Case Report
Craniofacial fibrous dysplasia and aneurismal bone cyst in a patient with McCune-Albright syndrome. A case report and review of the literature
Displasia fibrosa craneofacial y quiste óseo aneurismático en una paciente con síndrome de McCune-Albright. Presentación de un caso y revisión de la literatura
Julián Castro Castroa,
, Juan Manuel Villa Fernándezb, Jesús Patricio Agulleiro Díazc
Corresponding author
a Servicio de Neurocirugía, Complexo Hospitalario Universitario de Ourense, Instituto de Investigación Sanitaria Galicia Sur, Ourense, Spain
b Servicio de Neurocirugía, Complexo Hospitalario Universitario de A Coruña, A Coruña, Spain
c Servicio de Neurocirugía, Complexo Hospitalario Universitario de Santiago, Santiago de Compostela, A Coruña, Spain
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Retrospective cohort study assessing prognostic factors for functional outcome in adult patients" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "es" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "256" "paginaFinal" => "267" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tumores intradurales-extramedulares. Estudio retrospectivo de cohorte valorando factores predictores de «<span class="elsevierStyleItalic">outcome</span>» en pacientes adultos" ] ] "contieneResumen" => array:2 [ "en" => true "es" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0020" "etiqueta" => "Fig. 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 2610 "Ancho" => 2140 "Tamanyo" => 169403 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0080" class="elsevierStyleSimplePara elsevierViewall">Distribution of pre and postoperative McCormick score results. At the last evaluation, 96% of patients had McCormick grades less than 3. 90% (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>183) of patients improved preoperative McCormick score, 8.8% (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>18) remained stable, and less than 2% worsened their neurological status (3 patients).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Mauro Ruella, Guido Caffaratti, Amparo Saenz, Facundo Villamil, Rubén Mormandi, Andrés Cervio" "autores" => array:6 [ 0 => array:2 [ "nombre" => "Mauro" "apellidos" => "Ruella" ] 1 => array:2 [ "nombre" => "Guido" "apellidos" => "Caffaratti" ] 2 => array:2 [ "nombre" => "Amparo" "apellidos" => "Saenz" ] 3 => array:2 [ "nombre" => "Facundo" "apellidos" => "Villamil" ] 4 => array:2 [ "nombre" => "Rubén" "apellidos" => "Mormandi" ] 5 => array:2 [ "nombre" => "Andrés" "apellidos" => "Cervio" ] ] ] ] ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2529849623000333?idApp=UINPBA00004B" "url" => "/25298496/0000003400000005/v1_202309042000/S2529849623000333/v1_202309042000/en/main.assets" ] "en" => array:19 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case Report</span>" "titulo" => "Craniofacial fibrous dysplasia and aneurismal bone cyst in a patient with McCune-Albright syndrome. A case report and review of the literature" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "268" "paginaFinal" => "272" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Julián Castro Castro, Juan Manuel Villa Fernández, Jesús Patricio Agulleiro Díaz" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Julián" "apellidos" => "Castro Castro" "email" => array:1 [ 0 => "juliancastrocastro@yahoo.es" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Juan Manuel" "apellidos" => "Villa Fernández" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "Jesús Patricio" "apellidos" => "Agulleiro Díaz" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Neurocirugía, Complexo Hospitalario Universitario de Ourense, Instituto de Investigación Sanitaria Galicia Sur, Ourense, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Neurocirugía, Complexo Hospitalario Universitario de A Coruña, A Coruña, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Neurocirugía, Complexo Hospitalario Universitario de Santiago, Santiago de Compostela, A Coruña, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Displasia fibrosa craneofacial y quiste óseo aneurismático en una paciente con síndrome de McCune-Albright. Presentación de un caso y revisión de la literatura" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 817 "Ancho" => 1255 "Tamanyo" => 148706 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A) Axial CT section with the “ground glass” appearance of the bones of the facial skeleton and the skull base. B) Coronal reconstruction of the CT showing a lesion with cystic content with erosion of the right orbital roof. C) MRI image, coronal section in T2-weighted sequence showing the cystic content of the lesion and the displacement of the orbital contents.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Fibrous dysplasia (FD) is a rare bone disorder consisting of the replacement of normal bone by an excessive proliferation of fibrous connective tissue with non-functional bone structures. FD has three clinical patterns: monostotic, polyostotic, and that associated with McCune-Albright syndrome (MAS).<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">MAS is a rare disease, characterised by skeletal lesions, skin hyperpigmentation and hyperfunctioning endocrinopathies. It accounts for less than 7% of cases of bone dysplasia and its estimated prevalence is between 1/100,000 and 1/1,000,000. MAS tends to progress slowly, but very rarely it can be aggressive or, in 0.9–4% of cases, it can undergo malignant transformation.