Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 28
Filter
1.
Adv Ther ; 2024 May 15.
Article in English | MEDLINE | ID: mdl-38748332

ABSTRACT

Achondroplasia is a lifelong condition requiring lifelong management. There is consensus that infants and children with achondroplasia should be managed by a multidisciplinary team experienced in the condition. However, many people are lost to follow-up after the transition from paediatric to adult care, and there is no standardised approach for management in adults, despite the recent availability of international consensus guidelines. To address this, the European Achondroplasia Forum has developed a patient-held checklist to support adults with achondroplasia in managing their health. The checklist highlights key symptoms of spinal stenosis and obstructive sleep apnoea, both among the most frequent and potentially severe medical complications in adults with achondroplasia. The checklist acts as a framework to support individuals and their primary care provider in completing a routine review. General advice on issues such as blood pressure, pain, hearing, weight, adaptive aids, and psychosocial aspects are also included. The checklist provides key symptoms to be aware of, in addition to action points so that people can approach their primary care provider and be directed to the appropriate specialist, if needed. Additionally, the European Achondroplasia Forum offers some ideas on implementing the checklist during the transition from paediatric to adult care, thus ensuring the existing multidisciplinary team model in place during childhood can support in engaging individuals and empowering them to take responsibility for their own care as they move into adulthood.

2.
J Pediatr Orthop B ; 2024 Feb 19.
Article in English | MEDLINE | ID: mdl-38375856

ABSTRACT

The main objective of this study was to retrospectively evaluate and compare the outcomes and complications of displaced closed tibial fractures in children treated by CRC (closed reduction and casting), elastic stable intramedullary nailing (ESIN) or external fixation (EF). One hundred twenty-three consecutive children were treated for displaced closed tibia shaft fracture from July 2014 and January 2020 at two different institutions. Seventy-five of them met the inclusion criteria and were included in the study: 30 (40%) patients were treated with CRC, 33 (44%) with ESIN, and 12 with EF (16%). All clinical and radiographic outcomes and complications were registered and compared. The three groups did not differ with regard to gender, affected side, fracture site and associated fibula fracture. The age at the time of treatment in the CRC group was statistically lower than in ESIN and EF groups (8.43 ±â€…3.52 years vs. 10.39 ±â€…2.56 years vs. 11.08 ±â€…3.55 years, respectively). Immobilization time and time to partial and total weight bearing were significantly reduced in ESIN and EF groups compared to CRC group (P < 0.05). Overall, no statistically significant differences were found between the three groups regarding complication rate and clinical and radiographic outcomes between the three groups. However, in CRC group, 3 patients (10%) had secondary fracture displacement and underwent ESIN. Surgical treatment is not contraindicated in children with displaced tibia shaft fractures. EF and ESIN provide earlier mobilization and weight-bearing recovery than CRC. However, apart from that, nonoperative treatment was as efficacious as surgical treatment.

3.
Orphanet J Rare Dis ; 18(1): 219, 2023 07 27.
Article in English | MEDLINE | ID: mdl-37501185

ABSTRACT

Foramen magnum stenosis is a serious, and potentially life-threatening complication of achondroplasia. The foramen magnum is smaller in infants with achondroplasia, compared with the general population, and both restricted growth in the first 2 years and premature closure of skull plate synchondroses can contribute to narrowing. Narrowing of the foramen magnum can lead to compression of the brainstem and spinal cord, and result in sleep apnoea and sudden death. There is a lack of clarity in the literature on the timing of regular monitoring for foramen magnum stenosis, which assessments should be carried out and when regular screening should be ceased. The European Achondroplasia Forum (EAF) is a group of clinicians and patient advocates, representative of the achondroplasia community. Members of the EAF Steering Committee were invited to submit suggestions for guiding principles for the detection and management of foramen magnum stenosis, which were collated and discussed at an open workshop. Each principle was scrutinised for content and wording, and anonymous voting held to pass the principle and vote on the level of agreement. A total of six guiding principles were developed which incorporate routine clinical monitoring of infants and young children, timing of routine MRI screening, referral of suspected foramen magnum stenosis to a neurosurgeon, the combination of assessments to inform the decision to decompress the foramen magnum, joint decision making to proceed with decompression, and management of older children in whom previously undetected foramen magnum stenosis is identified. All principles achieved the ≥ 75% majority needed to pass (range 89-100%), with high levels of agreement (range 7.6-8.9). By developing guiding principles for the detection and management of foramen magnum stenosis, the EAF aim to enable infants and young children to receive optimal monitoring for this potentially life-threatening complication.


