li maya.doc

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V.7. Penegakan Diagnosis a. Anamnesis tambahan - Riwayat kehamilan - Infeksi - Asfiksia intrauterin - Preeklampsia/eklampsia - Gaya hidup (rokok, kunsumsi obat- obatan, gizi) - Radiasi - Riwayat perinatal - Prematuritas - Perdarahan otak - Ikterus (hiperbilirubinemia) - Anoksia/hipoksia / Asfiksia neonatorum - Trauma lahir - Riwayat postnatal - Trauma kapitis - Meningitis/ensefalitis - Racun : logam berat, CO - Riwayat keluarga - Terjadi pada anak sebelumnya - Terjadi pada keluarga yang lain - Bisa beraktivitas? Terbatas? Tidak bisa sama sekali? b. Pemeriksaan fisik tambahan

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Page 1: li maya.doc

V.7. Penegakan Diagnosis

a. Anamnesis tambahan

- Riwayat kehamilan

- Infeksi

- Asfiksia intrauterin

- Preeklampsia/eklampsia

- Gaya hidup (rokok, kunsumsi obat-obatan, gizi)

- Radiasi

- Riwayat perinatal

- Prematuritas

- Perdarahan otak

- Ikterus (hiperbilirubinemia)

- Anoksia/hipoksia / Asfiksia neonatorum

- Trauma lahir

- Riwayat postnatal

- Trauma kapitis

- Meningitis/ensefalitis

- Racun : logam berat, CO

- Riwayat keluarga

- Terjadi pada anak sebelumnya

- Terjadi pada keluarga yang lain

- Bisa beraktivitas? Terbatas? Tidak bisa sama sekali?

b. Pemeriksaan fisik tambahan

Pemeriksaan taraf perkembangan sesuai umur kronologis (pra skrining

dan skrining), terutama pada bayi berisiko tinggi CP dilakukan tiap bulan.

c. Pemeriksaan penunjang

- Pemeriksaan mata dan pendengaran segera dilakukan setelah

diagnosis CP ditegakkan.

- Pungsi lumbal harus dilakukan untuk menyingkirkan suatu proses

degeneratif. Pada CP likuor serebrospinalis normal.

Page 2: li maya.doc

- Pemeriksaan Elektro Ensefalografi dilakukan pada penderita

kejang atau pada golongan hemiparesis baik yang berkejang

maupun yang tidak.

- Foto kepala (X-ray) dan CTScan.

- MRIuntuk melihat infark yang terjadi di otak

- Penilaian psikologik perlu dilakukan untuk menentukan tingkat

pendidikan yang diperlukan.

- Pemeriksaan metabolik untuk menyingkirkan penyebab lain

retardasi mental.

- Selain pemeriksaan di atas, kadang-kadang diperlukan pemeriksaan

arteriografi dan pneumoensefalografi

V.8. Diagnosis banding

Tabel 4. Diagnosis Banding

No Deskripsi CP

(Spastic

quadriple

gic)

Muscular

Atrophy

Down

Syndrome

Spina Bifida

1 Kelahiran Preterm + +/- +/- +/-

2 APGAR score

- 1 (menit 2)

- 5 (menit 5)

(Apgar score

rendah)

+

-

- -

3 Asphyxia saat

kelahiran

+ - + -

4 Berat bayi kurang

dari 2000g.

+ - +/- -

Page 3: li maya.doc

5 Bayi laki-laki + + - -

6 Gangguan

perkembangan

motorik.

+ Gangguan

tidak tampak

sehingga

anak tersebut

bisa berjalan.

+ Abnormalit

as CNS

7 Masalah dalam

pemakanan(susah

menelan)

+ + + -

8 Kejang -/+ + + +

V.9. Diagnosis Kerja

A. Cerebral Palsy

1. Definisi

Kelainan pada posture dan pergerakan akibat lesi pada otak yang belum mature

2. Etiologi

a) Prenatal

1) Malformasi congenital

2) Infeksi dalam kandungan (TORCH)

3) Asfiksia intrauterine

4) radiasi

b) Natal

1) Anoksia

2) Hipoksia

3) Trauma lahir

4) Perdarahan intracranial

5) Prematuritas

c) Post natal

1) Trauma kepala

Page 4: li maya.doc

2) Infeksi (misalnya meningitis bacterial, abses serebri,dll)

