Surgical correction of unsuccessful derotational humeral osteotomy in obstetric brachial plexus palsy: Evidence of the significance of scapular deformity in the pathophysiology of the medial rotation contracture

Background

The current method of treatment for persistent internal rotation due to the medial rotation contracture in patients with obstetric brachial plexus injury is humeral derotational osteotomy. While this procedure places the arm in a more functional position, it does not attend to the abnormal glenohumeral joint. Poor positioning of the humeral head secondary to elevation and rotation of the scapula and elongated acromion impingement causes functional limitations which are not addressed by derotation of the humerus. Progressive dislocation, caused by the abnormal positioning and shape of the scapula and clavicle, needs to be treated more directly.

Methods

Four patients with Scapular Hypoplasia, Elevation And Rotation (SHEAR) deformity who had undergone unsuccessful humeral osteotomies to treat internal rotation underwent acromion and clavicular osteotomy, ostectomy of the superomedial border of the scapula and posterior capsulorrhaphy in order to relieve the torsion developed in the acromio-clavicular triangle by persistent asymmetric muscle action and medial rotation contracture.

Results

Clinical examination shows significant improvement in the functional movement possible for these four children as assessed by the modified Mallet scoring, definitely improving on what was achieved by humeral osteotomy.

Conclusion

These results reveal the importance of recognizing the presence of scapular hypoplasia, elevation and rotation deformity before deciding on a treatment plan. The Triangle Tilt procedure aims to relieve the forces acting on the shoulder joint and improve the situation of the humeral head in the glenoid. Improvement in glenohumeral positioning should allow for better functional movements of the shoulder, which was seen in all four patients. These dramatic improvements were only possible once the glenohumeral deformity was directly addressed surgically.     

The utility of superficial abdominal reflex in the initial diagnosis of scoliosis: a retrospective review of clinical characteristics of scoliosis with syringomyelia

Background

The incidence of spinal deformity in children with Prader-Willi syndrome (PWS) is high, with 86% of these patients found to have a significant structural scoliosis; however, there are very few case reports describing surgical treatment for this deformity.

Methods

The authors reviewed a case series consisting of 6 patients who underwent spine surgery for scoliosis. Children's mean age at index surgery was 12 years and 10 months (range, 10 to 15 yrs). Clinical evaluation revealed the typical phenotypic features of the PWS in all of the patients; 4 subjects had a karyotype confirmation of PWS. Major structural curves showed preoperative mean Cobb angles of 80.8° (range, 65° to 96°). Hybrid instrumentation with sublaminar wires, hooks and screws was used in the first 2 patients, while the remaining 4 were treated with titanium pedicle screw constructs.

Results

The mean clinical and radiological follow-up was 3 years and 10 months (range, 2 years to 9 years). Major complication rate was 50%. One patient who developed a major intraoperative complication (paraparesis) prevented spinal fusion to be obtained: the neurologic deficit resolved completely after instrumentation removal. Solid arthrodesis and deformity correction in both coronal and sagittal plane was, however, achieved in the other 5 cases and no significant curve progression was observed at follow-up. Another major short-term complication was encountered 3 months after surgery in a patient who experienced the detachment of a distally located rod and required correction through revision surgery and caudal extension by one level. Cervico-thoracic kyphosis was seen in 1 patient who did not require revision surgery.

Conclusions

Spine reconstructive surgery in patients with PWS is rare and highly demanding. The best method of reconstruction is posterior multilevel pedicle screw fixation. Moreover, even with modern techniques, the risk of complications is still high. These new techniques, however, have shown to improve the postoperative course by allowing for immediate mobilization without any brace or cast. The use of the growing rod techniques, requiring repeated surgeries, should be carefully evaluated in each single case.     

Methodology of evaluation of morphology of the spine and the trunk in idiopathic scoliosis and other spinal deformities - 6th SOSORT consensus paper

Background

Comprehensive evaluation of the morphology of the spine and of the whole body is essential in order to correctly manage patients suffering from progressive idiopathic scoliosis. Although methodology of clinical and radiological examination is well described in manuals of orthopaedics, there is deficit of data which clinical and radiological parameters are considered in everyday practise. Recently, an increasing tendency to extend scoliosis examination beyond the measure of the Cobb angle can be observed, reflecting a more patient-oriented approach. Such evaluation often involves surface parameters, aesthetics, function and quality of life.