Subject(s)
Achondroplasia , Bone Diseases , Sleep Apnea Syndromes , Child , Infant , Humans , Adolescent , Child, Preschool , Foramen Magnum/surgery , Constriction, Pathologic/diagnosis , Constriction, Pathologic/complications , Achondroplasia/diagnosis , Achondroplasia/therapy , Achondroplasia/complications , Sleep Apnea Syndromes/diagnosis , Spinal Cord , Bone Diseases/complications
4.
J Pediatr Orthop B ; 32(1): 66-71, 2023 Jan 01.
Article in English | MEDLINE | ID: mdl-35635539

ABSTRACT

The role of proximal tibiofibular joint (TFJ) transfixion during Ilizarov tibial lengthening is still debated in the literature. The aim of this study was to show that TFJ transfixion can be avoided with no impact on patient outcome. All achondroplastic patients undergoing tibia lengthening from 1999 to 2018 were included. Patients were divided in two groups: group A (11 patients) submitted to proximal TFJ transfixion and group B (22 patients) not submitted to TFJ transfixion. The results showed a statistically significant difference in proximal fibular migration (PFM) between the two groups of patients, with a mean PFM of 9.2 mm (range, 1-20 mm) and of 21 mm (range, 2.7-76 mm) in group A and in group B, respectively. We can state that PFM up to 41 mm in our series did not affect joint function and stability. There are no statistically significant differences in the other radiological parameters. Analysis of functional scores (Knee Society, Tegner-Lhysolm, Association for the Study and Application of Methods of Ilizarov) did not reveal significant differences between the groups. The scores of the Achondroplasia Personal Life Experience Scale were also similar in the two groups, demonstrating a good quality of life. There are no differences between the two groups in terms of clinical-functional outcome of the knee, radiographic results, and quality of life. Transfixion of proximal TFJ may not be necessary, but it is important to avoid premature consolidation of the fibular osteotomy.


Subject(s)
Ilizarov Technique , Humans , Quality of Life
5.
Orphanet J Rare Dis ; 17(1): 318, 2022 08 20.
Article in English | MEDLINE | ID: mdl-35987833

ABSTRACT

BACKGROUND: Achondroplasia is a genetic condition that can cause complications across the lifespan. While complications in childhood are well documented, the natural history of achondroplasia in adults has, until recently, been relatively lacking, and little is known about the care they receive or how they access it. The European Achondroplasia Forum undertook two exploratory surveys, one for healthcare professionals (HCPs) and one for patient advocacy group (PAG) representatives, to gain an understanding of current practices of the transition process of individuals with achondroplasia from paediatric to adult services and how adults perceive their care. RESULTS: Most HCP respondents followed up more children than adults, and 8/15 responded that individuals did not transition to an adult multidisciplinary team (MDT) after paediatric care. Of 10 PAG respondents, none considered the experience of transition to adult services as good or very good and 50% considered it to be poor or very poor. A total of 64% (7/11) described the coordination of transition to adult services as "Not satisfactory" or "Poor". HCPs and PAG representatives largely agreed on the core specialists involved in adult care (orthopaedic surgeons, physiotherapists, rehabilitation specialists, rheumatologists, clinical geneticists). However, there was a discrepancy in the understanding of healthcare needs outside of this, with PAG representatives selecting neurosurgeons and genetic counsellors, while HCPs selected pulmonologists and obstetricians/gynaecologists. There was agreement between HCP and PAG respondents on the key barriers to effective care of adults with achondroplasia, with lack of an adult MDT, lack of interest from individuals in accessing care, and less experience in adult than paediatric MDTs ranking highly. CONCLUSIONS: This study indicates that the care and follow up of adults with achondroplasia is challenging. Individuals are often lost to, or decline, follow up as they leave paediatric care, and it is largely unknown how, where, and why adults with achondroplasia access care later in life. Lifelong, multidisciplinary specialist care led by an identified physician should be accessible to all individuals with achondroplasia. It is important to ensure barriers to optimal care are addressed to enable access to appropriate care for all individuals with achondroplasia.