3) kernicterus

d) Idiopatik

3. Epidemologi

Cerebral palsy terjadi pada 1-2 dari 1000 bayi

4. Factor resiko

a) 10 kali ditemukan pada bayi premature

b) BBLSR <1500 gram

c) Kepala kecil (mikrocephali)

d) Kehamilan letak sungsang

e) Kejang segera setelah lahir

5. Manifestasi klinis

Bergantung pada klasifikasinya :

a) Tipe spastic (50% dari kasus Cerebral palsy), otot-oto menjadi kaku dan lemah

Kekakuan yang terjadi dapat berupa :

1) Kuadriplegia (kedua lengan dan tungkai)

2) Diplegia (kedua tungkai)

3) Hemiplegia (lengan dan tungkai pada salah satu satu tungkai)

b) Tipe diskinetik (20% dari semua kasus CP)

1) Otot lengan, tungkai dan badan secara spontan bergerak perlahan

menggeliat tak terkendali, tetapi bisa juga timbul gerakan kasar dan

mengejang.

2) Gerakan khorea athetosis (gerakan yang tidak terkontrol )

3) Gerakan menghilang bila anak tidur

4) Intelegensia biasanya normal

5) Bermasalah dalam berjalan,duduk dan berbicara dengan jelas

6) Bermasalah dalam mengontrol otot wajah.

c) Tipe ataksik (10% dari semua kasus CP)

1) Tremor

2) Langkah goyah dengna kedua tungkai terpisah jauh

3) Gangguan koordinasi dan gerakan abnormal

4) Memiliki masalah dalam keseimbangan dan koordinasi

Page 5: li maya.doc

5) Bersifat sebentar karena akan berubah nantinya ke tipe spastic dan

diskinetik

d) Tipe campuran (20% dari semua kasus CP)

Gejala lain yang bisa ditemukan :

a) Kecerdasan dibawah normal

b) Retardasi mental

c) Kejang/epilepsy

d) Refleks primitive menetap atau lambat hilang

e) Gangguan menghisap atau makan

f) Gangguan perkembangan motorik

g) Tidak bisa mempertahankan berat badan

h) Gerak menjadi terbatas (otot kaku)

Page 6: li maya.doc

Gambar 3. Patofisiologi Cerebral Palsy pada Amri

B. Mikrosefali

1. Definisi:

Suatu keadaan lingkar kepala yang < 42 cm atau kurang dari standar

deviasi 3 dibawah angka rata-rata. Atau, tidak tumbuhnya jaringan otak

dengan gejala retardasi, ukuran kepala lebih kecil daripada normal akan tetapi

bentuk masih normal. Secara patologis terdapat kelainan seperti hipoplasia

serebri, pakigiria, mikrogiria, porensefali, atrofi serebri dan sebagainya.

2. Etiologi:

a. Genetik

b. Didapat:

B. Antenatal pada morbili, penyinaran, sifilis, toksoplasmosis,

kelainan sirkulasi drah janin atau tidak diketahui

penyebabnya.

C. Intranatal akibat perdarahan atau anoksia

Page 7: li maya.doc

D. Pascanatal dini setelah ensefalitis, trauma kepala dan

sebagainya.

3. Faktor resiko

a. Ibu alkoholisne

b. Ibu dengan fenilketonuria

c. DM maternal

d. Penggunaa Obat-obatan terlarang oleh ibu

4. Gejala klinis:

a. Kepala lebih kecil daripada normal, sekunder akibat jaringan otak yang

tidak tumbuh.

b. Sutura kranialis menutup sebelum waktunya, kadang-kadang ubun-

ubun besar dan kecil terbuka.

c. Didapatkan retardasi mental.

d. Mungkin didapatkan juga gejala motorik berupa diplegia spastik,

hemiplegia dan sebagainya.

e. Terlambat bicara dan kadang-kadang didapatkan kejang.

C. Kurang Energi Protein (KEP)

1. Definisi:

KEP adalah gangguan gizi yang disebabkan oleh kekurangan protein dan atau

kalori, serta sering disertai dengan kekurangan zat gizi lain.

