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1.
Acta Orthop Belg ; 82(3): 599-609, 2016 Sep.
Article En | MEDLINE | ID: mdl-29119903

Treatment of tibial osteomyelitis with infected, necrotic, unstable bone segments (Cierny-Mader Type IV local osteomyelitis) includes débridement and segmental resection, which results in long bone defects. Reconstruction may be with distraction osteogenesis. Segmental bone transport and acute compression and distraction techniques are two main distraction osteogenesis techniques used in the treatment of Type IV local tibial osteomyelitis. In this retrospective, four-center study we compared these two techniques during a 15-year period. 29 patients treated using segmental bone transport technique and 45 patients were treated using acute compression and distraction technique. The mean age (p= 0,34) and the mean bone loss with preoperative shortening (P=0,08) and the mean number of previous operation (p=0,06) were not different in these two groups. . At latest followup, functional and radiographic results were evaluated There was no difference between two technique on the Paley's scoring system ( p=0,33) and in the total number of complication(p=0,16). Mean external fixator index was lower in the second group ( p=0.02 ). Both techniques can be used safely; however, the acute compression distraction technique may provide greater patient satisfaction because of shorter external fixator index, although future studies will be needed to determine whether this is.


External Fixators , Osteogenesis, Distraction/methods , Osteomyelitis/surgery , Tibia/surgery , Adolescent , Adult , Chronic Disease , Debridement/methods , Female , Humans , Male , Middle Aged , Retrospective Studies , Treatment Outcome , Young Adult
2.
Bone Joint J ; 97-B(11): 1577-81, 2015 Nov.
Article En | MEDLINE | ID: mdl-26530664

A retrospective study was performed in 18 patients with achondroplasia, who underwent bilateral humeral lengthening between 2001 and 2013, using monorail external fixators. The mean age was ten years (six to 15) and the mean follow-up was 40 months (12 to 104). The mean disabilities of the arm, shoulder and hand (DASH) score fell from 32.3 (20 to 40) pre-operatively to 9.4 (6 to 14) post-operatively (p = 0.037). A mean lengthening of 60% (40% to 95%) was required to reach the goal of independent perineal hygiene. One patient developed early consolidation, and fractures occurred in the regenerate bone of four humeri in three patients. There were three transient radial nerve palsies. Humeral lengthening increases the independence of people with achondroplasia and is not just a cosmetic procedure.


Achondroplasia/surgery , External Fixators , Humerus/surgery , Osteogenesis, Distraction/methods , Activities of Daily Living , Adolescent , Child , Disability Evaluation , Female , Follow-Up Studies , Humans , Male , Osteogenesis, Distraction/adverse effects , Retrospective Studies , Treatment Outcome
3.
Haemophilia ; 20(6): 879-83, 2014 Nov.
Article En | MEDLINE | ID: mdl-25143070

Haemophilia, a bleeding disorder, causes recurrent intra-articular bleeding of the joints result-ing in chronic haemophilic arthropathy with fixed knee flexion deformity. Mid-long-term results (between 2002 and 2006) of deformity correction in haemophilic patients with Ilizarov type circular external fixators were retrospectively evaluated. There were six patients (five haemophilia A and one haemophilia B). The mean age was 14.7 years (range, 8-22 years) at the time of initial surgery. The mean knee flexion contracture was 45 degrees (range, 30-75 degrees). The mean arc of motion was 58.3 degrees (range, 40-100) before the surgery. The mean duration of follow-up was 8 years (range, 5.5-10 years). The mean duration of external fixation was 4.4 months (range, 2.5-10.5 months). Full extension of the knee joint was obtained in all patients in the early postoperative period. No bleeding, neurological or vascular complications were encountered. The mean amount of recurrence in knee flexion contracture was 10 degrees (range, 0-15 degrees). The amount of the correction was significant (P = 0.0012) and the mean arc of motion was 51.6 degrees (range, 25-90 degrees) that show a decrease of 6.7 degrees (P = 0.04) at the end of follow-up. The circular external fixator is an important, safe and less invasive alternative surgical treatment modality with low recurrence rate. Using the external hinges and distraction during the correction has a protective effect on the joint. It requires a team-work consisting of a haematologist, an orthopaedic surgeon and a physical therapist.


