External Malleolus Research Articles

Treatment Strategy for a Closed Tibial Pilon Fracture Concomitant with a Closed Lateral Malleolus Fracture That Occurred in a Context of Low Energy Trauma: A Case Report of a 43-Year-Old Man

Tibial pilon fractures are rare and challenging fractures of the lower limb that account for about 1% of all lower limbs fractures and up to 10% of tibial fractures. They are challenging in terms of treatment outcomes. The injury is generally caused by high energy axial trauma from motor vehicle accidents or a fall from height. The type of definitive fixation is still a great challenge for surgeons and is dictated by the soft tissue injury and energy imparted to the fracture. In this case report, we describe the combination of a closed tibial pilon fracture and a closed lateral malleolus fracture occurring in an unusual context for this type of injury in a 43-year-old-man. It actually occurred in a context of low energy trauma of the ankle during a friendly soccer match. A one step internal fixation method was chosen for definitive treatment. For this, two screws were used for the fixation of the tibial pilon fracture and the external malleolus was fixed with two Kirschner wires. Post-operative follow-up was uncomplicated.

Kirschner Wire Internal Fixation of the Medial Tibiotalar Joint for Indirect Repair of Deltoid Ligament Injury: A Retrospective Comparative Study.

Ankle joint fractures are often accompanied by medial deltoid ligament rupture. There is controversy over whether or how to treat deltoid ligament rupture. This study was aimed to explore the feasibility of repairing the medial deltoid ligament using Kirschner wire internal fixation of the medial tibiotalar joint combined with external fixation. Forty-six patients with ankle fractures involving deltoid ligament rupture, treated between October 2012 and February 2021, were retrospectively evaluated. Twenty-five patients were treated with a Kirschner wire to fix the tibiotalar joint and indirectly repair the deltoid ligament as the repaired group. Twenty-one patients underwent reduction and fixation of internal and external malleolus fractures, and the deltoid ligament was not repaired in the unrepaired group. The American Orthopaedic Foot and Ankle Society (AOFAS) ankle-hindfoot score, visual analog scale (VAS), Medical Outcomes Short Form 36-item questionnaire score (SF-36), and Medial clear space perpendicular (preoperative, postoperative, final follow-up) were used for functional evaluations and reduction assessments. Mann-Whitney test were used to compare the differences between the groups. The follow-up time was 13-112 months with a mean of 59.32 months for the repaired group and 11-94 months with a mean of 53.43 months for the unrepaired group. There was no significant difference in the operative time or intraoperative blood loss between the two groups (p > 0.05). At the last follow-up, the AOFAS ankle-hindfoot and SF-36 scores of the repaired group were significantly higher than those of the non-repaired group (p < 0.05). Moreover, the VAS pain score was significantly lower and the Medial clear space perpendicular was significantly narrower in the repaired group than that in the unrepaired group. Tibiotalar joint fixation using Kirschner wires is a simple and effective technique that can indirectly reduce and repair the deltoid ligament and stabilize the ankle.

Use of hyperoxidized oils-based medical devices for the wound healing in post-surgical patients.

Postoperative injuries of the lower limb represent a challenging complication after surgery. The use of advanced dressings, local flaps, reconstructions with grafts or dermal substitutes represent the most common therapeutic solutions. With the present paper, we describe a case of a postoperative wound of the leg treated with the use of hyperoxidized oils-based medical device: NOVOX©. An 88-year-old woman presented in September 2022 an ulcer on her left leg, on the external malleolus. Authors decided to treat the lesion with NOVOX© in the form of a dressing pad. The controls initially lasted every 48 hours, subsequently every 72 hours and in the last month only once a week. The progressive clinical examination showed a global reduction of the wound area. According to our experience, the novel oxygen-enriched oil-based dressing pad (NOVOX©) is simple to use, secure, and successful in treating old patients receiving postoperative therapy for leg ulcers.

