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PURPOSE: To compare patient characteristics, operative time, estimated blood loss (EBL), postoperative length of hospital stay (LOS) and complications after insertion and removal of submuscular plates (SMPs) versus flexible nails (FNs) for paediatric diaphyseal femur fractures. METHODS: We reviewed records of 58 children (mean age, 7.7 years SD 2.0) with diaphyseal femur fractures who underwent treatment with SMPs (n = 30) or FNs (n = 28) from 2005 to 2017 (mean follow-up, 22 months SD 28). Patients with pathological fractures or musculoskeletal comorbidities were excluded. Alpha = 0.05. RESULTS: Insertion of FNs was associated with shorter operative time (ß = -24 mins) and less EBL (ß = -38 mL) (both, p < 0.001) compared with insertion of SMPs, after adjusting for fracture type and time from beginning of study period. Removal of FNs was also associated with shorter operative time (ß = -15 min) compared with removal of SMPs (p < 0.001). EBL during removal was similar between groups (p = 0.080). The FN group had a shorter LOS after insertion (ß = -0.2 d) compared with the SMP group (p = 0.032). Four patients treated with SMPs and three treated with FNs developed surgical site infections. Two patients treated with SMPs and seven treated with FNs experienced implant irritation that resolved with removal. No other complications occurred. CONCLUSION: Compared with SMPs, FNs were associated with shorter operative time (for insertion and removal), less EBL (for insertion) and shorter post-insertion LOS in patients with diaphyseal femur fractures. LEVEL OF EVIDENCE: III.
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Aims: The aim of this study was to compare the outcomes of surgery using growing rods in patients with severe versus moderate early-onset scoliosis (EOS). Patients and Methods: A review of a multicentre EOS database identified 107 children with severe EOS (major curve ≥ 90°) treated with growing rods before the age of ten years with a minimum follow-up of two years and three or more lengthening procedures. From the same database, 107 matched controls with moderate EOS were identified. Results: The mean preoperative major curve was 101° (90 to 139) in the severe group and 67° (33° to 88°) in the moderate group (p < 0.001), which was corrected at final follow-up to 57° (10° to 96°) in the severe group and 40° (3° to 85°) in the moderate group (p < 0.001). T1-S1 height increased by a mean of 54 mm (-8 to 131) in the severe group and 27 mm (-4 to 131) in the moderate group at the initial surgery (p < 0.001), and by 50 mm (-17 to 200) and 54 mm (-11 to 212), respectively, during distraction (p = 0.84). The mean number of complications per patient was 2.6 (0 to 14) in the severe group and 1.9 (0 to 10) in the moderate group (p = 0.040). Five patients (4.7%) in the severe group and three (2.8%) in the moderate group developed a neurological deficit postoperatively (p = 0.47). Conclusion: Severe EOS can be treated effectively using growing rods, but the risk of complications is high. Cite this article: Bone Joint J 2018;100-B:772-9.
