Secondary (Late) Developmental Dysplasia of the Hip with Displacement: From Case Studies to a Proposition for a Modified Diagnostic Path.

(1) Background. This paper presents a case of hip joints that were initially described as either normal or physiologically immature in four successive ultrasound examinations using the static method by Graf; however, the final treatment of the patient involved multiple hip reconstruction surgeries. (2) Case presentation. The patient was born with an Apgar score of 10 and did not exhibit neurological diseases that could deform and lead to pathological dislocation of the right hip joint. The subsequent analysis of medical data revealed that the hip luxation was due to secondary (late) developmental dysplasia of the right hip. (3) Conclusion. The article emphasizes the importance of early diagnosis and treatment standards for developmental dysplasia of the hip (DDH). The development of uniform international medical guidelines for the diagnosis, treatment, and prevention of hip dysplasia, along with the unification of DDH-related terminology, would allow for more effective management of DDH cases and reduce the cost of patient treatment.

Problems, Complications, and Factors Predisposing to Failure of Fassier-Duval Rodding in Children With Osteogenesis Imperfecta: A Double-center Study.

BACKGROUND: Although Fassier-Duval (FD) rods have been used for almost 2 decades, knowledge of factors predisposing to their failure is limited. Thus, the purpose of this study was to: (1) present the most common complications of FD rodding, (2) present tips on how to avoid or overcome them, and (3) identify factors predisposing to treatment failure. METHODS: Fifty-eight rod segments in 19 patients with osteogenesis imperfecta (mainly type III) underwent analysis with a median follow-up (FU) time of 4.4 years. We assessed the total number of complications clinically and radiographically. Next, the possible predisposing factors leading to failure were assessed using the Mann-Whitney U test. In addition, we evaluated the cutoff age for the increased rate of complications using the Youden index. A P<0.05 was considered significant. RESULTS: The total number of complications reached 44.8%. The most common complications included: migration of the male or female implant (45.7% and 25.7% out of the total number of complications, respectively), bone fracture with bending of the rod (8.6%), and rotational deformities (8.6%). Significant differences in patients' ages at the time of surgery were found between the group with and without complications (P=0.04), while sex, segment treated, preceding surgeries, length of FU, FD rod diameter, and length of bisphosphonate treatment were not significant. The Youden index showed that the risk of complications rose significantly in patients treated when younger than 5.5 years of age (P<0.05). CONCLUSIONS: This series displays the effectiveness and utility of FD rods at a median FU of over 4 years. Complication rates were comparable with the existing literature, with a notable increase in the number of side effects observed in younger patients (below the age of 5). LEVEL OF EVIDENCE: Level IV-therapeutic study.

Limitations in Use of Elastic Stable Intramedullary Nailing (ESIN) in Children with Disorders of Bone Mineralization.

BACKGROUND: Elastic intramedullary nails (ESIN) have been the treatment of choice in many long bone fractures in children for more than 20 years. The introduction of ESIN has drastically reduced tissue traumatization during fracture fixation procedures and decreased the risk of growth cartilage damage, as well as allowing for preservation of the natural biology of closed fracture healing. The objective of the present report is to draw attention to a small group of patients with bone mineralization disorders, who consequently demonstrate decreased mechanical resistance of the skeletal system, in whom indications for using ESIN fixation are limited. MATERIAL AND METHODS: The study group consisted of 6 patients who met the criteria for using ESIN fixation, but did not demonstrate a satisfactory outcome. The inclusion criteria included age below 18 years, appropriate ESIN nail insertion technique with correct calculation of nail diameter (2/5 of the medullary canal diameter), body mass < 50 kg and achieving appropriate prebending that is the prerequisite of three-point intramedullary support. The patients' medical records and radiographs were analyzed and they were invited for clinical and radiological follow-up examinations. RESULTS: All six patients demonstrated a gradually increasing bending deformity of the long bone axis. Additionally, in three patients, there was intrusion of terminal parts of the nails into the bone. In one patient with bone fibrous dysplasia, the proximal epiphysis was perforated by the flattened ends of the nails. In all patients, the removal of the ESIN fixation was followed by single or double-level corrective osteotomies and Rush pin fixation. CONCLUSION: In cases of long bone fractures in children with metabolic bone disorders in whom the bone structure is weakened and the bones themselves are easily deformed, more rigid intramedullary fixation with Rush or Fassier-Duval type nails as primary osteosynthesis should be considered.

Severe Thoraco-lumbar Kyphoscoliosis Associated with Osteoporosis in Siblings - Case study.