Application status of open-wedge high tibial osteotomy assisted by three-dimensional printing patient-specific cutting guides

To review the application of three-dimensional (3D) printing patient-specific cutting guides (PSCG) in open-wedge high tibial osteotomy (OWHTO). The domestic and foreign literature about the use of 3D printing PSCG to assist the OWHTO in recent years was reviewed, and the effectiveness of different types of 3D printing PSCG to assist OWHTO was summarized. Many scholars design and use different 3D printing PSCGs to confirm the precise positioning of the osteotomy site (the bone surface around the cutting line, the "H" point of the proximal tibia, the internal and external malleolus fixators, etc.) and the correction angle (the pre-drilled holes, the wedge-shaped filling blocks, the angle-guided connecting rod, etc.) during operation, and all of them achieve good effectiveness. Compared with conventional OWHTO, 3D printing PSCG assisted OWHTO has many obvious advantages, such as shortening the operation time, and the frequency of fluoroscopy, and being closer to the expected preoperative correction, etc. However, the effectiveness between different 3D printing PSCGs still need to be discussed in the follow-up studies.

Analysis of the efficacy of a modified posteromedial approach for Klammer III posterior Pilon fractures

PurposeTo analyze the curative effect and technical points of a modified posteromedial approach in the treatment of Klammer III posterior Pilon fracture. MethodsA retrospective analysis of patients with Klammer III posterior Pilon fractures were conducted in our department from January 2018 to December 2019. Before the surgery, the patients were fully relieved of swelling and pain, and a comprehensive examination was carried out. The posteromedial approach exposed the posterior and medial fracture block of the distal tibia. According to the fracture of external malleolus, it is determined whether to combine a lateral incision and protect tendons and vascular nerves by a retractor, and then perform a fracture reduction and internal fixation. Postoperatively, the patients were treated with analgesia, detumescence, anticoagulation and rehabilitation exercise. The American orthopaedic foot and ankle society (AOFAS) score and visual analogue score were recorded at regular follow-up after surgery. A t-test was used for the comparison of the preoperative and final AOFAS score. ResultsThere were 7 male and 13 female (n = 20) included in the study, aged 22 to 88 years (average age 54.2 years). The injury mechanisms were falling from a height (n = 7), traffic accident (n = 6), walking injury (n = 2) and heavy injury (n = 5). The postoperative follow-up duration was 12–24 months (mean 16.95 months). The AOFAS score of the 20 patients before and after surgery were compared. The preoperative AOFAS score was 38.90 ± 3.91, and the final AOFAS score was 80.55 ± 4.20, (p < 0.001). The mean final visual analogue scores at rest, active and weight-bearing walking were 0.30, 0.85 and 1.70, respectively. One patient reported poor postoperative wound healing and required a return to hospital for debridement and anti-infection treatment. ConclusionIn the treatment of Klammer III posterior Pilon fractures, the modified posteromedial approach can fully expose the fracture block and the collapsed articular surface of the medial malleolus, achieve good reduction and internal fixation with limited injury of the tendon and vascular nerves, and have a better prognosis.

Safety and efficacy of riluzole in spinocerebellar ataxia type 2 in France (ATRIL): a multicentre, randomised, double-blind, placebo-controlled trial

