Surgery : Part II
External Surgery
The late presentation, extensive nature and aggressive biology of a wide variety of sinonasal tumors necessitates surgery in the management of the majority of cases. The significant improvements in imaging with CT and MRI have allowed us ever-increasing preoperative accuracy with regard to the extent of the lesions. This improved imaging, combined with improved pathology-diagnostic techniques, has allowed us to more appropriately use the wide variety of surgical techniques available, which vary from endoscopic to major open skull base resection. Larger procedures may involve removal of intracranial structures, the bone of the skull base itself, and extensive components of the subcranial area when disease involves the orbit, nose, sinuses, and adjacent tissues. The surgeon, preferably part of a multidisciplinary team, attempts to combine a definitive cure with preservation of function, cosmesis, and quality of life.
The surgery in these areas is often complex and may require the careful coordination of several members of the multidisciplinary team to safely carry out the resection and reconstruction (should the latter be necessary). Much has been written about these evolving surgical techniques, but most of the variants of approaches to the anterior and anterolateral areas of the skull base by open surgery have now been with us for a sufficient time for us to have substantial long-term figures with regard to morbidity, mortality, and cure rates. Endoscopic surgery continues to be increasingly utilized but, as yet, the long-term outcomes of a substantial number of patients, particularly those treated for malignant disease, remain limited.
Despite our ever-increasing experience with these operative procedures, cure is still not possible in a significant proportion of these patients and is nearly always at a price of some morbidity. While the last three decades of craniofacial surgery have revealed low mortality and morbidity figures, it is to be hoped that endoscopic procedures, newer forms of radiotherapy, new chemotherapy and novel molecular biological agents will have an increasing amount to offer.
This book is not intended to replace the many excellent manuals published in recent years of operative surgery techniques for nose, sinus, and skull base tumors, but the following represents an assessment of the respective roles and limitations of the different surgical options.
When Tumors of the Upper Jaw was published in 1993, we stated that the immensely improved optics afforded by rigid and flexible endoscopes had greatly facilitated our examination technique and the hope was that this might ultimately lead to earlier diagnosis of disease. Sadly, while this may have occurred in some fortunate individual cases, the rarity of these diseases continues to be associated with a late clinical presentation as a consequence of both patients and doctors ignoring the early innocuous symptoms of sinonasal malignancy. The simple and extremely common symptoms of nasal block, discharge, and facial pain are often present for periods of between 6 months and 1 year, and it is only with advent of bleeding, facial swelling, orbital symptoms, and oral problems, or the appearance of a mass that prompts presentation of the patient. Endoscopes have allowed us to carry out far more accurate, specific, and multiple biopsies of many lesions, but there are still many patients in whom only imaging reveals the extensive nature of the disease and in whom obtaining appropriate biopsies can still be difficult. A high index of suspicion has to be maintained both in primary practice and in ENT outpatient clinics to detect these rare cases. Unilateral nasal and sinus symptoms, particularly in association with unpleasant nasal discharge or bleeding and inappropriate facial pain and paresthesia, should always be carefully assessed. Two further groups of patients are worthy of note in that, with the increase in modern scanning available around the world, asymptomatic patients may have lesions detected during investigation for other, unrelated problems. For those undertaking tertiary referral care, it is unfortunately the case that a substantial number of patients have had prior failed primary treatment and are referred with persisting/recurrent disease.
Clinical Features
Unilateral nasal symptoms of recent onset require as a minimum a good quality nasal examination and evaluation of any orbital, oral, or neurologic symptoms. If a short course of appropriate medical therapy does not produce a prompt resolution, patients should be referred for specialist assessment.
Following further examination in an appropriate ENT clinic, preferably with rigid and/or fiberoptic telescopes, further imaging studies may be indicated.