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Aneurysmal bone cysts (ABC) are rare solitary benign bone lesions, usually arising from the metaphysis of long bones, and rarely involving the skull. They consist of vascular spaces separated by septa of connective tissue. They are usually primary lesions, although they have been described as secondary, particularly to giant cell tumours. ABC secondary to FD or MAS are extremely rare.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,5</span></a> Primary orbital ABC associated with FD have only been described on three other occasions in the literature.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">This case report involves a female patient with MAS and ABC with orbital involvement. We discuss the clinical and imaging findings, as well as the treatment of this rare entity, and provide a brief review of the literature.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0025" class="elsevierStylePara elsevierViewall">This was a 21-year-old woman with no relevant previous medical history, who attended the Accident and Emergency Department of our centre due to symptoms of right proptosis and right-sided headache. She had no relevant previous medical history.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Obvious on physical examination was the deformity in the patient's right orbitofrontal region (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A and B), which the patient reported having had for several years. She had right exophthalmos with normal ocular motility and normal visual field by confrontation test.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">Also noteworthy was the patient's short stature (147<span class="elsevierStyleHsp" style=""></span>cm) and the presence of hyperpigmented “café-au-lait” spots on her back, predominantly on the right (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>C). After directed questioning, the patient stated she had her first vaginal bleeding at around 5 or 6 years of age.</p><p id="par0040" class="elsevierStylePara elsevierViewall">A neuroimaging study was requested with computed tomography (CT) and magnetic resonance imaging (MRI). CT of the brain (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A) showed a diffuse thickening of the skull bone with a “ground glass” appearance, involving the calvaria as well as the facial region and the base of the skull. There were areas of consolidation and other lithic areas. Also striking was a cystic-like lesion overlying the right orbit, with erosion of the orbital roof and the cranial vault (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>B). In the MRI (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>C) images of this same lesion were suggestive of a cystic lesion with a double level of fluid inside, with low signal on T1 and hypersignal on T2.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0045" class="elsevierStylePara elsevierViewall">Bone scintigraphy with metastable technetium 99 3,3-diphosophono-1,2-propanodicarboxylic acid (Tc99m-DPD) revealed multiple foci of uptake in the axial and peripheral skeleton.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Laboratory tests showed normal hormone levels, including serum oestradiol, FSH and LH. The patient had elevated alkaline phosphatase (1223 IU/L, range 40–150<span class="elsevierStyleHsp" style=""></span>IU/L) with normal calcium, phosphate and vitamin D levels.</p><p id="par0055" class="elsevierStylePara elsevierViewall">In view of the clinical and radiological findings, a right orbitofrontal craniotomy was performed. On raising the scalp, the wall of the ABC could already be seen eroding the frontal bone (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). The cyst was completely resected (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>B), with reaming of part of the dysplasia, particularly towards the optic canal. Finally, the skull was reconstructed with the patient's own bone, titanium mesh and calcium phosphate cement (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>C and D).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0060" class="elsevierStylePara elsevierViewall">The patient’s postoperative recovery was uneventful, with adequate control of skull pain and improvement in facial morphology.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Histological analysis revealed fibroblast proliferation composed of benign areas of fibrous bone with others of irregular bone formation. Also found was a cystic structure with haemorrhagic spaces lined by multinucleated giant cells similar to osteoclasts. The results were compatible with FD with ABC.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0070" class="elsevierStylePara elsevierViewall">MAS was first described separately by Donovan McCune and Fuller Albright in children. It is a relatively rare disease with an estimated prevalence of between 1/100,000 and 1/1,000,000, and is more common in women. It was initially defined by the presence of polyostotic FD, “café au lait” spots and early puberty. However, it was later described that other endocrine disorders could be associated, such as hyperthyroidism, excess growth hormone, renal phosphate wasting and Cushing's syndrome.