Aim of the study

To investigate current recommendations of experts on methodology of evaluation of the patient with spinal deformity, essentially idiopathic scoliosis.

Methods

Structured Delphi procedure for collecting and processing knowledge from a group of experts with a series of questionnaires and controlled opinion feedback was performed. Experience and opinions of the professionals - physicians and physiotherapists managing scoliosis patients - were studied. According to Delphi method a Meeting Questionnaire (MQ) has been developed, resulting from a preliminary Pre-Meeting Questionnaire (PMQ) which had been previously discussed and approved on line. The MQ was circulated among the SOSORT experts during Consensus Session on "Measurements" which took place at the Annual Meeting of the Society, totally 23 panellists being engaged. Clinical, radiological and surface topography parameters were checked for agreement.

Results

90% agreement or more was reached in 35 items and superior than 75% agreement was reached in further 25 items. An evaluation form was proposed to be used by clinicians and researchers.

Conclusion

The consensus was reached on evaluation of the morphology of the patient with idiopathic scoliosis, comprising clinical, radiological and, to less extend, surface topography assessment. Considering the variety of parameters indicated by the panellists, the Cobb angle, yet the gold standard, can be seen neither as the unique nor the only decisive parameter in the management of patients with idiopathic scoliosis.     

Prevalence of accessory deep peroneal nerve in referred patients to an electrodiagnostic medicine clinic

Background

Variations in the branching of posterior cord are important during surgical approaches to the axilla and upper arm, administration of anesthetic blocks, interpreting effects of nervous compressions and in repair of plexus injuries. The patterns of branching show population differences. Data from the African population is scarce.

Objective

To describe the branching pattern of the posterior cord in a Kenyan population.

Materials and methods

Seventy-five brachial plexuses from 68 formalin fixed cadavers were explored by gross dissection. Origin and order of branching of the posterior cord was recorded. Representative photographs were then taken using a digital camera (Sony Cybershot ^R, W200, 7.2 Megapixels).

Results

Only 8 out of 75 (10.7%) posterior cords showed the classical branching pattern. Forty three (57.3%) lower subscapular, 8(10.3%) thoracodorsal and 8(10.3%) upper subscapular nerves came from the axillary nerve instead of directly from posterior cord. A new finding was that in 4(5.3%) and in 3(4%) the medial cutaneous nerves of the arm and forearm respectively originated from the posterior cord in contrast to their usual origin from the medial cord.

Conclusions

Majority of posterior cords in studied population display a wide range of variations. Anesthesiologists administering local anesthetic blocks, clinicians interpreting effects of nerve injuries of the upper limb and surgeons operating in the axilla should be aware of these patterns to avoid inadvertent injury. A wider study of the branching pattern of infraclavicular brachial plexus is recommended.     

The direct cost of "Thriasio" school screening program

Background

Adolescent girls treated with a brace for scoliosis are submitted to prolonged stress related to both the disease and the therapy. Currently proposed quality of life questionnaires are focused on the outcome of therapy. Bad Sobernheim Stress Questionnaire (BSSQ) enables monitoring of patients being under treatment with a brace or exercises. The aim of the study was to assess the stress level in conservatively managed scoliotic girls using BSSQ.

Materials and methods

111 girls, aged 14,2 ± 2,2 years, mean Cobb angle of the primary curve 42,8° ± 17,0° and mean Bunnell angle of 11,4° ± 4,5° were examined with two versions of BSSQ (Deformity and Brace). The analysis considered the type of treatment, curve location, correlation of the total score with age, Cobb angle and Bunnell rotation angle.

