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Repeated fractures are not unusual in the course of forearm fractures in children. 49 cases were reviewed in this work. Some of them result from insufficient duration of the plaster. Other fractures are due to the weakness of the forearm bones after the fractures especially when there is a residual malalignment. The findings of this work emphasize the importance of the reduction even when the displacement is moderate. Open reduction and internal fixation is reserved in only rare cases.
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PMID:[Recurrent fractures of both bones of the forearm in children]. 54 83

A retrospective study of 23 acute and 6 chronic acromioclavicular dislocations treated by surgical transfer of the distal 1/2 inch of the coracoid process with the attached conjoined tendon of the coracobrachialis and short head of the biceps to the clavicle revealed 14 excellent, 14 good and one fair result. Results were determined according to symptoms, range of motion at the shoulder and elbow, strength, anatomic reduction, and return to previous activities. Although most patients with this injury are treated conservatively, this procedure is reserved for the athlete or manual laborer below age 45 years, especially with involvement of the dominant-extremity. The 29 cases were evaluated 20--108 months following surgery. Thirteen additional cases with less than 18 month follow-up have also been good or excellent. Weakness and pain have not been as pronounced following this procedure in vigorous individuals as have been noted after conservative treatment. Few postoperative complications developed, and early return to competitive athletics was possible.
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PMID:Acromioclavicular dislocations: treatment by transfer of the conjoined tendon and distal end of the coracoid process to the clavicle. 70 27

The introduction of the ventricular inhibited pulse generator with the feature of rate hysteresis has been associated with a variety of rhythm disturbances, some causing serious concern. This pulse generator has two different pacing rates: (1) the automatic rate, which is the interval between two successive paced beats (usually 860 msec or 70/min), and (2) the hysteresis interval, which results in a 1,000 msec delay between a sensed cardiac contraction and the next pacemaker discharge. The hysteresis interval after a sensed signal may result in long pauses that may predispose to the development of serious cardiac arrhythmias. Two examples of this type of complication were recently observed. One patient had bigeminal rhythm with mechanically ineffective cardiac contractions and an effective cardiac rate of 35/min; he experienced dyspnea and weakness during these prolonged episodes. Another patient had repeated episodes of ventricular fibrillation. The cardiac arrhythmias were not controlled by antiarrhythmic agents, and both patients required replacement of the pulse generator. The proposed advantages of pulse generator hysteresis are (1) prolongation of battery life, and (2) maintenance of effective atrial transport; these advantages may be outweighed by undesirable cardiac arrhythmias that may be associated with this mode of pacemaker function. Rate hysteresis cardiac pacemakers should be reserved for patients having predominantly sinus rhythm without ventricular irritability. In patients with frequent ectopic ventricular activity, atrial fibrillation or high degree atrioventricular block, the rate hysteresis pacemaker offers no advantage over the conventional demand pacemaker. For patients with frequent ectopic ventricular activity not easily controlled by antiarrhythmic agents, consideration should be given to the use of a permanent demand pacemaker with external rate control, which may provide greater flexibility in arrhythmia management.
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PMID:Undesirable cardiac arrhythmias associated with rate hysteresis pacemakers. 99 5

The present series of thirty patients has led us to certain conclusions concerning the management and treatment of patients with myasthenia gravis. The use of cholinesterase inhibitors alone is reserved for those patients with purely ocular myasthenia whose deficits can be satisfactorily corrected with those agents. Some of those with ocular involvement may be disabled; and in light of our excellent results with that small group, as well as similar findings presented by Fischer et al., patients with disabling or refractory ocular myasthenia should be considered for treatment with prednisone. All other patients with myasthenia are given a course of oral corticosteroids (prednisone) initially at high doses, with subsequent tapering to maintenance, alternate-day low-dose therapy. Cholinesterase inhibitors are used as needed while the patient is receiving corticosteroids. We now anticipate that patients will exhibit sustained improvement within the first two weeks, reaching maximal improvement at about three months. Exacerbations of myasthenic weakness may occur in the early phases of treatment. Such exacerbations have been commonly mild and occur with a mean onset at 5 days, and have a mean duration of 6 days. Most patients have been able to tolerate an alternate-day schedule of prednisone therapy when maintenance levels were achieved. The effective maintenance dose has been determined as the smallest dose of prednisone which allows the patient to maintain maximal improvement. Following the establishment of maximal improvement, patients have been considered for thymectomy. In our experience, the sternum-splitting procedure has been tolerated extremely well by patients exhibiting marked imporvement or remission while on corticosteroids. In those patients where thymectomy is contraindicated, irradiation of the thymus might be considered. Patients are continued on maintenance steroid therapy following surgery for a period of time that has been arbitrary. Currently, we consider an attempt to discontinue steroids at approximately one year reasonable. Should the patient relapse after discontinuation of the medication, oral corticosteroid treatment is reinitiated. Consideration is given to the possibility of recurrent thymus in patients who repeatedly fail to maintain a remission when steroids have been stopped. Our experience has not permitted us to draw firm conclusions concerning how long a time high-dose daily steroid treatment should be continued in patients who show no favorable response to that therapy. Other modes of treatment, such as courses of parenteral ACTH, methyl prednisolone, dexamethazone, or antimetabolites might be considered if there is no response after 12 weeks of high-dose, daily corticosteroid therapy.
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PMID:Long-term prednisone followed by thymectomy in myasthenia gravis. 106 98