The incidence of scoliosis among patients with certain systemic diseases is much higher than in the general population. Moreover, the onset of the scoliosis is in early childhood before the age of 5 and the deformity reaches extreme values. We present the clinical course of two siblings with multiple musculoskeletal deformities, osteoporosis, severe kyphoscolisis and an undiagnosed systemic disease. The onset of scoliosis was in the first months of life of both children, with a marked progression about the 8th month of life. Due to lower limb deformities, ambulation was delayed until the 5th year of life in the male sibling, and the girl remains non-ambulant. Both children had osteoporosis, which caused numerous fractures of the upper and lower limbs. Due to progression of the spinal deformity the boy underwent a posterior hemispondylodesis with instrumentation at the age of 7. The girl also underwent surgery at the age of 7, but instrumentation could not be placed successfully due to inadequate bone quality. The last follow-up to date has been at the age of 12 years for the female patient and 20 years for the male patient. The spinal deformity in the female has not progressed during the last 2-3 years. She has been on bisphosphonate therapy for two years and no new fractures have been noted. The male patient has undergone multiple surgeries for lower limb deformities and is an independent walker. His scoliosis remains stable, but a minor progression of kyphosis has been noted in the last year. The history of the two patients shows that not all early-onset deformities can be effectively treated and that osteoporosis is a crucial obstacle to this treatment.

Chronic recurrent multifocal osteomyelitis (CRMO) in children - single-centre clinical observations and experience.

BACKGROUND: CRMO is characterized by chronic bone pain of recurrent nature. The onset is usually at the beginning of the second decade of life. Bony lesions may be visible on radiographs, although sometimes a CT or MRI scan is necessary to visualise them. The aim of study was analyse symptoms, diagnostic difficulties and results of accessory investigations in patients suffering from CRMO. MATERIAL AND METHODS: Medical records of 42 patients with CRMO were analysed to assess: age at onset of the disease, symptoms reported, location of lesions and abnormalities in laboratory and imaging studies. RESULTS: The medical records of 42 children, including 16 boys (38%) and 26 girls (62%), were analysed. The median age at onset was 10.5 years. The first symptom was bone pain in 34 patients (81%), with the remaining 8 (19%) presenting with widened bone contour in the absence of pain. Initially, only 5 (12%) subjects had multiple foci, whereas the vast majority (88%) presented with a single lesion. During exacerbations, osteocalcin levels were significantly elevated, increasing to a mean of 34 ng/ml (max. 68, min.14.6) against a reference upper limit of 13.7 ng/ml. Diagnostic difficulties and complications were found in 18 children. CONCLUSIONS: 1. Familiarity with CRMO in orthopaedic clinical practice is indispensable in differential diagnosis with other bone diseases, including those suggestive of malignancy. 2. Correctly diagnosed CRMO is managed conservatively as a treatment of choice.

Dysplasia epiphysealis hemimelica - diagnostics and treatment in pediatric patients.

BACKGROUND: Dysplasia epiphysealis hemimelica, also known as Trevor-Fairbanks disease, is a rare developmental disorder The objective of this article is to present own observations and experience in treating patients with dysplasia epiphysealis hemimelica. MATERIAL AND METHODS: Six children with dysplasia epiphysealis hemimelica were treated in years 1990-2007. The mean age of observation was 8.5 years (from 3 to 19 years). Analysis of medical and radiological documentation of patients was performed to collect data on symptoms, disease location, management and outcomes. RESULTS: The main symptoms reported by patients included limited range of motion of the affected joints with pain (66%) and deformed joint outline (34%). Four patients were subjected to surgical treatment while conservative treatment was applied in the other two. Lated complications were observed in two patients after surgical intervention (50%). In patients undergoing conservative treatment, one positive outcome and one negative outcome involving complete hip ankylosis, were observed. CONCLUSIONS: Correct diagnosis is very important as it may save the patient from unnecessary surgery and, if the surgery is necessary, it may help in performing it correctly. In patients presenting with joint pains, joint deformations, and tuberous lesions in joints possibility of dysplasia epiphysealis hemimelica should be taken into account. The treatment should start with conservative treatment, particularly physical therapy applied in the region of pain. If pain, joint deformation or limited range of motion of the affected joint persist, surgical treatment consisting of complete excision of the lesion should be taken into account.

Diagnostic difficulties: chronic recurrent multifocal osteomyelitis and lymphoma. One or two diseases?

This paper presents a case report of a 15-year-old boy admitted to the hospital for persistent right knee pain and limping. The clinical appearance of his condition was the basis on which diagnosis of chronic recurrent multifocal osteomyelitis was made. Continuous deterioration in the clinical status also led to a suspicion of the presence of other pathological processes. A bone biopsy was performed and the results of histopathology showed diffused large cell B lymphoma. Chemotherapy was initiated; nevertheless, the patient's general state worsened gradually and subsequent pancytopenia resulted in septicemia and multiorgan failure that led to the patient's death. This paper reports the exceptional diagnostic challenges that emerged from the obscure course of the disease.