Imaging
This evaluation plays a key role in the pretreatment planning of both benign and malignant lesions involving the nose, paranasal sinuses, nasopharynx, and adjacent skull base. Both CT and MRI are frequently required as they are complementary when assessing lesions in this area and both are required to gain maximum accuracy in assessing the local regional extent of the involved tissues (Fig. 19.4).1
The individual imaging for each of the pathologies is outlined in the respective chapters and in Chapter 5. Since 1986, our own protocol has been to employ preoperative imaging by a combination of high-resolution contrast-enhanced CT (in coronal and axial planes) with 3 planar MRI enhanced with gadolinium diethylenetriamine pentaacetic acid (Gd-DTPA). This combination of imaging gives the best overall assessment of the tumors. It is important to understand that pain management may be required to allow a person to undergo this type of scanning and occasionally sedation is indicated as some patients confined in a magnetic resonance or CT scanner can find the experience very upsetting if they suffer from claustrophobia and the extent and nature of the procedure has not been explained to them.
Additional evaluation by modalities such as ultrasound or PET-CT scanning may be indicated depending on the tumor type and extent. It is important to discuss the outcome of the scans and any additional testing with the patient as well as at the multidisciplinary meeting with all colleagues. This is best done in association with the results of pathology from any biopsies.
Imaging may be of further importance as an added parameter to intraoperative guidance and is essential for long-term follow-up. However, posttreatment imaging undertaken with a different modality from the preoperative imaging, on a different machine with different settings, and then reported on by a different radiologist (often without access to the original imaging and no multidisciplinary discussion with the treating team) can be a complete disaster. Inappropriately early posttreatment imaging before treatment changes have resolved (less than 12 weeks and often longer) can also be seriously misleading. The patient′s and the team′s desire for early confirmation of “cure” can be disastrous. Additionally, too-early “repeat” posttreatment biopsy, again before a minimum of 12 weeks post treatment, can cause considerable confusion and distress if the pathologist reports what seems to be “residual” disease, when in fact this is nonviable.
All scanning techniques—CT, MRI, PET—become far more reliable at predicting “cure” after 4 months and preferably 6 months post treatment.
Histopathology
The diversity of pathology found within tumors of the nose, paranasal sinuses, nasopharynx, and adjacent skull base, is as great as any area of the human body. This is clearly documented in the WHO classification of tumors, Pathology and Genetics of Head and Neck Tumors edited by Leon Barnes et al in 2005—outlined in Table 4.2 on pages 42–44.
This list of tumors has been added to in this book with a variety of further conditions with potential for high morbidity and, in some cases, mortality.
Perioperative Patient Care—The Multidisciplinary Team
The multidisciplinary head and neck surgical team has existed for more than 30 years in our institution and combined clinics with our radiation oncology colleagues date back to 1952. However, in many institutions around the world, the multidisciplinary team is a relatively recent thing and, in some countries, is still not the accepted way of working. Within the United Kingdom, the head and neck surgical multidisciplinary team meets on a weekly basis, but in the last 10 years, there has been an increasing emphasis on an additional skull base team comprising those surgeons from ENT, neurosurgery, plastics, and maxillofacial with a particular interest in this group of patients. For more than 30 years our multidisciplinary teams have included colleagues from pathology, radiology, and radiation oncology and, at times, varying levels and numbers of specialist surgeons. Senior nurses, including over the past 15 years clinical nurse specialists, head and neck trained nurses, dieticians, social workers, nutritionists, speech therapists, physiotherapists, and ward clerks, have all been involved within the team. The key worker in the team has changed in emphasis from the senior ward sister to the clinical nurse specialist, but this situation varies in different hospitals and different countries. It remains extremely important that a particular individual can be in constant contact with the patient, whether at home or within the hospital. In many instances, a busy multidisciplinary group requires a coordinator to carry out administration, improve communications, mobilize test results, and collate/produce detailed documentation for multidisciplinary team meetings. The coordinator should ensure that data collection, both locally and nationally, are completed and logged. In addition, some coordinators are involved in clinic scheduling and research organization, although there are, unfortunately, multidisciplinary teams in the United Kingdom without coordinators and various members of the team undertake many of these functions.
