The procedure of pelvic exenteration was first described in its present form by Brunschwig in 1948. Over the years it has been used mainly in the treatment of advanced and recurrent carcinoma of the cervix. Its primary role at the present time is the management of the numerous patients who develop recurrent cancer of the cervix following primary radiotherapeutic treatment. It has been estimated that between one third and one half of patients with invasive carcinoma of the cervix will have residual or recurrent disease after treatment. Approximately one quarter of these cases will develop a central recurrence which may be amenable to exenterative surgery. However, pelvic exenteration as a therapy for recurrent cancer of the cervix has not been widely accepted and many patients will succumb to their disease having been through the process of radiotherapy followed by chemotherapy and other experimental treatments without being given the formal opportunity of a curative procedure. The published results of exenterative procedures show an acceptable primary mortality of approximately 3-4% and an overall survival/cure rate of 30-60%. The procedure is also applicable to a wide range of other pelvic cancers including cancer of the vagina, vulva and rectum, both for primary and secondary disease. It is less often applicable to ovarian epithelial cancers and melanomas and sarcomas because of their tendency for widespread metastases.
The surgery involved is extensive and postoperative care is complex; as a consequence, the operation has become part of the repertoire of the advanced gynaecological oncologist working in a centre with a wide experience of radical surgery. The procedure does demand of the surgeon considerable expertise and flexibility: virtually no two exenterations are identical, and considerable judgment and ingenuity are required during the procedure in order to achieve a comprehensive removal of all tumour. With small recurrences, more limited procedures may be carried out with a degree of conservation of structures in and around the pelvis. With extensive procedures and particularly following extensive radiotherapy, complete clearance of all organs from the pelvis (total exenteration) together with widespread lymphadenectomy may be essential in order to achieve a cure. There is now considerable evidence that even in patients with node metastases at the time of exenteration a significant survival rate can be achieved.
Exenterative surgery should be considered for both advanced primary pelvic carcinoma and recurrent disease. Many patients will be eliminated from the possibility of surgery at an early stage because of complete fixity of the tumour mass to the bony structures of the pelvis. The only exception to this rule is the
The limits of resection for (A) anterior and (B) total exenteration
The limits of resection for (A) anterior and (B) total exenteration rare circumstance in which a vulval or vaginal cancer is attached to one of the pubic rami: the ramus can be resected and a clear margin around the cancer obtained. In general terms exenterative surgery should not be used as a palliative, except perhaps in the presence of malignant fistulas in the pelvis when it may significantly improve the quality of the patient's life without any significant extension to her life. It is important that the surgical team including nurses and ancillary workers are confident in their ability to manage not only the extensive surgery involved but also the difficult, testing and sometimes bizarre complications that can sometimes occur after exenteration. The average age of patients who are subject to exenteration is 50-60 years, but the age range is wide—from early childhood through to the eighth or ninth decade.
It is frequently difficult following radiotherapeutic treatment to be certain that the mass palpable in the pelvis is due to recurrent disease and not to radiation reaction or persistent scarring associated with infection or the effects of adhesion of bowel to the irradiated areas.
In recent years both computed tomography (CT) and more recently magnetic resonance imaging (MRI) have been used extensively in the preoperative assessment of patients for many oncological procedures. The considerable difficulties of assessing CT scans in patients who have had preceding surgery or radiotherapy are a particular problem in patients being assessed for exenteration. Some clinicians feel that CT scanning is useful, whereas the author has not found the level of reliability to be acceptable. There will be many individual variations from centre to centre depending upon the skills available to the clinician. A tissue diagnosis is essential prior to embarking on exenterative surgery, and needle biopsy, aspiration cytology or even open biopsy at laparotomy will be required. As distant metastases tend to occur with recurrent and residual disease, it is sometimes helpful to perform scalene node biopsies and radiological assessments of the pelvic and para-aortic lymph nodes together with fine-needle aspiration, in order to assist with the assessment. The mental state of the patient is also important, but should not in itself be a bar to the performance of such surgery.
A Maylard or high transverse incision
If there are metastases in extrapelvic lymph nodes, abdominal viscera, lungs or bones there appears to be little value in performing such major surgery. However, there is evidence that patients with pelvic lymph node metastases may well survive, and a good quality of life is reported in a small but significant percentage of such patients.