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Alargamiento Óseo/métodos , Dispositivos de Fijación Ortopédica/efectos adversos , Diseño de Prótesis/efectos adversos , Escoliosis/cirugía , Columna Vertebral/cirugía , Alargamiento Óseo/efectos adversos , Niño , Preescolar , Bases de Datos Factuales , Femenino , Humanos , Lactante , Imanes , Masculino , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Fusión Vertebral/métodos , Resultado del TratamientoRESUMEN
BACKGROUND: Primary bladder closure of classic bladder exstrophy (CBE) is a major operation that occasionally requires intraoperative or postoperative (within 72 h) blood transfusions. OBJECTIVE: This study reported perioperative transfusion rates, risk factors for transfusion, and outcomes from a high-volume exstrophy center in primary bladder closure of CBE patients. STUDY DESIGN: A prospectively maintained, institutional exstrophy-epispadias complex database of 1305 patients was reviewed for primary CBE closures performed at the authors' institution (Johns Hopkins Hospital) between 1993 and 2017. Patient and surgical factors were analyzed to determine transfusion rates, risk factors for transfusions, and outcomes. Patients were subdivided into two groups based upon the time of closure: neonatal and delayed closure. RESULTS: A total of 116 patients had a primary bladder closure during 1993-2017. Seventy-three patients were closed in the neonatal period, and 43 were delayed closures. In total, 64 (55%) patients received perioperative transfusions. No transfusion reactions were observed. Twenty-five transfusions were in the neonatal closure group, yielding a transfusion rate of 34%. In comparison, 39 patients were transfused in the delayed closure group, giving a transfusion rate of 91%. Pelvic osteotomy, delayed bladder closure, higher estimated blood loss (EBL), larger pubic diastasis, and longer operative time were all associated with blood transfusion. In multivariable logistic regression, pelvic osteotomy (OR 5.4; 95% CI 1.3-22.8; P < 0.001), higher EBL-to-weight ratio (OR 1.3; 95% CI 1.1-1.6; P = 0.029), and more recent years of primary closure (OR 1.1; 95% CI 1.0-1.2; P = 0.018) remained independent predictors of receiving a transfusion (Summary Table). No adverse transfusion reactions or complications were observed. DISCUSSION: This was the first study from a single high-volume exstrophy center to explore factors that contribute to perioperative blood transfusions. Pelvic osteotomy as a risk factor was unsurprising, as the osteotomy may bleed both during and immediately after closure. However, it is important to use osteotomy for successful closure, despite the increased transfusion risk. The risks accompanying contemporary transfusions are minimal and osteotomies are imperative for successful bladder closure. CONCLUSIONS: More than half of CBE patients undergoing primary closure at a single institution received perioperative blood transfusions. While there was an association between transfusions and osteotomy, delayed primary closure, larger diastasis, increased operative time, and increased length of stay, only the use of pelvic osteotomy, higher EBL-to-weight ratio, and recent year of closure independently increased the odds of receiving a transfusion on multivariate analysis.
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Extrofia de la Vejiga/cirugía , Transfusión Sanguínea/estadística & datos numéricos , Femenino , Predicción , Humanos , Recién Nacido , Masculino , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
Bone disease, specifically low bone mineral density, is a common and undertreated complication that begins during childhood in patients with cystic fibrosis (CF). This case describes a male baseball player, aged 14years, with undiagnosed CF who sustained a left midshaft femoral fracture while running toward base; 8months later, he sustained a right midshaft femoral fracture under similar conditions. After the second fracture, further evaluation revealed low bone mineral density and CF. There is no previously published report of pathologic fractures occurring in the femoral shaft in an athlete with undiagnosed CF. Patients with CF have a higher fracture rate. Low-energy fractures of major bones in athletically active individuals should be viewed with suspicion for an underlying process.
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Béisbol/lesiones , Fibrosis Quística , Errores Diagnósticos/prevención & control , Fracturas del Fémur , Fémur , Absorciometría de Fotón/métodos , Adolescente , Densidad Ósea , Fibrosis Quística/complicaciones , Fibrosis Quística/diagnóstico , Fibrosis Quística/fisiopatología , Fracturas del Fémur/etiología , Fracturas del Fémur/fisiopatología , Fémur/diagnóstico por imagen , Fémur/patología , Fracturas Espontáneas/etiología , Fracturas Espontáneas/fisiopatología , Humanos , MasculinoRESUMEN
The bladder exstrophy-epispadias complex (EEC) represents a spectrum of rare and surgically correctable congenital anomalies. Classic bladder exstrophy (CBE) stands between epispadias and cloacal exstrophy (CE) in the severity spectrum, and is the most commonly encountered type. CBE involves congenital defects of the bladder, abdominal wall, pelvic floor, and bony pelvis. With the growing understanding of the detrimental effects of radiation in children, magnetic resonance imaging (MRI) is progressively been utilized in the preoperative work-up and post-surgical follow-up of these patients. MRI provides valuable information for planning and evaluating the optimal surgical techniques for closure of CBE. The aim of this paper is to provide a review of the two- (2D) and three-dimensional (3D) MRI features of CBE including a detailed analytical description of the anatomy of the pelvic floor in affected patients.
