- THIS MATERIAL IS PUBLISHED AND PROTECTED BY U.S. COPYRIGHT LAW - REPRODUCTION PROHIBITED UNLESS FOR PERSONAL USE, EXCEPTING AUTHOR PERMISSION - NEUROPATHY and ARTHROPATHY IN THE INSENSITIVE FOOT PRESENTED IN FOUR PARTS PART THREE Peter F. Kelly, D.P.M., F.A.C.F.A.S. Diplomate, American Board of Podiatric Surgery Fellow, American College of Foot and Ankle Surgeons TABLE OF CONTENTS PART ONE Abstract 3 Introduction 4 Summary of Development of the Neuropathic Joint 4 The Progression of Joint Destructive Hyperlaxity 5 The Clinical Appearance of Diabetic Neuropathy 8 The Histochemical Basis of Diabetic Neuropathies 10 Hereditary Peripheral Neuropathies 12 Acquired Peripheral Neuropathies 13 The Ulcerative Neurotropic Foot 15 PART TWO 17 Abstract 18 Vascular Findings in Diabetic Neuropathy 19 Physical Exam 20 Clinical Findings of Charcot Joints 21 Radiologic Findings of Charcot Joints 21 Diagnostic Methods 22 PART THREE 26 Abstract 27 Principles of Therapy 28 Surgical Management 32 Conservative Management 33 Medical Management 37 Summary 38 ----------------------------------------------------------- ABSTRACT In the first of three parts of this paper, the more frequent etiologies of the various neuropathies causing arthropathies found in Podiatric Medicine were discussed. Section two surveyed a variety of diagnostic modalities along with the clinical features useful in assessing the nature and extent of the neuropathies to the physician. Section three covers medical and surgical therapeutic measures relating to the specific nature of the neuropathic symptomatology. PRINCIPLES OF THERAPY The most common sequellae of peripheral neuropathy in lower extremity are foot ulcerations and osteoarthropathy. By understanding what causes the damage to these tissues, the practitioner will be able to administer the most effective way of protection. The determination of the forces creating destructive pressures on soft tissue during normal ambulation is nearly impossible because transducers which measure only the vertical component of sheer stress do not corellate quantitatively with the assessment of stress. Much of the trauma is due to shear stresses resulting from simultaneous vertical and horizontal or rotary forces. More than six hundred pounds per inch square is needed to produce a break or tear in human skin. However, if there is some shearing stress with it, only 200 pounds per square inch is necessary to be painful to limbs with normal sensation. Such shear stress occurs at the edge of the area of distribution and results twisting or tearing of tissues.(44,45) Five types of tissue damage are described by Brand: 1. Continuous pressure causing a focal ischemia leading to necrosis, 2. Concentrated high pressures causing a cutting or crushing of tissue, 3. Heat or cold causing burning or frost bite, 4. Repetitive mechanical stress, and 5. Pressure on infected tissues. Specific recommendations are given to the neurotropic patient which addresses these categories. To avoid continuous pressure, the patient is advised to never wear new shoes for more than two hours at a time and then to observe for any area of of redness, swelling or irritation. Frequent changing of shoes, at midday and again in the evening, will to reduce the possibility of specific pressure sites over prolonged periods of time. To reduce concentrated high pressure, the problem is not in dealing with excess force, but rather having a normal amount of force distributed over a reduced area. It is important to recommend to the patient with insensitive limbs the practical applications of every-day living so that they can learn to think ahead and prevent such stresses. Practical applications such as never to walking barefoot and always shaking out a shoe before putting it on in case foreign object fall into the shoe during the night. Patients with insensitive limbs should take extra care to their environment. Areas of normal travel may present as an accommodating walking surface, but in reality may prove to have intollerably high or low temperatures. Barefoot walking on sandy beaches seems an excellent surface to walk on for those having plantar calluses, but the high density silicone constituents of sand retains a deadly amount of heat for soft tissue. Foot problems may be also caused by burns from the floor of a car. In this case thick carpeting to accommodate the car floor to the needs of the patient should be installed in order to provide thermal insulation from the floorboard. Research by Brand has shown that repeated mechanical stresses on normal soft tissue results in moderate levels of inflammation. The repetition continued on inflammed tissue intensifies inflammation, bringing in many new cells full of lysozomal enzymes into the area. Autolysis resulting in focal necrosis will occur in the area at the center of the stress. The necrosis starts deep beneath the skin and will later form a fistula or sinus tract to the surface. This area of necrosis is frequently predisposed by a hyperkaratosis, which initially acts as a biological protection but later turns into a source of pressure. The remedy is lowering the pressure and redistributing the pressure, lowering the number of repetition by walking shorter distances or taking more frequent periods of rest with periodic examinations for inflammation, redness and swelling. When a patient walks on a wound, the