Hereditary neuropathy with liability to pressure palsies: a case report.

Hereditary neuropathy with liability to pressure palsy is a rare autosomal dominant disorder characterized by multiple episodes of focal demyelinating neuropathies after minor trauma to peripheral nerves. It usually appears in early adulthood with recurrent attacks of pain, numbness, and muscular weakness along the distribution of the clinically affected nerve. Segmental demyelination and thickenings of the myelin sheath are the pathologic findings. Electrophysiologic studies show a nonuniform mild demyelinating neuropathy with prolonged distal latencies. Genetic tests are available to aid in diagnosis as molecular analysis has identified a deletion in the chromosome 17p11.2 in the majority of these patients. There is a paucity of information in the orthopaedic literature regarding hereditary neuropathy with liability to pressure palsy. A case report is presented of a patient with this disorder to promote awareness and recognition that this entity should be considered in patients with multiple nerve palsies.

Acute injuries of the distal radioulnar joint.

Distal radioulnar joint injuries can occur in isolation or in association with distal radius fractures, Galeazzi fractures, Essex-Lopresti injuries, and both-bone forearm fractures. The authors have classified DRUJ/TFCC injuries into stable, partially unstable (subluxation), and unstable (dislocation) patterns based on the injured structures and clinical findings. Clinical findings and plain radiographs are usually sufficient to diagnose the lesion, but axial CT scans are pathognomonic. Diagnostic arthroscopy is the next test of choice to visualize stable and partially unstable lesions. Stable injuries of the DRUJ/TFCC unresponsive to conservative measures require arthroscopic debridement of the TFCC tear, along with ulnar shortening if there is ulnar-positive variance. Partially unstable injuries, on the other hand, are treated with direct arthroscopic or open repair of the TFCC tear, once again, along with ulnar shortening if ulnar-positive variance is present. Unstable injuries include simple and complex DRUJ dislocations. A simple DRUJ dislocation is easily reducible but may be stable or unstable. In complex dislocation, reduction is not possible because there is soft tissue interposition or a significant tear. After the associated injury is dealt with, treatment for complex injuries requires exploration of the DRUJ, extraction of the interposed tissue, repair of the soft tissues, and open reduction and internal fixation of the ulnar styloid fracture (if present and displaced). The early recognition and appropriate treatment of an acute DRUJ injury are critical to avoid progression to a chronic DRUJ disorder, the treatment of which is much more difficult and much less satisfying.

A comparison of patients with different types of syndactyly.

We performed a retrospective review of finger syndactyly releases at Shriners Hospital for Children, Houston Unit, between January 1983 and January 1993. This study was performed in an attempt to compare the long-term postoperative function in patients after release of syndactyly resulting from Poland's syndrome with that in patients with idiopathic forms of syndactyly. Only patients with one involved hand were included in this study. The contralateral hand was used as a control. Twenty-seven patients with only one hand involved underwent syndactyly release during this period. Of these, 13 patients who underwent a total of 30 syndactyly releases were available for evaluation. For each patient, the type of syndactyly was determined. Each patient was subjected to a detailed physical examination and participated in occupational-therapy modalities. We noted statistically significant differences in function between operated-on and control hands in the Poland's group, whereas operated-on hands affected with idiopathic forms of syndactyly did not demonstrate significantly different function compared with contralateral controls. These data suggest that functional deficits in hands affected by Poland's syndrome are attributable to more than the syndactyly alone. Hands affected by idiopathic forms of syndactyly are likely to have little postoperative functional deficit.

Detection of submaximal effort by use of the rapid exchange grip.

To assist in distinguishing patients with truly decreased hand grip strength from those deliberately not gripping the dynamometer at maximal capacity, a rapid exchange grip strength test was devised and tested under four conditions. Part I, 100 normal subjects undergoing static grip testing and the rapid exchange grip test. Part II, 45 patients chosen at random from physical therapy with various hand injuries tested using only the static grip test. Part III, a blind control study on 15 normal subjects instructed to fake an injury to either the right or left hand. This group was given both the static and rapid exchange grip test. Part IV, a retrospective evaluation of 45 patients seen in a private hand practice who had both the static and rapid exchange grip test. After the dynamometer had been set to the position at which the patient had previously achieved maximal grip strength, the patient was instructed to rapidly alternate hands while gripping the dynamometer. Uninjured subjects had consistently lower rapid exchange grip test scores than previous scores at the same setting (negative rapid exchange grip). Average rapid exchange grip test scores were higher than previous scores (positive rapid exchange grip) when subjects were instructed to fake an injury with one hand. We conclude that if maximal performance has not been achieved on the static test, the rapid exchange grip shows a significant increase in grip strength on the affected side. More patients claiming worker's compensation had positive rapid exchange grips and the average score was higher than that of patients not claiming worker's compensation.(ABSTRACT TRUNCATED AT 250 WORDS)

Sportsmedicine forum.