Results

The BSSQ Deformity revealed the median of 17 points in patients managed with exercises (from 4 to 24 points), 18 in patients managed with a brace (from 8 to 24 points) and 12 in patients before surgery (from 3 to 21 points). Braced patients who completed both questionnaires (n = 50) revealed significantly higher score with BSSQ Deformity (median = 18) comparing to BSSQ Brace (median = 9). There was a correlation between the total score of BSSQ Deformity and the Cobb angle (r = -0,34), Bunnell primary curve rotation (r = -0,34) and Bunnell sum of rotation (r = -0,33) but not with the age of patients.

Conclusion

Scoliotic adolescents managed with exercises and brace suffered little stress from the deformity. The brace increased the level of stress over the stress induced by the deformity. The stress level correlated with clinical deformity (Bunnell angle), radiological deformity (Cobb angle) and the type of treatment (exercises, bracing, surgery). Bad Sobernheim Stress Questionnaires are simple and helpful in the management of girls treated conservatively for idiopathic scoliosis.     

Real time noninvasive assessment of external trunk geometry during surgical correction of adolescent idiopathic scoliosis

Study design

Retrospective study.

Objective

To evaluate the radiological results of fusion with segmental pedicle screw fixation in juvenile idiopathic scoliosis with a minimum 5-year follow-up.

Summary of background data

Progression of spinal deformity after posterior instrumentation and fusion in immature patients has been reported by several authors. Segmental pedicle screw fixation has been shown to be effective in controlling both coronal and sagittal plane deformities. However, there is no long term study of fusion with segmental pedicle screw fixation in these group of patients.

Methods

Seven patients with juvenile idiopathic scoliosis treated by segmental pedicle screw fixation and fusion were analyzed. The average age of the patients was 7.4 years (range 5–9 years) at the time of the operation. All the patients were followed up 5 years or more (range 5–8 years) and were all Risser V at the most recent follow up. Three dimensional reconstruction of the radiographs was obtained and 3DStudio Max software was used for combining, evaluating and modifying the technical data derived from both 2d and 3d scan data.

Results

The preoperative thoracic curve of 56 ± 15° was corrected to 24 ± 17° (57% correction) at the latest follow-up. The lumbar curve of 43 ± 14° was corrected to 23 ± 6° (46% correction) at the latest follow-up. The preoperative thoracic kyphosis of 37 ± 13° and the lumbar lordosis of 33 ± 13° were changed to 27 ± 13° and 42 ± 21°, respectively at the latest follow-up. None of the patients showed coronal decompensation at the latest follow-up. Four patients had no evidence of crankshaft phenomenon. In two patients slight increase in Cobb angle at the instrumented segments with a significant increase in AVR suggesting crankshaft phenomenon was seen. One patient had a curve increase in both instrumented and non instrumented segments due to incorrect strategy.

Conclusion

In juvenile idiopathic curves of Risser 0 patients with open triradiate cartilages, routine combined anterior fusion to prevent crankshaft may not be warranted by posterior segmental pedicle screw instrumentation.     

Human immunodeficiency virus seroconversion presenting with acute inflammatory demyelinating polyneuropathy: a case report

Introduction

Electrocardiogram (ECG) abnormalities in patients with blunt chest trauma are diverse and non-specific, but may be indicative of potentially life-threatening conditions.

Case presentation

We report a rare case of pneumopericardium with extreme ECG abnormalities after blunt chest trauma in a 22-year-old male. The diagnosis was confirmed using computed tomography (CT) scanning. The case is discussed, together with its differential diagnosis and the aetiology of pneumopericardium and tension pneumopericardium.

Conclusion

Pneumopericardium should be distinguished from other pathologies such as myocardial contusion and myocardial infarction because of the possible development of tension pneumopericardium. Early CT scanning is important in the evaluation of blunt chest trauma.     

In lumbosacral plexus injuries can we identify indicators that predict spontaneous recovery or the need for surgical treatment? Results from a clinical study on 72 patients

Background

Post-traumatic lumbosacral plexus injuries seem to be rare events, spontaneously recovering in high percentage: as surgery is often challenging and results in poor outcome, many Authors have advocated conservative treatment only. Nevertheless surgery should not be ruled out: in invalidating injuries, it can restore basic function in the lower extremities. Therefore, it might be necessary to establish guidelines for the management and the indication to surgery in such cases. This study aims to identify indicators predicting spontaneous recovery or the need for surgery.

