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A 3 Age - month Age - old Age infant from Personal_background the Personal_background UAE Personal_background with a genetically History - confirmed History diagnosis History of History Jarcho History - Levin History syndrome History and dependent History on History mechanical History ventilation History since History birth History was admitted Clinical_event to our centre Nonbiological_location for the implantation Therapeutic_procedure of Therapeutic_procedure VEPTRs Therapeutic_procedure .
We received an intubated Therapeutic_procedure patient, mechanically Detailed_description ventilated Therapeutic_procedure in a volume Detailed_description - controlled Detailed_description mode Detailed_description , with a 0.30–0.45 fraction Lab_value of Lab_value inspired Lab_value oxygen Lab_value (FiO2) ( peak Lab_value inspiratory Lab_value pressures Lab_value (PIP) Lab_value of Lab_value 23–45  cm Lab_value   H2O Lab_value , positive Lab_value end Lab_value - expiratory Lab_value pressure Lab_value (PEEP) Lab_value of Lab_value 7 Lab_value   cm Lab_value   H2O Lab_value , backup Lab_value respiratory Lab_value rate Lab_value (RR) Lab_value of Lab_value 34 Lab_value ).
A chest Biological_structure X Diagnostic_procedure - ray Diagnostic_procedure of the thorax Biological_structure was performed on admission Clinical_event (figure 1A), and a Date month Date after Date the first surgery Therapeutic_procedure (figure 1B), showing improvement of the bilateral Detailed_description posterior Detailed_description atelectasis Disease_disorder in the base Biological_structure of Biological_structure the Biological_structure lungs Biological_structure .
Owing to the difficulty of progressing in the weaning process from mechanical ventilation after the VEPTRs were implanted to expand the thorax, a bronchoscopy Therapeutic_procedure was performed, showing bronchomalacia Disease_disorder predominantly in both Biological_structure upper Biological_structure lobar Biological_structure bronchi Biological_structure .
As prolonged Detailed_description mechanical Detailed_description ventilation Therapeutic_procedure was expected, a tracheostomy Therapeutic_procedure was previously performed.
After an initial CT Diagnostic_procedure scan (figure 2A), a follow-up CT Diagnostic_procedure scan (figure 2B) a Date month Date after Date the surgery showed a resolution of the posterior Detailed_description - basal Detailed_description atelectasis Disease_disorder , coinciding with a decrease Lab_value of FiO2 Diagnostic_procedure to 0.21 Lab_value .
Postoperatively, the patient was on the Servo Detailed_description - i Detailed_description ventilator Therapeutic_procedure in a volume Detailed_description - controlled Detailed_description mode Detailed_description and needed variable Detailed_description PIPs Diagnostic_procedure between Lab_value 25 Lab_value and Lab_value 45 Lab_value   cm Lab_value   H2O Lab_value and PEEP Diagnostic_procedure values of 7–11  cm Lab_value  H2O.
During the attempts to wean the patient off the ventilator in the following months, he was unable to activate the inspiratory flow trigger and required significant sedoanalgesia Medication to adapt to the ventilator due to repeated episodes of desaturation Sign_symptom , on some occasions with bradycardia Sign_symptom , associated to bronchial Disease_disorder collapse Disease_disorder .
He continued to have asynchrony Sign_symptom which required boluses of sedation Medication in addition to existing medications.
He initially received fentanyl Medication and midazolam Medication through a continuous Administration infusion Administration pump Administration with progressive Dosage increase Dosage in Dosage the Dosage doses Dosage as well as continuous Dosage cisatracurium Medication as a muscular Medication relaxant Medication to adapt to mechanical ventilation (figure 3).
As clinical Sign_symptom stabilisation Sign_symptom was achieved, the muscular Medication relaxant Medication was withdrawn and a progressive Dosage decrease Dosage of sedation Medication was initiated along with a change in the drug regimen.
During this period, the patient developed an ocular Sign_symptom flutter Sign_symptom which was attributed to a pharmacological cause after neurological alterations were ruled out with normal Lab_value EEG Diagnostic_procedure , ophthalmoscopy Diagnostic_procedure , metabolic Diagnostic_procedure workup Diagnostic_procedure and cranial Biological_structure MRI Diagnostic_procedure .
At 5 Date   months Date of Date age Date , the NAVA Therapeutic_procedure mode Therapeutic_procedure started to improve Lab_value the patient's adaptation Diagnostic_procedure to Diagnostic_procedure the Diagnostic_procedure ventilator Diagnostic_procedure through the use of a neural Therapeutic_procedure trigger Therapeutic_procedure .
The NAVA Therapeutic_procedure catheter Therapeutic_procedure additionally costs Detailed_description around Detailed_description €200 Detailed_description per Detailed_description catheter Detailed_description ; according Other_entity to Other_entity the Other_entity manufacturer Other_entity it Other_entity should Other_entity be Other_entity changed Other_entity every Other_entity 5 Other_entity   days Other_entity .