Stress fractures in children.

BACKGROUND: Presentation of own experiences, observations and results of treatment of patients with stress fractures treated in the Department of Paediatric Orthopaedics. The authors present an algorithm of diagnosing and treatment of stress fractures. MATERIAL AND METHODS: Over the period 1998 - 2010, 38 patients were hospitalised (40 fractures) with the final diagnosis of stress fracture. Personal patients' data, case histories and management preceding the diagnosis of the disease, diagnostic and therapeutic methods, as well as the results of treatment were collected on the basis of medical documentation. Standard diagnostic procedure consisted of: clinical and radiological examination, laboratory tests as well as computed tomography (CT). In 11 cases, the procedure included also scintigraphy and in two children MRI scan was performed. RESULTS: Complete healing of fractures was finally achieved in all patients CONCLUSIONS: In case of suspected stress fracture, it is necessary to perform a series of radiograms, which should present the picture of bone rebuilding and incorporation of the periosteal callus. If plain radiograph is inconclusive we should consider MRI , CT or scintigraphy scan. The results of laboratory tests should not show any abnormalities. In case of confirmed stress fracture nonweight-bearing of the affected extremity is recommended as well as introducing of close clinical-radiological monitoring. The results of MRI examination should be interpreted very cautiously, be cause it can be misleading. The observation strategy ("wait and see") should be implemented. However, the lack of the regression of pain sensation, bone rebuilding features and incorporation of periosteal callus or the appearance of visible bone destruction (lysis) should raise questions and present an indication for taking biopsy of the laesion.

[Ischial tuberosity fractures in children]. / Zlamania guza kulszowego u dzieci.

BACKGROUND: Ischial tuberosity fractures in children are a form of avulsion fractures caused by the strong thigh muscles of the back group (ischiotibial muscles). OBJECTIVE: Presentation of observations covering the diagnostic difficulties, treatment and follow-up of ischial tuberosity fractures in children. MATERIAL AND METHODS: 6 children (one girl and five boys), average age at the time of injury - 13.6 years (12-15.5 years). An analysis of medical and radiological documentation of patients. RESULTS: Two patients with chronic pain were suspected of ischial bone tumor, one was suspected of Perthes disease, and only 3 were sent to the Traumatology Department immediately after the football injury. All patients were treated conservatively. Complete healing of fractures was finally achieved in all patients - (fibrous union in two cases) and finally, after an average period of 9 months, the pain subsided. CONCLUSIONS: There is a discussion in medical literature about the difficulties in the diagnosis of ischial tuberosity fractures, which were primarily unrecognized. Radiological picture of significant bone rebuilding may suggest neoplastic lesions. In the literature dominates the attitude of conservative treatment. Only in cases of large displacement of fracture and chronic ailments caused by pressure on the sciatic nerve, surgery should be considered.

[Intervertebral disc calcification in children]. / Zwapnienia krazka miedzykregowego u dzieci.

THE AIM: Evaluation of children with intervertebral disc calcification (IDC) and own experience. MATERIALS AND METHODS: A retrospective analysis of 7 patients (2 girls and 5 boys) with detected, in the years 1990-2009, IDC. Age at the onset of symptoms (neck pain in 6 patients, torticollis in 4, hip pain in one) was on average 8.7 years (5-13 years). The diagnosis was based on review of radiographs of the spine, which revealed the presence of calcifications within the intervertebral discs in the cervical (4 patients), cervical and thoracic (2 patients) and thoracic spine (1 patient). The mean level of IDC on average was 3.3 spaces (1-6). RESULTS: After conservative treatment, including use of nonsteroidal anti-inflammatory drugs and rest, the symptoms subsided within 1-2 weeks. 4 patients had a return of pain in the neck, in one year, but the symptoms were milder and resolved within a few days. The mean observation period was 9 years (3.5-16 years). In 4 patients, changes disappeared completely, while in the remaining three there was a very discrete calcifications. One patient, after 6 years, had a pain in the neck, and CT showed minor lytic changes on the surface of the vertebral bodies between which calcification occurred. DISCUSSION: s IDC is a rare disease of the spine in children, but should be taken into account in cases of vertebral pain. Usually the disease affects children before the age of 10 and locates in the cervical spine. But it can be located in every segment of the spine. Most patients have multilevel location. Acute phase of the disease requires conservative treatment. Rarely used surgical treatment should be considered only in patients with persistant neurological symptoms. The natural course of the disease is mild, and over the years calcification gradually disappear spontaneously.