A Forum For Our Readers Sportsmedicine Forum is intended to provide a sounding board for our readers. Perhaps you have a special way to treat a common medical problem, or you may want to air your views on a controversial topic. You may object to an article that we have published, or you may want to support one. You may have a new trend to report, identified through an interesting case or a series of patients. Whatever your ideas, we invite you to send them to us. Illustrative figures are welcomed. Address correspondence to Sportsmedicine Forum, The Physician and Sportsmedicine, 4530 W 77th St, Minneapolis, MN 55435.

A universal tunneling device.

A universal tunneling device is described herein using a trocar chest tube. For the surgeon, it is readily available, simple, precise and effective. For the patient, this procedure is quick and traumatic.

Postlaminectomy arteriovenous fistula.

Forty-nine cases of postlaminectomy arteriovenous fistula have been reported. Perforation of the anterior spinal ligament by the pituitary rongeur during discectomy with resultant simultaneous damage to artery and vein is causative. Whether aorta, cava, and/or iliac vessels are involved depends upon the level of laminectomy, the angle of the instrument, and anatomic variations in aortocaval bifurcation. Nine patients presented early after operation and were diagnosed promptly. The 40 patients who presented from months to years following laminectomy to physicians unfamiliar with this entity often had a distressing delay in diagnosis. High output congestive heart failure, particularly in a young person, and the characteristic abdominal and back bruit, should arouse suspicion. Arteriography confirms the diagnosis and allows planning for the operative repair. Though potentially disastrous, the operative correction of a major arteriovenous fistula may be done safely if standard principles of vascular surgery are followed. When combined with technical hints regarding clamp placement, transvascular repair, balloon catheter use, vein preservation, and the multiple inventive uses of the Dacron vascular prosthesis, a successful outcome should be expected.

Cystic adventitial disease of the common femoral artery.

Cystic adventitial disease consists of a collection of clear gelatinous material within an aberrant synovial-type cyst located in the subadventitial plane of the wall of a major artery. Recurrent minor trauma is presumed to potentiate mucin production and enlargement of the cyst with resultant arterial occlusion and symptoms of ischemia. It occurs most commonly in the popliteal artery in young men; forty-seven such patients have been reported on. Extrapopliteal disease is distinctly less frequent, occurring three times in the external iliac, three times in the radial, once in the ulnar, and once in the common femoral artery. Surgical therapy of choice is evacuation of the gelatinous material and abrasion of the lining of the cyst. Grafting is occasionally necessary. Long-term results are excellent and recurrence is uncommon.

Adenomatous polyps of the ileocecal valve: report of three cases.

Lesions of the ileocecal valve are uncommon and include various inflammatory diseases, submucosal fatty infiltration, edema, and neoplasia. Of the neoplastic lesions, adenomatous polyps are distinctly rare, despite their frequency in the remainder of the colon. Three patients with adenomatous polyps of the ileocecal valve are added to the seven previously reported cases. One had a large pedunculated polyp, another had polyps resulting from familial polyposis, and the third had circumferential adenomatous polypoid hyperplasia and the ileocecal-valve syndrome. Although many lesions of the valve produce the characteristic symptoms, the diagnosis of the ileocecal-valve syndrome is seldom made. Most ileocecal-valve lesions are incidental findings on barium-enema studies and may be carefully followed. The indications for operative intervention are suspicion of malignancy and the presence of troublesome symptoms. If malignancy is likely, a right colectomy should be done. Segmental resection is the procedure of choice for the ileocecal-valve syndrome. Adenomatous polyps may require simple polypectomy, segmental resection, or even subtotal colectomy. Following recognition of these unusual disorders the exact management is individualized.