosing of intraoperative antibiotics secondary to large blood loss and extended time of the surgery.
In patients who are malnourished, preoperative oral or total parenteral nutrition (TPN) should be given until the prealbumin level is in the normal range. Postoperative TPN is usually given to all patients to promote healing and prevent anastomotic leaks.
Abscesses and anastomotic leaks are common complications. Placement of drains at the time of surgery can reduce these complications. Postoperative abscesses or leaks can also be managed conservatively by interventional radiology.
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