Riluzole has been reported to be beneficial in patients with cerebellar ataxia; however, effectiveness in individual subtypes of disease is unclear due to heterogeneity in participants' causes and stages of disease. Our aim was to test riluzole in a single genetic disease, spinocerebellar ataxia type 2. We did a randomised, double-blind, placebo-controlled, multicentre trial (the ATRIL study) at eight national reference centres for rare diseases in France that were part of the Neurogene National Reference Centre for Rare Diseases. Participants were patients with spinocerebellar ataxia type 2 with an age at disease onset of up to 50 years and a scale for the assessment and rating of ataxia (SARA) score of at least 5 and up to 26. Patients were randomly assigned centrally (1:1) to receive either riluzole 50 mg orally or placebo twice per day for 12 months. Two visits, at baseline and at 12 months, included clinical measures and 3T brain MRI. The primary endpoint was the proportion of patients whose SARA score improved by at least 1 point. Analyses were done in the intention-to-treat population (all participants who were randomly assigned) and were done with only the observed data (complete case analysis). This trial is registered at ClinicalTrials.gov (NCT03347344) and has been completed. Between Jan 18, 2018, and June 14, 2019, we enrolled 45 patients. 22 patients were randomly assigned to receive riluzole and 23 to receive placebo. Median age was 42 years (IQR 36-57) in the riluzole group and 49 years (40-56) in the placebo group and 23 (51%) participants were women. All participants presented with moderate-stage disease, characterised by a median SARA score of 13·5 (IQR 9·5-16·5). The primary endpoint, SARA score improvement of at least 1 point after 12 months, was observed in seven patients (32%) in the treated group versus nine patients (39%) in the placebo group, with a mean difference of -10·3% (95% CI -37·4% to 19·2%; p=0·75). SARA score showed a median increase (ie, worsening) of 0·5 points (IQR -1·5 to 1·5) in the riluzole group versus 0·3 points (-1·0 to 2·5) in the placebo group (p=0·70). No serious adverse event was reported in the riluzole-treated group whereas four patients in placebo group had a serious adverse event (hepatic enzyme increase, fracture of external malleolus, rectorrhagia, and depression). The number of patients with adverse events was similar in both groups (riluzole 16 [73%] patients vs placebo 19 [83%] patients; p=0·49). We were able to recruit 45 patients moderately affected by spinocerebellar ataxia type 2 for this trial. Riluzole did not improve clinical or radiological outcomes in these patients. However, our findings provide data on progression of spinocerebellar ataxia type 2 that might prove to be valuable for the design of other clinical trials. French Ministry of Health.

Surgical treatment of anterolateral malleolar pain combined with subfibular ossicles by ankle arthroscopy

To evaluates the clinical outcome of the ankle arthroscopic resection of subfibula ossicles combined with modified Broström technique for pain on the external malleolus caused by subfibular ossicles in 15 patients from April 2014 to January 2019 treated in the Tianjin First Central Hospital. The patients included 6 males and 9 females, aged from 24 to 56 years, and the symptoms lasted for a mean of 7.8 months(1.0-27.0 months) before the operation. With a 12-month follow-up, the visual analog scale (VAS) pain score, American Orthopedic Foot and Ankle Society (AOFAS) score and Karlsson-Peterson (KP) score were used to assess the clinical outcome. The VAS pain score reduced from 5.5±1.3 to 1.6±1.1, the AOFAS score increased from 68.0±9.0 to 86.5±7.1, and the KP score improved from 65.2±14.0 to 86.9±8.1 after the operation, respectively; and all the three assessment scores improved significantly (all P<0.05). No cases presented postoperative complications such as non-union of the incision, skin necrosis, and infection, etc. This method is an ideal cure for pain on the external malleolus caused by subfibular ossicles, which has the advantages of less trauma and quick recovery.

Variación de la baricidad en la mezcla de anestesia espinal para cesárea, ¿tiene algún efecto en la calidad anestésica? Estudio aleatorizado, doble ciego, controlado

IntroductionThis study was conducted to compare the effectiveness subarachnoid anaesthesia with bupivacaine plus fentanyl at different concentrations of dextrose for caesarean section. MethodsA double-blind, randomised study, which included 60 patients, aged between 18 and 40 years, ASA I-II for caesarean section class 3 or 4, divided into 3 groups, designated A, B, and C corresponding to dextrose 2, 4 and 6%, respectively, bupivacaine 6.25 mg plus fentanyl 25 μg. Demographic characteristics were quantified, episodes of hypotension and bradycardia onset and duration of sensory block and motor block, surgeon and maternal satisfaction, adverse events and the effect of anaesthesia on the foetus and neonate. A p < .05 was considered statistically significant. ResultsThere was no difference in the demographic data, the three-anaesthetic mixture with dextrose were sufficient to provide level and duration of anesthesia for surgery. Maternal hypotension events were lower in group C (p < 0.05), the same group had lower external malleolus deep sensitivity (p < 0.05), only 5 cases were consistent with neonatal asphyxia, however the Apgar scores values were greater than 7 points, with satisfactory development. ConclusionsThe three anaesthetic mixtures for subarachnoid anaesthesia with bupivacaine-fentanyl are clinically effective; however, the greater the amount of dextrose used the better block quality.