It was required for Duration 7 Duration   months Duration ; nevertheless the catheters Therapeutic_procedure were Therapeutic_procedure changed Therapeutic_procedure every Frequency 15 Frequency   days Frequency without observing a deterioration Sign_symptom of Sign_symptom the Sign_symptom Edi Sign_symptom signal Sign_symptom .
According Other_entity to Other_entity the Other_entity literature, Other_entity Jarcho Other_entity - Levin Other_entity syndrome Other_entity does Other_entity not Other_entity appear Other_entity with Other_entity mental Other_entity retardation Other_entity and Other_entity life Other_entity expectancy Other_entity is Other_entity pretty Other_entity long, Other_entity so Other_entity ethically, Other_entity we Other_entity considered Other_entity that Other_entity this Other_entity patient Other_entity should Other_entity have Other_entity an Other_entity opportunity Other_entity to Other_entity overcome Other_entity his Other_entity thoracic Other_entity insufficiency Other_entity if Other_entity it Other_entity were Other_entity technically Other_entity possible Other_entity .
Tachypnoea Sign_symptom was initially observed with a RR Diagnostic_procedure of 55 Lab_value without other signs of increase in his work Sign_symptom of Sign_symptom breathing Sign_symptom and his RR Diagnostic_procedure eventually returned to normal Lab_value for his age ( 40 Lab_value ) over the next few days.
The synchrony Sign_symptom achieved with the ventilator Therapeutic_procedure allowed a progressive decrease of the sedoanalgesia Medication he received until it Coreference was completely withdrawn in 10 Date   days Date ; and the need for extra boluses of sedation Medication was reduced to zero.
Concurrently to the reduction of sedation, his ocular Sign_symptom flutter Sign_symptom disappeared and he showed significant progress Lab_value in his psychomotor Diagnostic_procedure development Diagnostic_procedure .
The settings used were: NAVA Lab_value level Lab_value of Lab_value 1 Lab_value   cm Lab_value   H2O/µV Lab_value , PEEP Lab_value 11 Lab_value   cm Lab_value   H2O Lab_value , Edi Lab_value trigger Lab_value 0.5 Lab_value  µV.
The patient had tidal Diagnostic_procedure volumes Diagnostic_procedure ( Vt Diagnostic_procedure ) of 6–7  mL/kg Lab_value (figure 3).
After Date 10 Date   days Date on the NAVA Therapeutic_procedure mode Therapeutic_procedure , he did not require any type of sedation Medication and the sporadic episodes of bronchial Disease_disorder collapse Disease_disorder were resolved with a quick increase of PEEP Diagnostic_procedure to 20 Lab_value   cm Lab_value   H2O Lab_value or manual Detailed_description ventilation Therapeutic_procedure with the self Detailed_description - inflating Detailed_description bag Detailed_description .
Once he was stabilised on the NAVA Therapeutic_procedure mode Therapeutic_procedure , his PIPs oscillated between Lab_value 15 Lab_value and Lab_value 45 Lab_value   cm Lab_value   H2O Lab_value with a NAVA Lab_value level Lab_value of Lab_value 0.4 Lab_value and Edi Diagnostic_procedure peaks Diagnostic_procedure between Lab_value 15 Lab_value and Lab_value 100 Lab_value  µV.
A posterior Detailed_description pulmonary Biological_structure CT Diagnostic_procedure scan performed 2 Date   months Date after Date the first surgery, 1 month after starting NAVA and prior to the first thoracic expansion, showed a significant reduction in the posterobasal Detailed_description atelectases Disease_disorder previously observed (figure 2B).
When he turned 1 Date   year Date old Date , after several attempts with different Therapeutic_procedure devices Therapeutic_procedure Trilogy Detailed_description 100 Detailed_description (Philips) Detailed_description , Astral Detailed_description 150 Detailed_description (ResMed) Detailed_description , the patient finally tolerated mechanical Detailed_description ventilation Therapeutic_procedure with a home Detailed_description ventilator Detailed_description ( Monnal Detailed_description T-50, Air Detailed_description Liquide Detailed_description ) in a volume Detailed_description assisted/controlled Detailed_description mode Detailed_description with Detailed_description the Detailed_description following Detailed_description settings Detailed_description : Vt Lab_value 80 Lab_value   mL Lab_value (9 Lab_value   mL/kg) Lab_value , PEEP Lab_value 10 Lab_value   cm Lab_value   H2O Lab_value , flow Lab_value trigger Lab_value 0.5 Lab_value   L/min Lab_value .
His PIPs Diagnostic_procedure were around Lab_value 30 Lab_value   cm Lab_value  H2O.
He did not tolerate Sign_symptom a decrease Lab_value of PEEP Diagnostic_procedure below Lab_value 9 Lab_value   cm Lab_value   H2O Lab_value or short Detailed_description disconnections Detailed_description from the ventilator Therapeutic_procedure .
Currently, the patient is 19 Date   months Date old Date , is able Sign_symptom to Sign_symptom walk Sign_symptom and his neurodevelopment Diagnostic_procedure seems to be normal Lab_value .