18 research outputs found

    Persistent Müllerian Duct Syndrome

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    Intramuscular myxoma of the cervical paraspinal muscle

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    Myxoma is a neoplasm of mesenchymal origin composed of undifferentiated stellate cells in a myxoid stroma. This tumor can develop in a variety of locations. Myxomas that arise from skeletal muscles are called intramuscular myxomas. They usually occur in large skeletal muscles. Only ten cases of these benign tumors involving the neck muscles were reported in literature. Of them, only three were located at the paraspinal muscles. A 64-year-old woman presented with occiptal and neck pain over 5 years noted an expansive painful lesion located at posterior cervical region with progressive volume increase in the last 12 months. Image exams revealed a large mass located in the left posterior region of the neck in contact with the C2, C3 and C4 laminae with no invasion of the vertebrea. Tumor total removal was performed through normal muscle margins and the vertebral periosteum was scraped. The tumor was encapsuleted, lobulated with a gray-white appearance. The histological examination yielded the diagnosis of intramuscular myxoma. Follow-up at 1 year showed complete resolution of preoperative symptoms and no evidence of local recurrence. In conclusion, although rare, intramuscular myxoma should be included in differential diagnosis of cervical paraspinal tumors. We reported the fourth case of intramuscular myxoma in the paraspinal musculature of the neck. Despite its benign characteristics, local recurrence was reported after subtotal resection. Tumor total removal should be the goal of surgery

    N-terminal-pro-B type natriuretic peptide as a useful tool to evaluate pulmonary hypertension and cardiac function in CDH infants

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    Objective: In congenital diaphragmatic hernia (CDH) the severity of pulmonary hypertension (PH) is considered, by several authors, determinant of clinical outcome. Plasmatic N-terminal-pro-B type natriuretic peptide (NT-proBNP) might be useful in diagnosis and management of PH in newborns, although its interest in CDH infants remains to be defined. Early NT-proBNP levels were assessed in CDH infants and correlated with cardiovascular echocardiographic parameters. Patients and Methods: 28 newborns, CDH and age-matched controls were enrolled in a prospective study. Clinical condition, NT-proBNP plasmatic levels, echo parameters of PH and biventricular function were assessed at 24 h after delivery as well as survival outcome. Results: Estimated mean pulmonary pressure and NT-proBNP were significantly higher in CDH than control infants. NT-proBNP significantly correlated with estimated pulmonary artery pressure, right ventricular Tei index, and tricuspid E/A ratio. Additionally, we found that CDH infants with NT-proBNP >11,500 pg/ml experienced a worse prognosis. Conclusions: We demonstrated that PH is associated with NT-proBNP elevation and diastolic impairment in CDH infants. Early elevations in NT-proBNP levels seem to alert for a subset of CDH infants with worse prognosis.This study was funded by Fundação para a Ciência e Tecnologia (FCT) through the research project SAU-OBS/56428/200
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