Bone Marrow Mononuclear Cells (BM-MNCs) and Human Autologous Bone Marrow Plasma Concentrate (HA-BMPC) for diabetic foot ulcer refractoratory to conventional treatment. A case report

Background & Aim An ulcer is any open lesion on the skin or mucosa with loss of substance. Stem cells derived from bone marrow, with recognized plasticity and proliferative capacity, are determined to repair the function of injured tissues. Diseases of the arteries, arterioles and capillaries experienced a cause of death at all ages. Precisely, the development obtained in regenerative cell therapy has allowed treating conditions as well as diseases associated with the skin. Methods, Results & Conclusion This is an original case report from our Institute for Regenerative Medicine in Argentina where a point of care procedure started with the extraction of 110 ml of bone marrow was performed by puncture in the left posterior iliac crest under live fluoroscopy by using 5 ml of local anesthesia in a sterile procedure room.The bone marrow obtained was preserved in a sterile blood collection bag next to 2,500 IU of sodium heparin diluted in 0.1 ml every 10 ml of marrow. It was transported under sterile conditions in the laboratory of advanced therapies, where reception was received by a certified doctor in Regenerative Medicine and where cell separation was performed by density gradient of Ficoll Paque Premium and direct centrifugation through the GE Healthcare protocol under system ThermoFisher Scientific laminar flow in positive pressure sealed room. A cell count dose of 900,000,000 Bone Marrow Mononuclear Cells (BMMNCs) diluted with human autologous bone marrow plasma concentrate (H-BMPC) in a concentration of 15 ml was obtained, with a 99% viability. On the same day, asepsis of the ulcer site to be treated (4 × 6 cm) was performed, placement of sterile fields, resection of fibrotic edges through the use of a scalpel and the application of the cells in 15 injections of 1 ml with needle 30G, without local anesthesia, covering the entire surface of the ulcerative zone of the left external malleolus. After the application, the compressive bandage was performed for 5 days in a row, and it was then indicated that the ulcer was washed every 24 hours with the replacement of the compressive bandage until its closure. No type of antibiotic therapy was indicated. Complete closure of the left lower limb ulcer was observed after 110 days of the stem cell implantation. The use of bone marrow Mononuclear Cells in an autologous way is a safe application therapy, with scientific evidence both pre and clinical worldwide, and could be effective for the treatment of closure of diabetic ulcers of lower limbs.

Distal radius T-plate for 18 patients with vertical shear medial malleolus fractures

To explore clinical effects of distal radius T-plates in treating vertical shear medial malleolus fractures. From March 2014 and March 2016, clinical data of 18 patients with vertical shear medial malleolus fractures were retrospectively analyzed, including 12 males and 6 females aged from 22 to 63 years old with an average of (41.3±5.2) years old; 6 patients were on the left side and 12 patients were on the right side; 5 patients combined with external malleolus fractures and 13 patients combined with external malleolus and posterior malleolus fractures. All patients were treated with distal radius T-plate fixation. Fracture healing time, loss of reduction, stability of internal fixation, occurrence of osteoarthritis were observed, postoperative AOFAS score at 12 months was used to evaluate clinical effects. All patients were followed up from 18 to 36 months with an average of (22.5±4.3) months. All incisions healed well at stageⅠ. Review of X-ray showed that ankle joints were got anatomically reset. All fractures healed well ranged from 12 to 18 weeks with an average of (13.4±2.4) weeks. After surgery, patients resumed normal walking from 12 to 17 weeks with an average of (14.5±1.3) weeks. No complications such as loss of reduction, loosening or rupture of internal fixation, nonunion of fracture, radiographic appearance of osteoarthritis occurred during following up. AOFAS scores was 92.4 ±6.7 at 12 months after operation, and 15 patients got excellent result, 3 moderate. Distal radius T-plates for treatment of vertical shear medial malleolus fractures have advantages of firm fixation, conforming to biomechanical requirements, better matching with plate anatomy, and less soft tissue stimulation. It could achieve early function exercise, obtain good recovery of function, and it is an ideal choice for the treatment of vertical shear medial malleolus fractures.