wound will become infected. Continued walking on an infected wound spreads the infection through soft tissue planes, to bones and joints, and may result in severe destruction, sometimes necessitating amputation. Therapy for infected lesions is immediate complete bed rest for a few days so that the swelling may be reduced by elevating the foot. Antibiotic medication should be started immediately. Once the acute stage is over, a total contact molded plaster cast is applied and the patient allowed to walk. Used a generously padded cast. The dorsum of the cast should be cut approximately to the level of the metatarsal phalangeal joints to prevent excess pressure across the dorsum of the foot. The cast is changed at weekly intervals and then the foot is placed in extra-depth shoes as described. A close watch should be kept on the ability of the ulcer to reduce and heal over as this is otherwise as indication for surgical resection or bone resection. Periodic radiologic examination of Charcot joint changes and periodic vascular physiological examinations are performed using including continuous wave Doppler ultrasound.(46) Chronic ulcerations and infections are discouraging to the patient, but these patients often have ample capacity to regenerate tissues and are good healers. They should be encouraged that they will heal well if proper recommendations are followed.(47) Four categories of foot conditions are seen in Hansens's disease as described Enna and co-workers. However this system is highly appropriate to the staging and treatment of the diabetic foot.(48) Category 1: The foot has decreased or absent sensations but there are no soft tissue or skeletal deformities. Specific stress-concentrated areas can be treated by a microcellular rubber insole with extra-depth shoes to eliminate pressure over the dorsum of the foot and accommodate the larger toe box and the microcellular rubber insole. Patient education, periodic foot and shoe assessment are necessary. Category 2: The foot is insensitive and scarred on the plantar surface. There is a thinning of soft tissue underneath the metatarsal heads and at the distal ends of the toes. This category is similar to that of category 1, but with a reduced shock absorbing capacity of the soft tissues. A combination insole of molded polyethylene foam and microcellular rubber is recommended if scarring under the metatarsal head is pronounced or if the patient is an active walker. Shoe requirements are generally the same as in category 1, with the addition of a metatarsal bar, to protect the metatarsal heads adequately and redistribute weight from off of the metatarsal heads to the diaphyseal region. Caution is given to these patients against walking barefoot over hard surfaces, even for short distances as from the bedroom to the bathroom. These patients are highly subject to trauma in areas of bony projection. Category 3: Soft tissue thinning and scarring with bony deformities of the metatarsal heads or toes. This may be a result of bone reabsorption due to distal neuropathic arthropathy or secondary to infection. With this foot it is essential to have molded soft insoles with relief areas accommodating pressure on bone prominances. Essential to prevention of plantar ulcers is a semi-rigid inner sole to support and maintain the shape of the molded insole. Category 4: Extensive bone reabsorption has occurred with a shortening of the foot. Osteophytic proliferation in the tarsal and metatarsal shafts produces multiple soft tissue pressure points from inside the foot. Complete digital reabsorption may occur, and a close relationship between conservative and surgical management is necessary for patients in this category. Ankle range of motion is important in prevention of plantar ulceration. Patients with equinus will place more stress on the distal end of the shortened foot. The same therapeutic modalities as in category 3 are used with the addition of toe fillers to prevent excess motion of the foot in the shoe and metatasal bars or wedges to shift the stress more proximally. Custom-made shoes are the most effective footwear for these patients. A rigid rocker bottom sole will also distribute weight- bearing stresses proximally off of the end of the foot. The problem with conventional shoes is that the toe break is located distal to the hallux in a shortened foot as opposed to beneath the metatarsal phalangeal joint in a normal foot.(49) SURGICAL MANAGEMENT Successful therapeutic attainment of foot function in the non&- ambulatory patient usually involves surgery. These procedures are done to make their feet shoeable and prevent further pressure breakdown areas and pain. The intent of the surgical procedures is to remove the unopposed deforming forces and improve foot position to a manageable conservative level at which time correction by orthoses may help maintain a certain minimum of functioning and ambulation. Otherwise the muscle deterioration is unrelentless and progressive despite a patient's being confined to a wheelchair. Surgical procedures to correct these fixed contractures should not be performed unless the following indications are present: severe pain, skin breakdown or ulcerations, and the inability for the foot and ankle to accept reasonably costing and available shoe wear, including ankle-foot orthosis which are essential after the surgical releases. A study by Hsu and Jackson suggests that the most common indication for surgical correction of the foot is a pressure over the dorsal lateral aspect of the foot. For patients having Duchenne pseudohypotropic muscular dystrophy (DMD), spinal muscular atrophy (SMA), polio, etc., correctional procedures consist of the following: tendoachilles lengthening done as a first step, release of the posterior tibial tendon just proximal to the medial malleolus with occasional indication for transfer through the interosseous membrane and a variety of other releases including anterior tibial tendon release depending on the results of manual muscle testing. A long leg cast, then subsequently short leg cast holds the feet in corrected position which is then followed by an ankle foot orthosis (AFOs).