Managementul Sas In Pw

38
MANAGEMENTUL SAS IN PRADER - WILLI Stefan Mihaicuta UMF V. Babes Clinica de Pneumologie

Transcript of Managementul Sas In Pw

Page 1: Managementul Sas In Pw

MANAGEMENTUL SAS IN PRADER - WILLIStefan Mihaicuta

UMF V. Babes

Clinica de Pneumologie

Page 2: Managementul Sas In Pw

Caz

S. M., n. 20.09.1988, Carei, SM In noiembrie 2008, 150 cm, 105 kg, Circumferinta gat 39 cm, circumferinta

abdominala 121, Circumferinta bazin 141 cm HTA 120/70 mm Hg Trimisa de prof. dr. Puiu Maria

Page 3: Managementul Sas In Pw

Apnei raportate, fara sforait. Fara somnolenta diurna excesiva, Epworth 3 Rar cefalee matinala, Somn agitat, nicturie 1x/noapte, cu treziri din somn. Fara modificari ORL (deviatie sept, polipoza

nazala, uvula hipertrofica), Malampati III. Tratament cu Levotiroxin (hipotiroidism)

Page 4: Managementul Sas In Pw

Epworth Sleepiness Score

In poziţia şezând  Vizionare TV

Aşezat, inactiv, într-un loc public (cinema, sedinţe, sală de aşteptare, etc.)  Pasager într-o maşină, o oră fără oprire

Aşezat pe spate pentru odihnă diurnă

Aşezat şi discutând cu cineva

Aşezat liniştit după un prânz fără alcool

În maşină, la un stop

 

Page 5: Managementul Sas In Pw

PSG – traseu normal

Page 6: Managementul Sas In Pw

PSG cu apnee centrala

Page 7: Managementul Sas In Pw

PolisomnografiePatient name : SERESAN Acq : 707

First name : MARIA Type : Adult

Sex : F Started : 11/4/2008 at 10:18:13 PM

Birth date : 9/20/1988 Stopped : 11/5/2008 at 7:01:13 AM

Patient age : 20 years Duration : 8:43:00 (523.0 min)

Recording duration : 523.0 min Recording start -> end

TIB : 523.0 min Light off -> Light on

SPT : 488.5 min Sleep Onset -> Last Sleep Page

TST : 469.0 min REM + NREM + MVT (during SPT)

WK before sleep : 32.0 min WK from Light off to Sleep onset

WK during sleep : 19.5 min SPT - TST

WK after sleep : 0.0 min WK from Last sleep page to Light on

TWK duration (tot) : 51.5 min All WK summed

REM duration : 55.0 min REM (during TIB)NREM duration : 416.0 min S1 + S2 + S3 + S4 (during TIB)SWS duration : 78.5 min S3 + S4 (during TIB)Movement : 0.5 min MVT (during TIB)

Durations

Page 8: Managementul Sas In Pw

Caracteristicile somnului

From Light off (min) From Sleep onset (min)

Sleep onset 34.5 -

S1 32.0 12.5S2 34.5 0.0

S3 144.5 110.0S4

REM 253.5 219.0

Latencies

Sleep efficiency 1 : 89.7% 100 x TST/TIB

Sleep efficiency 2 : 96.0% 100 x TST/SPT

Sleep efficiency 3 : 28.5% 100 x (N3+REM)/TST

Inter-sleep WK : 4.0% WK in sleep / SPT

Page 9: Managementul Sas In Pw

Evenimente respiratoriiCA OA MA Sum Ap HYP Events

Settings (sec) 10.0 10.0 10.0 - 10.0 -

Number 4 47 1 52 376 428

Max (sec) 15.5 22.5 12.0 22.5 53.0 53.0

Mean (sec) 13.4 12.0 12.0 12.1 16.0 15.5

Tot duration (min) 0.9 9.4 0.2 10.5 100.0 110.5

TST (469.0 min)

% of TST 0.2 2.0 0.0 2.2 21.3 23.6

Index [#/h TST] 0.5 6.0 0.1 6.7 48.1 54.8

Page 10: Managementul Sas In Pw

PSG – hipopnee cu desaturari

Page 11: Managementul Sas In Pw

PSG

Page 12: Managementul Sas In Pw

REM #/h (REM)

NREM #/h(NREM)

TST #/h (sleep)

RDI 31.6 57.8 54.8

Central apnea total duration

: 1.68 minutes (0.3% of sleep)

Total number of central apnea

: 12 (1.5 CA/h (sleep))

Sigh rate : 8%

Periodic breathing total duration

: 1.57 minutes (0.3% of sleep)