Chondromyxoid fibroma: unusual location

Chondromyxoid fibroma is a rare bone tumor with cartilaginous differentiation which presents less than 1% of bone tumors. It is a benign lesion and occurs in the fibula in less than 10% of the cases. A 25-year-old with no medical history, right-hander, and long-distance running athlete who consulted in our orthopedic department for right ankle pain. This symptom dates back to 6 weeks and considerably hampered his professional sports activities. This mechanical ankle pain is next to the external malleolus without irradiation and subsides with usual analgesics. The clinical examination was poor. There was no swelling. Bony landmarks were painless. Motion range was normal. The neurovascular exam did not find any abnormalities. X-rays showed a rounded and well-defined lytic bone lesion located in the external malleolus and classified Lodwick Ia (A,B). Due to the benign aspect, we decided to perform an excisional biopsy with a cancellous autograft. The gesture was performed by a lateral approach under loco-regional anesthesia. The graft was taken from the proximal tibial epiphysis through a small internal incision. The patient resumed his training after 2 months, and returned to his professional sports level after 3 months. At the last follow-up of 6 months, the patient had not reported any symptoms and the radiological control was satisfying (C,D).

Ankle joint dislocation treating dislocated trimalleolar fractures accompanied with the complex posterior malleolus fracture without separation of the tibiofibular syndesmosis.

To explore the therapy of ankle joint dislocation treating dislocated trimalleolar fractures accompanied with the complex posterior malleolus fracture without separation of the tibiofibular syndesmosis and improve surgical results.Patients who had dislocated trimalleolar fractures accompanied with the complex posterior malleolus fracture without separation of the tibiofibular syndesmosis were retrospectively analyzed and 30 patients were enrolled the study. They were all treated by ankle joint dislocation and the surgical results were evaluated by the Baird–Jackson ankle scoring system. Longitudinal curved incision in medial malleolus was made in ankle joint dislocation and subluxation was automatically formed by appropriate traction of ankle joint. The talus and the distal end of internal and external malleolus were pushed the outside to form the lateral dislocation of the ankle joint. After fully revealed the posterior malleolus and distal articular surface of the tibia, the anatomical reduction of comminuted bones with joint cartilage and posterior malleolus was achieved by fixed with absorbable screw or Kirschner wire. Internal and external malleolus fracture was fixed by the conventional approach.The average follow-up period was 13 months. According to the Baird–Jackson ankle scoring system, the excellent and good result was 28 cases, fair was 2 cases which the excellent and good rate was 93.3% without talar necrosis in any cases.Ankle joint dislocation can achieve the satisfactory results in treating dislocated trimalleolar fractures accompanied with the complex posterior malleolus fracture without separation of the tibiofibular syndesmosis. Ankle joint dislocation can make joint cartilage restore accurately with excellent results.

Distance Between the Malleoli and the GroundA New Clinical Method to Measure Leg-Length Discrepancy.

The aim of this work is to introduce a useful method for the clinical diagnosis of leg-length inequality: distance between the malleoli and the ground (DMG). A transversal observational study was performed on 17 patients with leg-length discrepancy. Leg-length inequality was determined with different clinical methods: with a tape measure in a supine position from the anterior superior iliac spine (ASIS) to the internal and external malleoli, as the difference between the iliac crests when standing (pelvimeter), and as asymmetry between ASISs (PALpation Meter [PALM]; A&D Medical Products Healthcare, San Jose, California). The Foot Posture Index (FPI) and the navicular drop test were also used. The DMG with Perthes rule (perpendicular to the foot when standing), the distance between the internal malleolus and the ground (DIMG), and the distance between the external malleolus and the ground were designed by the authors. The DIMG is directly related to the traditional ASIS-external malleolus measurement (P = .003), the FPI (P = .010), and the navicular drop test (P < .001). There are statistically significant differences between measurement of leg-length inequality with a tape measure, in supine decubitus, from the ASIS to the internal malleolus, and from the ASIS to the external malleolus. This new method (the DMG) is useful for diagnosing leg-length discrepancy and is related to the ASIS-external malleolus measurement. The DIMG is significantly inversely proportional to the degree of pronation according to the FPI. Conversely, determination of leg-length discrepancy with a tape measure from the ASIS to the malleoli cannot be performed interchangeably at the level of the internal or external malleolus.

