Caudal Regression Syndrome-A Narrative Review: An Orthopedic Point of View.

Abnormalities in cellular differentiation during embryo-fetal period may lead to various malformations of the spine. Caudal regression syndrome (CRS) is a group of defects with premature growth/development termination of the vertebral column. CRS can be divided into three types: sirenomelia, complete absence of the sacrum and partial absence of the sacrum. Genitourinary and gastrointestinal anomalies are common, with neurogenic bladder and bowel incontinence. Treatment of patients with CRS is complex and multidisciplinary and should be comprehensive. The most common orthopedic problems are: spinal deformity (kyphosis and scoliosis), spinopelvic instability and lower limbs deformities.

Upper Limb after Forearm Lengthening in Patients' and Physicians' Perspective.

BACKGROUND: The goal of this study was to perform a functional (subjective) and radiological evaluation of patients who had undergone forearm lengthening by distraction osteogenesis years before. MATERIAL AND METHODS: Eleven patients with forearm shortening of various etiology were enrolled. They had undergone a total of 21 lengthening procedures. A retrospective analysis of radiological data was conducted and a subjective evaluation was accomplished by using a modified QuickDASH-9 questionnaire. RESULTS: Average bone lengthening was 3.54cm, and mean lengthening index was 40.53day/cm. Eight pa-tients answered the questionnaire at a mean of 7.8yrs after the treatment. The mean questionnaire score was 9.75pts (of 36). Four patients rated the overall function of the affected limb as improved following distraction, while 3 patients were not able to see any improvement. One patient reported that the lengthening had impaired limb function. With regard to cosmetic aspects, 4 patients reported a worsening after the lengthening procedure while 3 patients reported improvement and 1 patient did not note any changes. CONCLUSIONS: 1. Despite deformities and functional limitations, patients after forearm lengthening only occasionally suffered from moderate intensity pain. 2. The radiological outcomes were positive and the rate of complications was low. 3. The radiological outcomes did not match patient-declared functional and cosmetic results.

Intramedullary stabilization and over-nail lengthening as two-stage treatment of femoral nonunion with shortening. Case study.

Nonunion is one of the most serious complications of long bone fractures. It may be accompanied by a shortening of the segment. The authors describe the case of a 21-year-old woman with a post-traumatic nonunion with shortening of the femur. Treatment was divided into two stages: first, a previously placed nail was removed and new intramedullary stabilization was carried out while bone defects were filled with a bone graft substitute and platelet rich plasma was administered. After the nonunion had healed, the femur was lengthened over an external fixator and an intramedullary nail, resulting in equality of limb length. After eight years of follow-up, the lower limbs remain equal with a properly aligned long axis of the lower limb operated on and a full range of motion in the joints. The treatment strategy described in our article may be an alternative to one-stage surgery if the patient does not consent to it or in the presence of contraindications, but it is associated with a longer treatment time and necessity of additional surgeries.

Use of recombinant activated factor VII for reduction of perioperative blood loss during elective surgical correction of spine deformity in a Jehovah's Witness. Case report.

Planned surgical procedures at patients who refuse allogenic blood transfusion because of religious convictions are important problem, not only medical but also ethical and juristical. At the study authors report the successful use of activated recombinant factor VII (rFVIIa) for the reduction of perioperative blood loss in four years old child - Jehovah's Witness, who had planned Torode kyphectomy. Applied perioperative management together with preparing to surgery with erythropoietin allowed for reduction of blood loss and avoiding of blood transfusion. Authors state, that appropriate perioperative proceeding makes a possibility of safe surgical procedures also at patients who refuse the transfusion.

[Regeneration formation index--new method of quantitative evaluation of distraction osteogenesis]. / Wspólczynnik powstawania regeneratu--nowa metoda ilosciowej oceny przebiegu dystrakcyjnej osteogenezy.

