Endoscopic third ventriculostomy in children with chronic communicating congenital hydrocephalus: a single-center cohort retrospective analysis.

OBJECTIVE: The aim of this study was to analyze the role of endoscopic third ventriculostomy (ETV) in the treatment of pediatric chronic communicating congenital hydrocephalus (CCCH). MATERIAL AND METHODS: This retrospective study comprised a series of 11 children with CCCH treated with ETV. Data were recorded on gender, history, presenting symptoms, age at surgery, complications during surgery, clinical evolution, ETV survival, and follow-up period. Radiological variables including ventricular and cephalic diameters were also recorded to determine a series of ventricular indexes in magnetic resonance imaging (MRI) before and after the ETV procedure. The procedure was considered to be successful when there was clinical stability or improvement accompanied by a reduction in the radiological indexes in the postoperative control images, such that there was no need to place an extrathecal cerebrospinal fluid shunt. RESULTS: Over a mean follow-up period of 35.8 months (range: 6-108 months) from the ETV procedure, three patients required shunt placement; one of these was due to early failure in an 8-month old girl, the only patient younger than 12 months in our series. The radiological indexes were reduced in all patients except for one of the cases of ETV failure. The mean ETV survival among the successful cases was 32.1 months (range: 6-108 months), whilst that of the failed cases was 16 months (range: 6-108 months). CONCLUSION: Although studies with larger sample sizes are needed, ETV appears to be a promising option for the treatment of this type of patient with CCCH.

Cervical myelomeningocele with CSF leakage: a case-based review.

BACKGROUND: The cystic spinal dysraphism of the cervical and upper thoracic region (CDCT) accounts for only 3.9 to 8% of spina bifida cystica lesions. The presence of external cerebrospinal fluid (CSF) leakage is infrequent and very few authors have reported about surgical complications. CASE REPORT: We present the case of a new born diagnosed of CDCT C1-C3 with a stalk of fibrovascular tissue, CSF leakage, hydrocephalus, and type Chiari II malformation, discuss about the chosen surgical technique and the associated complications, and make a review of the literature focusing on the main aspects of CDCT. CONCLUSIONS: The CDCT with a stalk of neuroglial and/or fibrovascular tissue originates from the dorsal surface of the spinal cord and penetrates into a cervical cystic sac. The presence of CSF leakage, hydrocephalus, and/or type Chiari II malformation can influence to decide the most appropriate surgical technique.

Suprasellar ganglioglioma presenting as a middle cerebral artery infarction.

BACKGROUND: Gangliogliomas are a relatively rare neoplasm with a major incidence in the pediatric population. As the temporal lobes are the most common site, patients usually present with seizures. CASE REPORT: We report the case of a 9-year-old child with an extensive suprasellar ganglioglioma presenting with a cerebral infarction due to direct compression of the medium cerebral artery. CONCLUSIONS: Suprasellar lesions can compress adjacent vascular structures, so an accurate diagnosis is necessary for an early treatment.

Perianal Pediatric Crohn Disease Is Associated With a Distinct Phenotype and Greater Inflammatory Burden.

OBJECTIVES: Data on the outcomes of children with perianal Crohn disease (pCD) are limited, although its presence is often used for justifying early use of biologics. We aimed to assess whether pCD in children is associated with more severe outcomes as found in adults. METHODS: Data were extracted from the ImageKids database, a prospective, multicenter, longitudinal cohort study. The study enrolled 246 children at disease onset or thereafter. All patients underwent comprehensive clinical, endoscopic, and radiologic evaluation at enrollment; 98 children had repeat evaluation at 18 months. RESULTS: Of the 234 included patients (mean age 14.2 ±â€Š2.4 years; 131 [56%] boys), 57 (24%) had perianal findings, whereas only 21 (9%) had fistulizing perianal disease. Children with pCD had reduced weight and height z scores compared with non-pCD patients (-0.9 vs -0.35, P = 0.03 and -0.68 vs -0.23, respectively; P = 0.04), higher weighted pediatric CD activity index (32 [interquartile range 16-50] vs 20 [8-37]; P = 0.004), lower serum albumin (3.6 ±â€Š0.7 vs 4.5 ±â€Š0.8, P = 0.016), and higher magnetic resonance enterography global inflammatory score (P = 0.04). Children with pCD had more rectal (57% vs 38%, P = 0.04), and jejunal involvement (31% vs 11% P = 0.003) and a higher prevalence of granulomas (64% vs 23%, P = 0.0001). Magnetic resonance enterography-based damage scores did not differ between groups. Patients with skin tags/fissures only, had similar clinical, endoscopic, and radiologic characteristics as patients with no perianal findings. CONCLUSIONS: Pediatric patients with pCD with fistulizing disease have distinct phenotypic features and a predisposition to a greater inflammatory burden.