2. Klasifikasi

Menurut WHO:

a. KEP ringan   : > 80-90% BB  ideal terhadap TB (WHO-CDC)

b. KEP sedang : > 70-80% BB  ideal terhadap TB (WHO-CDC) 

c. KEP berat : < 70% BB ideal terhadap TB (WHO-CDC)

Klasifikasi Berdasarkan etiologi:

- Malnutrisi primer

- Malnutrisi sekunder

Secara klinis KEP :

- Kwashiorkor

- Marasmus

Page 8: li maya.doc

- Marasmus-kwashiorkor

3. Dampak KEP

- gangguan pertumbuhan

- atrofi otot

- penurunan kadar albumin serum

- penurunan hemoglobin

- penurunan sistem kekebalan tubuh

- penurunan berbagai sintesa enzim

Penegakan diagnosis

1. Anamnesis

a. Riwayat selama kehamilan

1) Kehamilan letak sungsang

2) Kehamilan kembar

3) Riwayat infeksi pada ibu

4) Riwayat penyakit pada ibu (seperti hipertensi, DM)

b. Riwayat selama melahirkan

1) Apakah ada trauma saat lahir

c. Riwayat setelah melahirkan

1) Apakah bayi lahir premature

2) Berapa berat bayi saat lahir

3) Apakah bayi mengalami asfiksia

4) Apakah bayi mengalami kejang

5) Apakah bayi mengalami hiperbilirubinemia

d. Riwayat keluarga dengan kondisi serupa

e. Riwayat obat-obatan

f. Riwayat pembedahan

g. Riwayat trauma

h. Bagaimana perkembangan anak sebelum mengalami keluhan

i. Sampai usia berapa ia terlihat normal

j. Apakah ada riwayat kejang? Bila ada, berapa kali?

2. Pemeriksaan neurologis

a. Tertundanya perkembangan kemampuan motorik.

Page 9: li maya.doc

b. Refleks infantil (misalnya menghisap dan terkejut) tetap ada meskipun

seharusnya sudah menghilang.

c. Tremor otot atau kekakuan tampak dengan jelas, dan anak cenderung melipat

lengannya ke arah samping, tungkainya bergerak seperti gunting atau gerakan

abnormal lainnya.

3. Pungsi lumbal untuk menyingkirkan penyebab suatu proses degenerative dan

meningitis (pada CP : CSS normal)

4. MRI kepala à menunjukkan adanya kelainan struktur maupun kelainan bawaan

5. CT scan à untuk identifikasi adanya perdarahan, kelainan struktur, maupun

kelainan bawaan

6. EEG berguna untuk mengevaluasi severe hypoxic-ischemic injury. EEG

merupakan alat penting pada diagnosis seizure disorder. Jika CP tidak disertai

kejang (epilepsy atau epileptic syndrome), EEG tidak diindikasikan (AAN, 2004)

7. Tes genetik untuk mengetahui adakah kecenderungan CP diturunkan

Diagnosis banding berdasarkan jenis penyakit:

1. Cerebral Palsy (CP)

2. Sindrom Down

3. DMD

4. Spina Bifida

5. Defisiensi Enz. Arginase

Subtipe CP:

1. Spastik

2. Diskinetik (koreoatetoid)

3. Ataksia

Tabel 6. Diagnosis banding

CP tipe

spastic

CP tipe

diskinetic

CP tipe

ataxic

Sindrom

down

DMD(duscen

t muscle

distropy

kasus

Jenis

kelamin

Laki-laki >

perempuan

Laki-laki

>perempu

Laki-laki

>perempu

Laki-

Page 10: li maya.doc

an an laki

Motorik

kasar(dudu

k dan

merangkak

)

Terlambat

dan statis

Terlambat

dan statis

Terlambat

dan statis

Terlambat

atau

normal

Normal/

sedikitTerlam

bat padaawal

umur,

selanjutnya

mengalami

kemunduran

progresif

Belum

bisa

pada

usia 12

bulan

Anak

pertama

>62,5% >62,5% >>>62,5% _ _ +

Usia ibu <30thn <30thn <30thn >35thn _ 22 thn

Persalinan

spontan

87,5% 87,5% 87,5% _ _ +

Usia

kehamilan

75%

aterm/preter

m

75%

aterm/pret

erm

75%

aterm/pret

erm

aterm aterm Preterm

36

minggu

Antenatal

care

canggih(ra

diasi)