Nodular lymphangitis caused by Mycobacterium marinum

Abstract A five-year-old boy was presented to a local walk-in clinic with an infection in the area of the external malleolus of the right foot, for which amoxicillin was prescribed. Upon completion of the course of the antibiotic, the inflammation in the primary area of the infection subsided but the multiple sites of nodular lymphangitis in the surrounding area persisted. Examination of those sites lead to the identification of Mycobacterium marinum as the cause of the coinfection.

Posterior Arthroscopic Subtalar Arthrodesis.

Although subtalar joint arthrodesis may be achieved through open or arthroscopic approaches, we have found that posterior arthroscopic subtalar arthrodesis has technical advantages compared with other approaches. Clinically assess the foot and ankle with regard to remaining motion, previous scarring, and associated deformities, ensuring that no concomitant procedure will be needed in the supine position. Use spinal or general anesthesia. Place the posterolateral portal lateral to the Achilles tendon at the level of the tip of the lateral malleolus, according to the original technique described by van Dijk et al. Place the posteromedial portal medial to the Achilles tendon at the level of the tip of the lateral malleolus. With the help of the shaver, look for the landmarks of the posterior arthroscopy, going from the posterolateral part of the subtalar joint toward the flexor hallucis longus tendon, which is medially located, while exposing the tibiotalar joint. Sometimes an accessory portal, just anterior to the tip of the external malleolus, may be needed to achieve a distraction of the anterior part of the subtalar posterior facet. To perform this concomitant step, which generally is not necessary, use an arthroscopic forceps to insert a 5 by 20-mm autograft or allograft in the subtalar joint. Use two cannulated screws with a 7.3-mm diameter; we prefer stabilization screws to compression screws. Always clean out the posterior compartment of the ankle during the PASTA procedure, as is done during surgical treatment for posterior impingement syndrome, and note that both posterior impingement syndrome and subtalar degenerative changes may thereby be easily addressed through this procedure. A short posterior leg splint is worn for three to ten days, followed by another non-weight-bearing cast for another three weeks. Since the appearance of the original article, a total of forty-one unilateral subtalar joints in twenty-five men and sixteen women, seen between May 2007 and December 2012, with isolated subtalar arthritis or talocalcaneal coalition without any other major hindfoot arthritis were treated by posterior arthroscopic subtalar arthrodesis, and all subtalar joints except for two were considered radiographically fused at the first attempt after an average of 6.7 weeks (range, six to ten weeks).IndicationsContraindicationsPitfalls & Challenges.

Subtalar dislocation: management and prognosis for an uncommon orthopaedic condition.

Subtalar dislocations were first described in 1811 as the simultaneous dislocation of the talo-calcaneal and talo-navicular joints without any tibio-talar or talar neck associated fractures. They were classified in 1853 as: medial, lateral, posterior and anterior based on the displacement of the foot in relationship to the talus. These are uncommon injuries, representing approximately 1% of all traumatic injuries of the foot and 1-2% of all dislocations, being associated with high energy trauma.Closed reduction of these dislocations should be performed as early as possible to avoid further damage to the skin and neurovascular structures. If this is not possible, then open reduction without further delay is recommended. Irreducible injuries have been reported in 0 to 47% of cases. Open dislocations represent between 46 and 83% of all cases, and have been associated with poor prognosis. Associated fractures have a high incidence, the most frequent ones are the posterior process of the talus, talar head, external malleolus, medial malleolus and the tubercle of the fifth metatarsal.These types of injuries are not faced by orthopaedic surgeons on a daily basis and having a source of information on how to manage and what to expect is important. We present an up-to-date literature review on the epidemiology, clinical presentation, radiologic assessment, treatment options and prognostic factors of these uncommon injuries.