INTRODUCTION: One of the most common techniques for limb lengthening is the Ilizarov method. The course of osteogenesis is usually monitored using classic X-ray in this way determining the moment of fixator removal. Classic x-ray evaluation is subjective and therefore errors may easily be encountered. New techniques for objective and quantitative evaluation of radiologic documentation are needed. Computer assisted image analysis offers this possibility. AIM OF PAPER: Aim of this prospective study is to determine a quantitative, measurable method of regenerate description during distraction osteogenesis, based on classic radiography and computer assisted image analysis. MATERIAL AND METHODS: Material consists of 40 patients, in whom long bone lengthening procedures were performed. Altogether 40 lengthening were performed, 16-femur, 20-tibia, 4-humeral. Radiographic data, obtained in standard conditions, were than analyzed digitally. A mathematical bone model was created and later compared with obtained regenerate images. Computer processing lead to determining a new value, called regeneration formation index (RFI). For the model bone, the value is one. RESULTS: Average femur lengthening was 5.4 cm, tibia lengthening was 5.6 cm, and humeral 8.0 cm. During distraction osteogenesis the RFI, at the end of distraction phase, and the beginning of stabilization phase reached the lowest values, to increase steadily with the progress of osteogenesis, finally at the end of stabilization phase reaching the original values. The pace of RFI increase correlated with the presence of potential complications that require surgical treatment. CONCLUSION: Quantitative evaluation of regenerate using the regeneration formation index allows to precisely analyze the course of distraction osteogenesis, particularly to precisely estimate the timing of fixator removal and avoid complications.

Results of simple excision technique in the surgical treatment of symptomatic accessory navicular bones.

INTRODUCTION: Accessory navicular bones might cause not only cosmetic problems but also be a reason of discomfort and pain. In case of inefficient conservative treatment symptomatic accessory naviculars are treated surgically. AIM OF PAPER: Presentation of results of simple excision of symptomatic accessory navicular. MATERIAL AND METHODS: Material consists of 22 patients (34 feet), 17 women and 5 men, treated surgically between 1992 and 2006. Mean age at surgery was 14.1 years (9-22 years). Accessory navicular type I was present in 5 feet (14.7%), type II in 17 (50%) and type III-in 12 (35.3%). Main symptom was localized pain on the medial arch of the foot, in the height of navicular bone. Surgery consisted of simple accessory navicular excision and if needed partial resection of navicular bone. The mean follow-up period was 5.6 years (1-13 years). We analyzed: intensity of pain (VAS score system), daily and sport activity. Subjective results were analyzed using a questionnaire. RESULTS: The questionnaire was returned from 21 patients: 9 patients had total pain relief, 11 considerable and one patient had persistent pain. Mean VAS results before surgery was 5.9 and 1.7 after surgery. Only one patient required analgesics occasionally. Complications were present in two patients (6.1%). All active patients returned to their sport activities. CONCLUSION: Surgical treatment of symptomatic accessory navicular by simple excision technique gives satisfying results, surgery is minimally traumatic and risk of complications low.

[Use of VEPTR (Vertebral Expandable Prosthetic Titanium Rib) device in the treatment of congenital spine and thorax deformities]. / Zastosowanie systemu implantów VEPTR (Vertebral Expandable Prosthetic Titanium Rib) w leczeniu wrodzonych deformacji kregoslupa i klatki piersiowej.

UNLABELLED: Congenital spine and thorax deformities are an interdisciplinary clinical problem. Apart from trunk deformity they may lead to respiratory or cardiovascular insufficiency. Surgical treatment should be implimented as soon as possible in order to improve posture, balance and further development. This treatment should not impair further growth of the young spine. This is possible with the VEPTR device. AIM OF PAPER: Aim of paper is presentation of initial results of surgical treatment of congenital spine deformities with the VEPTR system. MATERIAL, METHODS: We treated 3 patients, aged 5 to 14. All had severe congenital spine and thorax deformities. The VEPTR device was implanted in the following configurations: rib-rib in two patients and spine-rib in one patient. We evaluated: Cobb angle of the main curve, spine balance, respiratory function before and after surgical treatment. Followup was 12 months. RESULTS: Posture and balance of the spine improved in all patients. Curve correction was from 10% to 71%. In one patient with initial respiratory insufficiency symptoms subsided gradually. CONCLUSION: VEPTR device is indicated in treatment of severe congenital deformities of the spine and thoracic cage. It improves patients' posture, changes the shape of thorax wall and consecutively improves respiratory function and further development.