For a young trainee surgeon, the emphasis is often on acquiring the surgical expertise but an understanding of the natural history of the disease and the use of all aspects of the multidisciplinary team can involve considerably more work and study to obtain a full understanding. It is vital for the trainee to understand this requirement and in particular to study the varying natural history of these diseases.
Preoperative Counseling and Patient/Relation Education
The benign and malignant diseases outlined in this book often require frank and detailed discussion with the patients and other family members. The topics covered are many and varied but may include:
Pain control
The effects of any environmental exposure at work
Smoking, alcohol use, obesity, and lifestyle in general
Poor nutrition, weight loss
The individual fears of cancer of both the patient and family
The issues related to the upcoming surgery, radiotherapy, and/or chemotherapy
The early and long-term postoperative treatment in relation to breathing, speech, swallowing, cosmesis, eyesight, smell, taste, hearing, and restoration of normal or limited activities
Lack of information with regard to the potential changes, morbidity, and indeed mortality, often leads to inadequate ability of the patients and their families to cope, not only with the possibilities of even moderate or major surgery but with the issues of radiotherapy and chemotherapy. Obtaining informed consent from this group of patients requires care and time, and while this is not always easily available in a busy therapeutic, tertiary referral practice, it is extremely important to put aside this time and for all the members of the multidisciplinary team to be well informed with regard to the patient′s preoperative situation. A high proportion of these patients present with advanced disease with a far from certain outcome as to whether or not they can be cured; it is important to establish a real and definite area of cooperation and trust within the multidisciplinary team, the patient, and their family. With specific surgical procedures often being done by one or more surgeons, it is important that one, and preferably more, of the surgeons take a specific interest in each patient along with their key worker, who is usually the clinical nurse specialist. While multidisciplinary team efforts have been advocated for many years, it is still essential that a main operating surgeon and the patient have appropriate time together to discuss the overall situation, both pre- and postoperatively.
The Head and Neck Clinical Nurse Specialist
In the United Kingdom, the clinical nurse specialists are frequently former senior nurses and many of them have been ward sisters on head and neck and neurosurgical wards. As such, they are advocates for the patients and have considerable clinical nursing expertise, but added to this, many of them understand the roles of all the staff involved in the multidisciplinary team and can act as advisers, counselors, teachers, and researchers and generally liaise throughout the team. They are specifically able to provide continuity of care for the patients and must be involved from a point of early initial contact right through to discharge to home and subsequent communications with the family practitioner, visits to home, and the patient′s return to the clinic. This is a demanding but incredibly satisfying role for the right individual and another keystone for improved care for the patient. They are however, not a substitute for full and careful communication between the treating physician/surgeon and the patient.
Initial Assessment and Symptom Management
Patients with relatively early tumors of the nose, paranasal sinuses, and skull base may have little in the way of symptoms as alluded to above, but any that present late or having had previous failed treatment do have important problems with regard to pain control and symptoms such as epistaxis, epiphora or double vision, trismus, dental problems, and so on. While immediate consideration is given to establishing an accurate diagnosis if this has not already been undertaken, it is important before considering investigations, or certainly at the same time, for the consulting doctor and the team to address the patient′s pain control and other needs. Diseases of this area rarely result in major nutritional issues as most patients are still able to take food orally, unless the disease is so extensive as to involve the oral cavity or to produce severe trismus. However, weight loss, as with cancer in general, may already be significant and early assessment by the dietician, irrespective of the subsequent form of treatment, is essential.
Pain management is increasingly undertaken by specialist members of the team and morphine and its derivatives may be required immediately and, on occasion, immediate patient admission may be necessary to address the issues of pain and epistaxis. Only when these problems have been brought under control, should the team proceed with imaging, biopsies, and so forth.
Hospice Care
Unfortunately, advanced disease in these areas may be unresectable and inappropriate to be treated with either chemotherapy or radiotherapy; it is important that the multidisciplinary team agree on these issues and that the patient and the family receive immediate supportive care, and that any primary physicians receive adequate and early information with referral of the patient and family to an appropriate local hospice, if this exists in the country in which they present. When patients are transferred from hospital to hospice, or are returning to their home with appropriate primary care, pain control and nursing must be clearly communicated and documented to the ongoing carers.