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Extrofia de la Vejiga/patología , Epispadias/patología , Imagen por Resonancia Magnética , Huesos Pélvicos/anomalías , Diafragma Pélvico/anomalías , Extrofia de la Vejiga/cirugía , Preescolar , Epispadias/cirugía , Femenino , Estudios de Seguimiento , Humanos , Imagenología Tridimensional , Lactante , Recién Nacido , Imagen por Resonancia Magnética/métodos , Masculino , Diafragma Pélvico/cirugía , Periodo Posoperatorio , Periodo Preoperatorio , Calidad de Vida , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
Between 1996 and 2003 six institutions in the United States and France contributed a consecutive series of 234 fractures of the femur in 229 children which were treated by titanium elastic nailing. Minor or major complications occurred in 80 fractures. Full information was available concerning 230 fractures, of which the outcome was excellent in 150 (65%), satisfactory in 57 (25%), and poor in 23 (10%). Poor outcomes were due to leg-length discrepancy in five fractures, unacceptable angulation in 17, and failure of fixation in one. There was a statistically significant relationship (p = 0.003) between age and outcome, and the odds ratio for poor outcome was 3.86 for children aged 11 years and older compared with those below this age. The difference between the weight of children with a poor outcome and those with an excellent or satisfactory outcome was statistically significant (54 kg vs 39 kg; p = 0.003). A poor outcome was five times more likely in children who weighed more than 49 kg.
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Clavos Ortopédicos , Fracturas del Fémur/cirugía , Complicaciones Posoperatorias/etiología , Titanio , Adolescente , Factores de Edad , Análisis de Varianza , Peso Corporal/fisiología , Niño , Preescolar , Femenino , Fijación de Fractura/efectos adversos , Humanos , Masculino , Diseño de Prótesis , Factores de Tiempo , Resultado del TratamientoRESUMEN
BACKGROUND: The place of pelvic osteotomy in reconstructing bladder/cloacal exstrophy has been debated for some time; the experience with 'combined' osteotomy in primary and re-operative exstrophy closure at this institution is presented, with a discussion of the historical and scientific place of osteotomy in managing this condition. PATIENTS AND METHODS: Sixty-eight patients had bilateral vertical and transverse iliac osteotomy between 1992 and 2003, and with outcome data available. Of 58 patients with classic exstrophy, eight were newborns, eight were deliberately delayed primary closures, 36 were re-operative after previous failed closure and six were bladder neck reconstructions where the bladder outlet was very wide, such that bony closure was felt necessary for successful bladder neck coaptation. Of 10 patients with cloacal exstrophy, nine were primary closures and one was a re-operative closure. Data were collected relating to age at closure, complications and continence outcome. RESULTS: The mean (range) age (months) was 41 (5-179) for re-operative closures, 12.5 (3-32) for delayed primary closures, 64.1 (38-79) for bladder neck reconstruction, 51.4 (6-165) for cloacal exstrophy closure, and 15 (2-45) days for newborn exstrophy closure. There was a superficial wound infection in two patients, pin-site infection in one, loose pins in two, and two had transient femoral nerve palsy. In two patients the procedure failed and they required further re-operative closure with osteotomy. Sixteen patients are dry urethrally day and night, 12 have had and four are awaiting bladder augmentation, one has a colon conduit, and 35 are awaiting a definitive continence procedure. CONCLUSIONS: Osteotomy has a proven track record in the field of exstrophy reconstruction, and the benefit especially in re-operative closure is emphasized by the present results. The surgical morbidity with the 'combined osteotomy' is low, cosmetic results are excellent and the effect on success of closure is clearly advantageous.
