Síndrome de Gorham-Stout: doença do osso fantasma / Gorham-Stout syndrome: phantom bone disease

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.

Massive bone loss from fungal infection after anterior cruciate ligament arthroscopic reconstruction.

Although there are numerous reports of septic pyogenic arthritis after arthroscopic anterior cruciate ligament (ACL) reconstruction, there is limited information regarding the outcomes of fungal infection. We determined the outcomes of six patients with mycotic infection after regular ACL reconstruction. There were four males and two females with a mean age of 33 years. We determined the number of procedures performed, bone loss originating to control infection, and final reconstruction in these patients. An average of five arthroscopic lavage procedures had been performed at the referring centers. Fungal infection was diagnosed based on pathologic samples; five infections were the result of mucormycosis and one was Candida. After final débridement, the mean segmental bone loss was 12.8 cm. All patients were treated with intravenous antifungal coverage and cement spacers before final reconstruction. At final followup, all patients were free of clinical infection. Three had reconstruction with an allograft-prosthesis composite, two with hemicylindrical allografts, and one with an intercalary allograft arthrodesis. Despite the extremely unusual presentation of this complication, surgeons should be aware of potential and catastrophic consequences of this severe complication after ACL reconstruction.

Osteólitis primaria idiopática / Primary osteolysis

Las osteólisis primarias son entidades clínicas raras, se caracterizan por una resorción ósea lenta y progresiva capaz de afectar cualquier hueso del organismo, especialmente el carpo, tarso y falanges. Pueden presentarse en forma idiopática, o ser secundarias a diversos eventos, incluido el trauma. Sus manifestaciones clínicas son muy variadas, afectando además del componente óseo a la piel, el riñon, sistema vascular, etc. Nuestro grupo de investigación ha tenido especial interés en detectar y estudiar estos pacientes, por lo cual en esta publicación presentamos cinco casos con acroosteólisis primaria, de los cuales dos son una variante no descrita en la literatura y el otro caso poco frecuente, como lo es la osteólisis carpo-tarso sin compromiso renal. Hacemos una propuesta clasifícatoria para facilitar su entendimiento

Massive osteolysis of skull bones secondary to lymphangioma of paranasal sinuses and mastoid sinus: report of two cases in children.

Massive osteolysis (MO) is a rare condition in which progressive localized bone tissue resorption is associated with proliferating thin-walled vessels in the absence of inflammation. Rare cases have been reported to occur in the skull. This paper describes two patients with MO who presented with massive assymetric swelling of the skull. This was associated with extensive enlargement of the paranasal sinuses (frontal, ethmoidal, and sphenoidal in one and the mastoid air cells in the other). The second patient developed subcutaneous emphysema on several occasions and the Valsalva maneuver increased the swelling, indicating transmission of the air from the nasopharynx to the mastoid cells and from there to the subcutaneous tissue. In the first patient, the sinus mucosa was shown to be involved by an extensive lymphangioma, and a similar change was seen in the mastoid air cells (patient 2). We are proposing that MO of these two patients resulted from bone resorption due to progressive extension of sinus mucous lymphangiomata.

Acroosteolisis primaria

Describimos dos casos de acroosteólisis primaria: una mujer de 50 años y un hombre de 34 años quien además presentaba neurofibromas, osteopenia y cardipatía, la cual es una variedad no informada en la literatura. Proponemos una nueva clasificación para la entidad