Nutrition
The importance of nutrition in all forms of major head and neck tumor treatment cannot be overemphasized. As in other areas of cancer, there has been an increasing international literature over the last three decades that shows that it is important to establish nutritional parameters:
Prior to treatment
Throughout the treatment
During the posttreatment phase, particularly when combined therapy has been instituted
Patients with malignant disease of the nose, sinuses, and skull base are, unfortunately, no different from any others within the general population in that obesity, diabetes, respiratory, cardiovascular, and gastrointestinal disease and arthritis are all common accompanying factors. The overall weight of the patient and any loss or gain in recent times should be clearly established along with the present state of nutrition, which may require intervention and careful planning with correction prior to any major treatment considerations, either surgical, radiotherapeutic or chemotherapeutic. A nutritional plan is individualized for each patient and reassessed at regular intervals throughout any subsequent treatment. There is a wide variation in patients’ tolerance with regard to oral feeding or nasogastric or parental feeding with a wide variety of nutritional support and hence the need for a continuous and expert input if this is available. In those countries where dietetic/nutritionist expertise is not available these considerations must be taken on by the surgical team. Irrespective of the expertise of any surgeon, good postoperative results with low morbidity and mortality will not be obtained if operating on nutritionally compromised, dehydrated, and poorly prepared patients.
A proportion of patients with advanced disease of the skull base have significant cranial nerve deficits preoperatively, but these may be markedly increased following surgery and/or radiotherapy and may be accompanied by problems such as silent aspiration. In these patients, early assessment of these problems and their long-term care may be absolutely crucial to a successful outcome. They may require a full swallowing rehabilitation program and close cooperation between the nutritionists and speech therapists (speech pathologist).
Social Services
A proportion of the elderly patients who may present with diseases in this area will live on their own and may not have significant family support. For other patients, even ones who have significant family help, social service issues may need to be addressed from the outset and considered prior to any significant surgery. The important issues of coping on return to home may require adaptation. For instance, if it is necessary to remove the patient′s eye or maxilla or to carry out substantial facial reconstruction, then additional support may be necessary to help the patient cope with their subsequent return to home and, if possible, work. A patient′s comorbidity, dependence on alcohol, and drug history may also be part of the important mix of their requirements; social assessment and nursing evaluation needs, again, may be channeled through the clinical nurse specialist, depending on the circumstances within each given team or the country in which the patient is being treated. The patient may require a variety of aids both in and out of the home and help to deal with issues of disfigurement, mobility, ability to drive, and so on.
As in all forms of cancer surgery, occasionally, pre-treatment assessment may require expert psychiatric opinion and certainly this is necessary in a small number of patients who survive the more arduous forms of combined treatment and find it difficult to reestablish a reasonable quality of life.2 These issues cannot be ignored as the cure of many of these unfortunate patients is only the beginning of a long period of rehabilitation.
Dental Assessment; Osseointegration Prosthetics
The history of prosthetic rehabilitation after maxillectomy extends back to Syme in 1835. By the early part of the 20th century, prosthetic and dental restoration after maxillectomy were well developed, with Woodman′s seminal description of the technique of an initial plaster cast, a temporary denture, and subsequently a permanent denture, being described in 1923 (Fig. 19.5). These techniques have been continuously improved with the use of an ever-increasing range of modern materials so that in our own hospital, preoperative assessment and fashioning of an upper jaw prosthesis, which was subsequently placed at the time of the ablative surgery, dates back for more than 60 years (Fig. 19.6). In contrast, thorough pretreatment oral and dental evaluation by an oncologically orientated dentist documenting oral and dental pathology prior to any cancer treatment is far from universal. Many treating surgeons and physicians still fail to remember how important it is before treatment to reduce or remove any sources of potential infection and to consider osseointegrated implantation at the time of the ablative surgery to aid subsequent rehabilitation even if there is to be postoperative radiotherapy and chemotherapy. The surgeon, radiation oncologist, and medical oncologist should be able to assess oral pathology secondary to poor dental status, notably advanced periodontal disease, gross dental caries, poorly fitting existing dentures, and poor oral hygiene. As part of the initial assessment, the patient should be referred to a dental colleague to undertake thorough oral and dental assessment with appropriate radiographs, which may include occlusal, panoramic, periapical, and bitewing views. These are relatively easy and inexpensive investigations in comparison with CT and MRI and may be extremely important in the long-term care of the patient. This is particularly the case if an extensive nasal, paranasal, sinus, or skull base lesion involves the interior aspect of the upper jaw and prosthetic rehabilitation depends on stable anchorage of any prosthesis to remaining teeth on the opposite side (Fig. 19.7).