Method

The clinical and radiological data of 72 patients with a post-traumatic lumbosacral plexus injury were reviewed. A follow up equal or superior to 3 years is available in 42 cases.

Results

Lumbosacral plexus injuries mostly occurred during road accidents. The incidence of associated lesions was relevant: bone injuries were found in 85% of patients, internal lesions in 30% and vascular injuries in 8%. Lumbosacral trunk and sacral plexus palsies were the most frequent injury patterns. Root avulsions were revealed in 23% of cases and only in sacral plexus and complete lumbosacral plexus injuries: L5 and S1 were the roots more prone to avulsions. About 70% of cases recovered spontaneously, mostly in 18 months. Spontaneous recovery was the rule in lumbar plexus and lumbosacral trunk injuries (where root avulsions never occurred) or in sacral and complete lumbosacral plexus palsies due to compression injuries. The causative mechanism correlated with the injury pattern, the associated bone injury being often predictive of the severity of the nerve injury. Lumbosacral plexus injuries occurred in car crashes were generally associated with fractures causing compression on the nerves, thus resulting in injuries often amenable of spontaneous recovery. Motorcycle accidents implied high kinetic energy traumas where traction played an important role, as the high percentage of sacroiliac joint separations demonstrated (found in more than 50% of cases and always associated to root avulsions). Loss of sphincteral control and excruciating leg pain were also invariably associated with avulsions.

Conclusions

Clinical and radiological data can help to predict the occurrence of spontaneous recovery or the need for surgery in post-traumatic lumbosacral plexus injuries.     

The three-dimensional easy morphological (3-DEMO) classification of scoliosis – Part III, correlation with clinical classification and parameters

Background

The shape of the torso in patients with idiopathic scoliosis is considered to reflect the shape of the vertebral column, however the direct correlation between parameters describing clinical deformity and those characterizing radiological curvature was reported to be weak. It is not clear if the management proposed for scoliosis (physiotherapy, brace, surgery) affects equally the shape of the axial skeleton and the surface of the body. The aim of the study was to compare clinical deformity of (1) idiopathic scoliosis girls being under brace treatment for radiological curves of 25 to 40 degrees and (2) non treated scoliotic girls matched for age and Cobb angle.

Methods

Cross-sectional study of 24 girls wearing the brace versus 26 girls without brace treatment, matched for age and Cobb angle. Hypothesis: Patients wearing the brace for more than 6 months, when comparing to patients without brace, may present different external morphology of the trunk, in spite of having similar Cobb angle. Material. Inclusion criteria: girls, idiopathic scoliosis, growing age (10–16 years), Cobb angle minimum 25°, maximum 40°. The braced group consisted of girls wearing a TLSO brace (Cheneau) for more than 6 months with minimum of 16 hours per day. The non-braced group consisted of girls first seen for their spinal deformity, previously not treated. The groups presented similar curve pattern. Methods. Scoliometer exam: angle of trunk rotation at three levels of the spine: upper thoracic, main thoracic, lumbar or thoracolumbar. The maximal angle was noted at each level and the sum of three levels was calculated. Posterior trunk symmetry index (POTSI) and Hump Sum were measured using surface topography.

Results

Cobb angle was 34.9° ± 4.8° in braced and 32.7° ± 4.9° in un-braced patients (difference not significant). The age was 14.1 ± 1.6 years in braced patients and 13.1 ± 1.9 years in un-braced group (p = 0.046). The value of angle of trunk rotation in the main curvature was 8.4° ± 2.7°in braced and 11.4° ± 2.7° in un-braced patients (difference extremely significant, p = 0.0003). The value of the sum of angles of trunk rotation at three levels of the trunk was 12.8° ± 4.6° in braced and 16.5° ± 3.8° in un-braced patients (difference very significant, p = 0.0038). The POTSI did not differ significantly between the groups (p = 0.78), the Hump Sum values were not quite different (p = 0.07).