[Results of femoral lengthening over an intramedullary nail and external fixator]. / Wyniki wydluzania uda na gwozdziu sródszpikowym i stabilizatorze zewnetrznym.

INTRODUCTION: Current techniques of operative limb lengthening usually are based on distraction osteogenesis. One of the techniques is limb lengthening over an intramedullary nail. AIM OF PAPER: The goal of this study is to evaluate the results of femoral lengthening over an intramedullary nail. MATERIAL: Between 1999 and 200619 femoral "over nail" lengthenings were performed. There were 7 males and 12 females. Mean patients' age at surgery was 15.8 years, and mean initial femoral shortening was 5.1 cm. Operative technique consisted of one-stage implantation of intramedullary nail and external fixator. Ilizarov apparatus was used in 9 patients, monolateral fixator in 10 cases--ORTHOFIX in 9 patients, Wagner fixator--in 1 patient. Intramedullary nail was locked proximally with screws or Schanz pins from external fixator. After distraction phase, external fixator was removed and distal locking screws were applied. METHODS: Evaluation criteria: obtained lengthening, time of external fixator, treatment time, healing index, external fixation index, range of motion in hip and knee joints and complications according to Paley. RESULTS: The mean lengthening was 4.6 cm, and mean distraction time was 66.6 days. Mean time of external fixation was 115.5 days, and external fixation index was 26.2 days for centimeter. Healing index was 36.9 days for centimeter. In cases with monolateral fixator, healing index did not differ with the whole group. During treatment 18 complications occurred, for a rate of 0.9 complication per segment. CONCLUSIONS: Lengthening over an intramedullary nail reduces the time of external fixator. Over nail femoral lengthening can prevent axis deviation following regenerate bending. Complication rate is similar to lengthenings with the classic Ilizarov technique. There are no differences in the treatment time in relation to the type of external fixator.

[Femur lengthening with a telescopic intramedullary nail ISKD--method presentation and early clinical results]. / Wydluzanie kosci udowej przy zastosowaniu gwozdzia sródszpikowego samorozprezalnego ISKD--opis metody, wstepne wyniki leczenia.

INTRODUCTION: Limb discrepancy over 4 cm is an indication for limb lengthening. Surgical techniques rely on osteotomy, external stabilisation and gradual distraction. The ISKD (Intramedullary Skeletal Kinetic Distractor) nail allows distraction osteogenesis and no external fixator is required. AIM OF PAPER: Aims of paper are: presentation of limb elongation method with the ISKD device, and presentation of early own results. MATERIAL AND METHODS: Material consists of 5 patients, age 14-16 years, 3 boys and 2 girls, who underwent femur lengthening with the ISKD nail between 2005 and 2007. We evaluated: initial shortening, surgical procedure, complications, amount of lengthening, lengthening rate, distraction index, time of treatment and mobility of adjacent joints. RESULTS: Initial shortening was 4-11 cm. No surgical complications were observed, mean time of surgery was 145 minutes, mean blood loss--200 ml. In three patients difficulties with initial distraction required manipulations under general anaesthesia. Distraction was complicated in 3 cases--in two patients premature consolidation was noted; in one case the distraction rate was too high. Mean lengthening rate in the study group was 0.7 mm/day (0.6-0.7 mm/day). Mean distraction index was 41.7 days/cm (26.2-55 days/cm). Full weight bearing was allowed after mean 234 days (210-275 days). Transient decrease of adjacent joint mobility was observed. CONCLUSION: The fully implantable, telescopic ISKD eliminates the need of external fixation and associated complications. Early results of limb lengthening with ISKD are encouraging. Careful patient selection and preoperative planning is required. Further studies and longer follow-up periods are needed.

Lengthening of congenital forearm stumps.

The Ilizarov device and distraction osteogenesis method became very useful in correction and elongation of forearm defects. Two cases of forearm elongation with congenital transverse defect are described. The construction of the device is provided. During follow-up examination, 2 and 7 years after the treatment, good clinical results were achieved in both patients with the use of upper limb prosthesis employing the patient's own elbow joint. Presented application of the Ilizarov method can significantly improve possibilities for the use of prosthesis in patients with congenital upper limb defects and result in better cosmetic and functional outcome.