(50) It is unfortunate that there are high risks in this surgery, but this is because of the nature of the disability having generalized muscle weakness and advanced cardiomyopathy. This necessitates detailed multi-system medical clearances, with special considerations for anesthesia. For local treatment, excision, or realignment of the offending bone might be indicated. In addition to resecting old fibrotic tissue, ostectomy is indicated in patients having showing sharp osteophyte impingement on soft tissue due to hypertrophic tarsal anklylosis. Split thickness skin grafts or microsurgically anastomosed free flaps might be necessary regardless of surgical or conservative management. Molded shoes are indicated to try to avoid subsequent ulcerations or pathological fractures.(46,51-55) CONSERVATIVE MANAGEMENT The standard of palliative care for the diabetics is periodic debridement of calluses and nails as necessary. A multitude of specialized instruments is available and gentle, slow and gradual debridement of accumulated keratosis should be approached cautiously. Observations for subkeratotis ulcerations and preulcerative changes should be measured and documented. Toenails should be debrided to a reasonable thickness as they are often grossly thickened as a result of either poor circulation or fungal infection. A small bone cutter might be handy to keep on the tray for the palliative orthopedic work. Treatment of fungal nails if indicated by positive cultures or KOH preparation can be by administration of griseofulvin. Rarely should nail avulsion be resorted to in a diabetic. Most of the lesions lie under the metatarsal heads where weight bearing is highest at heel-off. Ulcers are found most frequently beneath the first metatarsal head, then the hallux, followed by the second and the third, then fifth metatarsal, and the heel. The lateral plantar boarder and the distal aspects of the toes are other expected areas for ulcerations.(47,44,56) Initial treatment for ulcers is soaking in either saline or betadyne-and- water and sterile dressing changes, with a surgical scrub brush scrubbing for five to ten minutes two or three times a day for debridement. The base of the ulcer should be scrubbed vigorously to stimulate hyperemia and free bleeding. Should a hyperkaratosis be present at the ulcer edge, sharp dissection is necessary to the point of free bleeding which will enable granulation tissue to grow. Antibiotics should be continued several days after negative cultures. Septic ulcers usually respond within seven to fourteen days. Patients with osteomyelitis take anywhere from three to six weeks. For topical ulcer therapy, polyurethane dressings are important in essentially maintaining a moist environment for healing. Polyurethane is a thin polymer membrane which is semi-permeable to oxygen, CO2 and water vapor. Its intent is to promote optimal conditions for wound healing that would otherwise exist beneath a naturally formed scab, but without the disadvantage of dehydration. The dressing is applied and left undisturbed for seven to ten days at which point exudate rich in proteins, hormones and enzymes, and most importantly, epithelialization and fibroblasts migration is promoted more efficiently while oxygenation is optimized. It has been the experience of some that there is striking reduction in treatment time and a reduction in costs expenditures during the ulcer therapy.(57) The loss of sudomotor function causes the skin to become dry and scaly. Cracks develop in the flexion creases and subsequent infections are quite likely. The patient should rehydrate the skin by daily water soaks for twenty minutes in mildly warm water twice a day, then treat the area to maintain hydration by covering with petroleum jelly. At night a method of decreasing the rate of evaporation for extremely anhydrotic feet is to place plastic bags over the feet after soaking and applying the petroleum jelly. This can be done conveniently during sleeping hours and will keep the skin supple and less susceptible to injury and infection.(58) Principles of shoe alterations and changing the location of stress is very important to apply to the neurotropic. Reduction or elimination of the stress of the plantar surface of the forefoot is most effectively accomplished by a custom-made shoe which has a rigid rocker bottom sole. There are certain features of this type of shoe. The insole and the upper part must be molded to a model of the particular foot. The shoe should be rigid and the distal end of the shoe turned upward. Lastly, the rocker axis must be near the center of the foot and the rocker bottom must be thick enough to minimize contact between the end of the shoe and the floor during the toe-off phase of gait. An additional advantage is the reduction of stride length thus earlier knee flexion as the weight line of the body progresses anterior to the rocker axis, reducing the impact of heel strike.