Reflex sympathetic dystrophy syndrome: Case report with a review of the literature

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<ul><li><p>Reflex sympathetic dystrophy syndrome Case report with a review of the literature </p><p>Michael Markof, D.M.D.,* and Anthony Farole, D.M.D.,** Philadelphia, Pa. </p><p>THOMAS JEFFERSON UNIVERSITY HOSPITAL </p><p>A case of reflex sympathetic dystrophy syndrome (RSDS) is presented in which the condition was exacerbated after the excision of impacted teeth. A review of the English language literature describes manifestations, distribution, etiology, pathogenesis, histology, and diagnosis of RSDS. Treatment modalities are reviewed, and recommendations for the management of the patient with a history of RSDS are detailed. (ORAL SURG. ORAL MED. ORAL PATHOL. 61:23-28, 1986) </p><p>R eflex sympathetic dystrophy syndrome (RSDS) is primarily a neurovascular pain dysfunction com- plex affecting the limbs. It has been known by many names and in various forms for at least 120 years. In well over half the patients reported, an antecedent event such as trauma, systemic disease, or unrelated surgery was implicated in the initiation or exacerba- tion of signs and symptoms. Psychogenic input by way of the pain gate of Melzack and Wall may greatly influence onset, progression, and resolution of this condition. </p><p>Pain and edema, disproportionately severe for the stimulus, lead to protective immobility, which, if allowed to continue, results in trophic skin changes, patchy osteoporosis, and permanent limb contrac- tures. There are no consistent laboratory findings that confirm the diagnosis; rather, such values serve to eliminate other diseases within the differential diagnosis. Steroids, sympathetic blockade, and phys- ical therapy are among the more popular modes of treatment at this time; all methods are claimed by their advocates to have superior rates of successful clinical resolution. However, there are no dependably curative treatments, and more than half the patients in a large study had residual symptoms after treat- ment.2 The childhood-adolescent form of RSDS, though comparatively rare, is much less severe and </p><p>*Chief Resident, Division of Oral and Maxillofacial Surgery. **Assistant Professor and Assistant Director, Oral and Maxillo- facial Surgery Residency Program. </p><p>has a much better prognosis, with few if any residual symptoms. </p><p>A case of RSDS is reported in which an acute exacerbation followed simple excision of impacted teeth under general anesthesia. </p><p>CASE REPORT </p><p>On Oct. 11, 1983, a 20-year-old white woman came to our institutions Division of Oral and Maxillofacial Sur- gery complaining of soreness of maxillary and mandibular third molar areas during the past week. Her medical history was remarkable for a 3-year history of RSDS (followed up by our hospitals Neurology Department), thoracic outlet syndrome (under care of the Orthopedic Department), anemia (followed by our hematologist), and hemoptysis of unknown cause (evaluated by our otolaryn- gology service). In addition, the patient had childhood asthma, which had been inactive without medication for the last 7 years, and mitral valve prolapse. She was taking diazepam, 4 to 10 mg, four times a day for spasms of the left neck and shoulder, and an oral iron supplement. She reported being allergic to cefazolin sodium (Ancef) and myelogram dye. </p><p>Physical examination revealed Horners syndrome on the left side (secondary to earlier cervical sympathectomy) and healthy oral tissues, except for pharyngeal edema, minor pericoronitis around the mandibular left impacted third molar, bilateral malpositioned maxillary third molars, and an impacted mandibular right third molar. Panoramic x-ray film demonstrated normal bony architec- ture with bilateral maxillary, completely impacted fourth molars. </p><p>Because of the patients mitral valve prolapse, elimina- </p><p>23 </p></li><li><p>24 Markoff and Farole Oral Surg. January, 1986 </p><p>tion of any source of infection and potential bacteremia was deemed necessary, and in light of the extensive medical history the patient was scheduled for admission for the excision of ail third and fourth molars. Penicillin was prescribed and the patient was instructed to use saline oral rinses in the time interval before admission. </p><p>On admission Oct. 18, 1983, a standard history was taken and a physical examination was given. Additional history was significant for several episodes of fever of unknown origin and exacerbation of RSDS in the left arm and leg. Surgical history revealed a scalenectomy, appen- dectomy, removal of the left first rib (to treat thoracic outlet syndrome), cervical sympathectomy, laparoscopy, arthroscopy of the left knee, fasciotomy, and lumbar sympathectomy between March 1981 and July 1983. </p><p>Her past medical history revealed that the patient suffered frequent, severe headaches with accompanying nausea and vomiting, which she claimed her neurologist related to her RSDS. The patient said she had chronic anemia during the previous 6 months, a left scapular bursa injury