La Pediatria medica e chirurgica : Medical and surgical pediatrics
-
Congenital giant melanocytic nevi of the scalp and forehead are rare lesions present at birth. These lesions are associated with risk of malignant transformation, but they primarily represent a psychological problem to both patient and parents and merit early excision and reconstruction. In this study we report our own experience: seven patients, aged 8 months to 9 years, with congenital pigmented nevi involving forehead and scalp, and a 4-year old patient with congenital pigmented nevus of periorbital region and nose were treated successfully with excision and expanded skin flap reconstructions. ⋯ We had no rupture, extrusion or infection of the skin expanders. Complications included eyebrow ptosis and asymmetry in two patients underwent correction at a final procedure. In our opinion tissue expansion is an excellent technique for the treatment of giant nevi of the scalp and forehead because it offers the best aesthetic and functional outcomes.
-
Many paediatric cardiac patients now survive to adulthood following early surgery. This population of adult patients with a congenital heart disease offer distinct challenges such as unusual anatomy and demands such as pregnancy and exercise tolerability not found in conventional paediatric or traditional adult interventional patients. ⋯ There has been a rapid growth in interventional cardiology techniques to treat adults with congenital heart disease, mirroring the rise of interventional cardiology as a cardiology subspecialty and the increasing population of adults with congenital abnormalities. We review current indications for adult congenital heart disease intervention and best practice, reviewing the patient spectrum commonly treated, devices used, and emerging treatments.
-
Cyanosis is negative predictor of survival in adult patients with congenital heart disease. When cyanosis is secondary to heart or lung disease, chronic hypoxiemia result in hematologic, neurologic, renal and reumatic complications. Is important,for the optimization of therapeutics procedure, the follow up of heart disease and of oxygen saturation and blood work. For the care of this patients are required specialized centers with a multidisciplinary team of experts.
-
Pulmonary Hypertension (PH) is definited by a mean pulmonary artery pressure (PAPm) >25 mmHg at rest. The Dana Point 2008 Revised Classification System represents the most recent classification system update with respect of various etiologies of PH. ⋯ Progressive vascular remodeling and increase in pulmonary vascular resistance (PVR) may ultimately lead to reversal of the shunt (pulmonary to systemic) causing cyanosis and determining the so-called Eisenmenger Syndrome (ES). Recent advances in the early diagnosis and medical targeted treatment of adult patients with CHD-PAH and ES can improve PAP, PVR and exercise tolerance, together with NYHA Class and survival, and may potentially reverse the vascular remodeling process in selected patients.
-
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.