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OBJECTIVES: To provide a complete look at the bony pelvis in children with classic bladder exstrophy: dimensions, orientation, and relationships. METHODS: Three-dimensional computed tomography was used in 6 boys and 1 girl, 5 of whom underwent primary closure and 2 who underwent reclosure at 4 and 8 months. These exstrophy pelves (intrapelvic angles and osseous dimensions) were compared with 26 age and sex-matched controls. RESULTS: The iliac wing angle was 11.4 degrees larger in the classic bladder exstrophy cases. The sacroiliac joint angle was 9.9 degrees more externally rotated in the exstrophy cases. The pelvis was rotated 14.7 degrees in the superoinferior plane in the exstrophy cases. The mean pubic diastasis was 4.2 cm (0.6 cm in controls). The inter-triradiate distance in the patients with classic bladder exstrophy averaged 6.0 cm (4.2 cm in controls). CONCLUSIONS: These new findings provide a better understanding of the bony pelvis, especially its posterior portion, in patients with classic bladder exstrophy. The results of this study revealed the orientation of the sacroiliac joints to be more externally oriented than previously thought and the pelvis to be rotated inferiorly, a previously unknown observation. Both of these factors will be important in the planning of newer osteotomies and pelvic reconstruction.
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Extrofia de la Vejiga/diagnóstico por imagen , Huesos Pélvicos/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Adulto , Extrofia de la Vejiga/cirugía , Estudios de Casos y Controles , Cóccix/diagnóstico por imagen , Cóccix/patología , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Huesos Pélvicos/patología , Hueso Púbico/diagnóstico por imagen , Hueso Púbico/patología , Rotación , Articulación Sacroiliaca/diagnóstico por imagen , Articulación Sacroiliaca/patología , Sacro/diagnóstico por imagen , Sacro/patologíaRESUMEN
Idiopathic scoliosis is a highly prevalent disorder, familial in nature, with marked clinical variability. The purpose of this study was to characterize idiopathic scoliosis in a large series of families to be used for a genome-wide search. One hundred thirty-one multigenerational families (892 individuals) with at least two affected individuals were studied. Data obtained included curve pattern, treatment, and back pain. Maximum curvature as a continuous variable was evaluated using t tests for dichotomous characteristics and linear correlation for continuous variables. An analysis of familial loading was done. Four hundred forty-four individuals were classified as affected (82% female; 18% male). The right thoracic and left lumbar curves had the highest mean curvature (49 degrees). Mean curve size was greater in individuals with back pain. Back pain was most prevalent in the right thoracic and left lumbar curve pattern. The Pearson correlation coefficient between the number of affected family members and the maximum degree of curvature was 0.16, suggesting that the greater the lateral curvature, the higher the proportion of family members affected with scoliosis. The sample population is consistent with those of previous studies in relation to gender and curve size. Statistically, the familial nature of this disorder is supported.
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Escoliosis/genética , Fenómenos Biomecánicos , Femenino , Humanos , Masculino , Linaje , Escoliosis/fisiopatologíaRESUMEN
The cauda equina syndrome in ankylosing spondylitis (the CES-AS syndrome) is marked by slow, insidious progression and a high incidence of dural ectasia in the lumbosacral spine. A high index of suspicion for this problem must be maintained when evaluating the patient with ankylosing spondylitis with a history of incontinence and neurologic deficit on examination. There has been disagreement in the literature as to whether surgical treatment is warranted for this condition. A meta-analysis was thus performed comparing outcomes with treatment regimens. Our results suggest that leaving these patients untreated or treating with steroids alone is inappropriate. Nonsteroidal antiinflammatory drugs may improve back pain but do not improve neurologic deficit. Surgical treatment of the dural ectasia, either by lumboperitoneal shunting or laminectomy, may improve neurologic dysfunction or halt the progression of neurologic deficit.