secondary to sympathectomy surgery, and numb- ness of the left shoulder, with swelling of the left arm, pain of the occiput and neck, and associated muscle spasms. A family history was noncontributory, and she neither drank alcohol nor smoked. </p><p>Physical examination was remarkable for the Horners syndrome of the left side and a II/VI systolic murmur. The following laboratory tests were performed: complete blood count with differential, prothrombin time, partial throm- boplastin time, sequential multiple analyzer,, sequential multiple analyzer,,, and urinalysis, all of which yielded results within the normal range, except for a white blood cell count of 3.8 X 10/mm3 and microcytic hypochromic anemia. </p><p>The patients neurologist was consulted to evaluate the patient before surgery. He recommended that her orthope- dist be consulted for an injection of anesthetic into the subscapular bursa to relieve the neck and occipital pains and that a medical service grant presurgical medical clearance. Such clearance was obtained. </p><p>Because of the patients low Hb/Hct, morning surgery was deferred until the hematology service assured us that this anemia was usual for our patient and that it posed no obstacle to the intended surgery; parenteral iron supple- mentation after surgery was recommended because the patients response to enteral iron was suboptimal. </p><p>On Oct. 20, 1983, the patient was given a general anesthetic via nasotracheal intubation for excision of four third and two fourth molars. The surgery and anesthesia were atraumatic and completed without difficulty. The patients head and neck were maintained in a neutral position and the arms were tucked against the sides throughout the intubation and surgery. She received 80 mg of methylprednisolone sodium succinate and 2 million units of aqueous penicillin intravenously before incision. On extubation in the operating room the patient complain- ed of spasm and severe pain in the neck, radiating into the fingers of the left hand, but sparing the thumb, and pins-and-needles paresthesia of the left arm with profound weakness. The Neurology Department was consulted </p><p>immediately from the recovery room; the impression was that of severe RSDS, rather than cervical myelopathy secondary to disc herniation. A cervical collar was placed and x-ray films were immediately taken of the cervical spine; they demonstrated no acute changes, Postoperative orders included continuation of the steroid, 80 mg every 4 hours and penicillin, 2 million units every 4 hours. For the remainder of the day the patients intraoral status was excellent but the pain of the left upper extremity necessi- tated increasing doses of narcotic for adequate analgesia. </p><p>On the first postoperative day the neurologist suspected that the symptoms represented exacerbation of RSDS and he recommended analgesics and EMG testing of the left arm. The orthopedist suspected cervical strain as the source of the pain and dysfunction and he recommended anti-inflammatory medication. The patient received a final dose of 80 mg of methylprednisolone sodium succinate, followed later by 80 mg of methylprednisolone acetate as a single intramuscular injection. </p><p>By the fourth postoperative day the patients mouth was healing well, but the pain and dysfunction of the left arm and neck remained unabated and the neurologist accepted her for treatment. EMG results indicated reduced volun- tary effort, as is seen in cases of extreme discomfort, without evidence of neuropathy or radiculopathy. The physical therapy service was consulted; it recommended range-of-motion therapy and moist heat to the left shoul- der and neck, followed by ice massage if moist heat was ineffective. The regimen was initiated the same day. The patient was given 300 mg of lidocaine via slow intravenous infusion by an anesthesiologist to reduce the pain and spasm; it gave only minor improvement. She was started on intravenous iron supplement for treatment of the iron- deficiency anemia. The patient received physical and occupational therapy sessions, and frequent analgesics with slightly improved function and pain control. </p><p>By the tenth day after surgery the patient started complaining of pain in the right shoulder, aggravated by function. Ice packs were applied to treat this problem. That night the patients temperature rose to 104 F, and this nocturnal fever was decrementally repeated, with her temperature returning to a normal level 3 days later; negative blood and urine cultures, chest x-ray films, and a negative antinuclear antibody titer suggested a viral cause. The patient also started complaining of bilateral medial knee pain. </p><p>Fourteen days after oral surgery the pains and dysfunc- tion were without change. The patient was interviewed by a psychiatrist from the hospitals chronic pain clinic. He suggested that the patient might have a hysterical type of personality and recommended hypnosis and consideration of propranolol for possible vasospasm. That day the patient was started on a tricyclic antidepressant for her increasing depression. By this time the patients