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1.
Medisur ; 21(2)abr. 2023.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1440651

ABSTRACT

Dentro de la clasificación de los síndromes de osteòlisis idiopática, la enfermedad de Gorham-Stout ocupa el cuarto lugar. Es un cuadro clínico caracterizado por la destrucción progresiva de tejido óseo y proliferación vascular, con angiomatosis y linfangiomatosis ósea que produce una osteòlisis progresiva del esqueleto, con pérdida de masa ósea en las áreas afectadas. Este artículo tiene como objetivo presentar un paciente pediátrico con síndrome de Gorham Stout atendido en el Hospital Provincial Pediátrico Universitario José Luis Miranda, de Villa Clara. Es una paciente femenina, de dos años de edad, con cuadro febril de una semana de evolución, aumento de volumen en región dorsal y pérdida de peso. Al examen físico se constataron palidez cutáneo-mucosa y lesión nodular indolora de tres centímetros en la región dorsal. Reapareció la fiebre y se observó marcado aumento de volumen en la región dorso lumbar. Mediante tomografía axial computarizada y resonancia magnética se evidenció osteòlisis de cuerpos vertebrales desde D8 a L2 y aumento de las partes blandas adyacentes. Las manifestaciones clínicas e imagenológicas obligaron a descartar etiologías infecciosas y neoformativas. Se descartaron las primeras y por cumplir con los criterios requeridos se concluyó como enfermedad de Gorham. Esta es una rara y peculiar condición patológica músculo-esquelética en la cual el hueso, virtualmente, se desintegra y es reemplazado por tejido conectivo vascular. Su etiología es especulativa, con una presentación clínica muy variable. Los estudios imagenológicos resultan de gran utilidad. Posee un pronóstico indeterminado a pesar de las opciones terapéuticas empleadas. Por ser un síndrome extremadamente raro se decidió la presentación del caso.


Within the idiopathic osteolysis syndromes classification, Gorham-Stout disease ranks fourth. It is a clinical picture characterized by the progressive destruction of bone tissue and vascular proliferation, with angiomatosis and bone lymphangiomatosis that produces a progressive osteolysis of the skeleton, with loss of bone mass in the affected areas. This article aims to present a pediatric patient with Gorham Stout syndrome treated at the José Luis Miranda University Pediatric Provincial Hospital in Villa Clara. A 2-years-old female patient with a one week fever evolution, increased volume in the dorsal region and weight loss. The physical examination revealed cutaneous-mucosal pallor and a three centimeters painless nodular lesion in the dorsal region. The fever recurred and a marked increase in volume was observed in the dorsal lumbar region. Computed axial tomography and magnetic resonance imaging revealed osteolysis of the vertebral bodies from D8 to L2 and an increase in the adjacent soft tissues. The clinical and imaging manifestations made it necessary to dismiss infectious and neoformative etiologies. The former were dismissed and because they fulfill the required criteria it was concluded as Gorham's disease. This is a rare and peculiar musculoskeletal pathological condition in which bone virtually disintegrates and is replaced by vascular connective tissue. Its etiology is speculative, with a highly variable clinical presentation. Imaging studies are very useful. It has an indeterminate prognosis despite the therapeutic options used. Because it is an extremely rare syndrome, the presentation of the case was decided.

2.
Acute Crit Care ; 33(2): 105-109, 2018 May.
Article in English | MEDLINE | ID: mdl-31723871

ABSTRACT

Gorham disease, also known as massive osteolysis, is characterized by unregulated proliferation of lymphatic or vascular capillaries within bone, resulting in destruction and replacement with angiomatous tissue. This disease can lead to chylothorax that can be lethal. Viscum album extract from European mistletoe is a complementary anti-cancer drug which is commonly prescribed in many European countries and is considered as a new generation of chemical agent for pleurodesis. A 14-year-old girl presented with dyspnea and chest pain. She was diagnosed as Gorham disease who was definitively treated with V. album extract for chylothorax that was refractory to standard conservative management.