Subject(s)
Achondroplasia , Achondroplasia/therapy , Adult , Child , Delivery of Health Care , Follow-Up Studies , Humans , Surveys and Questionnaires
6.
Orphanet J Rare Dis ; 17(1): 293, 2022 07 27.
Article in English | MEDLINE | ID: mdl-35897040

ABSTRACT

BACKGROUND: Achondroplasia is the most common form of skeletal dysplasia, with serious comorbidities and complications that may occur from early infancy to adulthood, requiring lifelong management from a multidisciplinary team expert in the condition The European Achondroplasia Forum guiding principles of management highlight the importance of accurate diagnosis and timely referral to a centre specialised in the management of achondroplasia to fully support individuals with achondroplasia and their families, and to appropriately plan management. The European Achondroplasia Forum undertook an exploratory audit of its Steering Committee to ascertain the current situation in Europe and to understand the potential barriers to timely diagnosis and referral. RESULTS: Diagnosis of achondroplasia was primarily confirmed prenatally (66.6%), at Day 0 (12.8%) or within one month after birth (12.8%). For suspected and confirmed cases of achondroplasia, a greater proportion were identified earlier in the prenatal period (87.1%) with fewer diagnoses at Day 0 (5.1%) or within the first month of life (2.6%). Referral to a specialist centre took place after birth (86.6%), predominantly within the first month, although there was a wide variety in the timepoint of referral between countries and in the time lapsed between suspicion or confirmed diagnosis of achondroplasia and referral to a specialist centre. CONCLUSIONS: The European Achondroplasia Forum guiding principles of management recommend diagnosis of achondroplasia as early as possible. If concerns are raised at routine ultrasound, second line investigation should be implemented so that the diagnosis can be reached as soon as possible for ongoing management. Clinical and radiological examination supported by molecular testing is the most effective way to confirm diagnosis of achondroplasia after birth. Referral to a centre specialised in achondroplasia care should be made as soon as possible on suspicion or confirmation of diagnosis. In countries or regions where there are no official skeletal dysplasia reference or specialist centres, priority should be given to their creation or recognition, together with incentives to improve the structure of the existing multidisciplinary team managing achondroplasia. The length of delay between diagnosis of achondroplasia and referral to a specialist centre warrants further research.


Subject(s)
Achondroplasia , Achondroplasia/complications , Achondroplasia/diagnosis , Adult , Europe , Female , Humans , Pregnancy , Prenatal Diagnosis , Referral and Consultation , Ultrasonography
7.
Hip Int ; 32(6): 813-819, 2022 Nov.
Article in English | MEDLINE | ID: mdl-33829904

ABSTRACT

BACKGROUND: The modified Dunn procedure (MDP) has risen enthusiasm in treating slipped capital femoral epiphyses (SCFE) due to the anatomic reduction and high patients' satisfaction rates at long-term follow-up. Main aim of this study is to compare clinical and radiographic outcomes of 2 cohorts with moderate to severe stable SCFE treated by MDP and in situ fixation. METHODS: Medical records were analysed to collect demographic data, comorbidities and time from slip to surgery. The collected postoperative data were: avascular necrosis (AVN); complications; progression of osteoarthritis and subsequent procedures. Southwick angles (SA), alpha angles and Klein line were measured on the preoperative x-rays, on the immediate postoperative period and at the latest follow-up. Outcomes scores were recorded by the following questionnaires: the Harris Hip Score, the Hip disability and Osteoarthritis Outcome Score, the Merle d'Aubigné and Postel score and the Western Ontario and McMaster Universities Arthritis Index. Kaplan-Meier survivorship curve was calculated. RESULTS: We compared 81 hips treated by MDP with 22 hips treated by in situ pinning (PS) for moderate/severe stable SCFE. No significant differences were found between the 2 groups in terms of age, BMI, comorbidities and preoperative slip angles. At the latest follow-up, postoperative anteroposterior mean slip angles were respectively 6.2 and 19.9° in MDP and PS group (p = 0.3). Slip angles in frog lateral view were 11° in the MDP group and 39.7° in the PS group (p = 0.2). MDP group achieved better correction angles on frog leg view (11° vs. 39.7°; p < 0.001). There was no statistically significant difference in the occurrence of AVN among both groups (19.7% MDP group vs. 31.8% PS group) (p = 0.2). CONCLUSIONS: The MDP in treating severe stable SCFE showed the best deformities corrections in conjunction with the highest functional scores at long-term follow-up and similar rates of osteonecrosis compared to in situ fixation.