• Pelvic side-wall spread: if the tumour has extended to the pelvic side-wall either in the form of direct extension or nodal metastases the prospects of a cure are extremely small and the surgeon must decide whether the procedure will materially improve the patient's quality of life. The triad of unilateral uropathy, renal nonfunction or ureteric obstruction together with unilateral leg oedema and sciatic leg pain is an ominous sign. The prospects of a cure are poor; readers are, however, referred to Chapter 12 for possible combination therapies. Perineural lymphatic spread is not visible on CT and can be a major source of pain and eventual death.
• Obesity is a problem with all surgical procedures, producing many technical difficulties as well as postoperative respiratory and mobilization problems. The more massive the surgery the greater are these problems.
In North America the majority of exenterations performed are total; in the author's series approximately half of his exenterations have been of the anterior type, removing the bladder, uterus, cervix and vagina, but preserving the rectum (Figure 1). For very small, high lesions around the cervix and lower uterus and bladder it may be possible to carry out a more limited procedure (a supralevator exenteration) retaining considerable parts of the pelvic floor. Posterior exenteration (abdominal perineal procedure) is rarely performed by gynaecological oncologists as this procedure tends to be the province of the general surgeon.
Probably the most important part of the preoperative preparation is the extensive counselling needed to make certain that the patient and her relatives, particularly her partner, understand fully the extent of the surgery and the marked effect it will have upon normal lifestyle, in particular the loss of normal sexual function when the vagina has been taken out. The transference of urinary and bowel function to the chosen type of diversionary procedure should be discussed, as should the possibility of reconstructive surgery of the vagina and bladder, and the significant risks of such extensive surgery must be honestly explained. During the course of this counselling the patient should be seen by a stoma therapist. The author finds it
Division of the round and infundibulopelvic ligaments and the beginning of the lateral pelvic dissection
Division of the round and infundibulopelvic ligaments and the beginning of the lateral pelvic dissection ideal for the patient to meet others who have had the procedure, to discuss on a woman-to-woman basis the real problems and feelings about exenteration.
The patient is usually admitted to hospital 2-3 days prior to the planned procedure to undergo high-quality bowel preparation. With the modern alternative liquid diets and antibiotic therapy, complete cleaning of the small and large bowel can be achieved very rapidly. The anaesthesiologist responsible for the patient's care will see the patient and explain the process of anaesthesia. The author prefers to carry out all radical surgery under a combination of epidural or spinal analgesia together with general anaesthesia. Cardiac and blood gas monitoring is essential. Although the majority of patients do not require intensive care therapy, its availability must be ensured prior to the surgical procedure. Prophylaxis against deep venous thrombosis is usually organized by the ward team utilizing a combination of modern elastic stockings and low-dose heparin which is initiated immediately following surgery.
The final decision to proceed with exenteration will not be made until the abdomen has been opened and assessment of the pelvic side-wall and posterior abdominal wall has been made, utilizing frozen sections where necessary. In the author's practice the procedure is performed by a single team. If plastic surgical procedures such as the formation of a neovagina are planned then a second plastic surgical team will carry out the necessary operation at the same time as the diversionary procedures are being performed by the primary team.
Once the patient has been anaesthetized and placed in the supine position in the operating theatre the abdomen is opened using either a longitudinal midline incision extending above the umbilicus, of a high transverse (Maylard) incision (Figure 2) cutting through muscles at the interspinous level. Exploration of the abdomen will confirm the mobility of the central tumour mass; thereafter the para-aortic lymph nodes and pelvic side-wall nodes are dissected (Figure 3) and sent for frozen section examination. Once the frozen sections show no extension of tumour the procedure of total exenteration can begin. At the same time as this initial intraoperative assessment the experienced exenterative surgeon will have opened tissue planes, including the paravesical, pararectal and presacral spaces to a deep level (Figures 4, 5) in the pelvis in order to become familiar with the full extent of the tumour. The dissection is achieved by opening the broad ligament: this can be done directly or the round ligament can be ligated and divided first. These dissections can be carried out without any significant blood loss and will yield considerable information. If it is not possible to proceed with the operation the abdomen may be closed at this stage as no significant trauma has been inflicted by the surgeon. Considerable experience and judgment is required in order to make this decision. Often the most difficult decision is to stop operating. Very occasionally, for example with some vulval cancers, resection of pubic bones may be attempted, but in general terms if there is bony involvement of tumour the procedure should be abandoned.