3.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-700225

ABSTRACT

Primary bone lymphoma(PBL)is rare and has no specific clinical features.A mouth-eaten pattern of bone destruction in metaphysic or subchondral bone and a large soft-tissue mass with little cortical destruction are important imaging features of primary bone lymphoma. The final diagnosis mainly depends on the histopathological features and the expression of specific proteins showing by immunohistochemical staining. It bears a good outcome after radiation and chemical therapy. The operation is not a routine therapy method.So, it is important to make accurate diagnosis before radiation and chemical therapy. In this article, we reviewed the clinical diagnosis, treatment, and prognosis of primary bone lymphoma.

4.
Colomb. med ; 47(4): 213-216, Oct.-Dec. 2016. tab, graf
Article in English | LILACS | ID: biblio-952886

ABSTRACT

Abstract Background: Gorham-Stout disease (GSD) is a rare disease of unknown etiology characterized by vascular proliferation that produces destruction of bone matrix. Case description: This case is about 43 year old woman who begins with pain in sternum, dyspnea, abdominal mass and, serous-hematic pleural effusion. Imaging tests were performed showing lesions on 6th and 10th left ribs archs. Later, a thoracotomy was performed observed absence of the end of the 6th and lung, pleural and costal biopsy was token. The histologic features described lymphatic vascular proliferation in bone tissue of chest wall. Other pathologies were excluded and in view of the findings, GSD diagnosis was made. Treatment and outcome: treatment was initiated with sirolimus achieving remission of the disease after the first month; however, because the presence of metrorrhagia the treatment was discontinued, reappearing symptoms afterwards. For that reason the treatment was restarted getting disappearance of the symptoms again, 4 weeks later. Clinical relevance: we present the first clinical cases of EGS with pleural effusion with response to sirolimus treatment that could be an alternative to the current therapy.


Resumen Antecedentes: La enfermedad de Gorham-Stout (EGS), es una enfermedad poco común, de etiología desconocida, caracterizada por la proliferación vascular que produce destrucción de la matriz ósea. Caso clínico: Se presenta el caso de mujer de 43 años que comienza con dolor en el esternón, disnea y tumoración abdominal junto con derrame pleural izquierdo de características serohemáticas como forma de presentación de una EGS. En pruebas de imagen que mostraron lesiones líticas en el 6º y 10º arcos costales izquierdos. Posteriormente se realizó toracotomía con biopsia pulmonar, pleural y costal observándose ausencia del extremo de la 6ª costilla. En el estudio histopatológico se describe proliferación vascular linfática en tejido óseo de pared costal. Se excluyeron otras patologías y se diagnosticó EGS. Tratamiento y resultado: Se inició tratamiento con sirolimus consiguiendo remisión completa desde el primer mes. Sin embargo, tras la suspensión del tratamiento por metrorragias, presentó reaparición de los síntomas. Se decide entonces reiniciar el tratamiento, consiguiendo nuevamente desaparición de los síntomas, tras 4 semanas de tratamiento. Relevancia clínica: Se presenta el primer caso clínico de EGS en edad adulta con derrame pleural asociado y con respuesta clínica a sirolimus, fármaco que podría ser una alternativa a la terapéutica actual.


Subject(s)
Adult , Female , Humans , Osteolysis, Essential/drug therapy , Sirolimus/therapeutic use , Immunosuppressive Agents/therapeutic use , Pleural Effusion/etiology , Pleural Effusion/drug therapy , Osteolysis, Essential/diagnosis , Osteolysis, Essential/physiopathology , Treatment Outcome
5.
Colomb Med (Cali) ; 47(4): 213-216, 2016 Dec 30.
Article in English | MEDLINE | ID: mdl-28348444

