Six cases of sporadic schwannomatosis: Topographic distribution and outcomes of peripheral nerve tumors.

The diagnosis of schwannomatosis is often overestimated and is based on the existence of multiple peripheral nerve tumors composed exclusively of schwannomas, in the absence of clinical signs of neurofibromatosis type 2 (NF2). Sporadic forms are much more frequent than familial forms. The objective of this study was to describe the distribution of peripheral nerve tumors and investigate the outcomes of schwannomas in the context of sporadic schwannomatosis. We conducted a retrospective study of patients who fulfilled clinical diagnostic criteria for sporadic schwannomatosis. Six patients were reviewed with a mean follow-up of 38.5months (27-60months). Patients' demographic, clinical, radiographic, and pathologic data were extracted. All patients underwent slit-lamp examination, enhanced brain magnetic resonance imaging (MRI) and a spinal MRI. Enucleation that preserved nerve continuity was performed in symptomatic patients. On average, patients were 36years of age at the time of diagnosis with no sex predominance. The topographic distribution of the peripheral nerve tumors was always unilateral and most frequently targeted the upper limb. In four cases, the tumors involved the same peripheral nerve exclusively. The average number of nerve tumors observed per patient was 4.7 (2-8). The outcome after enucleation was marked by the systematic appearance of new tumors. After enucleation, no recurrence or malignant transformation was observed at the final follow-up. There was no transition to a NF2 configuration. The absence of neurofibroma and NF2 criteria makes schwannomatosis a diagnosis of exclusion. While a good prognosis can be expected following enucleation, two risks related to neurofibromatosis type 3 (NF3) are worth monitoring: the transition to NF2, particularly in young patients, and the appearance of new tumors.

The role of imaging in isolated benign peripheral nerve tumors: A practical review for surgeons.

The diagnosis of nerve tumor(s) must be suspected in all cases of tumefaction or pain on the path of a nerve exacerbated by percussion. Solitary nerve tumors are primarily schwannomas, but other rare tumors may be present such as intraneural ganglion cysts of controversial origin. Preservation of nerve continuity is the underlying goal for any surgical procedure, irrespective of the type of tumor. Therapeutic outcomes are closely linked to tumor resectability; in most patients, the resectability of the tumor, its type and benignity can be predicted based on medical imaging. Comparison with the clinical examination and case-based reasoning is crucial. Consequently, the aim of this review was to examine the role of imaging in isolated benign peripheral nerve tumors, and provide the surgeon with a practical guide for its application in predicting the nature and resectability of nerve tumors.

[Multiple peripheral nerve tumors: update and review of the literature]. / Tumeurs multiples des nerfs périphériques: mise au point et revue de la littérature.

Multiple tumours of the peripheral nerves are seen only in neurofibromatosis. They are hereditary. They present and develop in a variety of different ways. Three main groups are distinguished: von Recklinghausen neurofibromatosis or type 1; bilateral acoustic neurofibromatosis or type 2 and schwannomatosis recently defined as type 3. The aim of this study was to clarify the clinical outcome of neurofibromatosis. The diagnosis is made purely on clinical grounds. Cranial MRI and slit lamp examination are useful for classification. Surgical management for peripheral nerve tumours is similar. Any new and rapid change noted at clinical examination (increase in volume, pain or neurological deficit) requires surgery because of potential malignant transformation of the neurofibroma into neurofibrosarcoma (type 1 only). The definitive treatment depends on the resectable character of the tumour which is usually only known after epineurotomy under operating microscope. In the event of resectable tumour (schwannoma) enucleation must be performed, preserving nerve continuity. In the event of unresectable tumour (neurofibroma), tumour resection is impossible without sacrificing nerve tissue. An epineurotomy must be performed. It prevents further deterioration. Interfascicular biopsy confirms the histological type. Our results are similar to those in other recorded studies. The unpredictable clinical course of neurofibromatosis makes prolonged follow-up mandatory.

Intraneural mucoid pseudocysts. A report of ten cases.

A mucoid pseudocyst of a peripheral nerve is a rare and benign tumour of controversial origin. We have reviewed ten patients with a mean follow-up of 3.2 years. The tumour affected the common peroneal nerve in eight and the ulnar nerve in two. The mean time between the onset of symptoms and diagnosis was 7.4 months (1.2 months to 2 years). On examination, there was pain in eight patients and swelling in seven. Motor deficit in the corresponding nerve territory was found in all. The diagnosis was usually confirmed by MRI. Treatment was always surgical. All the patients recovered, with a mean time to neurological recovery of 10.75 months. Recurrence was seen in only one patient and was treated successfully by further surgery. Our results are similar to those reported by other authors. A successful surgical outcome depends on early diagnosis before neurological damage has occurred.

[Complex fractures of the proximal end of the radius and ulna in adults: a new classification]. / Fractures complexes de l'extrémité supérieure des deux os de l'avant-bras chez l'adulte.

Unlike injuries involving only one of the forearm bones, complex lesions of the proximal end of the radius and the ulna are particularly unstable. Various situations-Monteggia fracture, transolecraneal dislocation, or fracture-dislocation-are encountered. The classification systems proposed to date and recalled here are insufficient, making it difficult to provide optimal therapy and also hindering comparison between published series. We propose a descriptive classification including all the anatomic varieties of complex fractures of the proximal end of the radius and the ulna. This classification is based on our experience with 38 cases and takes into account 4 basic elements: the height of the ulnar fracture line, the direction of the displacement of the proximal radius, the association of a fracture of the proximal radius and/or of the coronoid process.

