La Habana, Cuba. Con el uso de sutura polipropileno 4. Objective: to evaluate the surgical outcomes of the Crawford technique through the use of silastic and polypropylene 4. Those patients with prior surgery to treat this disease were excluded. Using polypropylene sutures 4.

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2017, Número 4

Arthrogryposis-renal dysfunction-cholestasis syndrome is a rare lethal disorder that involves multipl organ system. Three cardinal findings of this syndrome are arthrogryposis, renal tubular dysfunction and cholestasis. The other organ involvements including ichthyosis, central nervous system malformation, platelet anomalies, congenital heart defects and severe failure to thrive are sometimes associated with this syndrome. Clinical findings, organ biopsy and mutational analysis can help for diagnosing but there is no curative treatment except supportive care.


La artrogriposis nuevo protócolo de tratamiento

We report a new sporadic case of Freeman-Sheldon syndrome. The parents were not blood relatives. In addition, the patient showed other clinical manifestations such as a large bilateral inguinal hernia and thoracic cage abnormalities. The latter abnormality led to serious episodes of bronchopneumonia that delayed the surgical repair of bilateral inguinal hernia. Knowledge of the sonographic characteristics of deformities of the extremities is essential to reach an early prenatal suspected diagnosis of Sheldon-Freeman syndrome, especially in families with a history of the syndrome. We describe the preanesthetic management, anesthetic method and surgical technique performed when the child was aged 9 months. The delay was due to recurrent episodes of bronchopneumonia..

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