ABSTRACT

BACKGROUND: Gorham-Stout disease (GSD) is a rare disease of unknown etiology characterized by vascular proliferation that produces destruction of bone matrix. CASE DESCRIPTION: This case is about 43 year old woman who begins with pain in sternum, dyspnea, abdominal mass and, serous-hematic pleural effusion. Imaging tests were performed showing lesions on 6th and 10th left ribs archs. Later, a thoracotomy was performed observed absence of the end of the 6th and lung, pleural and costal biopsy was token. The histologic features described lymphatic vascular proliferation in bone tissue of chest wall. Other pathologies were excluded and in view of the findings, GSD diagnosis was made. TREATMENT AND OUTCOME: treatment was initiated with sirolimus achieving remission of the disease after the first month; however, because the presence of metrorrhagia the treatment was discontinued, reappearing symptoms afterwards. For that reason the treatment was restarted getting disappearance of the symptoms again, 4 weeks later. CLINICAL RELEVANCE: we present the first clinical cases of EGS with pleural effusion with response to sirolimus treatment that could be an alternative to the current therapy.


ANTECEDENTES: La enfermedad de Gorham-Stout (EGS), es una enfermedad poco común, de etiología desconocida, caracterizada por la proliferación vascular que produce destrucción de la matriz ósea. CASO CLÍNICO: Se presenta el caso de mujer de 43 años que comienza con dolor en el esternón, disnea y tumoración abdominal junto con derrame pleural izquierdo de características serohemáticas como forma de presentación de una EGS. En pruebas de imagen que mostraron lesiones líticas en el 6º y 10º arcos costales izquierdos. Posteriormente se realizó toracotomía con biopsia pulmonar, pleural y costal observándose ausencia del extremo de la 6ª costilla. En el estudio histopatológico se describe proliferación vascular linfática en tejido óseo de pared costal. Se excluyeron otras patologías y se diagnosticó EGS. TRATAMIENTO Y RESULTADO: Se inició tratamiento con sirolimus consiguiendo remisión completa desde el primer mes. Sin embargo, tras la suspensión del tratamiento por metrorragias, presentó reaparición de los síntomas. Se decide entonces reiniciar el tratamiento, consiguiendo nuevamente desaparición de los síntomas, tras 4 semanas de tratamiento. RELEVANCIA CLÍNICA: Se presenta el primer caso clínico de EGS en edad adulta con derrame pleural asociado y con respuesta clínica a sirolimus, fármaco que podría ser una alternativa a la terapéutica actual.


Subject(s)
Immunosuppressive Agents/therapeutic use , Osteolysis, Essential/drug therapy , Sirolimus/therapeutic use , Adult , Female , Humans , Osteolysis, Essential/diagnosis , Osteolysis, Essential/physiopathology , Pleural Effusion/drug therapy , Pleural Effusion/etiology , Treatment Outcome
6.
Paediatr Anaesth ; 25(7): 727-37, 2015 Jul.
Article in English | MEDLINE | ID: mdl-25736220

ABSTRACT

BACKGROUND: Primary osteolysis syndromes represent a number of rare conditions characterized by destruction and resorption of bone that is unrelated to neoplasia, infection, or trauma. AIM: To characterize the periprocedural course of patients with primary osteolysis syndrome undergoing procedures that require anesthesia care. METHODS: The medical records database from our institution was searched from 1976 to 2013 to identify patients with primary osteolysis syndromes who received anesthesia care. We reviewed demographic characteristics, comorbidities, and perioperative course of patients with different forms of primary osteolysis. A systematic review of the literature was performed to identify reports describing the anesthetic management of patients with these conditions. RESULTS: We identified 11 patients with a primary osteolysis syndrome who received 111 anesthetics. The patients' ages at the time of surgery ranged from 3 to 62 years. Difficult airway management was a common finding. On preoperative examination, difficulty with endotracheal intubation was predicted in 8 of 11 patients. Three patients had preexisting tracheostomies. One patient required multiple awake fiberoptic intubations. Another two patients where successfully intubated early in life via direct laryngoscopy, but eventually required awake fiberoptic intubations as the disease process caused progressive distortion of airway structures. Intraoperative complications were absent. In the postoperative period, three patients required prolonged mechanical ventilation due to airway swelling, respiratory failure, and infection. CONCLUSIONS: Airway management may be challenging in patients with primary osteolysis syndromes. Because the osteolytic process advances with aging and craniofacial dysmorphism may progressively worsen, the airway should always be reassessed before surgery with an appropriate strategy for potentially difficult intubation. In all patients with primary osteolysis, a potential for pathologic fracture must be considered and careful intraoperative positioning is warranted.