Increased levels of tumour necrosis factor alpha (TNFα) but not transforming growth factor-beta 1 (TGFß1) are associated with the severity of congenital hydrocephalus in the hyh mouse.

AIMS: Here, we tested the hypothesis that glial responses via the production of cytokines such as transforming growth factor-beta 1 (TGFß1) and tumour necrosis factor alpha (TNFα), which play important roles in neurodegenerative diseases, are correlated with the severity of congenital hydrocephalus in the hyh mouse model. We also searched for evidence of this association in human cases of primary hydrocephalus. METHODS: Hyh mice, which exhibit either severe or compensated long-lasting forms of hydrocephalus, were examined and compared with wild-type mice. TGFß1, TNFα and TNFαR1 mRNA levels were quantified using real-time PCR. TNFα and TNFαR1 were immunolocalized in the brain tissues of hyh mice and four hydrocephalic human foetuses relative to astroglial and microglial reactions. RESULTS: The TGFß1 mRNA levels were not significantly different between hyh mice exhibiting severe or compensated hydrocephalus and normal mice. In contrast, severely hydrocephalic mice exhibited four- and two-fold increases in the mean levels of TNFα and TNFαR1, respectively, compared with normal mice. In the hyh mouse, TNFα and TNFαR1 immunoreactivity was preferentially detected in astrocytes that form a particular periventricular reaction characteristic of hydrocephalus. However, these proteins were rarely detected in microglia, which did not appear to be activated. TNFα immunoreactivity was also detected in the glial reaction in the small group of human foetuses exhibiting hydrocephalus that were examined. CONCLUSIONS: In the hyh mouse model of congenital hydrocephalus, TNFα and TNFαR1 appear to be associated with the severity of the disease, probably mediating the astrocyte reaction, neurodegenerative processes and ischaemia.

Development and Validation of a Pediatric MRI-Based Perianal Crohn Disease (PEMPAC) Index-A Report from the ImageKids Study.

BACKGROUND: As part of the prospective multicenter ImageKids study, we aimed to develop and validate the pediatric MRI-based perianal Crohn disease (PEMPAC) index. METHODS: Children with Crohn disease with any clinical perianal findings underwent pelvic magnetic resonance imaging at 21 sites globally. The site radiologist and 2 central radiologists provided a radiologist global assessment (RGA) on a 100 mm visual analog scale and scored the items selected by a Delphi group of 35 international radiologists and a review of the literature. Two weighted multivariable statistical models were constructed against the RGA. RESULTS: Eighty children underwent 95 pelvic magnetic resonance imaging scans; 64 were used for derivation and 31 for validation. The following items were included: fistula number, location, length and T2 hyperintensity; abscesses; rectal wall involvement; and fistula branching. The last 2 items had negative beta scores and thus were excluded in a contending basic model. In the validation cohort, the full and the basic models had the same strong correlation with the RGA (r = 0.75; P < 0.01) and with the adult Van Assche index (VAI; r = 0.93 and 0.92; P < 0.001). The correlation of the VAI with the RGA was similar (r = 0.77; P < 0.01). The 2 models and the VAI had a similar ability to differentiate remission from active disease (area under the receiver operating characteristic curve, 0.91-0.94). The PEMPAC index had good responsiveness to change (area under the receiver operating characteristic curve, 0.89; 95% confidence interval, 0.69-1.00). CONCLUSIONS: Using a blended judgmental and mathematical approach, we developed and validated an index for quantifying the severity of perianal disease in children with CD. The adult VAI may also be used with confidence in children.

Central Venous Sinus Thrombosis in a Boy With Acute Severe Ulcerative Colitis.

Cerebral venous sinus thrombosis (CVST) in childhood is uncommon. Certain diseases predispose patients to CVST, such as inflammatory bowel disease (IBD), which is considered a risk factor for developing thrombosis, which in turn is considered an extraintestinal manifestation of IBD. The use of prophylaxis in certain patients is a controversial topic. We present the case of a 5-years-old child with ulcerative colitis, who presented with transverse sinus thrombosis immediately after colectomy. Considering the recent recommendations on prophylaxis in this disease, our patient and probably many others would benefit from establishing treatment with low-molecular-weight heparin. We believe that these recommendations should be known, with our case serving as an example, given that we are heading in a direction that has so far been controversial.