Teeth with potentially poor prognosis require extraction before postoperative chemotherapy or irradiation. With forward planning, this may be done under the same general anesthetic as the ablative surgery. Performing these removals at the time of the primary surgery may decrease the workload for the team and the waiting time for the patient. There is considerable potential room for improvement with regard to this aspect of patient care as subsequent osteoradionecrosis remains a serious and debilitating problem with a generally poor outcome. Dental assessment is frequently required in the postoperative and posttreatment period, particularly with patients who undergo additional irradiation because—even with the more modern techniques of IMRT—they may still experience some dry mouth (xerostomia) and an increased incidence of dental disease, particularly of the upper jaw in this group of patients. Patients may require continual encouragement to use simple measures such as a fluoride rinse and to attend their general dentist for routine care in the long term after their treatment.
There have been steady improvements in the extraoral application of osseointegrated techniques to allow improved retention of intraoral/maxillectomy/orbital/nasal/hemifacial prostheses in the last 20 years (see Chapter 21). While reconstruction of maxillectomy defects with free flaps is becoming increasingly popular in those countries able to undertake the technique, it does not always provide the best alternative to a well-constructed obturator prosthesis, particularly if the latter can be secured in an excellent manner on any remaining teeth and with additional osseointegrated implants. There are cases, particularly those patients who are edentulous, where the free flap reconstruction may offer an acceptable aesthetic and functional result, but in older patients the prolonged operating time may be a contraindication. In contrast, for some of the older edentulous patients and less capable individuals who are unable to maintain and accurately place a prosthesis, a free flap reconstruction may be beneficial. Careful consideration must be given to each individual patient.
Overview
Insufficient attention has frequently been paid in the past to understanding patients’ concerns with regard to proposed treatment of their nose, paranasal sinus, and skull base tumors. The multidisciplinary team needs to be sensitive to these issues and to understand that a patient′s ability to take in information under the stress of this situation is, to say the least, suboptimal. The use of medical terms rather than understandable everyday language is inappropriate, and the whole patient journey requires patients to be cared for and supported in a positive manner. The unique aspects of surgery in this region and the possibility of additional radiotherapy and chemotherapy require frank discussion with the patient and their family members; and the patient′s individual fears about why they have presented with these cancers and the issues around complications, morbidity, disfigurement, and overall changes from their normal activities require thorough discussion. Informed consent should be obtained by senior members of the team, preferably the consultant surgeon undertaking the procedure and with the clinical nurse specialist present. The patient, the relatives, and the team members will all benefit from the establishment of an atmosphere of mutual trust, particularly where the situation is likely to change over the passage of time, and there is always the possibility of failure of therapy or recurrence of disease in addition to the morbidity and occasional mortality.
Key Points
Surgery in these areas is often complex and requires the careful coordination of several members of a multidisciplinary team.
The rarity of these diseases, in contrast to their often common presenting symptoms, means that the majority of patients still present with advanced disease.
A high index of suspicion for unilateral nasal and sinus symptoms is needed in primary practice and with ENT outpatients to detect these rare diseases.
Both CT and MRI are usually required for imaging as they are complementary and provide maximum accuracy in assessing locoregional tumor extent.