Subject(s)
Arthroplasty, Replacement, Hip , Osteoarthritis , Slipped Capital Femoral Epiphyses , Humans , Slipped Capital Femoral Epiphyses/diagnostic imaging , Slipped Capital Femoral Epiphyses/surgery , Treatment Outcome , Radiography , Osteoarthritis/surgery , Retrospective Studies
8.
Nat Rev Endocrinol ; 18(3): 173-189, 2022 03.
Article in English | MEDLINE | ID: mdl-34837063

ABSTRACT

Achondroplasia, the most common skeletal dysplasia, is characterized by a variety of medical, functional and psychosocial challenges across the lifespan. The condition is caused by a common, recurring, gain-of-function mutation in FGFR3, the gene that encodes fibroblast growth factor receptor 3. This mutation leads to impaired endochondral ossification of the human skeleton. The clinical and radiographic hallmarks of achondroplasia make accurate diagnosis possible in most patients. However, marked variability exists in the clinical care pathways and protocols practised by clinicians who manage children and adults with this condition. A group of 55 international experts from 16 countries and 5 continents have developed consensus statements and recommendations that aim to capture the key challenges and optimal management of achondroplasia across each major life stage and sub-specialty area, using a modified Delphi process. The primary purpose of this first International Consensus Statement is to facilitate the improvement and standardization of care for children and adults with achondroplasia worldwide in order to optimize their clinical outcomes and quality of life.


Subject(s)
Achondroplasia , Quality of Life , Achondroplasia/diagnosis , Achondroplasia/genetics , Achondroplasia/therapy , Consensus , Humans , Mutation , Osteogenesis , Receptor, Fibroblast Growth Factor, Type 3/genetics
9.
Orphanet J Rare Dis ; 16(1): 333, 2021 07 31.
Article in English | MEDLINE | ID: mdl-34332609

ABSTRACT

Achondroplasia is the most common type of skeletal dysplasia, caused by a recurrent pathogenic variant in the fibroblast growth factor receptor 3 (FGFR3). The management of achondroplasia is multifaceted, requiring the involvement of multiple specialties across the life course. There are significant unmet needs associated with achondroplasia and substantial differences in different countries with regard to delivery of care. To address these challenges the European Achondroplasia Forum (EAF), a network of senior clinicians and orthopaedic surgeons from Europe and the Middle East representative of the achondroplasia clinical community, came together with the overall aim of improving patient outcomes. The EAF developed a consensus on guiding principles of management of achondroplasia to provide a basis for developing optimal care in Europe. All members of the EAF were invited to submit suggestions for guiding principles of management, which were consolidated and then discussed during a meeting in December 2020. The group voted anonymously on the inclusion of each principle, with the requirement of a 75% majority at the first vote to pass the principle. A vote on the level of agreement was then held. A total of six guiding principles were developed, which cover management over the lifetime of a person with achondroplasia. The principles centre on the lifelong management of achondroplasia by an experienced multidisciplinary team to anticipate and manage complications, support independence, and improve quality of life. There is focus on timely referral to a physician experienced in the management of achondroplasia on suspicion of the condition, shared decision making, the goals of management, access to adaptive measures to enable those with achondroplasia to access their environment, and the importance of ongoing monitoring throughout adolescence and adulthood. All principles achieved the 75% majority required for acceptance at the first vote (range 91-100%) and a high level of agreement (range 8.5-9.6). The guiding principles of management for achondroplasia provide all healthcare professionals, patient advocacy groups and policy makers involved in the management of achondroplasia with overarching considerations when developing health systems to support the management of achondroplasia.


