Procedural Sedation for Pediatric Patients With Spinal Muscular Atrophy

Procedural Sedation for Pediatric Patients With Spinal Muscular Atrophy Undergoing Intrathecal Treatment

Background and Aim: Spinal muscular atrophy (SMA) is a neuromuscular disease characterized by progressive symmetrical weakness and atrophy of proximal muscles causing from degeneration of anterior horn cells of spinal cord. Nusinersen must be administered intrathecally and this treatment is specially for spinal muscular atrophy. Procedural sedation is commonly enough for intrathecal treatment in children. In this retrospective study, the investigators aimed to present our experience in procedural sedation for the intrathecal treatment of patients with SMA 1,2 and 3 in our hospital.

Study Overview

• Status: Completed
• Conditions: Anesthesia; Child, Only; Muscular Atrophy, Spinal
• Intervention / Treatment: Other: Retrospective observational study

Detailed Description

Design: This research is a retrospective obsevational study. Methods: After approval Institutional Review Board, data from anesthesia charts and electronic medical records of 14 patients with SMA type I, II and III who underwent procedural sedation,such as; the demographics data of the patients (age, gender, weight, American Society of Anesthesiologist (ASA) Physical Status), which anesthetic agent was used and their doeses, during of anesthesia, presence of scoliosis, by whom the procedure was performed etc. was reviewed. All our patients with SMA type II and III who underwent intrathecal treatment are given sedation with different combination of anesthetic agent. Due to peripheral intravenous line was placed previously, intravenous induction was performed in all of our patients For procedural sedation, anesthesiologists used different combination of midazolam, ketamine, propofol, fentanyl or remifentanyl, depending on the patient's requirement. They were oxygenated with a face mask or nasal cannula while their spontaneous breathing continues. All patients were recovered in the recovery room.

Statistical analysis: The data were analyzed by using SPSS Statistics Software (SPSS 24, Chicago, IL, USA) Descriptive statistics were used in this study. Mean and standart deviation were calculated for Age (year), Weight (kilogram), Midazolam Dose (mg/kg), Fentanyl Dose (mcg/kg), Remifentanyl Dose (mcg/kg), Propofol Dose (mg/kg), Ketamine Dose (mg/kg) and Length of Stay in Recovery Room (minute) and frequency analysis were made for the number of intervention.

• Study Type: Observational
• Enrollment (Actual): 14

Contacts and Locations

Study Locations

• Turkey
  • Çankaya
    • Ankara, Çankaya, Turkey, 06800
      • Feyza Sever

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: No older than 18 years (ADULT, CHILD)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Pediatric patients younger than 18 years old with SMA 1,2 and 3 who were to undergo intrathecal treatment under procedural sedation in our clinic

Description

Inclusion Criteria:

• Pediatric patients younger than 18 years old with SMA 1,2 and 3 who were to undergo intrathecal Nusinersen treatment were included in this study.

Exclusion Criteria:

• Patients whose procedures were performed under fluoroscopic guidance in the radiology unit were excluded from the study because their procedures were performed in another hospital.
• Patients without a diagnosis of SMA

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Procedural sedation	The number of pediatric patients with SMA I,II and III who underwent intrathecal treatment required procedural sedation	4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.)
Anesthetics use	The anesthetic agents and their doses that used during procedure	4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.)
Age	Age in year	4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.)
Gender	Gender as female/male	4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.)
Weight	Weight in kilograms	4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.)
American Society of Anesthesiologists (ASA) physical status	ASA score	4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.)
Type of SMA	SMA I, SMA II and SMA III	4 years (The data of the patients were retrospectively analyzed between July 2017 and December 2021.)

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Presence of scoliosis	Yes or no	4 years The data of the patients were retrospectively analyzed between July 2017 and December 2021.)(
How many times intrathecal treatment was performed for each patient	The number of intrathecal treatments was administered for each patient over 4 years was recorded.	4 years The data of the patients were retrospectively analyzed between July 2017 and December 2021.)
By whom the procedure was performed	It was recorded who performed the lumbar puncture procedure from a neurologist, neurosurgeon, or anesthesiologist.	4 years The data of the patients were retrospectively analyzed between July 2017 and December 2021.)
Length of time spent in recovery room	Recovery time in minutes	4 years The data of the patients were retrospectively analyzed between July 2017 and December 2021.)

Collaborators and Investigators

• Sponsor: Ankara City Hospital Bilkent

Study record dates

Study Major Dates

• Study Start (ACTUAL): January 1, 2022
• Primary Completion (ACTUAL): January 31, 2022
• Study Completion (ACTUAL): January 31, 2022

Study Registration Dates

• First Submitted: February 8, 2022
• First Submitted That Met QC Criteria: March 18, 2022
• First Posted (ACTUAL): March 23, 2022

Study Record Updates

• Last Update Posted (ACTUAL): March 23, 2022
• Last Update Submitted That Met QC Criteria: March 18, 2022
• Last Verified: March 1, 2022

More Information

Terms related to this study

• Keywords: Children; Procedural sedation; Spinal muscular atrophy; Intratechal treatment
• Additional Relevant MeSH Terms: Central Nervous System Diseases; Nervous System Diseases; Neurologic Manifestations; Neuromuscular Diseases; Neurodegenerative Diseases; Neuromuscular Manifestations; Pathological Conditions, Anatomical; Spinal Cord Diseases; Motor Neuron Disease; Muscular Atrophy; Atrophy; Muscular Atrophy, Spinal
• Other Study ID Numbers: 2019-159

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No