Subject(s)
Airway Management/methods , Airway Obstruction/complications , Osteolysis/complications , Adolescent , Adult , Child , Child, Preschool , Craniofacial Abnormalities/complications , Female , Humans , Intraoperative Complications/prevention & control , Male , Middle Aged , Retrospective Studies , Syndrome , Young Adult
7.
Chinese Journal of Radiology ; (12): 458-463, 2015.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-467493

ABSTRACT

Objective To evaluate the clinical and radiological features of Gorham disease. Methods Clinical and radiological features of Gorham disease were retrospectively analyzed by reviewing the eleven cases from our hospital and the cases reported in the domestic literature in the past fifty years. The diagnoses of all these 11 patients were in accordance with the diagnostic criteria established by Wells and Gray et al. All patients had X?ray plain film, four had CT scan and five had MR examination, with one had additional contrast?enhanced MR examination. Results There were 7 males and 4 females, age ranged from 16 to 66 years with a median age of 32 years. There were six cases involving hand, one involving ulnar and radial bones, one involving acetabulum and three involving jaw bones. The main clinical manifestations were pain, swelling, limited activity, and focal muscular atrophy occurred in 7 cases. On X?ray plain films, the affected bone became thin and the cortexes were not smooth and became coarse in 6 cases. Local lucent area was seen in 5 cases and massive bone absorption was seen in 6 cases. The residual bones showed a tapering appearance in 1 case. Pathological fracture occurred in 1 case. On CT scans, the affected bone became thin and the cortexes became coarse in 4 cases. Local lucent area was seen in 1 case. Massive bone absorption was seen in 3 cases. The adjacent muscular atrophy and widened intermuscular fat space occurred in 2 cases (atrophic bone absorption). On MRI, normal signal intensity of bone marrow disappeared and demonstrated low signal on T1WI and high signal on T2WI. The signal could be homogeneous or heterogeneous. There were widespread strip and patchy high signal areas in the soft tissue around the absorption areas in 4 cases, which resembled the edema?like signal. In 1 case, there was irregular widespread soft tissue mass around the absorption areas with heterogeneous high signal on T2WI. The adjacent muscle showed atrophy, and the intermuscular fat space became wide. There were a total of 92 cases reported cases in the literature including our 11 cases. There were 63 males and 29 females. The onset age ranged from 10 to 40 years in 66/92(72%)cases. The lesion affected one bone in 24 cases, affected two or more bones in 68 cases, out of which 9 cases had single center distribution, 59 cases had multiple centers distribution. Pectoral girdle, pelvis, maxillofacial bones, and hand were the most common sites of involvement in decreasing order. Forty three cases had muscle atrophy and 8 cases had soft tissue mass. Conclusion Gorham disease should be considered when atrophic bone absorption in one bone or continuous bones occurs that does not match clinical symptoms, with soft tissue atrophy but no bone sclerosis or periosteal reaction in the osteolytic areas.

