The surgical management of acquired immune deficiency syndrome (AIDS).
WALTER J. BURDETTE
INTRODUCTION
Acquired immune deficiency syndrome (AIDS) has attracted widespread attention and fear because of its uniform fatality, its association with a controversial lifestyle, and its continued rapid increase in incidence. The groups in which the disease was originally prevalent included homosexuals, users of intravenous drugs, and recipients of blood carrying the virus. Now they also include heterosexuals and children of infected mothers. Racial and geographic disparity still exists. In the United States, the annual rates per 100000 population vary from 0.1 in South Dakota to 40.6 in New York state, 47.7 in Puerto Rico, and 71.2 in New York City. The geographic rankings in descending numbers of AIDS cases reported are the United States, Uganda, Zaire, Brazil, France, Malawi, Tanzania, Italy, Kenya, and Spain. So far the disease has yielded only to improvement in palliation.
Most cases of AIDS are treated by medical specialists, but many diagnostic and therapeutic problems require surgical participation. Experience with surgical management of AIDS has now been sufficiently extensive to provide some general guidelines for appropriate approaches to problems with a given set of clinical findings. In addition to the responsibilities associated with surgical treatment, the surgeon has the duty to protect personnel and himself from the dangers of infection prevalent in an environment where medical personnel are exposed to infected blood and body fluids and the danger of injury with a contaminated instrument.
HIV and AIDS
Acquired immune deficiency syndrome was first diagnosed in 1981, but subsequent retrospective studies have documented cases earlier than this. The search for the cause of the clinical syndrome resulted in the isolation of a virus from tissue by Montagnier et al. in 1983 which he called lymphadenopathy-associated virus (LAV). In 1984 Gallo et al. cultured a virus from AIDS patients and identified it as HTLV-III. The official name given at that time to the AIDS virus was HTLV-III/LAV, and this may be encountered in the early literature. However, the International Committee for Taxonomy of Viruses changed the name to human immunodeficiency virus (HIV), and the structure of this retrovirus is now known. Following invasion of a cell by this virus, viral RNA is converted to DNA by reverse transcriptase, which is integrated into the host genome, and undergoes replication. When this phenomenon occurs in helper/inducer CD4 lymphocytes, for which the virus has a predilection as the CD4 molecule acts as a receptor for the virus, they are destroyed as the newly produced viral particles are released. The cellular immune response of the host is impaired, leading to increased susceptibility to infections and to uncommon tumours in immunocompetent individuals. Patients infected with HIV virus do not show clinical symptoms when immunosuppression is not severe; they are said to be affected with AIDS-related complex (ARC) which later progresses to the clinical syndrome of AIDS. Viral infection can be screened by using an enzyme-linked-immunosorbent assay, but diagnosis of an individual case requires the Western blot test. Immunity is not conferred by infection with this virus.
DIAGNOSIS OF AIDS
When laboratory evidence of infection with HIV is present, a diagnosis of AIDS may be made with even a presumption of the following complicating diseases: Kaposi's sarcoma, infection with Pneumocystis carinii, cerebral lymphoma, non-Hodgkin's lymphoma, pneumonia, disseminated mycobacterial infection, cytomegalovirus retinitis, or toxoplasmosis of the brain (Figs. 1–11) 32,33,34,35,36,37,38,39,40,41,42. In the absence of a positive test for HIV infection, the diagnosis of AIDS may be made when there is definitive diagnosis of candidiasis of the oesophagus or tracheobronchial tree, non-Hodgkin's lymphoma, HIV encephalopathy, Kaposi's sarcoma, Pneumocystis carinii pneumonia, extrapulmonary cryptococcosis, extrapulmonary Mycobacterium avium or M. kansasii infection, prolonged cryptosporidiosis, cerebral toxoplasmosis, herpes simplex infection lasting longer than 1 month, progressive multifocal leucoencephalopathy, pulmonary lymphoid hyperplasia or pneumonia in a young child, or a T4 helper/inducer lymphocyte count below 400/mm³.