Conclusion

(1) Adolescent girls wearing the brace for idiopathic scoliosis of 25 to 40 degrees of Cobb angle, reveal smaller clinical rotational deformity of their back than non-treated girls having similar radiological deformity. (2) evaluation of the results of treatment for idiopathic scoliosis should consider parameters describing both clinical and radiological deformity.     

Une syringocèle révélée par un abcès périntéal

Purpose

The authors present a clinical report of a perforate syringocele diagnosed in a male patient and they review the clinical, radiological and therapeutic aspects of this disease.

Case report

A 50-year-old man attended the emergency room with perineal abscess. Computerized Tomography (CT) scan was used to establish the diagnosis. This patient also underwent urethrocystoscopy, sonography and retrograde urethrography.

Results

Treatment consisted of open surgical evacuation of the perineal abscess with bladder catheter. Urethrocystoscopy showed a large perforate syringocele in the urethra with no need for endoscopy marsupialization. No recurrence was observed at follow-up at 3 months.

Conclusion

Syringocele is a rare malformation which usually is asymptomatic. It can be congenital or acquired and can be classified into four morphological types. The most frequent treatment is endoscopic marsupialization of the syringocèle. Open surgical excision is recommended for complicated syringocèle with abscess or perineal pain.     

Prediction of peak pressure from clinical and radiological measurements in patients with diabetes

Background

Various structural and functional factors of foot function have been associated with high local plantar pressures. The therapist focuses on these features which are thought to be responsible for plantar ulceration in patients with diabetes. Risk assessment of the diabetic foot would be made easier if locally elevated plantar pressure could be indicated with a minimum set of clinical measures.

Methods

Ninety three patients were evaluated through vascular, orthopaedic, neurological and radiological assessment. A pressure platform was used to quantify the barefoot peak pressure for six forefoot regions: big toe (BT) and metatarsals one (MT-1) to five (MT-5). Stepwise regression modelling was performed to determine which set of the clinical and radiological measures explained most variability in local barefoot plantar peak pressure in each of the six forefoot regions. Comprehensive models were computed with independent variables from the clinical and radiological measurements. The difference between the actual plantar pressure and the predicted value was examined through Bland-Altman analysis.

Results

Forefoot pressures were significant higher in patients with neuropathy, compared to patients without neuropathy for the whole forefoot, the MT-1 region and the MT-5 region (respectively 138 kPa, 173 kPa and 88 kPa higher: mean difference). The clinical models explained up to 39 percent of the variance in local peak pressures. Callus formation and toe deformity were identified as relevant clinical predictors for all forefoot regions. Regression models with radiological variables explained about 26 percent of the variance in local peak pressures. For most regions the combination of clinical and radiological variables resulted in a higher explained variance. The Bland and Altman analysis showed a major discrepancy between the predicted and the actual peak pressure values.

Conclusion

At best, clinical and radiological measurements could only explain about 34 percent of the variance in local barefoot peak pressure in this population of diabetic patients. The prediction models constructed with linear regression are not useful in clinical practice because of considerable underestimation of high plantar pressure values. Identification of elevated plantar pressure without equipment for quantification of plantar pressure is inadequate. The use of quantitative plantar pressure measurement for diabetic foot screening is therefore advocated.     

Utilization trends of pedicle subtraction osteotomies compared to posterior spinal fusion for deformity: a national database analysis between 2008–2011

Background

Increased awareness regarding the importance of the sagittal spinal profile has led to more aggressive correction of sagittal malalignment. The utilization trends of pedicle subtraction osteotomy (PSO) for sagittal plane correction in spinal deformity surgery have not been well characterized.

Methods

A commercially available database (PearlDiver, Inc) was queried for both Private Payor and 5 % Medicare claims from 2008 to 2011. Revision and clarification of the coding guidelines for PSO were introduced in 2008. Patients who had a thoracic and/or lumbar PSO were identified using CPT codes (22206-22208). In order to appropriately interpret trends in PSO use, three comparison groups were identified. Patients who had a diagnosis of adult spine deformity were identified using ICD-9 codes. Patients who had fusion for spine deformity or posterior spine fusion were identified using CPT codes. Differences in annual utilization and demographics between these four groups were then compared.