Severe neonatal spondylometaphyseal dysplasia in two siblings.

We report on two siblings with a severe neonatal form of spondylometaphyseal dysplasia (SMD). Similar cases have been reported in four publications. Analysis of pedigree data from the original and present families suggests an autosomal recessive mode of inheritance, although parental gonadal mosaicism is also possible. The similarities in the phenotype between our patients and spondyloepimetaphyseal dysplasia congenita (SEMDC) and spondyloepimetaphyseal dysplasia Strudwick (SEMDS) type, indicated that these patients could have a defect in the COL2A1 gene. Molecular analysis of genomic DNA of these patients excluded this gene. Another potential candidate gene PTHR1, was also analyzed in the selected regions and no mutation was found. This gene is probably causative in the Jansen type of SMD, which shares some phenotypic features with the siblings whom we documented. Our results indicate that a new candidate gene for the reported form of SMD should be sought.

[Orthopaedic aspects of joint's pain and limping in children]. / Przyczyny ortopedyczne bólów stawowych i utykania u dzieci.

In this review article autor is discussing the ortopaedic causes of joint pain and limping in children. The paper presents orthopaedic diseases typical for the child's age. The attention was put on the possibilities of rare diseases including tumors and tumorlike conditions of bone which implies on the noncharacteristic symptoms.

Spondyloepimetaphyseal dysplasia with joint laxity, leptodactylic or Hall type: report of a case with normal face and literature review.

We have documented the clinical and radiological features of a girl with a severe form of spondyloepimetaphyseal dysplasia with joint laxity, leptodactylic or Hall type, which is associated with marked articular hypermobility. This condition is to be differentiated clinically from generalized hypermobility syndromes specifically Ehlers-Danlos syndrome and Larsen syndrome. The radiographic differential diagnosis is with the group of spondyloepimetaphyseal dysplasias specifically spondyloepimetaphyseal dysplasia with joint laxity and sponastrime dysplasia.

A variant or a "new" postaxial acrofacial dysostosis syndrome.

In this paper, we report a boy with remarkable phenotype and noteworthy radiographic abnormalities. He presented with distinctive facies, mesomelic shortening and asymmetry of the extremities, symmetrical ulnar and fibular ray absence in the hands and feet, with unique hypoplastic/dysplastic radiographic abnormalities. His mental development was normal. We believe that he represents a previously unreported form of the postaxial acrofacial dysostosis syndrome.

[Reconstructions of the teratogenic hip dislocation in childrens with AMC]. / Rekonstrukcja teratologicznego zwichniecia stawu biodrowego u dzieci w przebiegu Arthrogryposis multiplex congenita.

UNLABELLED: Hip diclocation is the cause of the mobility impairment in children with arthrogryposis. Hip joint reconstruction is a commonly accepted treatment method in unilateral dislocations, but opinions are divided in bilateral. AIM OF THE STUDY: Hip joint function assessment after reconstruction due to teratogenic dislocation in arthrogryposis. MATERIAL AND METHODS: In years 1988 to 2005 we performed 9 reconstructions of the teratogenic hip dislocation in 8 children with AMC (Arthrogryposis multiplex congenita). Mean age of children at the time of procedure was 3.5 years. Mean follow-up was 9.5 years. At final outcome the movement and the mobility were assessed according to modified Harris scale, as well as influence of other factor on ambulatory status. Radiological result was assessed according to Severin classification. RESULTS: Mean ROM in hip joint after operation decreased in all children comparing to preoperative assessment in all planes, except for adduction. In all ROM of the hip remained functional. At the final check up there was mobility improvement in 7 patients. The knee flexion contracture ipsilateral to the dislocated hip was stated only in 1 patient. There was no isolated relation between ambulatory status and hip and knee extensor muscles strength, residual deformities of the lower extremities and involvement of the upper extremities. There was no relation between radiological result and mobility. CONCLUSIONS: Decrease of hip ROM after the reconstruction in patients with AMC does not influence the mobility. The factor that influence that mobility ought to be considered altogether. Hip dislocation in patients with AMC may prevent occurrence of the knee flexion contracture deformity.

[The treatment of the hip cotructure with intertrochanteric osteotomy of the femur in children with Arthrogryposis multiplex congenita]. / Osteotomia miedzykretarzowa kosci udowej w leczeniu przykurczu stawu biodrowego u dzieci w przebiegu Arthrogryposis multiplex congenita.