OPERATIVE MANAGEMENT
Major operations are not often indicated in AIDS patients. However, biopsy, endoscopy of alimentary tract and bronchi, closed thoracotomy with thoracic drainage, and provision of vascular access are frequently required. In the early stages of the disease these patients can be treated as any other patient with symptoms, signs, and laboratory results indicating that operative management is required. Later the risk is increased and the usual clinical signs of disease may be lost. Patients with AIDS may require both elective and emergency abdominal operations, but there is a five-fold higher mortality rate following emergency rather than elective procedures. The most frequent abdominal emergencies are intestinal obstruction (including volvulus), gastrointestinal haemorrhage, perforation, and peritonitis. Elective procedures are usually those carried out for diagnosis in the presence of a mass or pain, splenectomy, operation to relieve partial or intermittent bowel obstruction, cholecystectomy for the treatment of Salmonella sepsis, and cytomegalovirus infections. Wound healing is not usually a problem following abdominal operations. Fortunately, more than twice as many operations are performed electively rather than as emergency procedures. When patients are operated on in an emergency, especially late in the disease, it may be advisable to use retention sutures and not to close the skin primarily. In reality, it is necessary to return to the measures used before the advent of antibiotic medications for management of surgical complications. The variety of procedures performed in any given facility varies widely. For example, those in descending order in one group of cases with AIDS were biopsy, including cerebral stereotactic localization with biopsy or culture, endoscopy, abdominal procedure, thoracic procedure, rectal operation, and insertion of sinus windows. The use of a vena cava filter when thrombophlebitis first occurs is advocated by some clinicians.
INFECTIONS IN AIDS
Many infections in patients with AIDS are similar to those found in other types of immune suppression, such as those associated with measures used to prolong survival of grafts and following chemotherapy. The clinical conditions occurring most often are those normally combated by helper (CD4) T cells and related cell-mediated immune responses. Infecting organisms which are normally damaged or eliminated by antibody-mediated and non-specific immune mechanisms do not constitute the same problem as such potentially lethal organisms as Pneumocystis carinii, Toxoplasma, and Cryptococcus. Abdominal symptoms encountered are usually the result of infections with cytomegalovirus, mycobacteria, or Salmonella typhimurium.
Infections with cytomegalovirus may result in perforation and bleeding; in the case of cytomegalovirus enterocolitis, the appearance of the mucosal surface may be somewhat deceptive and confused with ulcerative colitis. When operation is required on such bowel it is best not to reanastomose the gut since localized disease may not be apparent grossly on inspection of the mucosa. An enterostomy should be used instead. Retention sutures and allowing the skin to close by second intention should also be considered.
Mycobacterium avium intracellulare is one of the most frequent of the unusual infecting organisms encountered. In the case of lymphadenopathy and other manifestations of infections with M. avium, diagnosis by means other than biopsy of retroperitoneal nodes can be successful in most cases and should be attempted before an operative approach is undertaken. Diagnosis may be obtained from needle biopsies of liver and bone marrow and from blood cultures. Abscess of the spleen in patients with mycobacterial infection may necessitate splenectomy which may also be indicated for treatment of thrombocytopenia. Infections with Mycobacterium tuberculosis and M. kansasii also occur in AIDS patients, but not as often as M. avium infections. Other abscesses, infection of the liver, and generalized peritonitis also may be the result of infection with mycobacteria. The presence of intra-abdominal infection may necessitate ileocolic resection, drainage of abscesses, and exploratory operation.
Salmonella typhimurium infection may require vigorous antimicrobial therapy, and cholecystectomy may be necessary to control sepsis, particularly when gallstones are present. This organism can weaken the arterial wall, and may produce pseudoaneurysms of aorta, iliac, or femoral arteries requiring repair.