The diversity of pathology found within tumors of the nose, paranasal sinuses, nasopharynx, and skull base is greater than in any other area of the human body.
Multidisciplinary team-working improves the care and outcomes for these patients.
Young surgeons need to acquire a thorough knowledge of the pathology and natural history of these diseases.
Preoperative counseling of these patients and their relatives is essential.
A dedicated head and neck clinical nurse specialist is an invaluable member of the team.
Pain control management and nutritional assessment are essential in advanced disease.
Pretreatment dental assessment and prosthetic rehabilitation are important considerations in selected patients.
Thorough pretreatment of oral and dental disease to remove or reduce infection and to consider osseointegrated implantation is essential in many patients, but particularly those undergoing radiotherapy ± chemotherapy and maxillary sinus surgery.
Appropriate pre- and postoperative information, care, and support are essential for these patients.
Surgical Treatment
This component of the book is not designed to replace the many excellent major surgical manuals that are available on these subjects and clearly such issues as operative equipment and the positioning, prepping, draping, and overall planning of any operative procedure will depend on the individual operation. What is important in all cases is close cooperation and clear exchange of information with anesthesiology colleagues, particularly in the circumstances of patients with notable trismus or other possible airway difficulties. The positioning of any endotracheal tube or the establishment of a tracheostomy needs careful consideration depending on the approach to be used to the tumor.
Experienced circulating and scrub nurse personnel are an important requirement, as too is the recovery and postoperative nursing care. While many postoperative wards and intensive care units may contain highly qualified nursing staff, various problems can arise if they are not familiar with the specific requirements of patients undergoing surgery for tumors in these areas. Postoperative neurological monitoring, care of the airway, care and use of any prosthesis and any reconstruction by means of flaps and grafts all require appropriate understanding along with the general principles of wound care and postoperative patient management.
Key Points
Cooperation and clear exchange of information with anesthetic colleagues is essential.
Perioperative and specialist postoperative nursing care are essential.
Selection of a Particular Operative Procedure
In selecting a particular procedure for an individual patient, the site, size, histology, and natural history of the patient′s disease and any additional comorbidities are extremely important. Surgeons are influenced by their personal philosophy, past experience, and technical capability. A professional approach is required if patients who are curable are to be cured and the aim of any surgery is total removal of the tumor. Combined approaches to any cancer require the availability of a wide range of expertise and often individuals of compatible experience may not be available in most multidisciplinary clinics and democratic discussion may be weighted toward one particular discipline. In many countries, unfortunately, financial issues take part in the decision-making process and an undesirable, competitive element between different departments may further complicate the issues. However, if we are to improve the overall poor long-term control rates for many of these diseases, and the persistence of the crippling and mutilating effects of many surgical resections, then these procedures require careful consideration. Mere acquisition of multiple surgical technical skills does not necessarily produce appropriate selection for patients and this whole area is fraught with philosophical considerations. In particular, the advantages of successful surgery must outweigh the risks and subsequent morbidity and the surgeons must ask themselves whether they are able to perform the procedure to an accepted level of competence, bearing in mind what is the best treatment for any particular patient with any specific disease. If this is not the case, they should consider referring the case to a more experienced colleague or an alternative multidisciplinary team dealing specifically with these rare tumors.
Much of the surgery in this area aimed at cure or long-term palliation is associated with a degree of mutilation, and certainly notable interference with function. In some countries, such disabilities preclude social acceptance and facial disfigurement is still an important problem in most communities. Even excellent external prostheses only act as camouflage and considerable damage to the patient′s self-image may present a serious problem, with them requiring considerable support to return to work or other aspects of daily life (Figs. 19.8 and 19.9).
In contrast, however, electing a conservative procedure with little hope of cure may only allow the disease to progress and cause the patient far more in the way of symptoms prior to an unpleasant death. While the patient may pay a price for a “cure at any cost” philosophy, inadequate “debulking” and reliance on “chemotherapy” can produce devastating sequelae.