3.
Name : D/O Sgt Mozaffor
Age : 4½ years
Unit : 11 Sqn, BAF
Gender : Female
Religion : Islam
Address : Jashore
Date of Admission: 9th Aug 2019
Particulars of the Patient
4.
1. H/O recurrent convulsion for last three years
2. Frequent episodes of convulsion for last seven
days
Chief Complaints
5.
According to the father’s statement, the child was suffering
from recurrent episodes of convulsion for last three years.
Convulsions were characterized by involuntary jerky
movements of body followed by unconsciousness which lasted
for few minutes and frequency was 2-3 episodes/week. She was
advised to take anti-epileptic drugs by paediatric neurologist.
History of Present Illness
6.
She was convulsion free for last two and half years with
medications. For last seven days, she developed frequent
episodes of convulsion which were not associated with fever.
There was no history of perinatal asphyxia or any congenital
anomaly.
History of Present Illness …cont’d
7.
The child reported to CMH, Dhaka at 20 months of age and
on the basis of somatic growth (more than age), cognitive
delay, distinctive narrow long facies with large head
-- Diagnosed as a case of “Sotos Syndrome”
History of Past Illness
8.
Drug history : Tab. Sodium Valproate (300 mg) 1+0+1
Tab. Oxcarbazepine (75 mg) 1+0+1
Family history : Nothing contributory
Birth history : Normal Vaginal delivery (uneventful)
Immunization history : Immunized as per EPI schedule
9. Appearance : Dull looking with
Enlarged head
Body weight : 26 kg
Oedema : Absent
Dehydration : Absent
Lymph node : Not enlarged
Pulse : 98/min
Blood pressure : 100/60 mmHg
Respiratory Rate : 22 breaths/min
Temperature : 98.4ºF
General Examination
10.
a. Higher psychic functions–Hyperactive, Talkative.
Intelligence & Concentration were poor.
Cognition was below average.
b. Cranial nerves
c. Motor & Sensory functions NAD
d. Speech, Vision, Hearing
Neuro-developmental Examination
12.
D/O Sgt Mozaffor, 4½ years old, hailing from Jashore, reported
to CMH, Dhaka on 9th Aug 2019 with the complaints of
recurrent episodes of convulsion for last three years.
Convulsions were characterized by involuntary jerky
movements of body followed by unconsciousness which lasted
for few minutes, and frequency was 2-3 episodes/week. She
was convulsion free for last two and half years with
medications.
Salient Features
13.
For last seven days, she developed frequent episodes of
convulsion. She did not have any history of congenital
anomaly. On general examination she was dull looking with
enlarged skull and systemic examination revealed impaired
higher psychic functions.
Salient Features …cont’d
15.
CBC : Within normal limit
Urine RME : Normal
Thyroid profile : Within normal limit
Growth hormone : 2.2 ng/ml
S. FSH : 4.3 mIU/ml
S. LH : 0.1 mIU/ml
RBS : 4.6 mmol/L
Echo : Normal study
Investigations
16.
EEG :
The EEG in awake and sleep state is consistent with runs of
slow wave.
17. X-ray Wrist (B/V):
Appeared Pisiform bone indicate
advanced bony age
MRI of Brain:
Macrocephaly with cavum septum
pellucidum
19.
To exclude any pathology of Brain she was advised by
Paediatric Neurologist to repeat –
MRI of Brain under Anaesthesia.
Management Plan
20.
Smooth separation of child from parents.
Difficulty in IV cannulation.
Creating a friendly environment and undergo a
smooth induction.
Maintenance of spontaneous ventilation and
temperature during MRI
Lack of MRI compatible anaesthetic equipments.
Poor monitoring facilities.
Anaesthetic Challenges
21.
Smooth induction, maintenance and recovery.
Airway maintenance and adequate oxygenation.
Prevention of bradycardia and tachypnoea.
Prevention of over secretion and laryngospasm.
Maintenance of adequate fluid balance.
Prevention of hypothermia .