FR FR FR _ _ +

Tidak

langsung

menangis

>> + >> + >> + -/+ >>Langsung

menangis

+

APGAR Asfiksia

berat

Asfiksia

berat

Asfiksia

berat

-/+ -/+ Asfiksi

a berat

BBL BBLR BBLR BBLR BBLR >>normal 2000

gram

BBLR

Motorik

halus(belu

m bisa

makan

terlambat terlambat terlambat Normal/+

klo ada

kelainan

kongengita

Normal/

sedikitTerlam

bat padaawal

umur,

+

Page 11: li maya.doc

nasi) l lain selanjutnya

mengalami

kemunduran

progresif

Bicara

bahasa

Resiko

bertambah

pada

quadriplegi

Biasa

terjadi

karna otot

orofarig

kena

normal terganggu terganggu Bisa

mama

danpap

a

Riwayat

kejang

>>spastic

quadriplegi

jarang - -/+ Jarang sekali _

BB >>malnutrisi >>malnutr

isi

>>malnutr

isi

Klo +

gangguan

kongenital

pencernaa

n

-/+ 6,6 kg

2:mo

derate

pertumbuh

an

Terganggu

karna

gangguan

otot

pencernaan(

otot

orofaring),s

usah

menelan

Terganggu

karna

gangguan

otot

pencernaa

n(otot

orofaring)

normal -/+ -/+ tergang

gu

mikrosefali + pada

quadriplegi

Jarang,

karna

kognisinya

jarang

kena

-, karna

yang kena

otak yang

mengatur

keseimban

gan dan

depth

preseption

-/+ -/+

Page 12: li maya.doc

Gambaran

dismorfik

- - - + _ _

Gerakan

yang tidak

terkontrol(c

horeoatheto

sis

_ + _ -/+ _ _

Refleks

primitif(mo

ro,

menggengg

am, tendon

meningkat)

+ + + -/+ -/+ +

Kekuatan

kedua

lengan dan

tungkai

menurun menurun menurun Normal/

menurun

menurun 3,

normal

nya 5

Lengan dan

tungkai

kaku dan

susah

untuk

ditekuk

+ rigiditas

+ rigiditas

_

_

_

_

-/+

-/+

-/+

-/+

+

+

Kedua

tungkai

saling

menyilang

pada posisi

vertikal

rigiditas _ _ _ _ +

Page 13: li maya.doc

No Deskripsi Muscular

Atrophy

Down

Syndrome

Cerebral Palsy

(Spastic

quadriplegic)

Spina Bifida

1 Kelahiran

Preterm

+/- +/- + +/-

2 APGAR score

- 1 (menit

2)

- 5 (menit

5)

(Apgar score

rendah)

-

- + -

3 Asphyxia saat

kelahiran

- + + -

4 Berat bayi

kurang dari

2000g.

- +/- + -

5 Bayi laki-laki + - + -

6 Gangguan

perkembangan

motorik.

Gangguan

tidak

tampak

sehingga

anak

tersebut

bisa

berjalan.

+ + Abnormalitas

CNS

7 Masalah dalam

pemakanan(sus

ah menelan)

+ + + -

8 Kejang + + - +

Score Ashworth Scale  (1964) Modified Ashworth Scale  Bohannon &

Page 14: li maya.doc

Smith (1987)

0 (0) No increase in tone No increase in muscle tone

1 (1)

Slight increase in tone

giving a catch when the

limb was moved in flexion

or extension

Slight increase in muscle tone, manifested by a

catch and release or by minimal resistance at the

end of the range of motion when the affected

part(s) is moved in flexion or extension.

1+(2)

Slight increase in muscle tone, manifested by a

catch, followed by minimal resistance

throughout the reminder (less than half) of the

ROM (range of movement).

2 (3)More marked increase in

tone but limb easily flexed.

More marked increase in muscle tone through

most of the ROM, but affected part(s) easily

moved.

3 (4)

Considerable increase in

tone - passive movement

difficult.

Considerable increase in muscle tone passive,

movement difficult.

4 (5)Limb rigid in flexion or

extension.Affected part(s) rigid in flexion or extension.

Diagnosis Kerja

DEFINISI

Cerebral palsy adalah suatu gangguan atau kelainan yang terjadi pada suatu kurun

waktu dalam perkembangan anak, mengenai sel-sel motorik di dalam susunan saraf

pusat, bersifat kronik dan tidak progresif akibat kelainan atau cacat pada ja-

ringan otak yang belum selesai pertumbuhannya. Walaupun lesi serebral bersifat

statis dan tidak progresif, tetapi perkembangan tanda-tanda neuron perifer akan

berubah akibat maturasi serebral.