Spinal cord or cauda equina compression from prostatic cancer is an oncologic emergency necessitating prompt evaluation and treatment. The strong correlation between pretreatment motor status and treatment outcome underscores the importance of immediate treatment before further neurologic deterioration and before the damage to the spinal cord becomes permanent. Patients with known osseous metastases should be alerted by their clinicians to seek medical help within hours should they develop weakness in an extremity. Prompt MRI of the entire spine should be done prior to treatment. Myelography should be reserved for those patients who cannot undergo a technically adequate or expeditious MRI study. The convenience of MRI relative to myelography allows clinicians to diagnose actual or impending spinal cord compression earlier. High-dose steroids (dexamethasone) should be instituted immediately, and endocrine therapy should be started if not already in use. Ambulatory and moderately paraparetic patients seem best treated initially with radiation alone. Immediate surgical decompression should be used in patients with an expected lifespan of at least 6 months who deteriorate during radiation, who have had previous radiation to the involved site, or who have a potentially correctable unstable spine. In addition, paraplegic patients or severely paraparetic patients with recent neurologic deterioration should be treated with immediate surgical decompression if they are judged reasonably able to tolerate the surgery. These patients should then receive postoperative radiation treatment.
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PMID:Management of spinal cord compression secondary to metastatic prostatic carcinoma. 199 68

Chyloperitoneum is an extremely rare complication of abdominal surgery in children and a combined occurrence of chylothorax and chyloperitoneum after abdominal surgery has never been reported in children. Chylous ascites usually occurs as a result of operative trauma to the thoracic duct, cisterna chyli, or its tributaries. About one third of all patients with chylous ascites after retroperitoneal lymph node dissection also develop secondary chylothorax. Diaphragmatic defects have been shown to be responsible for the occurrence of chylothorax secondary to chyloperitoneum. Congenital diaphragmatic weakness may result in evagination of the peritoneum causing diaphragmatic blebs, the rupture of which results in the movement of the peritoneal fluid into the pleural cavity. In the authors' patient, the rent in the diaphragm that occurred during surgery was probably responsible for the chylothorax. The role of chemotherapy, if any, in the pathophysiology of this complication is unknown. Total parenteral nutrition (TPN) is a simple and effective treatment for postoperative chylous effusions. Surgical treatments such as abdominal exploration for the repair of leaking lymphatics and peritoneovenous shunt should be reserved for patients who fail TPN.
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PMID:Combined occurrence of chyloperitoneum and chylothorax after surgery and chemotherapy for Wilms' tumor. 255 73

Dermatomyositis in childhood is an uncommon disease, affecting muscle and skin. The disease usually has an insidious onset; the proximal muscle groups are classically more affected than the distal group. If left untreated, the disease will either spontaneously arrest or will progress until the child is completely bedridden, with death secondary to hypoventilation and aspiration. For a definitive diagnosis 3 or 4 of the following criteria (plus rash) are required: 1) symmetrical limb girdle weakness; 2) muscle biopsy evidence of myositis and muscle necrosis; 3) elevation of muscle enzymes; 4) electromyographic changes of myositis. The main pathologic feature of juvenile dermatomyositis is vasculitis affecting small arteries and veins of muscle, skin and gastrointestinal tract. Whether muscle from patients with polymyositis contains a specific auto antigen or is contaminated with an immunogenic infectious agent such as a virus (coxsackie virus, for instance) remains unclear. Childhood dermatomyositis is almost uniformly responsive to steroid treatment; there is a good chance of remission with minimal risk of secondary complications with an initial low dosage of prednisone (1 mg/kg/day). The use of additional drugs such as azathioprine, methotrexate or cyclophosphamide is reserved for patients who are either not completely responsive to steroids or difficult to wean off steroids. Cyclosporine A has been proposed to achieve a reduction in steroid dosage.
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PMID:[Dermatomyositis in children]. 271 39