Results

From the Private Payor database, 199 PSOs were identified with the number of PSOs increasing from 33 in 2008, to 61 in 2011, representing a 185 % increase. From the Medicare data, 102 PSOs were identified, increasing from 13 in 2008 to 32 in 2011, a 246 % increase. In contrast, from both databases, there was minimal to no increase in the incidence of adult spine deformity, fusion for spine deformity or posterior spine fusion over the study time interval.

Conclusion

Over the study time interval, there was up to a 3.2-fold increase in the utilization of PSOs while the diagnosis of adult spine deformity, fusion for spine deformity and posterior spine fusions had minimal to no increase.

     

Postural effects of symmetrical and asymmetrical loads on the spines of schoolchildren

Background

The Walter Reed Visual Assessment Scale (WRVAS) was designed to measure physical deformity as perceived by patients with idiopathic scoliosis. Previous studies have shown that the instrument has excellent internal consistency and a high correlation with the radiological magnitude of scoliotic curves. Nonetheless, it is not known whether the scale can discriminate between the various curve patterns of the deformity, or whether the deformities represented in the scale's drawings relate to the corresponding radiological deformities.

Methods

This study included 101 patients (86 women and 15 men; mean age 19.4 years) with idiopathic scoliosis. In a single visit, patients underwent standing PA radiography of the spine and completed the WRVAS. X-ray measurements included: 1) magnitude (Cobb angle) of the proximal thoracic curve (PT), main thoracic curve (MT), and thoracolumbar/lumbar curve (TL/L); 2) difference in shoulder level; 3) T1 offset from the central sacral line (T1-CSL); 4) apical vertebra (apV) rotation at the MT and TL/L curves and 5) apical vertebra offset of the MT and TL/L curves from the central sacral line. A variable designated Cobbmax was defined as the largest angle of the three curves (PT, MT or TL/L). Patients were grouped onto three patterns: Thoracic (TH Group)(n = 30, mean MT 42.1°, TL/L 20.9°); double major (DM Group) (n = 39, mean MT 38.6°, TL/L 34.4°) and thoracolumbar (TL Group)(n = 32, mean MT 14.3°, TL/L 25.5°). The magnitude of the curves in the TL Group was significantly smaller than in the other groups (P < 0.05). The Spearman partial correlation coefficient was determined between the score for each WRVAS question and the curve pattern, adjusting for the Cobbmax variable. The Spearman correlation coefficient was determined between the WRVAS items and shoulder imbalance, T1-CSL offset, MT Cobb angle, MT apV rotation, MT apV offset, PT Cobb, TL/L Cobb, TL/L apV rotation and TL/L apV offset.

Results

The median (interquartile range) of the total WRVAS score was 14 (IQR 6). No correlation was found between the curve pattern and the various scores on the scale (partial correlation coefficients ranged from -0.16 to 0.12). WRVAS drawings for items 1, 2, 4 and 7 correlated satisfactorily with the corresponding radiological measurements (correlation coefficients, 0.62, 0.3, 0.48 and 0.53, respectively). Items 3, 5 and 6 did not correlate with the radiological measurements (correlation coefficients -0.06, -0.07 and 0.05, respectively).

Conclusion

The profile of the individual WRVAS scores does not differentiate among specific curve patterns (thoracic, double major and thoracolumbar/lumbar). Moreover, some of the drawings (items 3, 5 and 6) do not correlate with the radiological deformity they were designed to measure.     

Ring chromosome 13 syndrome characterized by high resolution array based comparative genomic hybridization in patient with 47, XYY syndrome: a case report

Introduction

Percutaneous or endocavitory drainage of a diverticular abscess under radiological guidance often enables one to perform a one-staged resection and anastomosis (without stoma formation) instead of a two-staged procedure. It reduces the significant postoperative morbidity and mortality associated with the conventional emergency surgical management. However, radiological guidance is not always available due to limited resources during out-of-hours.

Case presentation

A 78-year-old Caucasian woman underwent transrectal drainage of a diverticular abscess performed with a pigtail catheter without radiological guidance. Technical details of the procedure are described and alternative options discussed.

