Bilateral slipped capital femoral epiphysis in a male adolescent with familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC), chronic renal failure, and severe hyperparathyroidism.

UNLABELLED: Slipped capital femoral epiphysis (SCFE) is the most common orthopedic hip disorder affecting otherwise healthy adolescents. The majority of SCFE cases are classified as idiopathic; rarely, it may be secondary to different endocrinopathies including hyperparathyroidism due to chronic renal failure (CRF). However, over the last decades, the association between SCFE and CRF has almost disappeared, probably due to better management of renal osteodystrophy. Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC, OMIM no. 248250) is a rare autosomal recessive tubulopathy characterized by renal wasting of calcium and magnesium leading to hypomagnesemia, hypercalciuria, nephrocalcinosis, and CRF. Patients usually show hyperparathyroidism before the onset of advanced CRF caused by FHHNC-related metabolic disturbances. We report on a 15-year-old patient with FHHNC and CRF who developed extreme hyperparathyroidism and high-grade bilateral SCFE after self-discontinuation of supportive treatment of underlying conditions. CONCLUSION: We believe that SCFE was caused not only by untreated CRF but also by metabolic disturbances related to FHHNC. To prevent this complication, careful management of disturbances of calcium, phosphate, and magnesium homeostasis seems to be crucial.

Clinical experience with the use of low molecular weight heparin in orthopaedic treatment of paediatric patients.

BACKGROUND: The objective of the paper is to describe our clinical experience with the use of low molecular weight heparin in children treated due to various orthopaedic conditions. MATERIAL AND METHODS: We have studied the treatment outcomes of 35 patients aged 11 to 18 years who were hospitalized in the Department of Paediatric Orthopaedics and Department of Traumatology and Emergency Medicine of the Medical University of Lublin and were treated with a low molecular weight heparin (LMWH). Short-term prophylaxis was applied in 33 patients during immobilization after such orthopaedic procedures as surgical correction of spine deformity and corrective osteotomy of the femoral and tibial bone. Two children received LMWH therapy after reduction and fixation of femoral bone fractures and long-term immobilization following ankle joint distortion with early-stage venous thromboembolism (VTE). RESULTS: None of the patients who received prophylactic treatment showed the clinical manifestations of thrombosis. The VTE symptoms subsided in children treated with the LMWH. Nadroparin was administered for both short-term prophylaxis and treatment, and was administered in doses proportionate to body weight and without prior determination of anti-factor Xa activity (anti-Xa assay). No side effects were observed. CONCLUSIONS: The use of the LMWH turned out to be effective and safe in thromboprophylaxis in paediatric patients.

Change of situation of a family with a child treated due to scoliosis.

INTRODUCTION AND OBJECTIVE: Scoliosis is a serious clinical problem which requires a systematic physical therapy and control of body balance - treatment from the moment of achieving skeletal maturity by a child. In the situation of neglect of such a management, the deformation of the spine often requires surgical intervention. The role of parents in the process of treatment of a child is undeniable. The study concerns the determination of socio-economic conditions and the engagement of parents with children treated due to scoliosis in Eastern Poland. MATERIAL AND METHODS: The study was conducted by means of a diagnostic survey. The study group consisted of 193 parents (148 females [76.7%] and 45 males [23.3%]) - a randomly selected sample of the parents of children who participated in scoliosis rehabilitation courses in rehabilitation centres in Eastern Poland. The significance of the relationships between variables was investigated by means of chi-square test for independence. The differences between the empirical and theoretical sample distribution was examined by means of chi-square goodness-of-fit test. The significance level was set at p=0.05. RESULTS: The study group covered 47.7% of inhabitants of rural areas and small towns, and 52.3% of inhabitants of medium-size and large cities. Respondents possessing university education provided their children a wider profile of health care; however, they neither supervised exercises at home nor paid attention to the maintenance of the normal body posture. The diagnosis of scoliosis in a child rarely affects the relationship between parents. The engagement of parents into rehabilitation treatment of their children is small. CONCLUSIONS: There is a relationship between social variables and engagement in the treatment of a child with scoliosis. There is a need to create a system of education for parents concerning scoliosis and the consequences of its negligence. The enrolment of a psychologist into the treatment team should be considered, which would provide support for the parents of children suffering from scoliosis.

Problems and complications in VEPTR-based treatment.