An 11-year-old girl with congenital myotonic dystrophy and infantile autism was reported. Her mother also suffered from typical myotonic dystrophy. Since her birth, the patient had been floppy, and showed bilateral talipes equinus at 1 year of age. Her subsequent psychomotor and speech development has been retarded. She showed autistic behavior and persistence to the sameness before 2 years old. She was admitted to Sawarabien at the age of 10 years. She could not talk anything but could understand simple, oral messages. Although she had severe degree of mental retardation, her ability for matching figures was relatively well reserved. Her autism was so manifest that it could not be explained by the degree of mental retardation. Neurological examinations revealed that she had facial diplegia, inverted V-shaped mouth, high-arched palate, talipes equinus, percussion myotonia of the tongue, generalized muscular atrophy and weakness, lordosis, areflexia, and congenital cataracta. The serum CPK was slightly elevated. EMG showed a myopathic pattern but did not show any myotonic discharge yet. The brachioradial muscle was biopsied and examined by light- and electron-microscopy. It mainly showed mild varieties of muscle fiber diameter and internal nuclei. Ultrastructurally, irregularly indented central nuclei and perinuclear degeneration of myofibrils associated with secondary lysosomes, lipid droplets and glycogen granules were revealed. Ventricular dilatation and some dysfunction of the brain were also revealed by CT scan and EEG respectively. The present case suggests that congenital myotonic dystrophy can be added into the disease group associated with infantile autism.
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PMID:[A case of congenital myotonic dystrophy with infantile autism]. 278 60

Postural brachial plexus compression neuropathy occurs more frequently than usually realized. The cause is multifactorial with certain predisposing anatomic and congenital factors that are not uncommon. An inciting event is required to cause a clinically significant syndrome. The event can be a specific traumatic episode or cumulative trauma leading to an adoption of a guarding posture, which results in a self-perpetuating cycle and brachial plexus nerve compression. The diagnosis and management may be complicated by concurrent vascular compression, concurrent reflex sympathetic dystrophy, and associated inflammatory musculotendinous conditions. Diagnosis relies on the appreciation of a peculiar symptom complex of pain and paresthesias. The important clinical signs are a supraclavicular Tinel's sign and a positive stress abduction test. Treatment includes exercises, patient education, and behavior modification. However, misdiagnosis can lead to inappropriate treatment, such as unnecessary carpal and cubital tunnel releases. Operative treatment is reserved for those severe cases that are resistant to extended and intense physical therapy. The preferred surgical technique involves an anterior, supraclavicular approach allowing for complete visualization and release of intrinsic and extrinsic nerve compression. Awareness is the key to making the diagnosis, and successful treatment requires a multidisciplinary approach. It is generally accepted that injuries to peripheral nerves result in serious losses of function. Paresthesias and motor weakness cause immediate functional limitation, and place the hand at risk for further injury. The system has little regenerative capacity, and the chance for recovery is poor even under the best circumstances. Therefore the treatment of acute nerve injuries can be difficult and frustrating.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Nerve injury complications. Management of neurogenic pain syndromes. 301 9

Scleromyxedema is a rare fibromucinous connective tissue disorder characterized by papular skin lesions associated with sclerosis and a serum monoclonal gammopathy. Little is known about either the natural history or the systemic manifestations of this disease. We reviewed the medical records of 19 patients with biopsy-proven scleromyxedema seen from 1950 to 1985 for evidence of systemic disease. There were 10 males and 9 females with a median age at diagnosis of 53 years. Monoclonal gammopathy was present in 13 patients. Eight patients complained of dysphagia; 3 had proximal esophageal dysfunction and 1 had total esophageal aperistalsis on barium swallow. Proximal muscle weakness was noted in 5, with an inflammatory myopathy in 3. Six patients complained of dyspnea on exertion. Of these, 5 had reduced diffusing capacity, 3 had reduced volumes, and 2 developed cor pulmonale. Pathologic changes characteristic of "scleroderma kidney" were demonstrated in 1 patient at postmortem. One patient had Raynaud's phenomenon and 2 had arthralgias/arthritis with noninflammatory synovial fluids. Although 8 of 12 patients treated with melphalan noted regression of their skin changes, no consistent improvement in the extracutaneous manifestations was demonstrated. Furthermore, 2 patients died of sepsis related to melphalan-induced myelosuppression, and 4 developed hematological malignancies following melphalan therapy. In conclusion, systemic manifestations in scleromyxedema are more prevalent than previously recognized, and can resemble those of scleroderma. Significant toxicity occurred with the use of alkylating agents in these patients, with treatment-related complications developing in 45% of patients treated with melphalan. The lack of definitive data regarding the natural history of this disease complicates the question of optimal therapy, but the use of alkylating agents should be reserved for those patients with severe debilitating skin disease.
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PMID:Scleromyxedema: a scleroderma-like disorder with systemic manifestations. 333 81


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