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Polirradiculopatía/cirugía , Espondilitis Anquilosante/cirugía , Adulto , Antiinflamatorios no Esteroideos/uso terapéutico , Humanos , Modelos Logísticos , Región Lumbosacra/cirugía , Masculino , Oportunidad Relativa , Polirradiculopatía/tratamiento farmacológico , Traumatismos de la Médula Espinal/diagnóstico , Traumatismos de la Médula Espinal/cirugía , Espondilitis Anquilosante/tratamiento farmacológico , Resultado del TratamientoRESUMEN
PURPOSE: We present the pelvic floor anatomy of the major pelvic floor musculature in classic bladder exstrophy, including the levator ani, obturator internus and obturator externus. By improving our knowledge of pelvic floor anatomy we hope to understand better the relationship of the pelvic floor to the bony anatomy as well as the role of osteotomy in changing pelvic floor anatomy to enhance urinary control after surgery. MATERIALS AND METHODS: 3-Dimensional computerized tomography was done in 6 boys and 1 girl, including 5 patients 2 days to 5 months old (mean age 7 months) undergoing primary closure and 2 who were 4 and 8 years old undergoing repeat closure. The pelvic floor musculature, including the levator ani, obturator internus and obturator externus, in these cases was compared to that in 26 age and sex matched controls. RESULTS: The levator ani musculature encompasses a significantly wider area of 9.5 cm.2 in patients with classic bladder exstrophy than in controls. The anterior segment of the levator ani was shorter (1.2 cm.) and the posterior segment of the levator ani was longer (2.5 cm.) than in controls. The degree of divergence of the levator ani in classic exstrophy was significantly more outwardly rotated (38.8 degrees) than controls. In addition, the transverse diameter of the levator hiatus was 2-fold that in our control group and in that of published controls, while the length of the hiatus was 1.3-fold that in normal controls. There was also significant flattening, involving a 31.7 degree decrease in steepness between the right and left halves of the levator ani, of the puborectal sling in classic bladder exstrophy versus controls. Because of these findings, there is more anterior superior rotation in the pelvic floor in exstrophy cases. The obturator internus was more outwardly rotated (15.1 degrees) in exstrophy and the obturator externus also showed more outward rotation (16.9 degrees) than in controls. CONCLUSIONS: This study provides better understanding of the pelvic floor anatomy in classic bladder exstrophy. Significant differences have been documented in the pelvic floor in classic bladder exstrophy cases and controls. Hopefully these differences may have a pivotal role in providing new insight into long-term issues, such as urinary and fecal incontinence, and pelvic organ prolapse, in classic bladder exstrophy.
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Extrofia de la Vejiga/diagnóstico por imagen , Extrofia de la Vejiga/patología , Diafragma Pélvico/diagnóstico por imagen , Diafragma Pélvico/patología , Tomografía Computarizada por Rayos X , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Tomografía Computarizada por Rayos X/métodosRESUMEN
Stickler syndrome is an autosomal dominant connective tissue disorder with a prevalence similar to that of Marfan syndrome. No previous study has examined hip pain or abnormalities in a large series of patients with Stickler syndrome. The purpose of this study was to describe hip abnormalities and their correlation with age and chronic hip pain in a cohort of 51 patients followed at the National Institutes of Health. Ten percent of patients had protrusio acetabuli, 21% coxa valga, and 34% of adults had hip osteoarthritis. Sixty-three percent of all patients and 79% of adults had chronic hip pain. In addition, 16% of adult patients had a history of femoral head failure during youth. Arthritic changes and adult age were associated with hip pain. In summary, hip abnormalities are commonly observed in Stickler syndrome. Young patients require careful evaluation of hip pain, and regular screening of children with Stickler syndrome may be indicated for early detection of hip complications.
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Anomalías Múltiples , Enfermedades del Tejido Conjuntivo , Articulación de la Cadera , Dolor/etiología , Adulto , Distribución por Edad , Enfermedades del Desarrollo Óseo/diagnóstico por imagen , Enfermedades del Desarrollo Óseo/etiología , Enfermedades del Desarrollo Óseo/genética , Enfermedades del Tejido Conjuntivo/diagnóstico por imagen , Enfermedades del Tejido Conjuntivo/epidemiología , Enfermedades del Tejido Conjuntivo/genética , Femenino , Pérdida Auditiva Sensorineural/etiología , Articulación de la Cadera/diagnóstico por imagen , Articulación de la Cadera/patología , Humanos , Masculino , Osteoartritis de la Cadera/diagnóstico por imagen , Osteoartritis de la Cadera/etiología , Radiografía , Enfermedades de la Retina/genética , SíndromeRESUMEN
OBJECTIVE: To describe two cases in which intraoperative monitoring of neurogenic 'motor' evoked potentials (NMEPs) did not identify a spinal cord injury that resulted in paraplegia. METHODS: Bilateral tibial nerve somatosensory evoked potential (SEP) and NMEP testing was performed in two patients during spinal deformity corrective surgery using standard stimulation and recording parameters. These potentials were obtained repetitively throughout the primary procedures and were performed again during a subsequent procedure that took place after the discovery of paraplegia. RESULTS: SEP and NMEP signals were preserved in both patients and no adverse events were identified during the initial procedures. Postoperatively, paraplegia was identified immediately upon recovery from anesthesia and preserved posterior column function was apparent on clinical exam. In the procedures following the discovery of paraplegia, SEP and NMEP signals remained comparable with signals elicited in the initial surgeries. CONCLUSIONS: Based on these cases and previously published experimental evidence, we conclude that while 'NMEPs' remain a useful second test of spinal cord function, they are not reliable indicators of motor tract function. An alternate term, such as 'spinally-elicited peripheral nerve responses' should be used.