Conclusion

In carefully selected patients, per-rectal drainage using a pigtail catheter can be performed without radiological guidance and the procedure offers a simple and effective way of controlling sepsis.     

Symptomatic pectus excavatum in seniors (SPES): a cardiovascular problem?: A prospective cardiological study of 42 senior patients with a symptomatic pectus excavatum

Objectives

There is no consensus among physicians as to whether or not pectus excavatum can produce symptoms sometimes even severe enough to justify a surgical procedure. The aim of this study was to assess the prevalence and severity of complaints and symptoms of senior patients with a pectus excavatum and to evaluate the results of surgical correction of the chest deformation.

Design

This is a prospective clinical study, case series.

Participants

The participants are 42 senior patients with a pectus excavatum and somatic complaints.

Methods

Cardiological screening included medical history taking, physical examination, electrocardiography, transthoracic echocardiography and treadmill cardiography. Complaints, symptoms and test results were arranged in a clinical score. Indication for a therapeutic surgical correction by a modified Ravitch operation was a high clinical score in combination with radiological evidence of cardiac compression on CT or MRI.

Results

The clinical picture of the 42 senior patients with a pectus excavatum showed complaints of fatigue and low exercise tolerance, shortness of breath, palpitations, inspiratory obstruction and sometimes chest discomfort or pain. The serious and sometimes invalidating complaints of 19 patients (45%) had started only in their fourth or fifth decade of life and were labelled in 12 patients (63%) as ‘Unexplained cardiovascular complaints’. To date, 11 patients have undergone surgical procedures. Symptoms were reduced substantially or had disappeared completely. All patients indicated that their health-related quality of life was significantly improved.

Conclusion

Recognising the clinical picture of SPES is relevant because surgical reconstruction of the chest can provide substantial relief of symptoms.     

Painful rib hump: a new clinical sign for detecting intraspinal rib displacement in scoliosis due to neurofibromatosis

Background

Spinal cord compression and associate neurological impairment is rare in patients with scoliosis and neurofibromatosis. Common reasons are vertebral subluxation, dislocation, angulation and tumorous lesions around the spinal canal. Only twelve cases of intraspinal rib dislocation have been reported in the literature. The aim of this report is to present a case of rib penetration through neural foramen at the apex of a scoliotic curve in neurofibromatosis and to introduce a new clinical sign for its detection.

Methods

A 13-year-old girl was evaluated for progressive left thoracic kyphoscoliotic curve due to a type I neurofibromatosis. Clinical examination revealed multiple large thoracic and abdominal "cafe-au-lait" spots, neurological impairment of the lower limbs and the presence of a thoracic gibbous that was painful to pressure at the level of the left eighth rib (Painful Rib Hump). CT-scan showed detachment and translocation of the cephalic end of the left eighth rib into the adjacent enlarged neural foramen. The M.R.I. examination of the spine showed neither cord abnormality nor neurogenic tumor.

Results

The patient underwent resection of the intraspinal mobile eighth rib head and posterior spinal instrumentation and was neurologically fully recovered six months postoperatively.

Conclusion

Spine surgeons should be aware of intraspinal rib displacement in scoliotic curves in neurofibromatosis. Painful rib hump is a valuable diagnostic tool for this rare clinical entity.     

Surgical outcomes of the brachial plexus lesions caused by gunshot wounds in adults

Background

The management of brachial plexus injuries due to gunshot wounds is a surgical challenge. Better surgical strategies based on clinical and electrophysiological patterns are needed. The aim of this study is to clarify the factors which may influence the surgical technique and outcome of the brachial plexus lesions caused by gunshot injuries.

Methods

Two hundred and sixty five patients who had brachial plexus lesions caused by gunshot injuries were included in this study. All of them were male with a mean age of 22 years. Twenty-three patients were improved with conservative treatment while the others underwent surgical treatment. The patients were classified and managed according to the locations, clinical and electrophysiological findings, and coexisting lesions.