Anaesthetic Goals
22.
After evaluating history, physical
examination, investigations and airway
assessment patient was accepted for
Anaesthesia in
ASA Grade: II
Mallampati class: II
Pre Anaesthetic Assessment
23.
Consent:
Informed written consent was taken from child’s father
Pre anaesthetic preparation:
NPO for 4 hours
Continue current medications
Pre Anaesthetic Assessment …cont’d
25.
Pre Medication:
Inj Atropine 300 mcg IV
Inj Ondansetron 3 mg IV
Induction:
Inj Ketamine 0.1 mg/kg IV
Inj Propofol 3 mg/kg IV As Ketofol
Conduct of Anaesthesia
26.
Precordial stethoscope
Portable Pulse Oximetry
Heart rate
SPO2
Respiratory rate and Chest movement
Temperature monitoring
Monitoring
29. In Postoperative ward –
NPO for 02 hours
IV fluid: Baby saline
Vital signs monitoring:
Heart rate
Respiratory rate
SPO2
Keeping the baby warm.
When the baby is fully awake – shifted to ward after 02 hrs.
Post Procedure Care
31. Sotos syndrome is autosomal dominant disorder.
The syndrome was first described in 1964 in by Juan F Sotos
Mutations in the NSD1 gene cause Sotos syndrome.
This syndrome is characterized by overgrowth and
advanced bone age.
Diagnosis is based on physical examination & other
symptoms.
Not a life-threatening disorder, treatment is symptomatic.
Incidence is approximately 1 in 14,000 births.
Sotos syndrome
32. Non operating room anesthesia (NORA) refers to administration
of sedation/anesthesia remote from hospital’s main operating
room to patients undergoing painful or uncomfortable
procedures.
Non Operating Room Anaesthesia (NORA)
41.
Inadequately trained or insufficient staff.
Poor communication system.
Difficulty in transportation of patients in case of
emergency.
No co-located recovery room.
Anaesthetic Challenges ...cont’d
42.
Narrow tunnel.
Limited access to the patient.
Keeping the patient calm and quite.
Claustrophobia.
Anaesthetic Challenges in MRI suite
43.
Difficulty in using ferromagnetic equipments ( risk of
accident by projectiles)
Nonavailability of MRI compatible
equipments.
Loud noise.
Allergic reactions to contrast dyes.
Cold environment.
Anaesthetic Challenges in MRI suite ...cont’d
44. Each location should have :
- Sufficient space.
- Skilled staff.
- Reliable source of oxygen, with a backup supply.
- Adequate and reliable source of suction.
- MRI compatible anaesthetic & standard monitoring aids .
- Adequate anaesthetic drugs, supplies, and equipments .
Recommendations
45.
All potentially hazardous articles (watches, rings, jewellery
and mobile phones) should be removed.
Patients should also be screened for potential risks like
cochlear implants, metallic implants and metallic vascular
clips.
Screening for the presence of ferromagnetic anaesthetic
equipments should be done before MRI procedures.
Recommendations …cont’d
46.
GA with Endotracheal Intubation/LMA
TIVA - common drugs used –
Ketamine with Propofol
Midazolam with Fentanyl
Propofol Infusion
Dexmedetomedine
Different Anaesthetic Techniques for
MRI
47.
Advantages:
Good control over airway
Protection against aspiration
Disadvantages:
Requires special setup
Laryngospasm
Post operative nausea, vomiting
GA with ETT/LMA
48.
Advantages:
Rapid onset & recovery
Effective and adjustable anaesthesia
Prevention of nausea and vomiting
Disadvantages:
Poor control over airway
Chance of aspiration
TIVA
50.
Non-operating room anaesthesia (NORA) is taking a vital &
versatile role in modern anaesthetic technique. NORA presents
setting specific challenges to safe anaesthesia care. Protocols and
interdisciplinary teamwork in well equipped setup can facilitate
safe, efficient and cost effective procedural care for NORA.
Conclusion