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,6</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">The mutation is located on chromosome 20q13 at the <span class="elsevierStyleItalic">guanine nucleotide-binding protein, alpha stimulating</span> (GNAS) locus. Substitution of arginine for cysteine or histidine leads to hyperactivation of GS〈-cyclic adenosine monophosphate (AMP). Due to a postzygotic mutation of the GNAS1 gene, it is considered a specific form of endocrine and non-endocrine neoplasia, with the affected cells distributed in a mosaic pattern. This contributes to the variability between patients in clinical manifestations and the severity of the disease. The overproduction and high concentration of cyclic AMP (cAMP) in bone activates abnormal proliferation and differentiation. FD classically affects the long bones, particularly the proximal femur. Both monostotic and polyostotic FD lesions involving long bones arise in the diaphysis or metaphysis, but much less often in the epiphysis. Vertebral bone involvement is common and can be associated with scoliosis. Craniofacial FD is also not uncommon, being reported in up to 60–70% of patients. The most common sign is that derived from the gradual painless enlargement of the involved bone, in this case, facial asymmetry, with its corresponding aesthetic deformity. Other symptoms result from constriction of cranial foramina or obliteration of bony cavities: anosmia; diplopia; proptosis; epiphora; strabismus; facial paralysis; tinnitus; nasal obstruction; malocclusion and interference with chewing and speech.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7–9</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Early puberty is the most common endocrine manifestation in MAS, being more common in females than in males. However, as previously mentioned, there are other associated endocrine disorders, including hyperthyroidism, pituitary adenomas, primary adrenal hyperplasia, hypophosphataemia and polycystic ovary.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Café-au-lait spots are caused by the over-production of melanin due to G〈/cAMP activation, and appear as irregular lesions along the midline of the body with a tendency to affect one side more than the other. They occur mainly in the frontal and posterior cervical areas, buttocks, chest, back, shoulders and pelvis.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">Similar clinical manifestations can occur in other conditions such as monostotic FD, Mazabraud syndrome, type I neurofibromatosis and Jaffe-Campanacci syndrome.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">ABC are benign pseudotumour-like lesions composed of several expansive cystic compartments filled with blood derivatives, with few endothelial elements. They most commonly occur as primary lesions, and are very uncommon secondary to FD and extremely rare secondary to MAS. They tend to involve the vertebrae and long bones and it is unusual for them to affect the skull. In the literature reviewed with simultaneous presentation of MAS and ABC, among those with cranial involvement, presentation has been reported at the orbital level, skull base and also in the occipital region.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> Although the mechanism of formation is unknown, one suggestion is that the development of this lesion may be due to an interruption in bone circulation in patients with FD.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">10,11</span></a> Cytogenetic data show that in ABC there is a rearrangement of chromosomal bands 16q22 and 17p13, showing a deregulated expression of the USP6 genes.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">In patients with MAS, treatment usually requires multidisciplinary teams, involving orthopaedic surgeons, neurosurgeons, maxillofacial surgeons, endocrinologists, dermatologists, ophthalmologists, etc, with personalised care required because of the heterogeneity of the syndrome.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4–6</span></a></p><p id="par0105" class="elsevierStylePara elsevierViewall">The treatment of craniofacial FD is also subject to debate. The initial approach is typically conservative. The most common indications are for cosmetic reasons, due to orbital involvement or neurovascular compression, and in some cases for histological confirmation of the lesion type. Treatment has been proposed with calcitonin, bisphosphonates or denosumab, which in some patients improve analytical parameters and pain, but they have not shown long-term evidence for control of the disease.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12,13</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">With regard to ABC, although cases of spontaneous healing have been reported, treatment is usually required, including surgical curettage with or without bone grafting, complete excision, selective arterial embolisation, radiotherapy, or a combination of these procedures. The role of embolisation in the treatment of ABC is to decrease vascularisation and reduce intraoperative blood loss. This treatment has only been successful in long bones and the pelvis. Total en bloc excision, if possible, provides the highest cure rate with a good prognosis and excellent functional results. Excision should include the entire cyst wall because partial resection is associated with a higher risk of recurrence.