After the comprehensive manual and visual assessment of the pelvis and the abdominal cavity, the surgeon proceeds by dividing the round ligament (if it is not already divided), drawing back the infundibulopelvic ligament and opening up the pelvic side-wall (Figure 6). The line of incision for removal of the entire pelvic organs begins at the pelvic side-wall, over the internal iliac artery, and will pass forward through the peritoneum of the upper part of the bladder, meeting with the similar lateral pelvic sidewall incision at the opposite side. The sigmoid colon will be elevated and at a suitable point will be transected, the peritoneal incision will be continued around the brim of the pelvis—with identification of the ureter as it passes over the common iliac artery— and will meet up with the similar incision on the opposite side. After the round ligaments have been divided and tied and the pelvic side-wall space opened, the infundibulopelvic ligament can also be identified, divided and tied. The incision is continued posteriorly and the ureters are separated and identified. If an anterior exenteration is to be performed the peritoneal dissection will be brought down into the pelvis to run across the anterior part of the rectum, just above the pouch of Douglas; this will allow a dissection from the anterior part of the rectum passing posteriorly around the uterosacral ligaments to the sacrum, releasing the entire anterior contents of the pelvis. For a total exenteration the dissection is even simpler: the mesentery of the sigmoid colon is opened and individual vessels clamped, divided and tied. The colon is divided, usually with a stapling device which allows the sealed ends of the colon to lie, without
Deepening the lateral pelvic dissection to reveal the pelvic spaces interfering with the operation in the upper abdomen (Figure 7). A dissection posterior to the rectum is then carried out from the sacral promontory, deep behind the pelvis; this dissection is rapid and simple and permits complete separation of the rectum from the sacrum. This allows complete and usually bloodless removal of the rectal mesentery including lymph nodes. Anteriorly, the bladder is dissected with blunt dissection from the cave of Retzius resulting in the entire bladder with its peritoneal covering falling posteriorly. This dissection is carried down to the pelvic floor, isolating the urethra as it passes through the pelvic floor (perineal diaphragm). As dissection is carried posteriorly into the paravesical spaces, the uterine artery and the terminal part of the internal iliac artery will become clearly visible. By steadily deepening this dissection the anterior division of the internal iliac will be isolated and the tissues of the lower obturator fossa identified; at this point, large exenteration clamps may be placed over the anterior division of the internal iliac artery and its veins (Figure 8). The ureter by this time will have been divided a short distance beyond the pelvic brim. The pelvic phase of the procedure is at this point completed and the perineal phase is now to be carried out.
The patient is placed in the extended lithotomy position and an incision made to remove the lower vagina (for an anterior exenteration) or the lower vagina and rectum (for a total exenteration) (Figure 9). Anteriorly the incision is carried through above the urethra just below the pubic arch to enter the space of the cave of Retzius which has been dissected in the pelvic procedure. The dissection is carried laterally and posteriorly, dividing the pelvic floor musculature, and the entire block of tissue is then removed through the inferior pelvic opening. Small amounts of bleeding will occur at this point, usually arising from the edge of the pelvic floor musculature. These can be picked up by either isolated or running sutures which will act as a haemostat.
Once the perineal dissection has been completed and haemostasis achieved, the surgeon's choice will depend on the preoperative arrangements made with the patient. If in the preoperative assessment period it was decided by the clinician and the patient that a neovagina should be formed, than at this point either the primary surgeon or the plastic surgeon will initiate the development of a neovagina. This may be in the form
The pelvic incision for a total exenteration of a myocutaneous graft using the gracilis muscle, or a Singapore graft may be used from alongside the vulva; other possible techniques involve the development of a skin graft placed within an omental pad, or transposition of a segment of sigmoid colon in order to form a sigmoid neovagina (see Chapter 21). For many patients, however, the desire to have a new vagina is a very low priority and it is surprising how frequently patients will put off these decisions until well after the time of exenteration. Surviving the cancer appears to be their uppermost desire. To this end the careful closure of the posterior parts of the pelvic musculature, a drawing together of the fat (Figure 10) anterior to that and a careful closure of the skin is all that is required. It is usually possible to preserve the clitoris, the clitoral fold and significant proportions of the anterior parts of the labia minora and labia majora so that when recovery is finally made the anterior part of the genitalia has a completely normal appearance. On some occasions patients will be able to have a neovagina formed some significant period of time following the exenteration. This is becoming the predominant pattern in the author's experience of some 89 cases.