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Potenciales Evocados Somatosensoriales/fisiología , Neuronas Motoras/patología , Paraplejía/diagnóstico , Traumatismos de la Médula Espinal/fisiopatología , Adulto , Niño , Reacciones Falso Negativas , Femenino , Humanos , Monitoreo Intraoperatorio , Actividad Motora/fisiología , Neuronas Motoras/fisiología , Valor Predictivo de las Pruebas , Nervio Tibial/fisiologíaRESUMEN
OBJECTIVE: To determine how well conventional radiographic findings can predict the presence of dural ectasia in Marfan patients. DESIGN AND PATIENTS: Twelve Marfan patients without dural ectasia and 21 Marfan patients with dural ectasia were included in the study. Five radiographic measurements were made of the lumbosacral spine: interpediculate distance, scalloping value, sagittal canal diameter, vertebral body width, and transverse process width. RESULTS: The following measurements were significantly larger in patients with dural ectasia: interpediculate distances at L3-L4 levels (P<0.03); scalloping values at the L1 and L5 levels (P<0.05); sagittal diameters of the vertebral canal at L5-S1 (P<0.03); transverse process to width ratios at L2 (P<0.03). Criteria were developed for diagnosis of dural ectasia in Marfan patients. These included presence of one of the following: interpediculate distance at L4 > or = 38.0 mm, sagittal diameter at S1 > or = 18.0 mm, or scalloping value at L5 > or = 5.5 mm. CONCLUSION: Dural ectasia in Marfan syndrome is commonly associated with several osseous changes that are observable on conventional radiographs of the lumbosacral spine. Conventional radiography can detect dural ectasia in patients with Marfan syndrome with a very high specificity (91.7%) but a low sensitivity (57.1%).
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Duramadre/diagnóstico por imagen , Síndrome de Marfan/diagnóstico por imagen , Adulto , Dilatación Patológica/diagnóstico por imagen , Duramadre/patología , Femenino , Humanos , Vértebras Lumbares , Masculino , Síndrome de Marfan/patología , Persona de Mediana Edad , Radiografía , SacroRESUMEN
32 patients with Marfan syndrome, diagnosed with DePaepe's criteria, volunteered for this study. All patients underwent standard anteroposterior radiographs of the lumbar spine. Interpediculate distances (IPD) at each level were compared to those of previously established norms. Criteria were developed to determine the presence of Marfan using "cut-off values" for the IPDs at each lumbar level. The IPDs were significantly larger in the Marfan patients at all lumbar levels. Cut-off values were calculated setting the specificity at 95% at each lumbar level. The cut-off value at L4 yielded the greatest sensitivity. We conclude that the IPDs are widened in patients with Marfan syndrome. The IPD at L4 is a good criterion for Marfan with specificity of 95% and sensitivity of 75%. One must consider using this as a skeletal criterion or a screening tool for Marfan.