Subject(s)
Achondroplasia , Quality of Life , Achondroplasia/therapy , Adolescent , Adult , Consensus , Europe , Humans , Longitudinal Studies , Receptor, Fibroblast Growth Factor, Type 3/genetics
10.
Ital J Pediatr ; 47(1): 179, 2021 Aug 28.
Article in English | MEDLINE | ID: mdl-34454557

ABSTRACT

BACKGROUND: Acute hematogenous osteomyelitis (AHOM) is an insidious infection of the bone that more frequently affects young males. The etiology, mainly bacterial, is often related to the patient's age, but it is frequently missed, owing to the low sensitivity of microbiological cultures. Thus, the evaluation of inflammatory biomarkers and imaging usually guide the diagnosis and follow-up of the infection. The antibiotic treatment of uncomplicated AHOM, on the other hand, heavily relies upon the clinician experience, given the current lack of national guidelines for the management of this infection. METHODS: A systematic review of the studies on the empirical treatment of uncomplicated AHOM in children published in English or Italian between January 1, 2009, and March 31, 2020, indexed on Pubmed or Embase search engines, was carried out. All guidelines and studies reporting on non-bacterial or complicated or post-traumatic osteomyelitis affecting newborns or children older than 18 years or with comorbidities were excluded from the review. All other works were included in this study. RESULTS: Out of 4576 articles, 53 were included in the study. Data on different topics was gathered and outlined: bone penetration of antibiotics; choice of intravenous antibiotic therapy according to the isolated or suspected pathogen; choice of oral antibiotic therapy; length of treatment and switch to oral therapy; surgical treatment. CONCLUSIONS: The therapeutic management of osteomyelitis is still object of controversy. This study reports the first Italian consensus on the management of uncomplicated AHOM in children of pediatric osteomyelitis, based on expert opinions and a vast literature review.


Subject(s)
Anti-Bacterial Agents/therapeutic use , Osteomyelitis/therapy , Child , Drainage , Drug Administration Schedule , Humans , Osteomyelitis/diagnosis , Pediatrics , Practice Guidelines as Topic
11.
Children (Basel) ; 8(7)2021 Jun 22.
Article in English | MEDLINE | ID: mdl-34206218

ABSTRACT

BACKGROUND: We aimed to investigate the variation of medical and surgical activities in pediatric orthopedics in Italy, during the year of the COVID-19 pandemic, in comparison with data from the previous two years. The differences among the first wave, phase 2 and second wave were also analyzed. METHODS: We conducted a retrospective multicenter study regarding the clinical and surgical activities in pediatric orthopedics during the pandemic and pre-pandemic period. The hospital databases of seven tertiary referral centers for pediatric orthopedics and traumatology were queried for events regarding pediatric orthopedic patients from 1 March 2018 to 28 February 2021. Surgical procedures were classified according to the "SITOP Priority Panel". An additional classification in "high-priority" and "low-priority" surgery was also applied. RESULTS: Overall, in 2020, we observed a significant drop in surgical volumes compared to the previous two years. The decrease was different across the different classes of priority, with "high-priority" surgery being less influenced. The decrease in emergency department visits was almost three-fold greater than the decrease in trauma surgery. During the second wave, a lower decline in surgical interventions and a noticeable resumption of "low-priority" surgery and outpatient visits were observed. CONCLUSION: Our study represents the first nationwide survey quantifying the impact of the COVID-19 pandemic on pediatric orthopedics and traumatology during the first and second wave.