Contracture/surgery , External Fixators , Hemarthrosis/pathology , Hemarthrosis/surgery , Hemophilia A/complications , Hemophilia B/complications , Knee Joint/pathology , Knee Joint/surgery , Adolescent , Adult , Child , Contracture/etiology , Follow-Up Studies , Hemarthrosis/etiology , Humans , Range of Motion, Articular , Retrospective Studies , Treatment Outcome , Young Adult
5.
Mol Syndromol ; 5(2): 81-6, 2014 Feb.
Article En | MEDLINE | ID: mdl-24715855

Heterozygous loss-of-function mutations of GDF5 are reported to cause hypoplasia/aplasia of certain skeletal elements (brachydactyly), and heterozygous gain-of-function mutations, occurring either on the gene itself or through the loss of its inhibitor noggin, result in joint fusion (symphalangism). We present here the clinical and molecular investigation of a family with disproportionate shortness of the second and third fingers which comprises 9 variably affected members spanning 4 generations. In this study, we performed clinical and radiographical examinations of 2 patients of this family, sequencing of GDF5 and 3D protein modeling of the wildtype and mutated polypeptide to predict the structural alteration. Diagnoses were compatible with familial brachydactyly type C. GDF5 analysis revealed a novel heterozygous in-frame indel mutation (c.803_ 827del25ins25), involving the propeptide domain of GDF5 that alters the number of random coil and beta-strand structures, creating a 1-turn-helix at the mutated site. The mutation described here is the second indel reported in GDF5. The previously published homozygous indel mutation affected the TGF-beta like domain and was associated with Du Pan syndrome. The novel mutation reported here presents further allelic heterogeneity and a probable intrafamilial variable clinical expressivity of GDF5.

6.
Br J Radiol ; 84(1004): 758-65, 2011 Aug.
Article En | MEDLINE | ID: mdl-21586507

Cerebrospinal fluid (CSF) spaces include ventricles and cerebral and spinal subarachnoid spaces. CSF motion is a combined effect of CSF production rate and superimposed cardiac pulsations. Knowledge of CSF dynamics has benefited considerably from the development of phase-contrast (PC) MRI. There are several disorders such as communicating and non-communicating hydrocephalus, Chiari malformation, syringomyelic cyst and arachnoid cyst that can change the CSF dynamics. The aims of this pictorial review are to outline the PC MRI technique, CSF physiology and cerebrospinal space anatomy, to describe a group of congenital and acquired disorders that can alter the CSF dynamics, and to assess the use of PC MRI in the assessment of various central nervous system abnormalities.


Arachnoid Cysts/diagnosis , Arnold-Chiari Malformation/diagnosis , Cerebrospinal Fluid/physiology , Hydrocephalus/diagnosis , Magnetic Resonance Imaging/instrumentation , Subarachnoid Space/pathology , Syringomyelia/diagnosis , Adolescent , Adult , Aged , Arachnoid Cysts/cerebrospinal fluid , Arnold-Chiari Malformation/cerebrospinal fluid , Child , Child, Preschool , Contrast Media , Female , Humans , Hydrocephalus/cerebrospinal fluid , Magnetic Resonance Imaging/methods , Male , Middle Aged , Subarachnoid Space/anatomy & histology , Syringomyelia/cerebrospinal fluid , Young Adult
7.
J Bone Joint Surg Br ; 93(1): 52-6, 2011 Jan.
Article En | MEDLINE | ID: mdl-21196543

We present the results of the surgical correction of lower-limb deformities caused by metabolic bone disease. Our series consisted of 17 patients with a diagnosis of hypophosphataemic rickets and two with renal osteodystrophy; their mean age was 25.6 years (14 to 57). In all, 43 lower-limb segments (27 femora and 16 tibiae) were osteotomised and the deformity corrected using a monolateral external fixator. The segment was then stabilised with locked intramedullary nailing. In addition, six femora in three patients were subsequently lengthened by distraction osteogenesis. The mean follow-up was 60 months (18 to 120). The frontal alignment parameters (the mechanical axis deviation, the lateral distal femoral angle and the medial proximal tibial angle) and the sagittal alignment parameters (the posterior distal femoral angle and the posterior proximal tibial angle) improved post-operatively. The external fixator was removed either at the end of surgery or at the end of the lengthening period, allowing for early mobilisation and weight-bearing. We encountered five problems and four obstacles in the programme of treatment. The use of intramedullary nails prevented recurrence of deformity and refracture.


