La Pediatria medica e chirurgica : Medical and surgical pediatrics
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Comparative Study
Neurofibromatosis type 1 clinical features and management.
Neurofibromatosis type 1 (NF1) or von Recklinghausen's disease is one of the most common genetic disorder. It is characterized by a wide variability and unpredictability of clinical manifestations involving multiple organ systems. Several complications affecting life expectancy can occur in affected patients. We reviewed genetic mechanisms, clinical aspects of the syndrome and multidisciplinary management required for NF1 patients.
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The aim of this paper was to report the management of a very rare malformative association of Left Pulmonary Artery (LPA) sling and Tracheal Lobe which is not still reported in scientific literature. ⋯ Current controversy in the management of Pulmonary Artery sling include surgical approach (median sternotomy versus left thoracotomy), use or non-use of cardiopulmonary bypass, and reimplantation versus translocation with distal tracheal resection. In our experience LPA reimplantation and tracheal lobe resection have been made easily and safely by the same sternotomy utilized for the cardio-pulmonary by-pass.