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">14,15</span></a></p><p id="par0115" class="elsevierStylePara elsevierViewall">In conclusion, we have presented a case which we consider to be very rare. MAS is a disease with a great variability of clinical manifestations, some of which involve the field of Neurosurgery, as in our patient due to the presence of FD and ABC with cranio-orbital involvement.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Funding</span><p id="par0120" class="elsevierStylePara elsevierViewall">This work received no funding from public, private or non-profit organisations.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflicts of interest</span><p id="par0125" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres1958140" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1685283" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1958141" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1685284" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Funding" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflicts of interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2022-01-30" "fechaAceptado" => "2022-06-23" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1685283" "palabras" => array:4 [ 0 => "Aneurismal bone cyst" 1 => "Fibrous dysplasia" 2 => "McCune-Albright syndrome" 3 => "Craniotomy" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1685284" "palabras" => array:4 [ 0 => "Craneotomía" 1 => "Displasia fibrosa" 2 => "Quiste óseo aneurismático" 3 => "Síndrome de McCune-Albright" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">McCune-Albright syndrome (MAS) is a rare heterogeneous genetic disorder that is characterized by a triad of polyostotic fibrous dysplasia (FD), café au lait spots (CAL), and multiple hyperfunctional endocrinopathies. In general, it is diagnosed clinically. From the triads, 2 of the findings are enough to make the diagnosis.</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Craniofacial fibrous dysplasia is a term that is used to describe the fibrous dysplasia, which was localized at the craniofacial skeleton and is common in MAS patients.</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Aneurysmal bone cyst (ABC) is a rare non-neoplastic bone lesion that involves mostly the long bones and vertebrae and may occur very rarely in the craniofacial bones. ABCs may occur as secondary bony pathologies in association with various benign and malignant bone tumors and with fibrous dysplasia. Secondary ABC occurring in craniofacial FD is extremely rare. We present the case of a 21-year-old patient treated at our center for a right orbital aneurysmal bone cyst associated with MAS and provide a review of the relevant literature.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">El síndrome de McCune-Albright (SMA) es un trastorno genético heterogéneo que se caracteriza por la tríada de displasia fibrosa (DF) poliostótica, manchas café con leche y múltiples endocrinopatías hiperfuncionales. En general, se diagnostica clínicamente. De la tríada, 2 de los hallazgos son suficientes para hacer el diagnóstico. La DF craneofacial es un término que se usa para describir la displasia fibrosa, que se localizaba en el esqueleto craneofacial y es común en pacientes con SMA.</p><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">El quiste óseo aneurismático (QOA) es una lesión ósea no neoplásica infrecuente que afecta principalmente a los huesos largos y las vértebras, y puede ocurrir muy raramente en los huesos craneofaciales. Los QOA pueden ocurrir como enfermedades óseas secundarias en asociación con varios tumores óseos benignos y malignos y con displasia fibrosa. El QOA secundario que ocurre en la DF craneofacial es excepcional. Presentamos el caso de una paciente de 21 años tratada en nuestro centro de un quiste óseo aneurismático orbitario derecho asociado a SMA y realizamos una revisión de la literatura relevante.</p></span>" ] ] "multimedia" => array:3 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 906 "Ancho" => 1305 "Tamanyo" => 166003 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A and B) View of the patient's face showing right exophthalmos and bulging of the right frontal region. C) Examination of the patient's back showing the “café-au-lait” spots predominantly on the right hand side.</p>" ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 817 "Ancho" => 1255 "Tamanyo" => 148706 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A) Axial CT section with the “ground glass” appearance of the bones of the facial skeleton and the skull base. B) Coronal reconstruction of the CT showing a lesion with cystic content with erosion of the right orbital roof. C) MRI image, coronal section in T2-weighted sequence showing the cystic content of the lesion and the displacement of the orbital contents.</p>" ] ] 2 => array:8 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 977 "Ancho" => 1255 "Tamanyo" => 361354 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A) Wall of the ABC eroding the frontal bone. Thin-walled cyst with xanthochromic contents. B) The defect produced by the ABC in the orbital bone and in the base of the skull is shown. 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