A Simple Endoscopic Score Modified for the Upper Gastrointestinal Tract in Crohn's Disease [UGI-SES-CD]: A Report From the ImageKids Study.

OBJECTIVE: There is no standardized endoscopic description of upper gastrointestinal [UGI] disease in Crohn's disease [CD]. We prospectively applied the Simple Endoscopic Score for CD [SES-CD] to the UGI tract as a planned sub-study of the multicentre prospective ImageKids study. We aimed to assess the utility of the UGI-SES-CD and its clinical significance in paediatric CD. DESIGN: Patients underwent an oesophagogastroduodenoscopy [EGD], ileocolonoscopy, and magnetic resonance enterography [MRE] with explicit clinical data recorded. SES-CD was scored at each region [oesophagus, stomach body, antrum, and duodenum]. Half of the patients were followed for 18 months, when a repeat MRE was performed. RESULTS: A total of 202 children were included 56% males, mean age 11.5 ± 3.2 years, median weighted Paediatric Crohn's Disease Activity Index [wPCDAI 25]). UGI-SES-CD score ranged 0-17, with 95 [47%] having a UGI-SES-CD ≥1; no narrowing was detected. UGI-SES-CD ≥1 was associated with higher: wPCDAI [32.5 vs 20; p = 0.03]; Physician's Global Assessment [PGA] of inflammation (45 mm visual analogue score [VAS] vs 30 mm VAS; p = 0.04); ileocolonoscopic SES-CD [10 vs 7; p = 0.004], faecal calprotectin [717 µg/g vs 654 µ/g; p= 0.046]; and radiological global assessment of damage by MRE [7 mm VAS vs 0; p = 0.04]. In all, 81 patients were followed for 18 months and no association was identified between initial UGI SES-CD and markers of disease course such as surgery, MRE assessment, or treatment escalation. CONCLUSION: UGI-SES-CD is an easily reported objective scoring system and is associated with a more severe disease phenotype but not with disease course.

A Distinct Metabolite Profile Correlates with Neurodegenerative Conditions and the Severity of Congenital Hydrocephalus.

In congenital hydrocephalus, cerebrospinal fluid accumulation is associated with increased intracranial pressure (ICP), ischemia/hypoxia, metabolic impairment, neuronal damage, and astrocytic reaction. The aim of this study was to identify whether a metabolite profile revealing tissue responses according to the severity of hydrocephalus can be detected. The hyh mutant mouse used for this study exhibits 2 different forms of hydrocephalus, severe and moderate. In a comprehensive investigation into the 2 progressions of hydrocephalus, mice with severe hydrocephalus were found to have higher ICP and astrocytic reaction. Several metabolites from the mouse brain cortex were analyzed with 1H high-resolution magic angle spinning nuclear magnetic resonance (1H HR-MAS NMR) spectroscopy. A differential profile for metabolites including glutamate and glutamine was found to correlate with the severity of hydrocephalus and can be explained due to differential astrocytic reactions, neurodegenerative conditions, and the presence of ischemia. The glutamate transporter EAAT2 and the metabolite taurine were found to be key histopathological markers of affected parenchymata. In conclusion, a differential metabolite profile can be detected according to the severity of hydrocephalus and associated ICP and therefore can be used to monitor the efficacy of experimental therapies.

Intracystic gadolinium-enhanced MRI in the evaluation of residual giant-cystic craniopharyngiomas in children: report of four cases.

Residual giant-cystic craniopharyngiomas are amenable to intracavitary bleomycin treatment. Radiologic identification of potential cyst leaks is of paramount for treatment decisions. This report describes our experience in the use of intracystic Gadolinium (Gd)-enhanced MR imaging to determine potential communications between the tumoral cysts and other intra-axial spaces in 4 pediatric patients with residual giant-cystic craniopharyngiomas in whom intracavitary bleomycin treatment was planned after the injection of .1-.2 mL of gadopentetate dimeglumine (Gd-DTPA). In three cases no leaks were found. In one case, whose previous water-soluble iodinated contrast-enhanced CT cystography was negative for leaks, intracystic Gd-enhanced MR showed intraventricular Gd enhancement. We conclude that MR imaging after intracystic administration of Gd-based contrast paramagnetic agents is useful in the detection of potential leaks in cases of giant residual craniopharyngiomas.