Limb lengthening in fibular hemimelia type II: can it be an alternative to amputation?

The purpose of our study was to analyze limb lengthening in fibular hemimelia type II. Ten patients underwent 16 tibia lengthenings. The mean tibia shortening was 5.8 cm. We used the Ilizarov technique in all cases. The mean follow-up time was 7.2 years. The mean lengthening was 23% of the former length. The healing index was 50.8 days/cm. In the final examination six patients were skeletally mature, equal limb length and functional foot positioning were achieved in four of them. Complications were observed during 14 lengthenings (87.5%). Although lengthening in fibular hemimelia is difficult, elongation with axis and foot correction may offer an alternative to amputation.

[Methods for the evaluation of bone regeneration during distraction osteogenesis]. / Metody oceny regeneratu kostnego powstajacego w czasie osteogenezy dystrakcyjnej.

During limb lengthening gradual reconstruction of bone tissue in distraction gap requires constant, systematic control and evaluation. This process is depicted descriptively and subjectively. Changes happening during osteogenesis are dynamic, they can stop, slow down or accelerate, and regenerate may be of various shape and form. In this work different methods of bone regenerate evaluation are presented. They may be divided on two basic groups. First group contains methods with X-radiation, and the second - without X-rays. Examinations that evaluate arising bone regenerate may be also divided on descriptive examinations and quantitative. Despite that different methods are used for distraction osteogenesis course estimation, classic radiological examination remains the basic one. The rest of them are most often supplementary examinations, useful in selected stages of treatment, for e.g. ultrasonography in first stage of regenerate arising. So it seems, that future is in quantitative regenerate evaluation using radiographs. Classic X-ray picture made in reproducible and repeatable conditions may be subjected to digital, quantitative image analysis, and acquired results enables for more precise and more balanced evaluation of new bone formation in distraction osteogenesis.

Slipped capital femoral epiphysis in identical twins - a case report.

Background. The pathoetiology of slipped capital femoral epiphysis (SCFE) has not been thoroughly established. Hormonal and genetic causes are the primary theories under discussion. There are some publications considering the occurrence of SCFE in identical twins. Material and methods. 15-year-old identical twins were admitted to our Department complaining of pain in the left groin and limping. A-P and axial x-rays presented slippage of the left capital femoral epiphysis. Results. Reposition and Kirschner pin fixation were performed in both patients with good clinical outcome. The follow-up period was 4 years. Conclusions. When SCFE occurs in one of a pair of identical twins it is prudent to take the second child under careful observation.

[The value of methods of bone regeneration evaluation in limb lengthening by the Wagner, Ilizarov methods and by physeal distraction]. / Wartosc metod oceny regeneracji kostnej w wydluzaniu konczyn metoda Wagnera, Ilizarowa i dystrakcji nasadowej.

Limb lengthening is a long-lasting process, and during new bone formation different complications may occur. Due to this, early diagnosis of disturbances of new bone formation leading to such complications is of importance. The goal of this study is to analyze already used methods of regenerate evaluation. Material consists of retrospective data of 237 patients, who underwent limb lengthening between 1983 and 2002 by one of three methods: Wagner method, Ilizarow method and physeal distraction. During femoral lengthening by Wagner method appropriate shape of regenerate according to Hamanishi was observed in 9 cases (29.0%), and during tibia lengthening--only in 1 case (6.7%). During femoral lengthening by physeal distraction appropriate shape of regenerate (A or B according to Hamanishi) was observed in 24 cases (77.4%), and during tibia lengthening--in 11 cases (78.6%). During femoral lengthening by Ilizarow method appropriate shape of regenerate was observed in 51 cases (72.9%), and during tibia lengthening--in 46 cases (66.7%). Only in Wagner method a correlation between abnormal regenerate shape and bone consolidation complications was noted. Methods of evaluation of bone regeneration during distraction osteogenesis give only descriptive assessment. So far parameters applied for evaluation of distraction osteogenesis in Ilizarow method and physeal distraction do not allow for detailed assessment of bone regeneration process.