Chronic Kidney Disease-Mineral and Bone Disorder/surgery , Familial Hypophosphatemic Rickets/surgery , Lower Extremity/surgery , Adolescent , Adult , Bone Nails , Chronic Kidney Disease-Mineral and Bone Disorder/diagnostic imaging , External Fixators , Familial Hypophosphatemic Rickets/diagnostic imaging , Female , Femur/diagnostic imaging , Femur/surgery , Follow-Up Studies , Humans , Lower Extremity/diagnostic imaging , Male , Middle Aged , Osteogenesis, Distraction/methods , Osteotomy/methods , Radiography , Tibia/diagnostic imaging , Tibia/surgery , Young Adult
8.
J Bone Joint Surg Br ; 92(1): 146-52, 2010 Jan.
Article En | MEDLINE | ID: mdl-20044694

We report the results of using a combination of fixator-assisted nailing with lengthening over an intramedullary nail in patients with tibial deformity and shortening. Between 1997 and 2007, 13 tibiae in nine patients with a mean age of 25.4 years (17 to 34) were treated with a unilateral external fixator for acute correction of deformity, followed by lengthening over an intramedullary nail with a circular external fixator applied at the same operating session. At the end of the distraction period locking screws were inserted through the intramedullary nail and the external fixator was removed. The mean amount of lengthening was 5.9 cm (2 to 8). The mean time of external fixation was 90 days (38 to 265). The mean external fixation index was 15.8 days/cm (8.9 to 33.1) and the mean bone healing index was 38 days/cm (30 to 60). One patient developed an equinus deformity which responded to stretching and bracing. Another developed a drop foot due to a compartment syndrome, which was treated by fasciotomy. It recovered in three months. Two patients required bone grafting for poor callus formation. We conclude that the combination of fixator-assisted nailing with lengthening over an intramedullary nail can reduce the overall external fixation time and prevent fractures and deformity of the regenerated bone.


External Fixators , Fracture Fixation, Intramedullary/methods , Fracture Healing/physiology , Leg Length Inequality/surgery , Osteogenesis, Distraction/methods , Tibia/surgery , Adolescent , Adult , Bone Nails , Female , Fracture Fixation, Intramedullary/rehabilitation , Humans , Leg Length Inequality/diagnostic imaging , Leg Length Inequality/rehabilitation , Male , Osteogenesis, Distraction/rehabilitation , Radiography , Tibia/abnormalities , Tibia/diagnostic imaging , Treatment Outcome , Young Adult
9.
J Med Imaging Radiat Oncol ; 54(6): 541-9, 2010 Dec.
Article En | MEDLINE | ID: mdl-21199431

Various types of lesions can occur within the corpus callosum (CC) which is a white matter tract communicating corresponding regions of the cerebral hemispheres. Magnetic resonance imaging is the modality of choice for the evaluation of the CC. In addition, diffusion weighted imaging and diffusion tensor imaging can provide additional information about the CC. The aim of this study is to illustrate the imaging features of the corpus callosum and its pathologies.


Corpus Callosum/anatomy & histology , Corpus Callosum/pathology , Magnetic Resonance Imaging/methods , Agenesis of Corpus Callosum , Anisotropy , Brain Diseases/pathology , Diffusion Magnetic Resonance Imaging/methods , Diffusion Tensor Imaging/methods , Humans
10.
Br J Radiol ; 82(977): e102-4, 2009 May.
Article En | MEDLINE | ID: mdl-19386952

Klippel-Trenaunay syndrome (KTS) is a rare disorder characterised by congenital vascular hamartomas, limb hypertrophy, lymphangiomas and atresia of lymph vessels with non-pitting oedema. A 6-year-old girl with KTS was referred to our hospital for evaluation of intractable seizures. In addition to findings consistent with KTS, we also found hemimegalencephaly, retroperitoneal lymphangioma and double inferior vena cava. All of these associations in the same patient with KTS are unique in the English literature. We report on the multidedector CT and MRI features of such an unusual case.


Kidney Neoplasms/diagnosis , Klippel-Trenaunay-Weber Syndrome/complications , Lymphangioma/diagnosis , Malformations of Cortical Development/diagnosis , Vena Cava, Inferior/abnormalities , Child , Female , Humans , Magnetic Resonance Imaging , Tomography, X-Ray Computed/methods
11.
Article En | MEDLINE | ID: mdl-18230374

The use of osseointegration for bone anchorage in the prosthetic management of craniofacial defects has been successfully applied for many years. Different methods can be used for reconstruction of the aural atresia. Nowadays plastic surgery for auricular reconstruction and the bone-anchored episthesis are used for correction of aural atresia. There have been several studies in the literature comparng these techniques. According to those studies, bone-anchored episthesis is an excellent alternative to plastic reconstructive surgery of the auricle. In the present study we present 2 cases with different etiologic factors. The patients' right external ears were absent. There was loss in hearing function in one of the patients. We applied external ear episthesis with a bone-anchored system.