ANGKA KEJADIAN

Page 15: li maya.doc

Insidensi cerebral palsy di Eropa (1950) sebanyak 2,5 per 1000 kelahiran hidup.

Sedangkan di Skandinavia sebanyak 1,2 - 1,5 per 1000 kelahiran hidup

Gilroy memperoleh 5 dan 1000 anak memperlihatkan defisit motorik yang sesuai

dengan cerebral palsy; 50% kasus termasuk ringan sedangkan 10% termasuk

berat. (Yang dimaksud ringan ialah penderita yang dapat mengurus dirinya sendiri,

sedangkan yang tergolong berat ialah penderita yang memerlukan perawatan

khusus)

25% mempunyai intelegensi rata-rata (normal), sedangkan 30% kasus

menunjukkan IQ di bawah 70

35% disertai kejang

50% menunjukkan adanya gangguan bicara

Laki-laki lebih banyak daripada wanita (1,4:1,0)

Insiden relatif cerebral palsy yang digolongkan berdasarkan keluhan motorik

adalah sebagai berikut: spastik 65%, atetosis 25%, dan rigid, tremor, ataktik I0%

ETIOLOGI

Penyebab cerebral palsy dapat dibagi dalam tiga periode, yaitu:

1) Pranatal (dari masa gestasi smpai saat lahir):

Malformasi kongenital.

Infeksi dalam kandungan yang dapat menyebabkan kelainan janin (misalnya;

rubela, toksoplamosis, sifilis, sitomegalovirus, atau infeksi virus lainnya).

Radiasi.

Toksemia gravidarum.

Asfiksia dalam kandungan (misalnya: solusio plasenta, plasenta previa, anoksi

maternal, atau tali pusat yang abnormal).

2) Natal (dari lahir sampai 1 bulan kehidupan):

Anoksialhipoksia.

Perdarahan intra kranial.

Trauma lahir.

Prematuritas.

3) Postnatal (dari bln pertama kehidupan sampai 2 tahun):

Trauma kapitis.

Infeksi misalnya : meningitis bakterial, abses serebri, tromboplebitis,

ensefalomielitis.

Page 16: li maya.doc

Kern icterus.

Beberapa penelitian menyebutkan faktor prenatal dan perinatal lebih berperan

daripada faktor pascanatal. Nelson dkk (1986) menyebutkan bayi dengan berat lahir

rendah, asfiksia saat lahir, iskemi prenatal, faktor genetik, malformasi kongenital,

toksin, infeksi intrauterin merupakan faktor penyebab cerebral palsy.

GAMBARAN KLINIK

Gambaran klinik cerebral palsy tergantung dari bagian dan luasnya jaringan otak

yang mengalami kerusakan.

1) Paralisis

Dapat berbentuk hemiplegia, kuadriplegia, diplegia, monoplegia, triplegia.

Kelumpuhan ini mungkin bersifat flaksid, spastik atau campuran.

2) Gerakan involunter

Dapat berbentuk atetosis, khoreoatetosis, tremor dengan tonus yang dapat bersifat

flaksid, rigiditas, atau campuran.

3) Ataksia

Gangguan koordinasi ini timbul karena kerusakan serebelum. Penderita biasanya

memperlihatkan tonus yang menurun (hipotoni), dan menunjukkan perkembangan

motorik yang terlambat. Mulai berjalan sangat lambat, dan semua pergerakan serba

canggung.

4) Kejang

Dapat bersifat umum atau fokal.

5) Gangguan perkembangan mental

Retardasi mental ditemukan kira-kira pada 1/3 dari anak dengan cerebral palsy

terutama pada grup tetraparesis, diparesis spastik dan ataksia.

Cerebral palsy yang disertai dengan retardasi mental pada umumnya disebabkan oleh

anoksia serebri yang cukup lama, sehingga terjadi atrofi serebri yang menyeluruh.

Retardasi mental masih dapat diperbaiki bila korteks serebri tidak mengalami

kerusakan menyeluruh dan masih ada anggota gerak yang dapat digerakkan secara

volunter. Dengan dikembangkannya gerakan-gerakan tangkas oleh anggota gerak,

perkembangan mental akan dapat dipengaruhi secara positif.

6) Mungkin didapat juga gangguan penglihatan (misalnya: hemianopsia, strabismus,

atau kelainan refraksi), gangguan bicara, gangguari sçnsibilitas.

