+ All Categories
Home > Documents > Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina...

Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina...

Date post: 17-Jul-2019
Category:
Upload: dinhque
View: 214 times
Download: 0 times
Share this document with a friend
25
Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany
Transcript
Page 1: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Surgery and Anesthesia in MPS II

Christina Lampe, MDCenter for Rare DiseasesDr Horst Schmidt Clinics

Wiesbaden, Germany

Page 2: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

First Symptoms

Reprinted from Wraith JE, et al. Genet Med. 2008;10(7):508-516.

Page 3: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Case Reportto demonstrate typical surgicalprocedures and complications In MPS II

Sebastian *1982

Eine der Lieblingsbeschäftigungen von Sebastian ist das Telefonieren mit Freunden. Freunde bedeuten ihm eine

Menge. Außerdem surft Sebastian gerne im Internet.

Als Sebastian 6 Jahre alt war, wurde bei ihm Morbus Hunter festgestellt. Im Laufe der Zeit verursachte Morbus

Hunter bei ihm immer mehr körperliche Beschwerden. Sebastian leidet auch unter starken Schmerzen, wie z.B.

Hüftschmerzen. Er hatte zahlreiche Bandscheibenvorfälle und musste bereits ein künstliches Hüftgelenk erhalten.

Heute sitzt er im Rollstuhl.

Sebastian, 30 Jahre , Bochum, Nordrhein-Westfalen , Deutschland

Ein Projekt der Stiftung Positive Exposure™

Page 4: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Case Report- Sebastian *1982

Family 1st child of healthy German parents 1 healthy brother *1983, 1 healthy sister *1995

Pregnancy/Birth Normal, spontaneous, normal weight and height

-3 years of age Normal mental and motor development Kindergarden

At the age of 3 years Adenoidectomy, tonsillectomy, t-tubes Rec. infections of the upper airways

Diagnosis

4 years: hepatosplenomegaly6 years: diagnosis [Hunter] was found

Page 5: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Case Report- Sebastian *1982

At the age of 9-17 years

Migraine

Hernia inguinalis repair

Glasses, glaucoma

Pain in hips and knees after 20 min walking

Wheelchair (recurrent use)

Torsion of the testis

Mastoidectomy

Problems in school (concentration)

Left school at the age of 18 years

Page 6: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

At the age of 18- 22 years

T-tubes

Wheelchair (full time)

Femoral head necrosis left

Wrist surgery (pain)

Hearing aids

MI I and MI II (enalapril and atenolol)

Osteotomy and extraction of 7 teeth

Hernia umbilicalis repair (incarceration)

CTS both sides

Herniation L2/3 and L5/S1

Coxarthrosis left

Case Report- Sebastian *1982

Start of ERT

Page 7: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

At the age of 24 -32 years

Hip replacement left - complications while extubation:

Tracheostomy (removal after 3 weeks)

Tracheal stenosis

Appendectomy

CTS decompression both sides

CPAP mask at night

Portacath

Heart valve replacement

CTS release both sides

Inguinal hernia

Inguinal hernia repair

Heart valve replacement

Change of the portacath

Case Report- Sebastian *1982

Eine der Lieblingsbeschäftigungen von Sebastian ist das Telefonieren mit Freunden. Freunde bedeuten ihm eine

Menge. Außerdem surft Sebastian gerne im Internet.

Als Sebastian 6 Jahre alt war, wurde bei ihm Morbus Hunter festgestellt. Im Laufe der Zeit verursachte Morbus

Hunter bei ihm immer mehr körperliche Beschwerden. Sebastian leidet auch unter starken Schmerzen, wie z.B.

Hüftschmerzen. Er hatte zahlreiche Bandscheibenvorfälle und musste bereits ein künstliches Hüftgelenk erhalten.

Heute sitzt er im Rollstuhl.