Calidad de vida relacionada con la salud en trabajadores sanitarios / Health related quality of life in health workers

El estado de bienestar en que vivimos en los países desarrollados hace que surja la necesidad de buscar herramientas que sean una imagen fiel del estado de salud de la población como es la medición de la calidad de vida relacionada con la salud. El objetivo de nuestro estudio es conocer la calidad de vida relacionada con la salud (CVRS) de los trabajadores sanitarios como medida de su estado de salud. Para ello hemos realizado un estudio transversal, prospectivo y descriptivo de una muestra de 542 trabajadores seleccionada por muestreo sistemático. Como variable dependiente se evaluó la CVRS a través del cuestionario SF-36. Este cuestionario evalúa el estado de salud a través de 7 escalas (Función física, rol físico, salud general, vitalidad, función social, rol emocional y salud mental) valoradas de 0 a 100 (siendo 100 el mejor valor) y de dos dominios globales estandarizados para población general siendo su valor 50, (índice general de salud física e índice general de salud mental). Se tiene en cuenta la edad y el sexo para el análisis de los resultados. Los resultados más relevantes obtenidos son: Edad media 46 años (DE 10,4), 82% mujeres. Función física: 88.7 (Población general-PG-: 84.7); Rol físico: 86.5 (PG: 83.2); Dolor: 71.9 (PG: 79); Salud general: 70 (PG: 68.3); Vitalidad: 66.1 (PG: 66.9); Función social: 86.4 (PG: 90.1); Rol emocional: 87.8 (PG: 88.6); Salud mental: 72.6 (PG: 73). Índice global de salud física: 51.9; Índice global de salud mental: 49.8. Los valores encontrados tanto para las escalas de salud física como mental son ligeramente mejores que los establecidos para la población general para el mismo rango de edad y sexo, salvo en la escala de dolor (AU)

The welfare state in whom we live in the developed countries does that there arises the need to look for tools that are a faithful image of the health of the population such as it is the measurement of the quality of life related to the health. The aim of our study is to know the health related quality of life (HRQOL) of the sanitary workers as measure of their health status. For it we have realized a cross-sectional, prospective and descriptive study of a sample of 542 workers selected by systematic sampling. The dependent variable was assessed HRQOL by the SF-36. This questionnaire assesses the health status across 7 scales (physical function, role physical, general health, vitality, social function, emotional role and mental health) from 0 to 100 (100 being the best value) and two global domains standardized for general population value being 50, (general index of physical health, and general index of mental health). It takes into account the age and the sex for the analysis of the results. The most important results obtained are: Middle Age 46 years (SD 10,4), 82 % women. Physical function: 88.7 (PG-general population: 84.7), role physical, 86.5 (PG: 83.2); Pain 71.9 (PG 79), general health: 70 (PG: 68.3); Vitality: 66.1 (PG: 66.9 ), social function: 86.4 (PG: 90.1), Role emotional 87.8 (PG: 88.6); Mental Health: 72.6 (PG 73). Global index of physical health: 51.9; global Index of mental health: 49.8. The values found so much for the scales of physical as mental health are lightly better than the established ones for the general population for the same range of age and sex, except in the scale of pain (AU)

[Liposarcoma of the perirenal cell]. / Liposarcoma de la celda perirrenal.

OBJECTIVES: To show the imaging tests characteristics and differential diagnosis of masses located within the retroperitoneal perirenal area. METHODS: Diagnostic imaging tests (KUB x-ray, ultrasound, helical CT scan with IV contrast) and follow-up in one case of perirenal cell liposarcoma. RESULTS: The study of the surgical specimen after renal tumorectomy in a patient with a solitary kidney resulted in the present diagnosis. CONCLUSIONS: Liposarcoma is a malignant neoplasia derived from fatty tissue. Approximately 13% are located in the retroperitoneal area, and less than 1/3 arise from perinephric fat. We report one case of liposarcoma located in the perirenal cell as an incidental finding during contralateral psoas muscle abscess follow-up in a patient with a solitary kidney after surgery for xanthogranulomatous pyelonephritis. Successive controls during the last year allowed us to evaluate the evolution of this lesion's.