13.
J Pediatr Orthop B ; 30(6): 535-539, 2021 Nov 01.
Article in English | MEDLINE | ID: mdl-32956283

ABSTRACT

Several mechanical factors have been related to slipped capital femoral epiphysis (SCFE). Main aim of this study is to investigate the acetabular coverage and acetabular version in unilateral SCFE hips in order to detect a potential pincer-type deformity as predisposing factor; second, we compared those measurements either to the contralateral, uninvolved hips either to a matched healthy control population. A total of 85 patients treated for unilateral SCFE were retrospectively reviewed. The lateral center-edge angle (LCEA) and the Tönnis angle were used to assess acetabular coverage, whereas acetabular retroversion was defined by positive prominent ischial spine (PIS), cross-over sign (COS) and posterior wall sign (PWS). Angles and signs of the affected hips were compared to the contralateral hips and to a matched cohort undergoing an abdominal/pelvic computed tomography for nonorthopedic-related diseases. Affected and unaffected hips of patients with unilateral SCFE had similar morphology in terms of LCEA 28.7° vs. 28° (P = 0.4), Tönnis angle 9° vs. 9° (P = 0.1) and retroversion signs with concomitant rate of PWS and COS 57.6% vs. 50.5% (P = 0.4), PIS 56.4% vs. 49.4% (P = 0.4). Matched healthy controls vs. the affected hips showed a lower LCEA (P < 0.001) and higher Tönnis angle (P < 0.001) in conjunction with a lower incidence of acetabular retroversion: PWS and COS 40% vs. 57.6% (P = 0.01), PIS 43% vs. 56.4% (P = 0.07). A significant retroversion and increased overcoverage were observed in SCFE patients compared to matched healthy controls. In unilateral SCFE, the involved and uninvolved hips showed a substantial symmetry.


Subject(s)
Slipped Capital Femoral Epiphyses , Acetabulum/diagnostic imaging , Cohort Studies , Hip Joint/diagnostic imaging , Humans , Retrospective Studies , Slipped Capital Femoral Epiphyses/diagnostic imaging , Slipped Capital Femoral Epiphyses/surgery
14.
Pathol Int ; 70(7): 452-457, 2020 Jul.
Article in English | MEDLINE | ID: mdl-32314513

ABSTRACT

We describe a unique case of skeletal and extraskeletal angiomatosis complicated by Kasabach-Merritt syndrome. The patient was a 3-year-old boy, who presented with involvement of both femurs and left tibia, as well as with soft tissue lesions of the left thigh. At birth, multiple hemangiomas of the soft tissues of the frontal and parietal scalp had been identified, together with a space-occupying lesion of the lung. Histologically, the skeletal and soft tissue lesions consisted of a proliferation of thin-walled, dilated blood vessels, with an endothelial lining devoid of atypia and exhibiting immunoreactivity for CD31 and CD34, while podoplanin and GLUT1 were negative. Whole exome sequencing performed on samples from the lesion of the femur, the tibia and the skin of the thigh, showed a GNAQ (c.286A>T:p.T96S) variant in all specimens, that was confirmed with digital droplet PCR. This case expands the clinical and pathologic spectrum of vascular proliferations showing similar molecular biology, characterized by GNAQ, GNA11 or GNA14 mutations.


Subject(s)
Angiomatosis/genetics , GTP-Binding Protein alpha Subunits, Gq-G11/genetics , Kasabach-Merritt Syndrome/genetics , Angiomatosis/pathology , Bone and Bones/pathology , Child, Preschool , Connective Tissue/pathology , Humans , Kasabach-Merritt Syndrome/pathology , Male , Mutation
15.
J Pediatr Orthop B ; 29(6): 611-617, 2020 Nov.
Article in English | MEDLINE | ID: mdl-31904740

ABSTRACT

Implantable intramedullary nail lengthening devices (e.g., PRECICE 2 system) have been proposed as alternative method to external fixation for lower limb lengthening surgery. The aim of this study was to analyse our outcomes and complications using the PRECICE 2 (P2) nail system and review them in light of the existing literature. A retrospective multicentre study was conducted on patients <18 years, who were treated for limb lengthening using the P2 system. The inclusion criteria were a limb length discrepancy ≥ 30 mm and a follow-up ≥6 months after the end of treatment. A total of 26 (15 males) patients were included, average age was 14.7 ± 2.3 years; 26 nails (21 femur, 5 tibia) were implanted. The average goal lengthening was 49.4 ± 12.4 mm, while average achieved lengthening was 44.4 ± 11.6 mm. Average distraction and consolidation indexes were 11.9 ± 2.1 days/cm and 25.1 ± 8.1 days/cm, respectively. Nail accuracy and reliability were 91.1% and 88.5%, respectively. A total of five problems (joint contractures), one obstacle (femur fracture) and three complications (hip joint subluxation, deep infection and nail running back) were encountered. The P2 nail system is a valid alternative to external fixator for limb lengthening in young patients with no significant angular or rotation deformities. Our study confirms a favourable complication rate and available evidence from literature suggests a lower complication rate than external fixator systems. Nevertheless, surgeons should keep a watchful eye on risk of joint subluxation and mechanical complications with intramedullary lengthening.