Sebastian, 30 Jahre , Bochum, Nordrhein-Westfalen , Deutschland

Ein Projekt der Stiftung Positive Exposure™

Page 8: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Summary Surgical Procedures

Adenoidectomy, tonsillectomy, T-tubes

Hernia repair (umbilical and inguinal)

Carpal tunnel release

Hip replacement

Dental surgery

Tracheostomy

Appendectomy

Mastoidectomy

Portacath

Appendectomy

Torsion of the testis

Heart valve replacement

Typical surgicalprocedures in MPS II?

Page 9: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Adenoidectomy, tonsillectomy, T-tubes 50% (median age 3 years)

Hernia repair (umbilical and inguinal) 50% (median age 3 years)

Carpal tunnel release 18% (median age 8.7 years)

Dental surgery 14% (median age 7.2 years)

Tracheostomy

Hip replacement

Appendectomy

Mastoidectomy

Portacath

Appendectomy

Torsion of the testis

Heart valve replacement

Typical Surgical Procedures in MPS (II)

51%50%50%

36%29%

18%14%7% 4% 3% 2% 2%

0 10 20 30 40 50 60

ear t

ubes

aden

oide

ctom

y

hern

ia in

guinalis

tons

illec

tom

y

othe

r

carp

al tu

nnel

dent

al sur

gery

intra

cran

ial s

hunt

trach

eoto

my

trigg

er fing

er re

leas

e

spine

deco

mpr

ession

feed

ing

tube

n= 527 patients HOS before July 23, 2009

Page 10: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Age at First Surgical Procedure In MPS (II)

N. Mendelson et al., Importance of Surgical History in diagnosing MPS II: Data from the HOS

Outcome Survey, Genetics in Medicine 2010

Page 11: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

MPS II... Surgical History as a Diagnostic Clue

• median of 3.0 operations per patient, median of 2.0 operations while undiagnosed (57% of patients)

• for the first time at a median age of 2.6 years

• repeated surgeries, especially for ENT, hernia and carpal tunnel syndrome, were very common.

Mendelsohn NJ, et al. Genet Med. 2010 Dec;12(12):816-22.

Hernia repair + ENT intervention

Carpal tunnel syndrome in childhood

Difficult intubation or inability to extubate

+

Page 12: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

ANESTHESIA IN MPSA CHALLENGE AND A BALANCE OF RISK !!

Page 13: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Anesthesia Complications Are Common in MPS

For 428 of 441 patients who were reported in HOS to have had surgical intervention:

• Difficulties with intubation were reported in 22.0%

• Unable to extubate 3.7%

Problems with intubation/extubation were reported to occur before diagnosis:

• Intubation: 18.6%

• Extubation: 7.7%

Mendelsohn NJ, et al. Genet Med. 2010;12(12):816-22.

Example MPS II:

Page 14: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Most frequent airway complaints in MPS

Berger et al JIMD 2013

Page 15: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Healthy versus affected epithelial tissue of thebronchi

...and additionally: MPS !!!

Page 16: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Specific Anesthesiological Risk Factors in MPS

Difficult airwaysdwarfism, short neck

large head, hypertelorismthickended and less flexible epiglottis, hyperplasia soft tissue, macroglossia

cervical spine flexibility,atlanto-axial instability

Limited cardio-respiratory resourceshemodynamically significant heart

changes, cardiomyopathyrecurrent infections of the airways

restrictive and obstructive lung disease

Difficult positioningdeformity of the chest cage

enlagred abdomencontractures

Limited cooperationMental retardation

Postoperative:intensive care

WeaningSwelling of the airways

Perioperative mortality 20%

Page 17: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Difficult Airway Management

Prepare an individual plan for each patient !

Page 18: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Preoperative Evaluation and Planning

• Cardiopulmonary status 1. Echo and ECG

cardiological complications are the

main cause of intraoperative death! 2. Pulmonary testing

• Conditions to intubate 3. Laryngoscopy

ventilation with a mask possible?

Size of the equipment?

Atlanto-axial instability? 4. MRI craniocervical junction

• Conditions to ventilate 5. CT scan airways

tracheal stenosis or malacia

Intraoperative positioning

• Conditions to extubate 6. Organizing management and team!

