A 7-year-old boy, who arrived in Milan from Sardinia where he was hospitalized in serious condition for degenerative dystonia, underwent an operation with magnetic resonance ultrasound (Mrgfus, Magnetic Resonance guided Focused Ultrasound) at the Besta Neurological Institute. The Milanese IRCCS announced this in a note: “For the first time in the world” the technique is “a mini-invasive procedure capable of carrying out lesion-type surgery under direct radiological control and with the aid of ultrasound , was used with excellent results on a 7-year-old child”, they explain from the facility. The little one is “better now”. The operation, we read, “avoided complications or fatal outcomes for the patient, already suffering from a genetically based degenerative dystonia (Pkan deficiency).
Dystonia is a motor disorder that causes abnormal postures and/or involuntary muscle spasms and which can have different causes, ranging from perinatal or post-natal damage to genetic forms, as in the case of the young patient, affected by a rare genetic form particularly aggressive. In the case of a dystonic state, very serious spasms occur which require deep sedation in intensive care.
Performing the procedure were Vincenzo Levi, neurosurgeon of the simple departmental structure (Ssd) of functional neurosurgery, and Giovanna Zorzi, child neuropsychiatrist and director of the complex structure (Sc) of child neuropsychiatry 1- Besta movement disorders, assisted by the direct anesthetic team by Marco Gemma, from the SC Neurology 1 – Movement Disorders team directed by Roberto Eleopra, from the SC Neuroradiology team of Marina Grisoli and from the SC Interventional Neuroradiology team of Elisa Ciceri.
“The child – reports Zorzi – presented a serious and rapid clinical deterioration due to the appearance of a dystonic state refractory to medical therapy which made it necessary to be admitted to intensive care, first in Sassari and then here at Besta. He was sedated, intubated and ventilated for almost a month with a high load of anti-dystonic and sedative drugs and with serious clinical complications, such as septicemia, septic thrombosis which were putting the patient’s life at high risk”.
The dystonic state, explains Levi, “is a rare and serious complication of pediatric dystonias. If not treated in time it can be fatal. When medical therapy is not effective, traditional neurosurgical interventions are successfully carried out (deep brain stimulation or radiofrequency pallidotomy) In this case it was not possible to carry out such invasive interventions given the child’s recurrent septic state. It was therefore decided, after obtaining the favorable opinion of the Ethics Committee, to subject the child to treatment using Mrgfus, a minimally invasive surgical procedure able to perform a bilateral pallidotomy under direct radiological control, using focused ultrasound”.
The procedure was carried out under general anesthesia “without complications – adds Levi – After 48 hours from the operation it was possible to lighten the sedation and after a week to transfer the child from the intensive care unit to the inpatient department, from which he will be discharged in the next few weeks in a compensation situation, having reacquired the ability to interact with the outside world”.
This technique “is used in the treatment of some movement disorders and is currently foreseen only for adult patients who are able to carry out the procedure without sedation and unilaterally – comments the general director of Besta, Angelo Cordone – The exceptional nature of the intervention , the first in the world, consists in having successfully performed a bilateral pallidotomy using Mrgfus in a child suffering from a dystonic state refractory to medical therapy, for which no other therapeutic option was feasible. A procedure never before performed on a child, which opens up a new, minimally invasive therapeutic perspective, which has rewarded our doctors with an exceptional result and makes Besta a global excellence in neuroscience”.
“The Besta is equipped with cutting-edge equipment, but above all with high-level medical teams who, in this case, saved the life of the little boy, who was already suffering from a rare disease”, added the president of the IRCCS, Andrea Gambini , highlighting that in this way we can “give hope to the sick, in this case to a child and his family”.
For the Welfare Councilor of the Lombardy Region, Guido Bertolaso, there is also another aspect to highlight: “The ability of our professionals to ‘look beyond’ and adopt innovative solutions for problems that unfortunately seem to have no possibility of being treated. I congratulate the Neuroscience specialists at Besta – he states – for their great competence and I thank them for this spirit of initiative placed at the service of those who entrust themselves to them for treatment. A particular and personal thought goes to the little patient and his family members to whom I wish all the serenity possible.”
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