AIM OF THE STUDY: Early-onset scoliosis with co-existing thoracic deformity is particularly difficult to treat, with early surgery usually being the only rational solution. Operative correction of the deformed spine and rib cage employs techniques enabling further growth of the structures, an example of which is the Vertical Expandable Prosthetic Titanium Rib (VEPTR), designed to provide dynamic stabilization of the spinal column and the thoracic cage. Our study aimed to present difficulties and complications related to VEPTR-based operative reduction of the spinal and thoracic deformities in the youngest child. MATERIAL AND METHODS: Twelve children aged 3 to 9 years (a mean age of 5.25 years) underwent sequential VEPTR-based surgery the Department of Paediatric Orthopaedics of the Medical University of Lublin. The subjects were qualified for the procedure on account of multiple congenital spine deformities (3 children), early-onset progressive neuromuscular scoliosis (4 children) and kyphosis secondary to a myelomeningocele (5 children). The number of procedures per child ranged from 4 to 10 and the follow-up was 10 months to 4 years (with a mean of 2.5 years). Treatment failures and complications comprised rib fractures (4 cases), perforation of the iliac ala by the ala hook (5 cases), pulmonary oedema (2 cases), hip joint destabilization (1 case), tissue reaction to the metal (4 cases), breakage of the implant (2 cases). RESULTS AND CONCLUSIONS: The specific character of the method makes it liable to a variety of difficulties and complications that may sometimes affect the outcome. Both the authors' experience and published data prove the clinical effectiveness of VEPTR. Nevertheless, it is only a temporary means that cannot disturb the strategic anatomical elements of the spine which are to be involved in subsequent conventional operative procedures. An awareness of the limitations and possible difficulties connected with VEPTR allows for some modifications with which the technique continues to be the most effective sequential treatment of thoracic and spinal deformities in the youngest child.

Enhancing bone healing during distraction osteogenesis with platelet-rich plasma.

UNLABELLED: Gradual limb lengthening with external fixators using distraction osteogenesis principles is the gold standard for treatment of limb-length discrepancy. However, long treatment time is a major disadvantage of the current lengthening procedures. Efforts to decrease the treatment include biological and biomechanical factors. Injection of platelet-rich plasma (PRP) is a biological method to enhance bone healing during distraction osteogenesis. We hypothesised that PRP can enhance bone healing during limb lengthening. We report our experience with the use of PRP during distraction osteogenesis. This retrospective study included 19 patients divided into the standard group of 10 patients who did not receive PRP and the PRP group of nine patients who received PRP at the end of the distraction phase. The study variables included external fixator time, external fixation index, and complications during treatment. The PRP group had statistically significantly shorter treatment time (p=0.0412). Injection of PRP into regenerate bone might be an effective method to shorten treatment time during limb lengthening and lead to better functional outcomes and improved patient satisfaction. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Diastematomyelia - a diagnostic and therapeutic problem. Case study.

BACKGROUND: Diastematomyelia is a type of dysraphism with a double or bifid spinal cord divided by an osseous septum. This defect often co-occurs with other developmental disorders of the skull or the atlanto-occipital junction. The course may be benign or aggressive. 2. CASE REPORTS: We describe two female patients treated in the Rehabilitation Clinic and the Orthopaedic Department at the Medical University of Lublin in the years 2004 - 2009. The first patient was diagnosed at the age of 20 years to have diastematomyelia at the L3 level and spina bifida occulta at L1- S5 and at the C1 arch. In the other patient, diastematomyelia at the L3 level and spondylolisthesis at L5-S1 were found at the age of 14 years. Initially both patients were treated for lumbosacral radicular syndromes. Physiotherapy intensified the pain. The patient with diastematomyelia and L5-S1 spondylolisthesis had L5-S1 segment stabilization performed at the age of 16. The pain subsided after the surgery. The other patient was instructed to stop rehabilitation, follow a balanced lifestyle, and refrain from physical work, which eliminated the pain. DISCUSSION: Managing a patient with diastematomyelia demands caution. Diagnosis of this defect requires a thorough cause-and-effect analysis of the presenting signs and symptoms of spinal dysfunction. The treatment should be dependent on local pain intensity (which is often not directly associated with the disorder) and on the degree of neurological dysfunction. 5. CONCLUSIONS: 1. A thorough clinical evaluation with spinal imaging prior to elective surgery for scoliosis and other spine deformities should be a standard procedure undertaken in order to avoid complications. 2. The treatment for diastematomyelia should depend on the intensity of local pain and on the level of neurological dysfunction. 3. Broadly understood rehabilitation is not always effective, often increasing the pain and/or neurological complaints.