Once the perineal phase is finished the legs can be lowered so that patient is once more lying supine and attention can be addressed to dealing with the pedicles deep in the pelvis. All that remains following a total exenteration will be the two exenteration clamps on either side of the pelvis and a completely clean and clear pelvis. The pelvic side-wall dissection of lymph nodes can be completed before dealing with the clamps and any tiny blood vessels that require haemostasis are ligated. As the exenteration clamps are attached to the distal part of the internal iliac arteries it is important that comprehensive suture fixation is carried out (Figure 11). This is usually readily and easily done, although occasionally the large veins of the pelvic wall can provide difficulties and the use of mattress sutures may be necessary in order to deal with
Exenteration clamps applied to the anterior division of the internal iliac arteries
Exenteration clamps applied to the anterior division of the internal iliac arteries these complex vascular patterns. Having completed the dissection of the pelvis the clinician now moves to produce either a continent urinary conduit or a Wallace or Bricker ileal conduit, and if the procedure has been a total exenteration a left iliac fossa stoma will be formed (see Chapters 2 and 9).
A problem which must be avoided is that of small bowel adhesion to the tissues of a denuded pelvis. This is particularly important when patients have previously had radiotherapy, as the risk of fistula formation in these circumstances is extremely high. A variety of techniques have been utilized to deal with this potentially life-threatening complication, including the placing in the pelvis of artificial materials such as Merselene (Ethicon, Edinburgh, UK), Dacron (DuPont) and Gortex sacs (WL Gore & Associates, Flagstaff, Arizona, USA), or even using bull's pericardium. Stanley Way in the 1970s described a sac technique in which he manufactured a bag of peritoneum which allowed the entire abdominal contents to be kept above the pelvis. This resulted in an empty pelvis, which from time to time became infected and generated a new problem, that of the empty pelvis syndrome. Intermittently over the years patching with the peritoneum has been used, but the most successful method appears to be the mobilization of the omentum from its attachment to the transverse colon leaving a significant blood supply from the left side of the transverse colon and allowing the formation of a complete covering of the pelvis by a soft 'trampoline' of omentum which will then
The perineal incisions for anterior and total exenterations stretch, completely covering and bringing a new blood supply into the pelvis. From time to time procedures such as bringing gracilis muscle flaps into the empty pelvis have been carried out to deal with the difficulty of a devitalized epithelium due to previous radiation.
It is the author's current preference to use an omental graft mobilizing the omentum from the transverse colon using a powered autosuture; this allows a broad pedicle to remain at the left-hand end of the transverse colon, maintaining an excellent blood supply to the omentum. This is brought down to the right side of the large bowel, dropping into the pelvis immediately to the left side of the ileal conduit which is anchored just above the sacral promontory. By careful individual suturing around the edge of the pelvis and sometimes by refolding the peritoneum upon itself, a complete covering of the true pelvis with a soft central 'trampoline' area can be generated (Figure 12). A suction drain is inserted below the omentum, which when activated will draw the omentum down into soft contact with the pelvic floor. The small bowel can thus come into contact with an area with a good blood supply, obviating the risk of adherence and subsequent fistula formation. At the end of the procedure the bowel is carefully oriented to make sure that no hernia can develop and the abdomen is closed with a mass closure. The stomas are dressed in theatre and their appliances put in place. The patient leaves the operating theatre and is then transferred back to the ward at the appropriate time.
Closure of the pelvic floor musculature
The postoperative care of exenterations is straightforward, essentially being a matter of maintaining good fluid balance, good haemoglobin levels and ideally a significant flow of urine of 2.5-3.5 litres per day. Bowel function often returns at the usual time of 2-4 days following the procedure, and a nasogastric tube (the author's preference) can be removed after 3-4 days; the return to oral intake, beginning with simple fluid, is initiated on the third day. During and following the procedure prophylactic antibiotic cover is maintained, as is subcutaneous heparin cover as prophylaxis against deep venous thrombosis. Mobilization should be rapid. Patients are usually discharged 10-15 days postoperatively, once they are used to dealing with the stomas and the ileal conduit tubes have been removed.
Most series show that the 5-year survival rate following exenteration is of the order of 40-60%; these figures depend very largely upon the selection of patients. A figure that is rather more difficult to obtain is the exact number who are assessed for exenteration but fail at one of the many hurdles that the patient must face before finally undergoing the procedure. It is therefore likely that the final, truly salvageable figure is an extremely low percentage. The value of exenteration procedures in patients who have lymph node involvement has been shown to be low but significant, and it is now many clinicians' practice to carry on with an exenterative procedure even in circumstances where one or two pelvic lymph nodes are involved by tumour.
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Suture of the internal iliac arteries and lateral pelvic pedicle
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Development of the 'omental pelvic floor': (A) omental incision; (B) soft 'trampoline' area
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