Comparison of radiological results of conservative and operative treatment by Salter osteotomy in severe cases of Perthes' disease.

Background. The goal of treatment in Perthes' disease is the creation of best conditions for good hip reconstruction. The aim of this work was the comparison of radiological results of conservative and operative treatment by Salter osteotomy in Perthes' disease. Material and methods. 193 hips with severe Perthes' disease were analyzed (Catterall group III, IV), 105 hips were treated conservatively, 88 - were operated. Minimal follow-up time was 5 years. Following data were evaluated: Wiberg angle, acetabular angle, acetabulum-head index, acetabular and epiphyseal index. Additionally, head sphericity according to Mose, results estimation according to Stulberg and presence of arthritic changes were evaluated. Results. In conservative treatment group, Mose good result was achieved in 25 hips (23,8%), fair result - in 54 (51,4%), and poor result - in 26 (24,8%). According to Stulberg classification, class I was in 25 hips (23,8%), class II - 37 (35,2%), class III - 28 (26,7%), class IV - 9 (8,6%), class V - 6 (5,7%). In operative treatment group, Mose good result was achieved in 45 hips (51,2%), fair result - in 19 (21,6%), and poor result - in 24 (27,2%). According to Stulberg classification, class I was in 39 hips (44,3%), class II - 26 (29,5%), class III - 13 14,7%), class IV - 8 (9,1%), class V - 2 (2,3%). Radiological parameters of femoral head coverage were better after operative treatment. The amount of arthritic changes after conservative treatment were greater than after surgery. Conclusions. 1. Operative treatment by Salter osteotomy creates better conditions for spherical femoral head reconstruction. 2. Patients with III and IV Catterall group should be treated surgically.

Salter osteotomy in Perthes' disease. Late radiological results.

Background. Surgical treatment in Perthes' disease has been used for many years, but the choice of operation method still remains controversial. The aim of this work is to analyze long-term radiological results at patients after Perthes' disease treated by Salter osteotomy. Material and methods. 113 hips were analyzed at 110 patients. Males were 90, females 20. Due to severity of changes in hips, patients were divided into groups according to Catterall: group II - 25 hips (22.1%), group III - 14 hips (12.4%), group IV - 74 hips (65.5%). Average patient's age at the time of surgery was 7.2 years. Follow-up time was 9 years (5-14years). Salter osteotomy was performed as described by its author. The material was analyzed according to age of disease onset, extensiveness of necrosis (Catterall classification), and the length of follow-up period. Radiographs taken before surgery and during last examination were evaluated. For results estimation, Mose criteria and Stulberg classification were applied. Results. According to Mose criteria, 63 hips (55.3%) had good result, 25 (21.9%) - fair and 25 hips (21.9%) - bad result. Using Stulberg classification, group I included 55 hips (49.4%), group II - 33 hips (29.1%), group III - 14 hips (12.3%), group IV - 8 hips (7.8%), group V - 2 hips (1.5%). Radiological parameters of head coverage improved after surgery. Arthrotic changes were noted in 8 hips (7.8%). Conclusion. Salter osteotomy assures good head coverage by the acetabulum and creates favorable conditions for spherical head reconstruction. Results after Salter osteotomy are permanent and do not depend on the length of follow-up period.

Long-term results of conservative treatment in Perthes' disease.

Background. Legg-Calvé-Perthes disease, due to its frequency and impact on hip function, is an important orthopedic issue. The goal of this research was to assess long-term outcome after conservative treatment in Perthes' disease. Material and methods. We studied 123 patients (135 hips), 105 males and 18 females, average follow-up 19.5 years (range 10-34), average age at onset 6.4 years. Conservative treatment involved non-weight bearing, abduction casts, orthoses and rehabilitation. The average treatment duration was 2.9 years. The range of hip motion, pelvic stability, limb length, pain, limping and physical activity were estimated. The patients were classified according to Catterall's criteria and age of onset. In x-rays the risk factors, Wiberg angle, and acetabulum-head index were measured. Outcome was assessed according to Mose and Stulberg, and arthritic changes were noted. Results. At follow-up there was full physical activity in 50 cases (40.7%). Limping was observed in 38 patients (30%), limb shortening in 27 (22%). The range of motion was limited in 37 hips (27%), and 13 hips were unstable (9.6%). There was a good Mose outcome in 36 hips (26.7%), fair in 70 (51.8%), and poor in 29 (21.5%). By Stulberg's classification, 35 hips were in class I (25.9%), 49 in class II (36.3%), 31 in class III (23%), 11 in class IV (8.1%), and 9 in class V (6.7%). Arthrotic changes were noted in 51 hips (37.8%). Conclusions. Twenty years after onset only 25% of the evaluated hips were normal. This suggests low effectiveness of treatment.