Results

The wounding agent was shrapnel in 106 patients and bullet in 159 patients. Surgical procedures were performed from 6 weeks to 10 months after the injury. The majority of the lesions were repaired within 4 months were improved successfully. Good results were obtained in upper trunk and lateral cord lesions. The outcome was satisfactory if the nerve was intact and only compressed by fibrosis or the nerve was in-contunuity with neuroma or fibrosis.

Conclusion

Appropriate surgical techniques help the recovery from the lesions, especially in patients with complete functional loss. Intraoperative nerve status and the type of surgery significantly affect the final clinical outcome of the patients.     

Rare causes of scoliosis and spine deformity: experience and particular features

Background

Spine deformity can be idiopathic (more than 80% of cases), neuromuscular, congenital or neurofibromatosis-related. However, there are many disorders that may also be involved. We present our experience treating patients with scoliosis or other spine deformities related to rare clinical entities.

Methods

A retrospective study of the records of a school-screening study in North-West Greece was performed, covering a 10-year period (1992–2002). The records were searched for patients with deformities related to rare disorders. These patients were reviewed as regards to characteristics of underlying disorder and spine deformity, treatment and results, complications, intraoperative and anaesthesiologic difficulties particular to each case.

Results

In 13 cases, the spine deformity presented in relation to rare disorders. The underlying disorder was rare neurological disease in 2 cases (Rett syndrome, progressive hemidystonia), muscular disorders (facioscapulohumeral muscular dystrophy, arthrogryposis) in 2 patients, osteogenesis imperfecta in 2 cases, Marfan syndrome, osteopetrosis tarda, spondyloepiphyseal dysplasia congenita, cleidocranial dysplasia and Noonan syndrome in 1 case each. In 2 cases scoliosis was related to other congenital anomalies (phocomelia, blindness). Nine of these patients were surgically treated. Surgery was avoided in 3 patients.

Conclusion

This study illustrates the fact that different disorders are related with curves with different characteristics, different accompanying problems and possible complications. Investigation and understanding of the underlying pathology is an essential part of the clinical evaluation and preoperative work-up, as clinical experience at any specific center is limited.     

Algorithms for the diagnosis and treatment of restless legs syndrome in primary care

Background

Restless legs syndrome (RLS) is a neurological disorder with a lifetime prevalence of 3-10%. in European studies. However, the diagnosis of RLS in primary care remains low and mistreatment is common.

Methods

The current article reports on the considerations of RLS diagnosis and management that were made during a European Restless Legs Syndrome Study Group (EURLSSG)-sponsored task force consisting of experts and primary care practioners. The task force sought to develop a better understanding of barriers to diagnosis in primary care practice and overcome these barriers with diagnostic and treatment algorithms.

Results

The barriers to diagnosis identified by the task force include the presentation of symptoms, the language used to describe them, the actual term "restless legs syndrome" and difficulties in the differential diagnosis of RLS.

Conclusion

The EURLSSG task force reached a consensus and agreed on the diagnostic and treatment algorithms published here.     

Place de l’embolisation percutanée de la varicocèle dans le traitement de l’infertilité

Objectives

To describe percutaneous embolization of varicocele and to evaluate the effect on fertility disorders.

Materials and methods

One hundred nineteen patients, aged 11 to 48 years, underwent percutaneous embolization for varicocele indicated by a fertility disorder in 23% of cases. Correction of the varicocele and resolution of associated pain, improvement of testicular trophicity and the effect on fertility were studied and a review of the literature was performed.

Results

Percutaneous embolization was performed using neuroleptanalgesia on an outpatient basis. The technique combined venous sclerotherapy and placement of coils in the gonadal vein. The technique was successful in 95% of cases and one complication was observed. At 3 months, the varicocele and related pain had resolved in 98% of treated patients. According to previous reports in the literature, the relations between varicocele and fertility disorders are still unclear, but improvement of fertility (semen quality and conception rates) after varicocele repair has been established.

Conclusion

Although the consequences of varicocele on fertility have not been fully elucidated, treatment of varicocele appears to be beneficial. Percutaneous embolization of varicocele is a safe and effective alternative to surgery. This technique, based on a urological and radiological consensus, is the first-line treatment for varicocele in our institution.