Page 13: Managementul Sas In Pw

PSG – apnei centrale, desaturari, flux respirator scazut

Page 14: Managementul Sas In Pw

Cardio-vascular

WK REM NREM S1 S2 S3 MVT

Duration (min) 51.5 55.0 416.0 86.0 251.5 78.5 0.5

Mean HR (BPM) 90.5 88.8 88.6 94.2 87.3 86.4 113.0

Stand. dev. 8.356 6.891 8.777 8.908 8.232 7.718 2.000

Coef. var. 0.092 0.078 0.099 0.095 0.094 0.089 0.018

Median (BPM) 89.000 87.000 87.000 94.500 86.000 85.000 112.000

IQ 8.000 4.000 10.000 12.000 8.000 5.000 2.000

# of LHR 0 0 16 0 11 5 0

LHR min (BPM) 57 57 58

# of HHR 0 7 34 9 18 7 0

HHR max (BPM) 124 186 135 186 130

Mean RR (BPM) 92.4 89.7 89.7 95.4 88.4 87.3 112.9

Stand. dev. 20.215 12.928 12.548 13.067 12.521 9.677 2.641

Coef. var. 0.219 0.144 0.140 0.137 0.142 0.111 0.023

Mean RR (msec) 663.0 676.0 677.6 636.9 687.4 694.6 531.7

Stand. dev. 130.181 56.643 72.330 65.540 70.506 67.921 12.488

Coef. var. 0.196 0.084 0.107 0.103 0.103 0.098 0.023

ECG fail (min) 0.0 0.0 0.0 0.0 0.0 0.0 0.0

Statistics

Page 15: Managementul Sas In Pw

Saturatia in O2WK REM NREM TOTAL

Fail duration (min)

0.2 0.0 0.0 0.2

Average (%) 98 97 96 96

Desat Index (#/hour) 8.7 29.7 27.2

Duration desat/hour 1.8 6.3 5.8

Desat max (%) 0 5 19 19

Desat max dur (sec) 0.0 20.0 45.0 45.0

Page 16: Managementul Sas In Pw

Desaturari

Longest continuous duration spent below 0%: 0.0 minute(s)

Lowest SpO2 (>= 2 seconds) [%]: 79

# Episodes (>= 5.0 minutes) SpO2 < 88 %: 0

Longest duration SpO2 < 88 % 0.0 minute(s)

Mean of the resp. event SpO2 min levels [%] : 93

Mean of the resp. event SpO2 min levels with desat [%] : 92

Minimum of the resp. event SpO2 min levels [%] 79

Page 17: Managementul Sas In Pw

Total number of WK or MVT episodes : 3

Arousal index : 5.8/h(sleep)

% of pages with arousal during sleep : 4.8

Number of arousals associated with leg movements : 0

Number of arousals NOT associated with leg movements

: 45

Page 18: Managementul Sas In Pw

Hipnograma completa

Page 19: Managementul Sas In Pw

DiagnosticSindrom de apnee in somn forma obstructiva (predominant hipopnei) cu indice de apnee- hipopnee 54,8/h, indice de desaturare 27/h, saturatia medie 96%. Tahicardie sinusala nocturna (88/min). Hipnograma cu alterarea structurii somnului, reducerea somnului REM.Scaderea duratei si eficientei somnului profund la 28%.  Se recomanda titrare CPAP in timpul somnului.

Page 20: Managementul Sas In Pw

HipnogramaHR,BPM

30507090110130150170190

SpO2,%

50

60

70

80

90

100

Stage

S4

S3

S2

S1

REM

MVT

WK

CA,sec

0

10

20

OA,sec

0

10

20

MA,sec

0

10

20

HYPO,sec

0

10

20

Night Hypnogram

10:18:13 PM 12 AM 1 AM 2 AM 3 AM 4 AM 5 AM 6 AM 7 AM

Page 21: Managementul Sas In Pw

ManagementFORMA CLINICĂ TRATAMENT

RECOMANDAT IAH > 30/H, CU SIMPTOME SEVERE NCPAP, DIETĂ, IGIENA

SOMNULUI

IAH < 30, CU SIMPTOME UŞOARE DIETĂ ŞI IGIENA SOMNULUI

IAH < 30, CU SIMPTOME SEVERE DIETĂ, IGIENA SOMNULUI, CPAP PROVIZORIU

IAH > 30/H, RISC CV IGIENA SOMNULUI, DIETĂ, CPAP

HIPERTROFIE ŢESUT MOALE

FARINGIAN SAU ANOMALII SCHELETALE INTERVENŢII CHIRURGICALE ORL

Page 22: Managementul Sas In Pw

Managementul opţiunilor în SASOformă severă

1. CPAP- opţiune de elecţie.

2. Altele – nonresponderi la CPAP, când nu este tolerat: scădere ponderală, poziţie non-supin.

3. Măsurile de a avansa mandibula:(i) dispozitive orale(ii) Osteotomia maxilomandibulară – f rar...

Doar la pacienţii tineri, slabi şi foarte motivaţi.

4. Intervenţia chirurgicală asupra căilor aeriene superioare (UPPP) şi ablaţia prin radiofrecvenţă - nu în cazurile severe.