8.
Trauma Mon ; 18(1): 41-5, 2013.
Article in English | MEDLINE | ID: mdl-24350149

ABSTRACT

INTRODUCTION: Gorham's disease is a rare musculoskeletal disease which causes progressive osteolysis and is characterized by massive bone destruction due to proliferation of vascular elements along with a great number of osteoclasts. The etiology of the disease is unknown. Gorham's disease is essentially rare in the forearm bones. As far as we know, only 2 cases of Gorham's disease of the forearm have been reported with 1 of them in the radius and the other starting in the radius and spreading to the lower portion of the humerus. CASE PRESENTATION: This case report shows that Gorham's disease may affect the ulna primarily and spread to adjacent bones despite the fact that there are no such reports in the literature. CONCLUSIONS: Gorham's disease has several manifestations as primary bone involvement. As in this rare case the ulna may be affected first and then the disease may spread to adjacent bones. More studies are needed to better recognize the behavior of this rare disease.

9.
Article in Korean | WPRIM (Western Pacific) | ID: wpr-92208

ABSTRACT

Gorham disease is a rare disorder characterized by proliferation of vascular channels resulting in destruction and resorption of osseous matrix. There is no standard treatment defined for this disease, and variable therapies such as medical, surgical, and radiation therapy have been used. Antiresorptive medication, such as bisphosphonate, is used in Gorham disease because they suppress the course of osteolysis and angiogenisis. We report a 9-year-old boy with Gorham disease, who was presented with recurrent hemothorax and treated by pamidronate. After treatment, he showed no recurrence of hemothorax for more than 2 years.


Subject(s)
Child , Humans , Diphosphonates , Hemothorax , Osteolysis , Osteolysis, Essential , Recurrence
10.
Rev. bras. ortop ; 45(6): 618-622, 2010. ilus, tab
Article in Portuguese | LILACS | ID: lil-574811

ABSTRACT

A síndrome de Gorham-Stout é uma doença que apresenta osteólise idiopática de um osso ou área contígua próxima. A etiologia é desconhecida, sendo uma condição rara, de difícil diagnóstico e tratamento controverso. Acomete pessoas sem distinção quanto à idade e ao sexo. Neste trabalho realizamos uma revisão bibliográfica da doença, dando enfoque específico no diagnóstico diferencial, e demonstramos o acompanhamento de um paciente com esta síndrome, desde o seu diagnóstico, tratamento e estado atual de evolução.


Gorham-stout syndrome is a disease that presents idiopathic osteolysis of a bone or contiguous area. The etiology is unknown. It is a rare condition, difficult to diagnose and with controversial treatment. This condition affects persons with no distinction as to age or sex. In this study, we conduct a bibliographic review of the disease, specifically focusing on differential diagnosis, and follow a patient with this syndrome from the time of its diagnosis, through treatment, to its current state of evolution.


Subject(s)
Humans , Male , Adolescent , Clavicle/physiopathology , Osteolysis, Essential/diagnosis , Osteolysis, Essential/etiology , Osteolysis, Essential
11.
Article in Chinese | WPRIM (Western Pacific) | ID: wpr-677182

ABSTRACT

Objective Toevaluatetheradiologicfindingsanditsclinicaluseinmassiveosteolysis (MOL) .Methods FourcasesofMOLwereretrospectivelyanalyzed .X rayexaminationwasperformedinfour, CTintwo ,andMRIinone .Results (1 )Singleskeletallesionwasdemonstratedinthreecases ,andmultiple lesionsinone .(2 )OnX ray ,tubebonelesionwasshowedastaperingintwo ,butbonedefectorbone disappearingwasfoundintheflatbonedisease .Onecasealsoassociatedwithchylothorax .(3)OnCT ,flatbone lesionhadataperingmargin ,riblesionswereshowedasflattenandirregularsurfacewithcorticalorsubcortical bonedefect.Surroundingsofttissueofskeletallesionswasatrophy .(4)NormalMRIsignalintensityofbone marrowdisappearedintwoskeletallesionsandhadalongT1andlongT2 signalintensitychanges .Conclusion X rayexaminationistheessentialmethodforMOL ,CTandMRIarecomplementaryone .

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