PO-0214 Complicated Kingella Kingae Osteoarthritis

Introduction Kingella kingae is increasingly being recognised as a common aetiology of osteoarticular infections, especially in younger children. Infections are usually mild with a favourable outcome. However, unusually complicated cases have been rarely reported. Case Report Three children with complicated K. kingae osteoarticular infection were identified. The age varied between 2 to 3 years-old and the diagnostic delay was 12 ± 6 days. All had lower limb claudication, low grade fever and normal-to-mild elevated acute-phase reactants. Radiologic exams showed unifocal bone lytic lesions compatible with abscess (astragalus, calcaneus and external malleolus). Cultures of bone exudate or joint fluid identified Kingella kingae . Osseous lytic lesions were drained in all cases and joint fluid was aspirated in one. Antibiotherapy with cephalosporins or amoxicillin-clavulanate was done: intravenous for 8 ± 2 days, followed by oral treatment for 26 ± 5 days (total duration 34 ± 6 days). There was complete clinical recovery at 3 month, with disappearance of the osteolytic lesions in two patients. Conclusions In these cases several characteristics of Kingella kingae infections are identified, as young age, mild clinical presentation, normal-to-mild elevated inflammatory markers, single focus and favourable response to antibiotics. Although K. kingae bone lytic lesions are rarely identified they could led to important sequels and should be vigorously treated.

Papillon-Lefèvre Syndrome: A Series of Six Cases in the Same Family

Papillon-Lefèvre syndrome (PLS) is a rare, autosomal recessive heterogeneous disorder, which is characterized by palmoplantar hyperkeratosis, early loss of primary and permanent teeth, and associated calcification of the dura mater. Herein we described six cases of PLS in the same family. In this series, six cases (two females and four males) with the mean age of 15.6 ± 10.4 years were recruited. Palmoplantar hyperkeratosis was detected in all of the cases, leading to a difficult and painful walking in two cases due to lesions on the soles. Skin lesions were sharply distinct from adjacent normal skin in all cases. Other skin lesions were located in the external malleolus (5/6), knee (4/6), elbow (4/6), toe and dorsal fingers (3/6), and the thighs (2/6). In three cases, all permanent teeth were exfoliated. In three others, no primary teeth remained. Severe gingivitis was observed in three patients. Radiologic study confirmed alveolar bone destruction in five cases. Delayed diagnosis and insufficient treatment of PLS patients can affect patient's life of by causing edentulism at a young age and may impose PLS patients to increased risk of social, psychological, and economical burdens.

A System Utilizing Metal Hydride Actuators to Achieve Passive Motion of Toe Joints for Prevention of Pressure Ulcers: A Pilot Study

This paper describes the influence of human toe movement on blood flow and the design of a toe joint passive motion system for preventing pressure ulcers. First, we measured lower extremity blood flow in the foot during active and passive motion of the toe to facilitate the design of new rehabilitation equipment. Also, the flexion and extension angles and the force of the toe joints were measured to determine appropriate specifications for the system. Increases in blood flow were observed at the external malleolus during movement. Flexion and extension angles and the force of the toe joints were found to differ significantly among participants. It is shown that a toe joint passive motion system can be effective in preventing pressure ulcers. On the basis of these results, a device using alloys of metal hydride (MH) as an actuator that is suitable for the system to initiate toe motion, was developed.

P3.30 Normative values for intraepidermal nerve fibers in a Portuguese population

Skin biopsy as a complementary in the diagnosis of small fiber neuropathy, has came to be increasingly used by different researchers. There is a need to establish normative standards that allow the comparison of biopsies performed in patients with biopsies performed in healthy individuals in the Portuguese population. Presentation of data collected in the counts of intraepidermal nerve fibers in skin biopsies from healthy volunteers without pathology, with descriptive statistics (mean and standard deviation). We performed two skin biopsies 3 mm in diameter by Punch biopsy of the lower limb. A distal, 10 cm above the external malleolus, and one proximal to 20 cm from the iliac crest. The material is fixed in paraformaldehyde, cryoprotected with 20% glycerol and cryopreserved at −80 °C. Are made serial sections of 50 μ and is made immunohistochemical staining with antibody to PGP 9.5 counts posterior fibers of the skin. We studied 19 individuals, 6 men and 13 women. For the proximal biopsies, the average is 30 fibers, 3 mm with a standard deviation of 9.55 fibers 3 mm distal to the biopsies, the average is 24.5 fibers 3 mm with a standard deviation of 10, 6 fibers, 3 mm; There is a lower concentration of fibers in biopsies distal compared with proximal biopsies. The results obtained allow to establish a normative database for the Portuguese population, though limited by the availability of volunteers. These data are important in the diagnosis of small fiber neuropathies.