The types of infection encountered in patients with AIDS varies, depending on location and facility. For example, in one series infecting agents encountered were Candida, Pneumocystis, cytomegalovirus, Mycobacteria, herpes, hepatitis viruses, Cryptosporidium, Staphylococcus, Histoplasma, organisms causing blastomycosis and lues, and Legionella. Others not found in this study but occurring elsewhere fairly frequently include Aspergillus, Listeria, Mucor, Nocardia, Strongyloides, Toxoplasma, Salmonella, and Zygomycetes.
TUMOURS IN AIDS
The two types of tumours encountered most frequently in AIDS patients and whose complications may require surgery are non-Hodgkin's lymphomas and Kaposi's sarcoma. Other tumors such as melanoma, astrocytoma, and basal and squamous cell carcinoma may also be found. Although first described as cutaneous nodules in the lower extremities of elderly Mediterranean males, Kaposi's sarcoma can be disseminated in AIDS patients, involving any organ, including the skin, gastrointestinal tract, liver, and lungs. When present in the alimentary tract the prognosis is poor; diagnostic endoscopic biopsy may or may not give positive results because of the location of the tumour in the submucosa. The presence of Kaposi's sarcoma in the gut can lead to dysphagia, abdominal pain, haemorrhage which can be massive, perforation, a syndrome similar to toxic megacolon and ulcerative ileocolitis, malabsorption syndrome, and bowel obstruction. Non-Hodgkin's lymphoma, a high grade B-cell lymphoma, can cause gastrointestinal bleeding, obstruction, perforation, and may present as an abdominal mass. Resection of the tumours or bypassing them usually yields the desired result. Radiation and chemotherapy possibly may accelerate a fatal outcome because of the resulting increase in immunosuppression.
PULMONARY PROBLEMS IN AIDS
Neoplasms and infections of the lung occur frequently in AIDS patients; life-threatening pneumonia affects approximately two-thirds of patients with the disease. Bronchoscopic biopsy and lavage have been a successful means for diagnosing diffuse lesions, but are less so with focal disease. Pneumocystis, cytomegalovirus, fungi, and atypical mycobacteria are the most common aetiological agents. When endoscopic biopsy or washings are not appropriate or successful in making a diagnosis of Kaposi's sarcoma or lymphoma, an open lung biopsy may be necessary. In patients with pneumothorax, closure of the pulmonary defect may be indicated when closed thoracotomy and thoracic drainage tube(s) fail, but caution must be exercised in adopting this option. Removal of a tumour of the lung early in the disease has been undertaken successfully without complications, and the opportunity should not be missed because of the fear of a fatal outcome at that time. Haemoptysis from an aspergilloma can be sufficiently severe to require thoracotomy with resection since bronchial artery embolization and intracavitary amphotericin have not been very successful treatments. Bronchial lavage and biopsy has reduced the number of thoracotomies necessary for diagnosing the nature of pulmonary infections and, at times, tumours. However, pneumothorax is not an uncommon complication of a vigorous approach to pulmonary biopsy via the bronchoscope. Closed thoracotomy and insertion of one or two thoracic tubes for closed drainage is then necessary. A balloon catheter may be useful for clearing the lumen of an obstructed thoracic drainage tube. Although the use of bronchoscopy for diagnosing infections in adults has almost superseded open lung biopsy, this is not necessarily the case in infants and children. Creation of a pericardial window for drainage can be done through a subxiphoid approach perhaps even by video-assisted thoracoscopy. The wisdom of placing a patient on a respirator is often debatable, although there may be sufficient recovery to discontinue this management early in the course of AIDS. Tracheostomy is almost never indicated because of the danger of overwhelming infection and difficulty in containing the contamination.
OTHER COMPLICATIONS OF AIDS
Neurological complications of AIDS includes the presence of intracerebral masses, encephalitis, meningitis, myelitis, radiculitis, and progressive multifocal leucoencephalopathy. Symptoms, including progressive dementia, are present in 40 per cent of patients. Infections with papovavirus, Varicella-zoster virus, cytomegalovirus, and herpes simplex, and non-viral infections such as cryptococcosis and tuberculosis can occur. Stereotactic localization, biopsy, and culture of accessible sites may be indicated occasionally.