BACKGROUND: The flexion contractures of the hips affect mobility in patients with Arthrogryposis Multiplex Congenita (AMC). There are only a few reports concerning correction of those deformities with femoral intertrochanteric osteotomy. AIM: The results' analysis of fixed hip flexion deformity correction with femoral intertrochanteric osteotomy in AMC patients. MATERIAL: 16 femoral intertrochanteric osteotomies were performed for hip contracture between 1992 and 2002 in 7 AMC patients. Mean follow up period was 6.5 years postoperatively. The hip contractures between 20 and 110 degrees making mobility impossible or difficult fulfilled the criteria for surgery. In 7 cases knee flexion deformity correction was performed simultaneously with the hip procedure. At the final check up, the hip ROM, the rate of deformity recurrence and patients' mobility were assessed. RESULTS: The mobility was affected in all children preoperatively. The range of deformity correction during the procedure was average 53 degrees. In 10 cases the improvement of mobility status was estimated postoperatively. The deformity recurrence occurred in 13 cases, in 4 of them required procedure to be repeated. The age in the recurrence group was lower and the contractures measured preoperatively more severe in comparison to the non recurrence group. CONCLUSIONS: Femoral intertrochanteric osteotomy tends to be efficient method of treatment of hip flexion contracture in children with AMC. Deformity correction has been achieved in all patients. The mobility status improvement is noted in majority of the patients, despite high rate of deformity recurrence postoperatively.

[Femoral head resection in dislocated hip in cerebral palsy children]. / Resekcja glowy kosci udowej w zastarzalym zwichnieciu stawu biodrowego u dzieci z mózgowym porazeniem dzieciecym.

The purpose of this study is to assess the outcome of proximal femoral resection in dislocated hip in cerebral palsy patients. Between 1993-2003 we treated 5 nonambulatory patients with quadriplegic form of cerebral palsy. We performed 6 resection of the proximal part of the femur. The average age at the surgery was 15.8 years (9-19.5 years) and average follow-up was 69 months (12-144 months). The indication to surgery was persistent hip pain. The results were good--the pain relieved, the movement in ,,the hip" was better and hygiene improved. Femoral head resection is radical, invasive method but we recommended it for selected group of severly spastic nonambulatory cerebral palsy patients with painful hip dislocation.

[Osteogenesis imperfecta--operative treatment on lower extremities in children with osteogenesis imperfecta]. / Osteogenesis imperfecta--leczenie operacyjne deformacji konczyn dolnych u dzieci z wrodzona lamliwoscia kosci.

The group of 141 children with osteogenesis imperfecta was treated in Orthopaedic Department of the University Children Hospital in Krakow, Poland. In 77 (54.6%) children from this group, we operated on lower extremities. Prophylactic operations, that were intramedullary Rush rodding, we performed in 19 cases (14 femurs and 11 tibias). Sofield-Millar procedures we performed in 58 children. We operated 321 times - there are 4 operations on average in one child. Average follow-up period was 6.7 years. We operated 473 long bones: 234 femurs and 239 tibias. We did 479 osteotomies. First operations were done at the age of 9 years on average (1.5-21 years). Further operations, 3 in each patient on average, we performed in period 37 months from one to another on tibias and 49 months on femurs. In all operated children we achieved full axis correction and their activity after operation improved. In order to assess that, we used the Bleck scale. In general, before operation, 54 (70%) children did not walk, and, in contrast, after operations 53 (69%) started walking. Operative treatment of the lower extremities in children with osteogenesis imperfecta improves their clinical physical abilities, quality of life and allows increase in activities.

[Operative management of long-bone of the upper limb in children with osteogenesis imperfecta]. / Osteogenesis imperfecta--leczenie operacyjne konczyn górnych.

The authors present their own experiences in operative treatment of the upper extremities in 24 children with osteogenesis imperfecta (oi) among 141 treated in years 1988-2002, in whom 34 operations were performed. In one subgroup were children presented with bone fractures: olecranon six, humerus shaft five, lateral condyle of the humerus one, humerus supracondylar fracture one. In second subgroup included children with upper limb deformities following procedures were performed: correction of cubitus varus three, humerus osteoclasis two, dislocated radial head resection, forearm correction thirteen. Results after operative management of fractures were very satisfying for all the children--proper fracture healing, axis of the bones was straight and a full mobility of the joints was achieved. In second group: partial deformation recurrence after cubitus varus correction, good result on the one side and lack of correction an the other after humerus osteoclasis; bad result in terms of elbow mobility after radial head resection; good results (despite complicated healing and wires migration) in terms of axis correction after forearm bones osteotomies. Surgery of the upper limbs in osteogenesis imperfecta is very challenging but it is worth to perform, as it improves function.