(49) The design of these devices is also supplemented by a Harris foot print recording. These foot print impressions are made by means of a painted paper imprint laid upon a white sheet of paper which is placed on a rubber mat. The transferred image of foot contact assumes a proportional size of the applied pressure. This tool can increase the physician's ability to evaluate and prevent many of the areas of increased pressure or maldistributions that occur on the plantar surface. A polyethylene-foam material, Plastazote, has been utilized in making insoles in normal custom-made footwear for patients with anesthetic feet. It is economical and cheaper than ordinary microcellular rubber shoes and represents a significant advance in the care of the insensitive foot. However before using Plastazote buildup, obtain footprints using the Harris footprint mat. Plastazote is warmed to temperature of 130 to 140 degrees farenheit for three to five minutes, after which point it becomes very flexible. A thin plastic sheating is placed between the Plastazote and the patient places their foot on the Plastazote while the technician exerts an even pressure on the patient's knee and over the toes for about one minute. The plantar impression is obtained and margins of the foot cut out. A combination of rubber dust, saw dust and rubber cement can be used on the bottom of the Plastazote to build the concave points to ground level. This is then used in conjunction with a custom-made shoe or boot.(59,60,61) Plastazote would seem to be the ideal answer from the standpoint of economy, rapidity of preparation and application to pressure distribution. However, it has been found with certain practitioners that Plastazote quickly loses its resilliance so that the area beneath the insole or under a pressure point can quickly become compressed to be as solid as the sole of the shoe itself. To remedy this, a higher density Plastazote cradle was then developed in England which was also molded to the shape of the foot, windows were cut out underneath the pressure points and filled in with 6mm of Neoprene. Neoprene has proven to be the most resilliant of the cushioning materials maintaining its recovery and provided that it's thick enough it will not "bottom out" under pressure. With these insoles the recurrence rate of ulcers has been negligible.(62) MEDICAL MANAGEMENT If abnormalities of flow are response for lesions of the neuropathic foot, what can be done to influence them? Lefaucher reported ligating the dorsalis pedis artery with resolution of ulcers in the neuropathic foot. Egotamine, an alpha-adrenergic agonist theoretically proves to be useful and has been shown to divert this blood flow from the arteriovenous anastomosies, however more research needs to be done in this area before such therapy is initiated in the treatment of the diabetic foot and microangiopathic peripheral vascular diseases.(63,64) For relief of the burning foot syndrome, pantothenic acid has been administered. To relieve the causalgic pain, cooling lotions, aspirin, propoxyphene and codeine are needed. However, increased vitamin dosages have not proven to be of a therapeutic benefit if the neuropathy is not secondary to avitaminosis. Trials of pyridoxine (vitamin B6) on neuropathic diabetic patients produce no objective improvement in sensory nerve conduction. Results of any improvement in these patients not suffering from avitaminosis represent a placebo phenomena. The only measurable improvement that may be obtained is in subjects suffering from true vitamin depletion.(62,65,66) SUMMARY The triphasic nature of the peripheral neuropathy, with regards to motor, sensory and autonomic function, produces multiple manifestations that may elude the clinician to easily diagnose other significant disease pathologies of the neurotropic patient, particularly the diabetic, while avoiding recognition of the Charcot joint entirely. These presentations may lead to a diagnosis of phlebitis, cellulitis, lymphedema, anhydrosis, in addition to the painful dysesthesias, motor loss, and painless ulcers. Oftentimes the correct diagnosis is not made at the initial visit and several doctors may be consulted before the diagnosis of Charcot joint is even considered in the differential. The surgical management of neuropathic arthropathy should attempt to focus, on a osseous reconstruction or realignment plan together with muscle balancing tendon transfer procedures. Local treatment may indicate soft tissue resection with ostectomy for non-closing ulcers or deformities. A surgical management of arthrodesis, arthroplasties, and osteosynthesis procedures, combined with dedicated physical therapy should have the intent of stabilizing and balancing the soft tissue structures. This will help in the absorbtion and distribution of shock so that it might be more uniformly directed through more appropriate, stronger portions of the foot and leg. Success of surgical procedures would be correspondingly increased. END PART THREE SEE REFERENCES PART FOUR