[Complex fractures of the proximal end of the radius and ulna in adults: a retrospective study of 38 cases]. / Fractures complexes de l'extrémité supérieure des deux os de l'avant-bras chez l'adulte.

PURPOSE OF THE STUDY: Complex lesions of the proximal end of the radius and ulna are uncommon and generally associate a fracture of the proximal ulna and a dislocation of the radial head, which also may be fractured. We assessed the effect of the type of fracture and treatment on functional outcome and complications. MATERIAL AND METHODS: Thirty-eight adults (25 men and 13 women) were treated for complex fractures of the proximal end of the radius and ulna. For 25 of them, mean follow-up was 2.4 years. For the ulna, there was an epiphyseal fracture in 10 cases, a metaphyseal-epiphyseal fracture in 16 and a diaphyseal fracture in 12. The coronoid process was fractured in 20 cases and the proximal radius in 19. There was an anterior displacement in 24 cases. There were 9 open fractures and 13 patients had another injury of the upper limb. Functional outcome was assessed with a 100 point scale using subjective (pain) and objective (active motion, muscle force, stability) criteria. RESULTS: For the 25 fractures with more than 1 year follow-up, outcome was very good in 8, good in 6, fair in 8 and poor in 3. The pain and muscle force scores followed a similar pattern. Motion appeared as the determining factor for good outcome. Seven early revisions (2 for deep infections, 2 for disassembly of the fixation system, 3 for insufficient fixation) were required among the 38 patients. Among the late complications, there were 3 nonunions, 5 misalignments, and 4 proximal radio-ulnar synostoses. Six elbows required revision to restore motion. DISCUSSION: Certain characteristics of the fractures were predictive of poor outcome: skin opening, association with a lesion of the homolateral upper limb, mirror lesion of the lateral condyle, metaphyseal-epiphyseal fractures, communitive fractures, presence of a fracture of the radial head or the coronoid process. CONCLUSION: Complex proximal fractures of both bones of the forearm threaten the functional prognosis of the upper limb due to the risk of stiffness. Successful treatment depends on three factors: stable anatomic reconstruction of the ulnar articulation, and reconstruction of the lateral column and the coronoid process, necessary for a stable elbow. In addition, early mobilization, possible with a stable osteosynthesis, is indispensable for recovering useful joint movement.

[Acute compartment syndrome in the thigh: 3 cases]. / Syndrome de loge aigü de cuisse. A propos de 3 cas chez 2 patients.

We report three cases of compartment syndrome in the thigh related to drug intoxications. Compartment syndrome is an uncommon condition often diagnosed late as it may occur in unconscious patients. The typical clinical presentation begins with pain of the lower limb refractory to major antagesics. The condition then progresses with sensoromotor involvement of the sciatic nerve. Rapid onset of acute renal failure in the setting of a crush syndrome may be life-threatening with a long-term risk of major sensoromotor sequelae. Surgical treatment with aponeurotomy is required rapidly to limit sequelae.

[Benign solitary tumors of the peripheral nerves]. / Tumeurs bénignes isolées des nerfs périphériques.

Solitary tumors of the peripheral nerves are uncommon and found to be benign in 90 p. 100 of the cases. They develop from the elements constituting the nerve and are generally schwannomas (80 p. 100). Other tumors are much more exceptional and exhibit wide histological variability. The diagnosis of a tumor of the peripheral nerve must be envisaged for all cases with tumefaction or pain on the path of a nerve exacerbated at percussion. Magnetic resonance imaging is the preferred exploration technique, particularly useful in case of a deep tumor. Preservation of nerve continuity is the underlying goal of the therapeutic strategy, irrespective of the type of tumor. Extricable tumors are to be distinguished from inextricable tumors. Extricable tumors (schwannomas, intranervous lipomas) displace nerve fiber bundles without penetrating into the bundle itself and can thus be resected without interrupting nerve continuity. Prognosis is excellent if no recurrence or degeneration occurs. In case of persistent symptoms, a new exploration may be required to search for other localized tumor(s) unperceived at the first procedure. Inextricable tumors (solitary neurofibromas, hemangiomas of the Schwann sheath, neurofibrolipomas) infiltrate the structural elements of the nerve fibers making complete excision impossible without altering the nerve fibers. Epineurotomy (associated with an interfascicular biopsy for pathology examination) allows decompression and can often provide symptom relief although moderate paresthesia may persist. Patients must be informed of this possibility prior to surgery. Any recent and rapidly evolving modification in the clinical findings is suggestive of recurrence and should be followed by revision exploration. Malignant degeneration has not been observed in solitary tumors to our knowledge. Our own experience with 51 cases is generally in agreement with reports in the literature.

Differentiating violent and nonviolent opiate-addicted reformatory inmates with the MMPI.

Used multivariate profile analysis and stepwise discriminant analysis in an effort to discriminate among four groups of male opiate-addicted reformatory inmates (N = 193) classified according to degree of criminal violence: (1) Bodily Violent (N = 19); (2) Potentially Bodily Violent (N = 25); (3) Materially Violent (N = 113); and (4) Nonviolent (N = 36). Profile analysis indicated that the four groups were very similar; a stepwise discriminant analysis significantly distinguished the Bodily Violent group from each of the other groups, but failed to differentiate successfully among the remaining three groups. A second stepwise discriminant analysis, in which Groups 2, 3 and 4 were combined, produced a significant discriminant function and correctly classified 68.4% of the Bodily Violent group and 75.9% of the combined Nonbodily Violent groups. The MMPI scales that contributed most to the latter prediction of group membership, in relative order of discriminating power, were: F, MA, D, PD, HS, PA and SI.