Ear, External , Plastic Surgery Procedures , Prostheses and Implants , Prosthesis Implantation/instrumentation , Adult , Ear Deformities, Acquired/rehabilitation , Humans , Male , Mastoid/surgery
12.
Br J Radiol ; 80(959): e287-9, 2007 Nov.
Article En | MEDLINE | ID: mdl-17989330

Replacement lipomatosis of the kidney (RLK) is an advanced form of renal sinus lipomatosis, in which infection, renal calculi and long-standing hydronephrosis are accompanied by renal parenchymal atrophy. The kidneys are usually poor or non-functioning. We present CT and MRI findings of an unusual focal RLK of a 52-year-old male, who was examined with the suspicion of renal malignancy.


Kidney Diseases/diagnosis , Lipomatosis/diagnosis , Contrast Media , Humans , Magnetic Resonance Imaging/methods , Male , Middle Aged , Tomography, X-Ray Computed/methods , Urography/methods
15.
Neuroradiol J ; 20(2): 233-6, 2007 Apr 30.
Article En | MEDLINE | ID: mdl-24299651

Spinal cord involvement, either isolated or together with brain, in Behçet's disease (BD) has been reported. In these cases the existence of the disease was previously known or the classical triad of disease such as oral and genital ulcers with uveitis/iritis was present. Here we describe a 22-year-old man in whom acute transverse myelitis diagnosed with MRI was the first finding of BD.

16.
Neuroradiol J ; 20(3): 359-63, 2007 Jun 30.
Article En | MEDLINE | ID: mdl-24299682

Central nervous system involvement in systemic mastocytosis (SM) is very rare. This case report describes the computed tomography and magnetic resonance (MR) imaging findings of central nervous system involvement in a patient with isolated bone marrow mastocystosis. Bone marrow infiltration in SM caused cranial nerve dysfunction and meningeal irritation secondary to narrowing of cranial apertures and meningeal involvement, respectively. MR imaging is the modality of choice in both detection and follow-up of SM and also useful for differential diagnosis and detection of complications.

17.
Neuroradiol J ; 20(6): 646-55, 2007 Dec 31.
Article En | MEDLINE | ID: mdl-24299999

The aim of this study was to evaluate the efficacy of (31)P magnetic resonance spectroscopy ((31)P-MRS) in the differentiation and staging of brain tumors. Fifteen volunteers and 44 patients with brain tumors (14 meningiomas, 13 low- and 17 high-grade gliomas) were prospectively evaluated by (31)P-MRS. The pH (r=0.493, p<0.001), [Mg(+2)] (r=0.850, p<0.001) PME/α-ATP (r=0.776, p<0.001), PDE/α-ATP (r=-0.569, p<0.001) and (PCr+ß-ATP)/Pi ratios were well correlated with tumor differentiation. High-grade gliomas had significantly higher pH (r=0.912, p<0.001) and [Mg(+2)] (r=0.855, p<0.001) and PME/α-ATP (r=0.894, p<0.001) ratio, and lower PCr/α-ATP (r=-0.959, p<0.001), Pi/α-ATP (r=-0.788, p<0.001) and PDE/α-ATP ratios (r=-0.968, p<0.001) than those of low-grade gliomas. Changes in (31)P-MRS parameters by the degree of malignancy are good indicators of increased anaerobic metabolism and hypoxia of tumoral tissue to compensate intratumoral energy deficiency. (31)P-MRS parameters are very useful for grading and differentiation of brain tumors.