Oral lesions may include candidiasis and hairy leucoplakia. Kaposi's sarcoma and mycobacterial infections can be localized in the upper alimentary tract, and both infections and tumours may lead to bleeding, ulceration, and obstruction that can be life threatening. Cervical lymphadenopathy may be troublesome, but biopsy does not often alter clinical management. Maxillary sinus infections may require drainage.
Infections of the soft tissues usually are caused by multiple aerobic and anaerobic organisms. Bacteria other than Clostridia also produce gas in these lesions, and pentamidine ulcers may be quite troublesome to treat. Proctocolitis is the most common problem for AIDS patients in some locations. Because of depressed immune responsiveness, operations in anal and rectal areas must not be undertaken except in unusual circumstances. A minor procedure may result in an overwhelming cellulitis and may require a colostomy.
THERAPEUTIC AGENTS USEFUL IN AIDS
Much research is in progress to develop drugs effective against HIV itself and against the infections that occur in AIDS patients. Of the former zidovudine (AZT) is widely used as a treatment of AIDS and is moderately effective, while those that are used currently to treat Pneumocystis carinii infections are pentamidine isothionate, dapsone, co-trimoxazole, and zidovudine. Others such as clindamycin and primaquine show some promise. Alone and in combination, a battery of drugs is required to produce any therapeutic response to infections with Mycobacterium avium. Those used most frequently are aminoglycosides, ciprofloxacin, clofazimine, ethambutal, imipenem, isoniazid, and the rifamycins. Gancyclovir is used for retinal and gastrointestinal damage resulting from infections with cytomegalovirus. Foscarnet will be licensed soon. Initially amphotericin B was the only drug at all effective for infections with Cryptococcus neoformans; itraconazole and fluconazole also show some promise. Pyrimethamine and sulphadiazine are used for treating Toxoplasma gondii infection. The extensive and prolonged damage that herpes simplex viruses 1 and 2 can produce in AIDS patients responds to acyclovir, provided that the infecting strains are not resistant. Chemotherapy and/or irradiation for the tumours that appear in AIDS patients follows the same protocols as for patients with the same tumours who do not have AIDS. Unfortunately treatment is limited by toxic effects, some of which are similar to those caused by HIV. The spectrum of drugs and manner of combining and administering them both to treat infections and tumours in AIDS, is rapidly changing, resulting in improved palliation, but no cures are available at present.
PROTECTION OF HEALTH-CARE WORKERS FROM HIV
The chief sources of HIV infection in health-care workers have been blood and tissues, semen, vaginal secretions, or other body fluids containing blood. Pleural, peritoneal, pericardial, amniotic, synovial, and cerebrospinal fluids are also potential sources of infectious virus. Contact with oral or other mucous membranes and skin lesions of patients, droplets that are airborne, and laboratory specimens containing the virus should also be avoided. There is no evidence that the virus occurs as an aerosol from the lungs.
Routes of transmission to the surgeon and other health-care workers are via mucous membranes, transcutaneously after prolonged contact or when dermatitis or other conditions have destroyed the normal dermal barrier, and percutaneously by injuries from sharp instruments contaminated with infected blood. The risk of transfer of infectious virus by a needle puncture has been calculated as 0.4 per cent. The transfer of virus to health-care workers has occurred most frequently during manual handling and loading of needles, hand-to-hand transfer of sharp instruments, during lengthy procedures associated with blood loss, emergencies, management of trauma, and obstetric, gynaecological, orthopaedic, vascular, and cardiothoracic procedures.