Subject(s)
Bone Lengthening/methods , Bone Nails , Leg Length Inequality/diagnostic imaging , Leg Length Inequality/surgery , Postoperative Complications/diagnostic imaging , Postoperative Complications/etiology , Adolescent , Bone Lengthening/adverse effects , Bone Lengthening/trends , Bone Nails/adverse effects , Bone Nails/trends , Child , Female , Humans , Male , Retrospective Studies , Treatment Outcome
17.
Foot Ankle Surg ; 25(5): 623-629, 2019 Oct.
Article in English | MEDLINE | ID: mdl-30321938

ABSTRACT

BACKGROUND: Treatment of complex foot deformities in growing children and young adult patients is challenging. The traditional approach consists of extensive soft tissues releases, osteotomies and/or arthrodesis. More recently, distraction osteogenesis has been proposed as an alternative strategy. The aim of this study was to describe our treatment strategy and report clinical outcomes of the patients affected from complex foot deformities treated by distraction osteogenesis and hexapod external fixator. MATERIALS AND METHODS: We retrospectively reviewed 10 consecutive patients with complex foot and ankle deformities treated from 2014 to 2016 at our unit. A TrueLok external fixator system was used in all patients. Final outcome was classified as good, fair and poor according to the criteria indicated by Paley and Ferreira. The results were also evaluated by the pre-operative and post operative American Orthopedic Foot and Ankle Score (AOFAS) and The Manchester-Oxford Foot Questionnaire (MOXFQ). RESULTS: A plantigrade foot was obtained in eight patients at the end of treatment, while in two patients a recurrence of the deformity was noted. Result was classified as good in 6 patients, fair in 2 patients, and poor in 2 patients. The AOFAS score improved from 33.9±21.2 pre-operatively to 67.25±15.1 post-operatively (p=0.005). A statistically significant improvement was observed for the MOXFQ score as well (from 60.6±23.3 to 33.0±25.2, p=0.020). CONCLUSIONS: Our study shows that the TrueLok hexapod external fixator is a safe and effective tool in treatment of complex rigid foot deformities. Nevertheless, deformity recurrence can be observed in some cases and treatment remains challenging. Distraction osteogenesis should be reserved as a salvage solution for particularly complex cases and should be performed at dedicated specialized centers.


Subject(s)
External Fixators , Foot Deformities/surgery , Osteogenesis, Distraction , Adolescent , Arthrogryposis/surgery , Child , Clubfoot/surgery , Female , Foot Bones/surgery , Foot Deformities/etiology , Humans , Male , Meningomyelocele/complications , Osteotomy/methods , Retrospective Studies
18.
J Pediatr Orthop B ; 28(3): 214-220, 2019 May.
Article in English | MEDLINE | ID: mdl-30531204

ABSTRACT

The aim of this study was to compare clinical and radiographical results for treatment of lower limb multiaxial deformities±limb length discrepancy (LLD) of at least 2 cm with the Truelok hexapod fixator system (TL-HEX). All consecutive cases of lower limb multiaxial deformities were included. Patients were divided in two groups: group 1, lower limb angular deformity+LLD less than 2 cm, and group 2, lower limb angular deformity+LLD of at least 2 cm. Only patients with age younger than or equal to 20 years and follow-up of 6 months after removal of the external fixator were included. A total of 27 (six femur and 39 tibia treated) and 20 patients (12 femur and 19 tibia) were enrolled in groups 1 and 2, respectively. Complete correction of the deformity was achieved in 90 and 96% of the patients in groups 1 and 2, respectively. There were no differences in terms of external fixator, maturation, and distraction indexes between the two groups and between different anatomical sites. Good to excellent functional results (ASAMI score) were obtained in 93% of patients in group 1 and 75% in group 2 (P=0.01). Complication rate was similar between the two groups (7.4 vs. 10%, respectively). Average follow-up after removal of the external fixator was 25.6 (range: 7.0-54.0) months. The TL-HEX external fixator system allows a predictable correction of complex lower limb deformities regardless of the presence of LLD. Although complication rate is similar between the two groups, lower functional outcomes can be expected in patients with significant preoperative LLD.