[Spontaneous anterior atlanto-axial subluxation--a case story]. / Samoistne przednie podwichniecie szczytowo-obrotowe--opis przypadku.

In this study, a case of 4.5 years' old girl with spontaneous dislocation of C1/C2 vertebrae probably due to congenital defect of C1-hypoplasia of the atlas anterior arch is presented. Treatment consisted of skull traction and slow reposition followed by surgical spinodesis C1 and C2. During examination after 1 year follow up period, patient was in good clinical condition, without any complaints. On radiograms the cervical spine was stable and of correct shape.

Indications and range of surgery in the treatment of spine metastatic tumours.

Background. Spine is the most common place of metastatic tumors in the skeletal system. Due to diagnostic problems and the risk of quickly increasing neurological defects, the treatment of metastatic spine tumors is a significant clinical problem. The goal of surgical treatment is decreasing pain, neurological improvement and achieving full spinal stability.
Material and methods. Material consists of 31 patients with metastases in spine, who underwent spine surgery. Tumors were placed in thoracic spine at 48% patients, at lumbar spine - 42% patients, and at cervical spine in 10% patients; one level was involved in 56% cases, two or more levels - in 44 % cases. Qualification for surgery contained: the type of primary tumor, the amount of metastases to the spine impairment of spinal biomechanics and overall patient's condition of the. Corporectomy with anterior stabilization (intervertebral cage) was performed at 13 patients, and with additional anterior implants at 2 cases. At 8 patients, with destabilization of posterior spinal column, in spite of corporectomy, posterior stabilization was done. Surgery from posterior approach was performed in 3 cases.
Results. After operation, we noted pain relief at 34% of patients, while in 14% of them transient increase of pain occurred. Neurological status worsened after surgery at 1 patient. We achieved proper spine stabilization, without the need of use of external orthoses at 30 patients. At one patients, reoperation with change of implants and the range of stabilization was necessary. The amount of complications correlated with patient's general condition at the time of surgery.
Conclusions. 1. The main condition of success in operative treatment of spinal metastatic tumors is individual patient's qualification for surgery, including the extent of disease and general patient's condition. 2. Good stabilization with use of implants is a necessary element of surgery of spinal metastases from both anterior and posterior approach.

Surgical treatment of cervical spine metastatic tumours - case report.

Background. Metastatic tumours to the cervical spine generate pathological fractures followed by spinal instability and are connected with significant risk of neurological defects.
The goal of this study was to asses the surgical treatment of 4 patients with metastatic tumours in the cervical spine.
Material and methods. Between 1999 and 2001 four patients underwent surgery due to metastatic tumours in cervical spine. Primary focus was following: breast - 2 patients, prostate - 1 patient, in one case primary tumor remained unknown. Spinal localization of tumors: axial dens, vertebral body and arch of C5, vertebral body of C6. Patients' age at the time of surgery ranged from 48 to 75 years. The severity of spinal cord lesion before treatment was estimated according to Frankel classification: type B - 1 patient, E - 3 patients. According to Harrington classification, we noted type IV in 3 cases and type V - in one case.
Tumour resection with anterior and posterior stabilization, with use of implants was performed at 2 cases, occipito-cervical stabilization was done at one case and tumor resection with anterior stabilization was done in one case.
Results. Patients' neurological status did not change after treatment. As a result of operative treatment, we achieved full spine stabilization in 3 cases. Destabilization of vertebral cage due to osteoporosis occurred in one case and reoperation with change of the implant was performed at the fifth day after primary surgery.
Conclusion. Performed operations enabled further specialistic oncologic and rehabilitation treatment.