[The attempt of identification of the essentials risk factors of venous thromboembolism after hip arthroplasty despite of pharmacological prophylaxis]. / Próba identyfikacji istotnych czynników ryzyka zylnej choroby zakrzepowo-zatorowej, wystepujacej po endoprotezoplastyce stawu biodrowego, mimo stosowania profilaktyki farmakologicznej.

Hip arthroplasty is of particular great risk of venous thromboembolism (VTE). The objective of this study was to identify the risk factors of VTE and the analysis of their influence on VTE appear. There were 213 patients after hip arthroplasty in the investigation group. VTE occurred in 15 patients (7.04%).We found Deep Venous Thrombosis (DVT) in eight patients (3.75%) and Pulmonary Embolism (PE) in seven (3.3%). The analysis showed statistically real dependence on patient age, obesity, veins varicoses of lower extremity and VTE occur. We also showed the significant influence of the time of the leg ultraposition time during the surgery on the frequency of VTE episodes, which hasn't been described jet in accessible literature. It is the only risk factor which may be limited by simply modification of surgery technique.

The vertical expandable prosthetic titanium rib (VEPTR) in the treatment of scoliosis and thoracic deformities. Preliminary report.

BACKGROUND: Severe deformities of the chest, early-onset progressive scoliosis, congenital defects of the ribs and the vertebral column may all give rise to the thoracic insufficiency syndrome (TIS), when the chest capacity is too low to fully support basic vital functions, leading to gradually progressive cardiopulmonary failure. Aim of study. This paper presents new possibilities for sequential correction of progressive deformities of the thorax and spinal column in skeletally immature children using a vertical expandable prosthetic titanium rib (VEPTR) system. MATERIAL AND METHODS: At the Department of Pediatric Orthopedics of the Medical University in Lublin, three children were treated surgically with VEPTR, a low-profile modular system allowing simultaneous correction of scoliosis and chest deformities. Two children (aged 8 and 9 years) required treatment for multiple congenital spinal deformities, while one (aged 7) had early progressive neuromuscular scoliosis. DISCUSSION: Existing surgical approaches based on the Harrington method do not prevent further progression of chest deformity. The ongoing sequential VEPTR treatment of our patients resulted in significant correction of thoracic and spinal deformities already in the first phase of the treatment, with considerably improved respiratory capacity. CONCLUSION: The vertical expandable prosthetic titanium rib appears to be the best alternative to other approaches to sequential treatment of chest and spine deformities currently in use.

[Congenital disorders of hemostasis in children with Perthes disease]. / Wrodzone zaburzenia ukladu hemostazy u dzieci z choroba Perthesa.

The level of selected parameters of the coagulation system and fibrynolysis (prothrombin time, partial thromboplastin time, fibrinogen level, albumin C system, V Leiden factor and III antithrombin level) in 25 children who had been treated with Perthes disease was evaluated. In three children prolonged prothrombin time occurred; in one child anomalous protein C system was noted. The remaining parameters were normal in all children. The investigation results reveal that congenital disturbances of the haemostasis system were not the cause of Perthes disease in 24 children.

[Femur head necrosis in haemophilia and after prolonged steroid therapy--description of two cases]. / Jalowa martwica glowy kosci udowej w przebiegu hemofilii oraz po leczeniu hormonami kory nadnercza--opis 2 przypadków.

Clinical and radiological course of femoral head necrosis of known aetiology (in a boy, aged 8, with haemophilia and in a girl, aged 13, after prolonged steroid therapy) was compared with the typical course in children with Perthes disease.

Testing the differences in bioelectrical potentials occurring in distractive osteogenesis: Human and animal clinical research.

Background. Metal intraosseous implants cause disturbances in tissue bioelectrity.
Material and methods. Our research involved a group of 28 patients treated by distractive osteogenesis and 12 dogs treated for long bone fractures with external fixation.
Results. During the process of limb lengthening excessive electrical potentials were generated, considerably exceeding the physiological level.
Conclusions. The observed effect may have a negative effect on osteogenesis and remodeling of the regenerating bone. The application of an external fixator did not significantly increase electrical potentials in animals.

An attempt to normalize excessive differences in electric potentials between the metal external distractor and the limb.

Background. The metal element of an external stabilizer cause changes in the electrical potential of tissue. During limb lengthening, large differences in potential are generated between the apparatus and the limb. According to some authorities this phenomenon may have a negative impact on the rebuilding of bone.
Material and methods. In 10 children undergoing limb lengthening by Ilizarov distractor, we applied an original device to eliminate undesirable electrical potentials produced by the introduction of metal distractor implants to tissues.
Results and Conclusions. It was found that the prototype "eliminator" effectively maintains the level of electrical charge within physiological limits.