18.
AJNR Am J Neuroradiol ; 27(9): 1983-6, 2006 Oct.
Article En | MEDLINE | ID: mdl-17032879

BACKGROUND: Reversible lesions in the splenium of the corpus callosum (SCC), caused by various agents such as influenza, rotavirus, Escherichia coli, mumps, and adenovirus, were previously defined in a handful of cases. We present 5 cases with transient diffusion restriction of the SCC associated with influenza A virus infection. MATERIALS AND METHODS: Five patients with influenza-associated encephalitis/encephalopathy and sudden-onset neurologic symptoms following a prodromal flulike episode were examined by MR and diffusion-weighted imaging (DWI). RESULTS: Three patients, who had drowsiness and new-onset convulsions, recovered spontaneously without any medication. In the other 2 seizure-free patients, 1 had trigeminal neuralgia and headache and the other had facial numbness and left upper monoparesis. All patients had round well-defined ovoid hyperintense splenial lesions (14.94 +/- 1.87 mm) on DWI with a significantly low apparent diffusion coefficient (ADC) of 0.41 +/- 0.05 x 10(-3) mm(2)/s compared with 0.84 +/- 0.01 x 10(-3) mm(2)/s of normal-appearing white matter. In the patient with a motor deficit, additional lesions were found in the cerebral deep white matter. The high signal intensity of the splenial and deep white matter lesions on DWI completely disappeared on follow-up studies, and ADC values also improved, returning to those of normal-appearing white matter on days 8-11. Clinically, all patients completely recovered on days 4-9. CONCLUSION: A transient lesion of the SCC is a significant but nonspecific finding. It is probably due to edematous and/or inflammatory changes of the SCC. It may be the only detectable change in patients with good prognosis, indicating a clinically mild form of encephalitis/encephalopathy.


Alphainfluenzavirus , Brain/pathology , Corpus Callosum/pathology , Diffusion Magnetic Resonance Imaging , Encephalitis, Viral/diagnosis , Influenza, Human/diagnosis , Adolescent , Adult , Age Factors , Child , Female , Humans , Male , Neurologic Examination , Remission, Spontaneous , Statistics as Topic
19.
Acta Radiol ; 47(7): 746-51, 2006 Sep.
Article En | MEDLINE | ID: mdl-16950716

PURPOSE: To determine the added value of multidetector computed tomography (MDCT)-assisted virtual bronchoscopy (VB) to axial MDCT and multiplanar reformatted (MPR) imaging with respect to conventional bronchoscopy in the evaluation of children with suspected foreign-body aspiration (FBA). MATERIAL AND METHODS: Twenty-one consecutive children (14 male, seven female, age 8 months-7 years, mean age 3.5 years) presenting with the suspicion of FBA were examined with 16-slice MDCT using 100-120 kV, 30-50 mA, 1-mm section thickness, 1.2 pitch ratio, and 0.6-1.0-mm reconstruction interval. Before CT examinations, chest radiographies were also obtained in all cases. VB and MPR imaging were carried out after MDCT examinations. RESULTS: Nine foreign bodies were identified by gold-standard conventional bronchoscopy. VB, thin-section axial MDCT, and MPR images identified eight of nine foreign bodies. CT scans with MPR images and VB did not reveal a chronic foreign body; and, in one patient, endobronchial mucous was diagnosed as the foreign body. The sensitivity, specificity, and accuracy of thin-section axial MDCT and MPR images alone in the diagnosis of FBA were 88.9%, 91.7%, and 90.5%, respectively. Results were the same as those obtained when VB was also included. CONCLUSION: MDCT with VB and axial/MPR images provide equally valuable information in children with suspected foreign-body aspiration and prevent unnecessary conventional bronchoscopic examinations. However, VB increases total examination time and cost, and it does not provide additional information over MPR images in the evaluation of foreign-body aspiration.


Bronchi , Bronchoscopy/methods , Foreign Bodies/diagnosis , Tomography, X-Ray Computed , Child , Child, Preschool , Female , Foreign Bodies/diagnostic imaging , Humans , Image Processing, Computer-Assisted , Infant , Male , Predictive Value of Tests , Radiography, Thoracic , Sensitivity and Specificity
20.
AJNR Am J Neuroradiol ; 27(8): 1735-7, 2006 Sep.
Article En | MEDLINE | ID: mdl-16971625

Psoas abscess secondary to tuberculous spondylodiskitis is usually a complication of thoracolumbar vertebrae disease. The psoas abscess may be difficult clinically to diagnose because of its rarity, insidious onset of the disease, and nonspecific clinical presentation. We report multidetector CT and MR imaging findings of a psoas abscess secondary to primary tuberculous spondylodiskitis of the spine from the T3 to L2 vertebrae, which presented as a flank mass.


Discitis/diagnosis , Lumbar Vertebrae/pathology , Psoas Abscess/diagnosis , Psoas Muscles , Thoracic Vertebrae , Tuberculosis, Spinal/diagnosis , Adult , Diagnosis, Differential , Humans , Magnetic Resonance Imaging , Male , Psoas Muscles/pathology , Thoracic Vertebrae/pathology , Ultrasonography
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