Presentación de un caso de hemicerebelitis pediátrica / Pediatric hemicerebellitis: a case report

La cerebelitis aguda es una de las causas principales de disfución cerebelar aguda en pediatría; puede ser infecciosa, postinfecciosa o posvacunal, y generalmente es de etiología viral. El diagnóstico es difícil porque la clínica presenta sólo algunos de los signos de patología cerebelosa y el líquido cefalorraquídeo puede ser normal. La afectación de un único hemisferio cerebeloso, hemicerebelitis, es muy infrecuente, hay publicados tres casos hasta el año 2003. La resonancia magnética muestra edema cerebeloso unilateral con realce que sigue la morfología de las folias; este patrón es de gran ayuda para el diagnóstico. Aunque es una entidad que se considera benigna, autolimitada y con buena evolución con tratamiento corticoide, a veces es necesaria la biopsia para diferenciarla de patología tumoral

Acute cerebellitis is one of the main causes of acute cerebellar dysfunction in pediatrics. It can be infectious or occur after infection or vaccination; it is usually viral in origin. It is difficult to diagnose because not all of the clinical signs of cerebellar pathology are present and CSF may be normal. Involvement of a single hemisphere of the cerebellum, hemicerebellitis, is very rare: only three cases were published before 2003 (en castellano dice: hasta 2003; si el año 2003 queda incluso, tiene que ser before 2004). MRI shows unilateral cerebellar edema and enhancement along the folia. This pattern is very helpful in the diagnosis. Although this entity is considered to be a benign, self-limiting disorder that responds well to corticosteroid treatment, it is sometimes necessary to differentiate it from tumors

Calcinosis cutánea en una paciente con lupus eritematoso sistémico

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Pielonefritis xantogranulomatosa en la infancia: una entidad rara / Childhood xanthogranulomatous pyelonephritis: an uncommon entity

La pielonefritis xantogranulomatosa es una enfermedad inflamatoria crónica infrecuente, caracterizada por la sustitución de tejido renal por un infiltrado celular de macrófagos cargados de lípidos. En la bibliografía existen pocos casos descritos en niños. La patogenia permanece desconocida, aunque hasta en un 76 por ciento se ha relacionado con obstrucción litiásica de la unión pieloureteral. Los gérmenes implicados con más frecuencia son Escherichia coli y Proteus mirabilis. Dado que ni sus características clínicas ni los hallazgos radiológicos son específicos, con frecuencia es infradiagnosticada. El diagnóstico definitivo es anatomopatológico y el tratamiento, la nefrectomía.Presentamos un caso diagnosticado tras el estudio anatomopatológico de la pieza renal, en un niño con pionefrosis y mala evolución, haciendo hincapié en la importancia de realizar el diagnóstico preoperatorio e incluir esta entidad en el diagnóstico diferencial de procesos obstructivo-infecciosos de tórpida evolución (AU)

Estudio por imagen del desarrollo de tumores linforreticulares en la ataxia telangiectasia y el síndrome de Nijmegen / Imaging study of lymphoreticular tumor development in ataxia-telangiectasia and Nijmegen breakage syndrome

La ataxia telangiectasia de Louis Barr (AT) es una enfermedad autosómica recesiva caracterizada por ataxia cerebelosa progresiva, telangiectasias oculocutáneas, inmunodeficiencia combinada con susceptibilidad a infecciones sinopulmonares y alta incidencia de desarrollo neoplásico. El síndrome de Nijmegen (SNJ) es una variante de la AT, también autosómico recesivo, que presenta ataxia cerebelosa, inmunodeficiencia combinada y tendencia al desarrollo tumoral. A diferencia de la AT no muestra telangiectasias y exhibe un fenotipo característico (talla corta, cara «de pájaro» y microcefalia). Ambas entidades se incluyen dentro de la clasificación de los síndromes con fragilidad o inestabilidad cromosómica que agrupan además al síndrome de Bloom y la anemia de Fanconi. Todos ellos muestran un aumento en la frecuencia de presentación neoplásica, principalmente tumores del sistema linfoide. Presentamos tres pacientes, dos con AT y uno con SNJ, que han desarrollado diferentes tipos de linfoma en el curso de la enfermedad, indicando los aspectos más relevantes desde el punto de vista clinicorradiológico (AU)

