Intraoperative Scintigraphy With Portable Gamma Camera for the Localization of Interaortocaval Paraganglioma.

ABSTRACT: A 15-year-old adolescent girl diagnosed of interaortocaval paraganglioma with a positive 123I-MIGB SPECT/CT and 1 unsuccessful prior surgery was operated on with the assistance of a handheld gamma camera. Once the lesion was located and removed, 2 images were taken, one of the surgical field (without 123I-MIGB uptake) and another of the tumor ex vivo (with high 123I-MIGB uptake), confirming that the lesion had been satisfactorily excised. This case highlights the use of a portable gamma camera as a useful tool to locate this rare tumor, with a SPECT/CT positive for 123I-MIGB and a difficult anatomical location suspected.

Osteocondromatosis sinovial de rodilla: una causa infrecuente de gonalgia en edad pediátrica / Synovial chondromatosis of the knee. A rare cause of knee pain in pediatric age

La osteocondromatosis sinovial es una metaplasia benigna de la membrana sinovial que afecta a 1 de cada 100 000 personas, en su mayoría adultos, y es extremadamente infrecuente en edad pediátrica. Predomina en grandes articulaciones, sobre todo la rodilla, y la sintomatología es, por lo general, inespecífica. Dado que la radiografía simple no suele ser concluyente, se recurre a la resonancia magnética nuclear y a la tomografía axial computarizada para orientar el diagnóstico. Se expone el caso de una paciente de 10 años de edad con gonalgia y dismorfia en la patela izquierda de seis meses de evolución, con diagnóstico de osteocondromatosis sinovial. Se presenta el caso dado que se trata de una entidad muy rara en niños, pero que requiere un tratamiento quirúrgico precoz para evitar sus posibles complicaciones, como la destrucción articular progresiva o la malignización a condrosarcoma.

Synovial osteochondromatosis consists of a synovial metaplasia which affects 1 per 100 000 people. It is a very rare disease among children. It typically affects large joints of the body, especially the knee. Due to the lack of specificity of the signs and symptoms and X-Ray images, imaging tests such as nuclear magnetic resonance or computerized tomography are frequently needed for diagnosis.We report a case of a ten-year-old female patient with a six months history of pain and deformity of left patella which was diagnosed with synovial osteochondromatosis. This case highlights the importance of clinical suspicion, not only because it is an extremely rare disease in children, but also because it needs a surgical treatment as soon as possible in order to avoid consequences it might have in pediatric age, as joint destruction or malignization to chondrosarcoma.

[Synovial chondromatosis of the knee. A rare cause of knee pain in pediatric age]. / Osteocondromatosis sinovial de rodilla: una causa infrecuente de gonalgia en edad pediátrica.

Synovial osteochondromatosis consists of a synovial metaplasia which affects 1 per 100 000 people. It is a very rare disease among children. It typically affects large joints of the body, especially the knee. Due to the lack of specificity of the signs and symptoms and X-Ray images, imaging tests such as nuclear magnetic resonance or computerized tomography are frequently needed for diagnosis. We report a case of a ten-year-old female patient with a six months history of pain and deformity of left patella which was diagnosed with synovial osteochondromatosis. This case highlights the importance of clinical suspicion, not only because it is an extremely rare disease in children, but also because it needs a surgical treatment as soon as possible in order to avoid consequences it might have in pediatric age, as joint destruction or malignization to chondrosarcoma.

La osteocondromatosis sinovial es una metaplasia benigna de la membrana sinovial que afecta a 1 de cada 100 000 personas, en su mayoría adultos, y es extremadamente infrecuente en edad pediátrica. Predomina en grandes articulaciones, sobre todo la rodilla, y la sintomatología es, por lo general, inespecífica. Dado que la radiografía simple no suele ser concluyente, se recurre a la resonancia magnética nuclear y a la tomografía axial computarizada para orientar el diagnóstico. Se expone el caso de una paciente de 10 años de edad con gonalgia y dismorfia en la patela izquierda de seis meses de evolución, con diagnóstico de osteocondromatosis sinovial. Se presenta el caso dado que se trata de una entidad muy rara en niños, pero que requiere un tratamiento quirúrgico precoz para evitar sus posibles complicaciones, como la destrucción articular progresiva o la malignización a condrosarcoma.

Case Report: Primary Peritonitis as the Onset of Pediatric Ménétrier's Disease.

Introduction: Primary peritonitis (PP) and Ménétrier's Disease (MD) are both rare conditions among pediatric population. Although about 150 MD cases have been described in the scientific literature to date, its onset with a PP is an unusual condition. Case Presentation: We present a case of an 11-year-old boy who was admitted to our unit because of abdominal pain and distension. Complementary tests showed ascites, bilateral pleural effusion, leukocytosis, increased acute phase reactants and hypoproteinemia with hypoalbuminemia. Laparoscopy ruled out appendicitis or visceral perforations and exposed purulent peritoneal fluid, compatible with PP. Biochemical stool analysis showed increased clearance of alpha-1-antitrypsin, which was consistent with a protein-losing enteropathy. Gastroscopy findings were compatible with MD. The clinical course was favorable and he had no recurrence after 12 months of follow-up. Conclusion: PP can be the first clinical manifestation of pediatric MD. Knowledge of MD and its generally benign nature in children is important in order to avoid excessive testing and unnecessary treatment.