Difficult venous access in a child with a severe form of scl
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Scleromyxedema, is a very rear condition of mucinous deposition and diffuse papular eruptions of the skin, accompanied by systemic complications.A case of a 12 years old boy whose disease first presented with multiple painful aphtous ulcers in his mouth, papular skin eruptions on his palms and soles and hyperthermia up to 38.3°C. Over a period of four years the disease advanced progressively with rapid spreading of the skin lesions on the boys extremities, trunk and neck. On our first contact with the patient, he was in a very altered health condition, severely underfed, almost paralized with rigid flexion of all of his proximal and distal joints. His skin was thick and unpenetrable, intravenous cannulation and administration of therapy, laboratory testing was impossible. Neck extension and mouth opening were severely limited. The positioning for central vein puncturing was impossible and therefore it was decided to place a central venous catheter under the control of ultrasound. The procedure took place in the operating theatre, under analgosedation with Chloral hydrate administered rectally and N2O/air mixture through a facial mask. The right internal jugular vein was cannulated using the out-of-plain technique, with an 18G catheter under the guidance of a 6 cm linear ultrasound probe....

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