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

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Surgery and Anesthesia in MPS II

Christina Lampe, MDCenter for Rare DiseasesDr Horst Schmidt Clinics

Wiesbaden, Germany

First Symptoms

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

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™

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

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

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

Start of ERT

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

Change of the portacath

Summary Surgical Procedures

Adenoidectomy, tonsillectomy, T-tubes

Hernia repair (umbilical and inguinal)

Carpal tunnel release

Hip replacement

Dental surgery

Tracheostomy

Appendectomy

Mastoidectomy

Portacath

Appendectomy

Typical surgicalprocedures in MPS II?

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

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

guinalis

al sur

er fing

ession

n= 527 patients HOS before July 23, 2009

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

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

ANESTHESIA IN MPSA CHALLENGE AND A BALANCE OF RISK !!

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:

Most frequent airway complaints in MPS

Berger et al JIMD 2013

Healthy versus affected epithelial tissue of thebronchi

...and additionally: MPS !!!

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%

Difficult Airway Management

Prepare an individual plan for each patient !

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

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

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

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

Anesthesia in MPS

Teamwork is needed:

Anesthesist Cardiologist pulmologist otolaryngologist surgeon intensive Care metabolic specialist

prepare an individual plan for eachpatient

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

BE AWARE….

• 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!

Thank You for Your Attention !!

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

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

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