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Antropometría/métodos , Vértebras Lumbares/diagnóstico por imagen , Vértebras Lumbares/patología , Síndrome de Marfan/diagnóstico por imagen , Síndrome de Marfan/patología , Adulto , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Síndrome de Marfan/complicaciones , Tamizaje Masivo/métodos , Tamizaje Masivo/normas , Persona de Mediana Edad , Radiografía , Valores de Referencia , Sensibilidad y Especificidad , Espondilolistesis/etiologíaRESUMEN
BACKGROUND: Classic bladder exstrophy is a developmental defect presenting at birth with a wide pubic separation and an exposed bladder; cloacal exstrophy involves, in addition, intestinal prolapse. Reconstruction requires several surgical procedures. The use of anterior iliac osteotomies in this process has not been reviewed in a large series. METHODS: We reviewed the results of eighty-six anterior innominate osteotomies performed in conjunction with genitourinary repair of classic and cloacal bladder exstrophy in eighty-two patients. Clinical outcome measures were successful bladder closure, achievement of continence, and maintenance of a normal gait. Radiographs of the pelvis were reviewed, and the pubic intersymphyseal diastasis (a measure of the reduction in tension on the anterior closure) was measured preoperatively and at three time-points postoperatively. Children with classic exstrophy who had undergone osteotomy and bladder neck reconstruction but not bladder augmentation were divided into four groups on the basis of the degree of continence. In addition, children with classic exstrophy were stratified according to age at the time of the osteotomy. The mean postoperative percent reduction in the amount of the original diastasis was determined for all groups. RESULTS: Children with classic exstrophy and those with cloacal exstrophy had correction of the diastasis after the osteotomy, with greater correction in those with classic exstrophy, presumably because of better bone quality. Daytime continence was achieved with anterior osteotomy and bladder neck reconstruction in 74% of the children for whom continence was a goal. However, no difference in the symphyseal diastasis or in the percentage of pubic reduction was detected among the four continence groups. Children who were older at the time of the osteotomy maintained better correction over time. Wound dehiscence or bladder prolapse occurred in 4% of the patients who had osteotomy and primary closure, and the only important complication of the osteotomies was transient palsy of the left femoral nerve in seven children. CONCLUSIONS: Anterior innominate osteotomy is an effective part of reconstructive repair of bladder exstrophy. The primary goals of the osteotomy are to reduce the tension in the closed bladder and the lower abdominal wall and to promote continence by restoring the sling of the pelvic floor muscles. These goals can be achieved in the majority of patients.
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Extrofia de la Vejiga/cirugía , Ilion/cirugía , Osteotomía/métodos , Huesos Pélvicos/anomalías , Adolescente , Factores de Edad , Extrofia de la Vejiga/complicaciones , Extrofia de la Vejiga/diagnóstico por imagen , Niño , Preescolar , Femenino , Marcha , Humanos , Ilion/anomalías , Ilion/diagnóstico por imagen , Lactante , Recién Nacido , Masculino , Huesos Pélvicos/diagnóstico por imagen , Huesos Pélvicos/cirugía , Complicaciones Posoperatorias , Sínfisis Pubiana/anomalías , Sínfisis Pubiana/diagnóstico por imagen , Sínfisis Pubiana/cirugía , Radiografía , Estudios Retrospectivos , Incontinencia Urinaria/etiología , Incontinencia Urinaria/cirugíaRESUMEN
STUDY DESIGN: Retrospective review of clinical and radiographic records of patients with Stickler syndrome. OBJECTIVES: To describe thoracolumbar spinal abnormalities and their correlation with age and back pain among patients with Stickler syndrome. SUMMARY OF BACKGROUND DATA: Stickler syndrome (hereditary arthro-ophthalmopathy) is an autosomal dominant connective tissue disorder characterized by skeletal, ocular, oral-facial, cardiac, and auditory manifestations. Prevalence is approximately 1 in 10,000 (similar to that of Marfan syndrome). No one has investigated spinal abnormalities in a large series of patients. METHODS: A single-center evaluation of 53 patients from 24 families with Stickler syndrome (age range, 1-70 years) in a multidisciplinary genetics clinic. Thoracolumbar radiographs were analyzed for spinal abnormalities and correlation with age and back pain. RESULTS: Thirty-four percent of patients had scoliosis, 74% endplate abnormalities, 64% Schmorl's nodes, 43% platyspondylia, and 43% Scheuermann-like kyphosis. Sixty-seven percent of patients and 85% of adults reported chronic back pain. Endplate abnormalities and Schmorl's nodes were associated with adult age; endplate abnormalities, Schmorl's nodes, and adult age were associated with back pain. Only one adult patient was free of spinal abnormalities. CONCLUSIONS: Spinal abnormalities are nearly uniformly observed in Stickler syndrome, progress with age, and are associated with back pain. Although common, scoliosis is generally self-limited (only one patient needed surgical treatment). Correct diagnosis of this syndrome facilitates early identification and management of other potentially severe systemic manifestations and genetic counseling for affected families. Moreover, recognition of Stickler syndrome allows accurate prognosis for skeletal abnormalities and anticipation of potential surgical complications.