Subject(s)
External Fixators/trends , Leg Length Inequality/diagnostic imaging , Leg Length Inequality/therapy , Leg/abnormalities , Leg/diagnostic imaging , Adolescent , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Male , Prospective Studies , Retrospective Studies
19.
Injury ; 49 Suppl 3: S8-S11, 2018 Nov.
Article in English | MEDLINE | ID: mdl-30415674

ABSTRACT

INTRODUCTION: Elastic stable intramedullary nailing (ESIN) is currently considered the gold standard in surgical treatment of femur and tibial shaft fractures in school age paediatric patients. Although elastic intramedullary nails are available in both titanium (Ti) and stainless steel (SS) alloy, titanium nails are most commonly used. Nevertheless, there is still contrasting evidence as to whether the use of Ti nails can offer better outcomes in terms of fracture healing and stability over SS nails. The aim of this study was to compare outcomes and complications of Ti and SS ESIN for femur and tibia shaft fractures in a population of school age paediatric patients. MATERIALS AND METHODS: All consecutive patients who underwent ESIN for femoral or tibial shaft fracture from June 2012 to May 2015 at our centre were retrospectively reviewed. Standard demographic data were collected. Pre-operative and post-operative X-rays were reviewed, complications were collected from patients charts. Patients were divided in two groups, titanium nails (Ti group) and stainless steel nails (SS group) and outcomes compared between the two. RESULTS: A total of 34 patients were included (17 patients Ti group, 17 patients SS group) with a total of 14 femur and 21 tibia fractures. Average age at surgery was 9.4 ± 2.5 years in Ti group and 10.4 ± 2.4 years in SS group (p = 0.21). The average time to bone healing was 3 months in Ti group, and 2.8 months in SS group (p = 0.63). At final follow-up (12 months), no patient showed a coronal plane or sagittal plane deformity >10° and >15°, respectively. Complication rate was similar between the two groups (24% Ti group, 22% SS group). CONCLUSIONS: We did not observe any significant difference in terms of bone healing, fracture mechanical stability, return to full activity, and complication rate between Ti and SS ESIN for paediatric femoral and tibial shaft fractures. While Ti nails remain a better choice for patients with metal allergy, SS nails may offer safe, effective, and cheaper alternative to Ti nails in school age femur and tibial shaft fractures.


Subject(s)
Bone Nails , Femoral Fractures/surgery , Fracture Fixation, Intramedullary/instrumentation , Stainless Steel , Tibial Fractures/surgery , Titanium , Child , Female , Femoral Fractures/diagnostic imaging , Femoral Fractures/physiopathology , Fracture Fixation, Intramedullary/methods , Fracture Healing/physiology , Humans , Male , Postoperative Complications/diagnostic imaging , Radiography , Range of Motion, Articular , Retrospective Studies , Tibial Fractures/diagnostic imaging , Tibial Fractures/physiopathology , Treatment Outcome
20.
Acta Orthop Traumatol Turc ; 52(4): 315-319, 2018 Jul.
Article in English | MEDLINE | ID: mdl-29248252

ABSTRACT

We report a nine-year-old boy with a type IIIa tibial hemimelia, according to the new Paley classification. We describe the x-ray findings, the surgical treatment technique, and the prognostic course of the patient. Descriptions of such cases are very infrequent in the literature and type of treatment is still object of debate.


Subject(s)
Ectromelia/surgery , Orthopedic Procedures/methods , Surgery, Computer-Assisted/methods , Tibia/abnormalities , Child , Ectromelia/diagnosis , Fluoroscopy , Humans , Male , Radiography , Rare Diseases , Tendon Transfer/methods , Tibia/surgery
SELECTION OF CITATIONS
SEARCH DETAIL
...