Ataxia-telangiectasia (AT), or Louis-Bar syndrome, is an autosomal recessive illness characterized by progressive cerebellar ataxia, oculocutaneous telangiectasias, immunodeficiency combined with susceptibility to sinopulmonary infections and high incidence of neoplastic development. Nijmegen breakage syndrome (NBS) is a variant of AT, is also an autosomal recessive illness that presents cerebellar ataxia, as well as combined immunodeficiency and a tendency toward tumor development. Contrary to Louis-Bar syndrome, it doesn't present telangiectasia and exhibits a characteristic phenotype (short stature, bird-like face and microcephaly). Both entities are classified as syndromes of chromosomal instability or chromosomal fragility, a group which also includes Bloom syndrome and Fanconi anemia. All of these show an increase in the frequency of neoplastic pathologies, mainly lymphoid tumors. We present three patients, two with AT and one with NBS, who developed different lymphoma types in the course of the illness. We highlight the most outstanding aspects from a clinical-radiological point of view (AU)

Colecistitis en un niño con sida: hallazgos ecográficos en una forma infrecuente de presentación en la infancia / Cholecystitis in a child with acquired immunodeficiency syndrome: ultrasonographic findings in a case of uncommon presentation during childhood

Presentamos el caso de un niño con sida en el que se diagnosticó una colecistitis alitiásica con ecografía. La ecografía abdominal fue esencial en el diagnóstico inicial y valoración de la respuesta al tratamiento. Este es un cuadro escasamente descrito en la bibliografía radiológica pediátrica. En nuestro paciente la causa de la colecistitis pudo ser infecciosa, planteando además la participación de la obstrucción linfática regional en la patogenia del cuadro descrito (AU)

Liposarcoma de la celda perirrenal / Perirenal cell liposarcoma

OBJETIVO: Mostrar las características de imagen y el diagnóstico diferencial de las masas localizadas en el área retroperitoneal perirrenal. MÉTODO: Estudio por imagen (RX abdomen, Ecografía, TC helicoidal con contraste intravenoso) diagnóstico y de seguimiento de un caso de liposarcoma de la celda perirrenal. RESULTADO: El estudio de la pieza quirúrgica de la tumerectomía renal en una paciente monorrena dio como resultado el caso presentado. CONCLUSIONES: El liposarcoma es una neoplasia maligna derivada del tejido adiposo; aproximadamente un 13 por ciento se localiza en retroperitoneo, y menos de un tercio se origina en la grasa perinéfrica, constituyendo el liposarcoma perirrenal. Presentamos un caso de liposarcoma localizado en la celda perirrenal como hallazgo incidental del seguimiento de un abceso del psoas contralateral en una paciente monorrena tras cirugía por pielonefritis xantogranulomatosa. Los controles en el último año han permitido valorar la evolución de la imagen de esta lesión (AU)

Síndrome de Noonan y quilotórax / Noonan syndrome and chylothorax

El quilotórax en la edad pediátrica generalmente se desarrolla en complicaciones posquirúrgicas de cirugía cardiotorácica y raramente se debe a malformaciones del sistema linfático asociado a síndromes dismórficos. Presentamos dos casos de Síndrome de Noonan con desarrollo de quilotórax neonatal. Ante pacientes con fenotipo Noonan y derrame pleural que se desarrolle en el período neonatal, sin antecedente de trauma obstétrico, está indicado descartar malformación linfática congénita y estudiar el derrame pleural para realizar tratamiento inicial conservador con dieta. Radiografía de tórax, ecografía y TC torácico muestran el derrame pleural y patrón parenquimatoso compatibles con quilotórax y linfangiectasias (AU)

El síndrome del hueso evanescente / Vanishing bone disease

El Síndrome de Gorham o hueso evanescente u osteolisis masiva es una patología rara con etiología y patogenia aún desconocidas. Acontece una reabsorción ósea progresiva y espontánea de uno o varios huesos contiguos alrededor de un foco, sin respetar los límites articulares. Puede afectar a cualquier parte del esqueleto, pero se ha descrito con mayor frecuencia en articulaciones del hombro y la pelvis. No se asocia historia familiar y la edad de presentación suele ser en adolescentes y adultos jóvenes. La regeneración del hueso no se produce aunque cese la progresión osteolítica. No se han descrito focos múltiples simultáneos no contiguos ni metástasis a distancia. La patología se considera benigna y la lisis ósea suele cesar tras unos años. El diagnóstico es de exclusión basándose en la evolución clínico-radiológica y en los hallazgos histológicos compatibles. Describimos un nuevo caso con los hallazgos clínicos, radiológicos e histopatológicos característicos del síndrome (AU)