neurologist noted, . The spinal x-rays, tomograms, EMG, are negative and . (he sees) no physiological reason for pain. He was concerned with the narcotic dosage and ordered a decrease of dosage and frequency. The patient complained often about this decrease in medication. </p><p>By Nov. 7, 1984, the patient reported a sudden absence </p></li><li><p>Volume 6 I Number 1 </p><p>Reflex sympathetic dystrophy syndrome 25 </p><p>of pain in the right shoulder. It was believed that at this time she would benefit from her home environment, with outpatient follow-up by a psychiatrist. Discharge was arranged with the patient on oral analgesics and with psychiatric follow-up. She was discharged the next day, 19 days after oral surgery, without improvement of the pain, dysfunction and paresthesia of the left arm. On follow-up more than a year after discharge the patient reported persistence of the pain and dysfunction. </p><p>REVIEW OF LITERATURE </p><p>RSDS represents a symptom complex that in one form or another and by a multitude of names was known since Mitchell et al3 reported it is wounded soldiers of the American Civil War in 1864. RSDS has numerous signs and symptoms, and may be considered a collective term under which various diseases have been included, for example, Steinbrok- ers shoulder-hand syndrome, Sudecks atrophy, posttraumatic osteoporosis, traumatic angiospasm, chronic traumatic edema, peripheral trophoneurosis, algodystrophy, and minor causalgia.4-8 </p><p>The classic signs and symptoms are pain and swelling in an extremity, trophic skin changes in the same limb, and typically a precipitating event that usually involves injury to a peripheral nerve.4, 5*9. O In addition, hypertrichosis and osteoporosis of the limb may be noted.5 The pain and dysesthesias are not restricted to the distribution of the injured nerve and often temporally endure beyond the nerve deficit. </p><p>The age and sex distribution reported in the 125 cases of Subbarao and Stillwell* agree with other studies; the incidence increased with age, peaking in the fifth decade. The male-female ratio is 1: 2.9.* The actual prevalence is not known, but Plewes* reported one case in every 2000 accidents (approxi- mately 12 a year) treated in a busy trauma center. De Takats4 estimated that 5% to 15% of all trauma cases will result in a form of minor to severe RSDS. </p><p>Initiation of RSDS has been attributed to trauma, often of a minor nature, drugs, nerve injury, surgery, carcinoma, cervical osteoarthritis, myocardial ische- mia and infarct, and cerebrovascular and other neurologic lesions. I, I33 I4 In the study by Subbarao et al.,* about 9.5% of the cases had no identifiable cause. </p><p>Several theories have arisen in an attempt to explain the various clinical stigmata and test results found in RSDS. Bonicals and others thought that damage to peripheral nerves led to a reflex aberra- tion of vasomotor function in autonomic efferent nerves. This may occur by cross-stimulation between sympathetic efferents and damaged, demyelinated, or unmyelinated sensory fibers by way of a choliner- </p><p>gic vasodilator chemical mediator.4 Livingstoni pos- tulated that this short-circuiting of neural transmis- sion occurred in self-sustaining neuronal loops within the spinal dorsal horn. More recently, the gate control mechanism of Melzack and Wall has been cited to explain the beginning of RSDS; specialized cells within the substantia gelatinosa of the spines dorsal column modulate sensory transmission to higher centers by closing the gate in response to large, myelinated afferent fiber input, and by open- ing this gate with input from small C fibers. In addition, fibers descending from higher neural cen- ters modulate this gate. Thus mild stimuli transmit- ted along C fibers may be felt as intense pain, especially when input from large fibers is faulty or when descending impulses fail to close the gate.l. IO. 13. I7 C-S involvement in RSDS is supported by a case of diffuse RSDS reported by Bentley and Hameroff and by consistent findings of bilateral limb involvement in RSDS.5 </p><p>De Takats4 pointed out that in the primary phase of RSDS a sympathetic block will relieve the pain because of its restriction to the first-order neurons. This is the vasodilation phase, with hot throbbing and burning pain, and is relieved by elevation and cold, wet packs. However, as with other long- standing painful states, the second-order sensory neuron becomes involved, making peripheral auto- nomic or somatic blocks ineffectual, and spinal anesthesia is the least anesthesia required for pain relief. This is the secondary or vasoconstrictive phase, with cold, tense, sweaty, glossy skin, limited range of motion secondary to pain and contracture, and patchy osteoporosis. RSDS may continue to the tertiary phase, with higher centers involved, namely, the reflex tuning of the hypothalamus to minor or subliminal stimuli, which results in exaggerated autonomic outbursts.18 When RSDS reaches the highest sensory level, drug addiction, insanity, and even suici...</p></li></ul>


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