Page 23: Managementul Sas In Pw

PSG

Page 24: Managementul Sas In Pw

Dispozitive

Page 25: Managementul Sas In Pw

CPAP

80% din pacienţi acceptă CPAP ca terapie de lungă durată la domiciliu

W. T. McNicholas, M. R. Bonsignore, Eur Respir J, January 2007; 29:156-178

Page 26: Managementul Sas In Pw
Page 27: Managementul Sas In Pw

CPAP: impactul asupra activităţii din timpul zilei

Engleman HM et al, Lancet 1994;343:572–575

Engleman HM et al, Thorax 1998;53:341–345

Redline S et al, AJRCCM 1998;57:858–865

Jenkinson C et al, Lancet 1999;353:2100–2105

, B a lle s t e r E e t a l AJRCCM 1 9 9 9 ;1 5 9 :4 9 5 – 5 0 1 , , 2 0 0 1 , 1 6 4 :6 0 8 -1 3M o n t s e r r a t e t a l A J R C C M

nCPAP aduce îmbunătăţiri semnificative

pe somnolenţă şi calitatea vieţii la pacienţii

cu simptome de SAS moderată sau severă

, , 2 0 0 0 , 1 6 1 :1 7 7 5 -8R D a v ie s a n d J S t r a d lin g A J R C C M

Page 28: Managementul Sas In Pw

Morbiditate cardiovasculară

Page 29: Managementul Sas In Pw

Cauze de HTA secundară Sindromul de apnee în somn HTA indusă de medicamente sau droguri Nefropatiile cronice Hiperaldosteronismul primar Boala renovasculară Corticoterapia cronică şi sindromul Cushing Feocromocitomul Coarctaţia de aortă Afecţiunile tiroidiene sau paratiroidiene

(The Seventh Report of the Joint National Comittee on Prevention, Detection, Evaluation and Treatment of High Blood Pressure. The JNC Report). JAMA 2003; 289: 2560-2572.

Page 30: Managementul Sas In Pw

Somers et al, NEJM 2005

Moarte subită

Page 31: Managementul Sas In Pw

Mortalitatea crescută la pacienţii care nu primesc CPAP

Doherty LS Chest. 2005;127:2076-2084Long-term Effects of Nasal Continuous Positive Airway Pressure Therapy on Cardiovascular Outcomes in Sleep Apnea Syndrome*

Chest. 2005;128:624-633.Mortality in Obstructive Sleep Apnea-Hypopnea Patients Treated With Positive Airway Pressure* Francisco Campos-Rodriguez,

Marti, S, Sampol, G, Muñoz, X, et al Mortality in severe sleep apnoea/hypopnoea syndrome patients: impact of treatment. Eur Respir J 2002;20,1511-1518

Page 32: Managementul Sas In Pw

Medical care

Initial management of hypotonia or poor feeding

Evaluation for hypogonadism or hypopituitarism

Management of obesity   Monitoring for scoliosis

Therapy for behavioral issues

Page 33: Managementul Sas In Pw

Surgical Care

Complications of obesity: gastric bypass Cryptorchidism Scoliosis intervention. Urgent surgical attention for abdominal issues:

cholecystitis, appendicitis, or acute gastric dilation with risk for progression to necrosis.

Tonsillectomy, adenoidectomy, or tracheostomy placement may be required in patients with obstructive sleep apnea.

Page 34: Managementul Sas In Pw

Consultations Geneticist for initial diagnosis and counseling   Developmental pediatrician for stimulation programs

Endocrinologist for management of hypogonadism

Nutritionist for dietary counseling

Ophthalmologist for management of strabismus

Pulmonologist for management of sleep apnea

Psychiatrist, psychologist, or both for management of behavioral issues

Page 35: Managementul Sas In Pw

Circumferinţa abdominală medie 120,06 cm

Obezitatea abdominală (plus ≥2 elemente) - element obligatoriu în noua definiţie a sindromului metabolic: peste 80 cm la femei şi peste 94 la bărbaţi la europeni

Lancet 2007; 369:2059-2061

Page 36: Managementul Sas In Pw

Diet +Activity

Limitation of access to foods Supplemental occupational and physical therapy to

promote acquisition of gross and fine motor skills and to strengthen spinal musculature in order to minimize scoliosis.

Encouragement of physical activity at home, at school (eg, increased physical education periods), and in the community (eg, Special Olympics)

Page 37: Managementul Sas In Pw

Follow-up

Inpatient evaluation and treatment for hypotonia and poor feeding during infancy.

Individuals with scoliosis and complications of obesity or pickwickian syndrome, may require inpatient therapy.

Patients with severe behavioral problems may merit admission to a facility staffed with individuals with long-term experience with PWS.

Page 38: Managementul Sas In Pw

Follow-up

Routinely monitor for symptoms of sleep apnea.

Obtaining a sleep study within the few months after initiation of growth hormone therapy at the first sign of symptoms.