Early extubation inside the operating theater, tracheotomy stand by, intensive care

Emergency management: postoperative complications, extubation

Page 19: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Intubation - Balance of Risk!• Premedication

anxiolysis, reduction of hypersalivation, oxygen

Cave: obstuctive sleep apnea

Cave: tachycardia by limited cardiological reserve

• Management of difficult airways

correct size of the equipment(standard sizes and medication dosages are not fitting!)

• Clear responsibilities

Calculation of medication dosing in advance

Emergency medication prepared

intubation in tracheostomy stand by ENT

Short-acting anethetics (total intraveneous anesthesia)

prophylaxis of edema of the airways

early extubation in tacheotomy stand by ENT

First choice: awake fiberoptic intubation

Page 20: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Challenge: Mental Retarded Patient

Limited cooperation concerning swallowing, breathing and tolerance of nasal intubation

Recommendations• quiet atmosphere

• oral fibertoptic intubation (cave: muscle relaxation without intubation)

• Careful titration of the medication: a few can be too much!

• Repeat local anesthesie (throat/nose) if necessary

• High risk of mask anesthesia!

• Only short-acting drugs

• Always tracheostomy standby

Dont´t hurry – time is not toxic

Florianliebt Musik und die Simpsons

Ein Projekt der Stiftung Positive Exposure™

Page 21: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Extubation- Balance of Risk again!

Same conditions like intubation

Extubation in tracheostomy standby

• Prophylactic therapy against edema of the mucosa: Fortecortin

• Inspection of the epiglottis after intubation und prior extubation

• Contact to patient possible? - having definitive protective reflexes(coughing, swallowing)

• Sufficient spontaneous breathing

• Step by step extubation(level of vocal folds – hypopharynx – mouth/nose)

Close communication betweenAnesthesiologist & Surgeon

Page 22: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Anesthesia in MPS

Teamwork is needed:

Anesthesist Cardiologist pulmologist otolaryngologist surgeon intensive Care metabolic specialist

prepare an individual plan for eachpatient

Page 23: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Anaesthesia in MPS

• Significant risk due to airway disease and anatomic changes (macroglossia,

narrowed airways, short neck, immobility of the jaw)

• Difficult intubation is common (tracheostomy standby)

• Postprocedure edema of the larynx can make extubation difficult

• Avoid bleeding- mucosa in MPS patients is very vulnerable

• Postoperative observation at an intensive care unit

Mariuszliebt Tomatensuppe

Ein Projekt der Stiftung Positive Exposure™

Page 24: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

BE AWARE….

Eine der Lieblingsbeschäftigungen von Sebastian ist das Telefonieren mit Freunden. Freunde bedeuten ihm eine

Menge. Außerdem surft Sebastian gerne im Internet.

Als Sebastian 6 Jahre alt war, wurde bei ihm Morbus Hunter festgestellt. Im Laufe der Zeit verursachte Morbus

Hunter bei ihm immer mehr körperliche Beschwerden. Sebastian leidet auch unter starken Schmerzen, wie z.B.

Hüftschmerzen. Er hatte zahlreiche Bandscheibenvorfälle und musste bereits ein künstliches Hüftgelenk erhalten.

Heute sitzt er im Rollstuhl.

Sebastian, 30 Jahre , Bochum, Nordrhein-Westfalen , Deutschland

Ein Projekt der Stiftung Positive Exposure™

• high anesthesia risk ! Also adult and mild affected MPS patients

• discuss necessity of each surgery andcombine different surgeries

• multidiscipliary team is needed foreach single surgery- prepare the colleagues!

• Prepare the patient!

Page 25: Surgery and Anesthesia in MPS II - Brains for Brain · Surgery and Anesthesia in MPS II Christina Lampe, MD Center for Rare Diseases Dr Horst Schmidt Clinics Wiesbaden, Germany

Thank You for Your Attention !!

Many thanks to Dr. Matthias Schäfer, who providedthe anesthesia knowledge in MPS !!!!


Recommended