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Anomalías Congénitas/patología , Vértebras Lumbares/anomalías , Escoliosis/etiología , Escoliosis/patología , Vértebras Torácicas/anomalías , Adolescente , Adulto , Anciano , Niño , Preescolar , Anomalías Congénitas/diagnóstico por imagen , Anomalías del Ojo/etiología , Anomalías del Ojo/patología , Cara/anomalías , Femenino , Humanos , Lactante , Vértebras Lumbares/diagnóstico por imagen , Vértebras Lumbares/patología , Masculino , Persona de Mediana Edad , Radiografía , Escoliosis/diagnóstico por imagen , Cráneo/anomalías , Vértebras Torácicas/diagnóstico por imagen , Vértebras Torácicas/patologíaRESUMEN
Rhizomelic chondrodysplasia punctata (RCDP) is a rare peroxisomal disorder leading to multiple developmental malformations, including skeletal deformity. Specifically, involvement of the vertebral bodies has been described. Presented here is a case of a two-year-old female child with RCDP leading to advanced cervical stenosis as detected on magnetic resonance imaging (MRI) studies of the cervical spine. The practicing clinician should be aware of the possibility of cervical stenosis secondary to RCDP and its impact on the management of the patient with this rare disease process.
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Condrodisplasia Punctata Rizomélica/complicaciones , Estenosis Espinal/etiología , Vértebras Cervicales/patología , Preescolar , Condrodisplasia Punctata Rizomélica/patología , Femenino , Humanos , Imagen por Resonancia Magnética , Canal Medular/patología , Estenosis Espinal/patologíaRESUMEN
STUDY DESIGN: This study examines pedicle widths, laminar thicknesses, and scalloping values for lumbosacral spine elements in Marfan volunteers. Comparisons were made between these measurements and norms as well as measurements between Marfan patients with and without dural ectasia. OBJECTIVES: To determine if the lumbosacral vertebral elements are altered in the patient with Marfan syndrome. SUMMARY OF BACKGROUND DATA: Several abnormalities have been noted in Marfan lumbar spine, including pedicular attenuation and widened interpediculate distances. This may be due to abnormalities of growth or presence of dural ectasia. Given the large numbers of Marfan patients requiring spinal surgery and the high postoperative failure rate, better understanding of the bony anatomy of Marfan lumbar spine is necessary, especially if use of instrumentation is anticipated. METHODS: Thirty-two volunteers with Marfan syndrome based on the Ghent criteria underwent spiral computed tomography of the lumbosacral spine. Images were evaluated for dural ectasia, and measurements of pedicle width, laminar thickness, and vertebral scalloping were made. RESULTS: Pedicle widths and laminar thicknesses were significantly smaller in Marfan patients at all levels (P<0.001). Mean pedicle widths at L1-L3 were smaller than the smallest available pedicle screw (5 mm). In Marfan patients with dural ectasia, laminar thickness from L5-S2 and pedicle widths at all lumbar levels were significantly reduced (P<0.01). Vertebral scalloping at S1 was significantly greater in Marfan patients with dural ectasia (P = 0.02). CONCLUSION: Lumbar pedicle width and laminar thickness are significantly reduced in Marfan individuals. Those with dural ectasia demonstrate increased bony erosion of anterior and posterior elements of lumbosacral spine. Preoperative planning and routine computed tomography scans are recommended when operating on Marfan lumbosacral spine.