Preventive measures include the identification of patients with AIDS and ARC. (When precautions are universal in suspected groups, this is not mandatory.) No surgeon or member of an operating team should participate if he or she have defects in skin and/or immune barriers. Operating gowns should prevent passage of fluids completely or an effective apron should be worn in addition to an ordinary gown. Helmet head covering and protective eye wear should also be used. Needles should be handled with instruments only, and there should be no hand-to-hand passing of sharp instruments. The use of staples and cutting cautery should be considered when feasible. During emergencies on the ward and in the emergency room and intensive care units the improper use and disposal of large-bore needles is a known source of transfer of HIV to health-care workers. The urgency of the moment should not cause the usual precautions to be abandoned. Double gloving is advisable in the operating room, and gloves should always be used when dressing wounds or examining orifices and when handling contaminated materials. Gloves should be discarded in an appropriate container immediately after use.
After a procedure has been completed and gloves removed, the hands must be washed thoroughly. If there has been contamination of broken skin or injury with sharp instrument or needle the area should be cleansed thoroughly but application of caustic material to the site is not recommended. Evidence for the effectiveness of immediate initiation of therapy with the antiretroviral agent, zidovudine (AZT), which inhibits the production of provirus, is incomplete. The person affected should be tested for HIV antibody at the time and periodically over the following 6 months, and should refrain from transmitting blood, semen, and milk or donating an organ to others for at least 12 weeks.
The usual measures for sterilizing instruments and customary cleaning of the operating room are adequate. Cleaners should wear protective gloves, gown, helmet head covering and eye wear or shield. Discarded needles should not be bent or replaced in an individual container. Both needles and disposable blades should be discarded in a puncture-proof, sealed container. A reliable system of transfer of specimens either to the laboratory or to a site of disposal should be adopted so that no specimen or other item contaminated with infected body fluids, blood, or tissue is lost or arrives in the laboratory without the nature of the material being known to the receptionist. When ventilation of the patient is necessary, disposable equipment should be available and mouth-to-mouth resuscitation should never be attempted.
Much has been published about the rights of the patient with AIDS for treatment and the rights of health-care personnel when managing their treatment. Knowledge of the methods of transmission and the dangers of infection with HIV virus and adoption of the precautions suggested while caring for these patients should reduce the danger of contracting AIDS sufficiently to remove most objections about risks of exposure and provide adequate numbers of personnel willing to provide the therapy required.
SUMMARY
The surgeon has a role to play in the clinical management and diagnosis of the protean manifestations of AIDS. Familiarity with its unusual features is required to ensure appropriate and effective management of complications requiring attention in a manner that avoids risk to the surgical team.
A wide range of unusual infections with clinical manifestations and the appearance of non-Hodgkin's lymphomas and Kaposi's sarcoma at multiple sites in patients with AIDS are problems encountered with increasing frequency by the surgeon. To make diagnosis and management more difficult, warning clinical signs often are effaced late in the disease because of deranged immune responses.
Biopsy, endoscopy, procedures to provide vascular access, and closed thoracotomy drainage are frequently required in the management of AIDS.
Exploration of the abdomen for relief of obstruction, drainage of abscesses, biopsy and resection of tumours, splenectomy, cholecystectomy, appendectomy, and colostomy and ileostomy may be required in the care of patients with AIDS. Emergency abdominal procedures have a much poorer prognosis than elective operations and often require deviation from usual modes of treatment. Anal and perineal procedures should not be undertaken except in exceptional circumstances. Tracheostomy is almost never advisable. Thoracotomy can be performed successfully in selected patients. Intubation requires careful evaluation of indications before initiating the use of a respirator in selected cases.
Operations should not be undertaken by those with defective dermal and immune barriers. Appropriate protective gowns, eye wear, and double gloves should always be used when any procedure is done at any location, and no manual handling of needles or hand-to-hand passage of sharp instruments is permissible. Exposure to the AIDS virus requires immediate cleansing of the site and subsequent testing for HIV, but the usefulness of immediate administration of zidovudine or other therapy is unknown.
FURTHER READING
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