Page 17: li maya.doc

7) Problem emosional terutama pada saat remaja.

KLASIFIKASI

Berdasarkan gejala klinis maka pembagian cerebral palsy adalah sebagai berikut:

1) Tipe spastis atau piramidal.

Pada tipe ini gejala yang hampir selalu ada adalah :

a) Hipertoni (fenomena pisau lipat).

b) Hiperrefleksi yang djsertai klonus.

c) Kecenderungan timbul kontraktur.

d) Refleks patologis.

Secara topografi distribusi tipe ini adalah sebagai berikut:

a) Hemiplegia apabila mengenai anggota gerak sisi yang sama.

b) Spastik diplegia. Mengenai keempat anggota gerak, anggota gerak bawah lebih

berat.

c) Kuadriplegi, mengenai keempat anggota gerak, anggota gerak atas sedikit lebih

berat.

d) Monoplegi, bila hanya satu anggota gerak.

e) Triplegi apabila mengenai satu anggota gerak atas dan dua anggota gerak bawah,

biasanya merupakan varian dan kuadriplegi.

2) Tipe ekstrapiramidal

Akan berpengaruh pada bentuk tubuh, gerakan involunter, seperti atetosis, distonia,

ataksia. Tipe ini sering disertai gangguan emosional dan retardasi mental. Di samping

itu juga dijumpai gejala hipertoni, hiperrefleksi ringan, jarang sampai timbul klonus.

Pada tipe ini kontraktunjarang ditemukan, apabila mengenai saraf otak bisa terlihat

wajah yang asimetnis dan disantuni.

3) Tipe campuran

Gejala-gejalanya merupakan campuran kedua gejala di atas, misalnya hiperrefleksi

dan hipertoni disertai gerakan khorea.

Page 18: li maya.doc

Berdasarkan derajat kemampuan fungsional:

1) Ringan:

Penderita masih bisa melakukan pekerjaanlaktifitas sehari-hari sehingga sama sekali

tidak atau hanya sedikit sekali membutuhkan bantuan khusus.

2) Sedang:

Aktifitas sangat terbatas. Penderita membutuhkan bermacam-macam bantuan khusus

atau pendidikan khusus agar dapat mengurus dirinya sendiri, dapat bergerak atau

berbicara. Dengan pertolongan secara khusus, diharapkan penderita dapat mengurus

diri sendiri, berjalan atau berbicara sehingga dapat bergerak, bergaul, hidup di tengah

masyarakat dengan baik.

3) Berat:

Penderita sama sekali tidak bisa melakukan aktifitas fisik dan tidak mungkin dapat

hidup tanpa pertolongan orang lain. Pertolongan atau pendidikan khusus yang

diberikan sangat Sedikit hasilnya. Sebaiknya penderita seperti ini ditampung dalam

rumah perawatan khusus. Rumah perawatan khusus ini hanya untuk penderita dengan

retardasi mental berat, atau yang akan menimbulkan gangguan social emosional baik

bagi keluarganya maupun lingkungannya.

embriologi system saraf

a) Perkembangan susunan saraf dimulai dengan terbentuknya neural tube yaitu

induksi dorsal yang terjadi pada minggu ke 3-4 masa gestasi, dan induksi ventral

yang berlangsung pada minggu ke 5-6 masa gestasi. Setiap gangguan pada masa

ini bisa mengakibatkan terjadinya kelainan kongenital seperti kranioskisis totalis,

anensefali, hidrosefalus dan lain sebagainya.

b) Fase selanjutnya terjadi proliferasi neuron, yang terjadi pada masa gestasi

minggu ke 24. Gangguan pada fase ini bisa mengakibatkan mikrosefali,

makrosefali.

c) Stadium selanjutnya yaitu stadium migrasi yang terjadi pada masa gestasi bulan

35. Migrasi terjadi melalui dua cara yaitu secara radial, sd berdiferensiasi dan

daerah periventrikuler dan subventrikuler ke lapisan sebelah dalam koerteks

serebri; sedangkan migrasi secara tangensial sd berdiferensiasi dan zone germinal

menuju ke permukaan korteks serebri. Gangguan pada masa ini bisa

mengakibatkan kelainan kongenital seperti polimikrogiri, agenesis korpus

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kalosum. Stadium organisasi terjadi pada masa gestasi bulan ke 6 sampai

beberapa tahun pascanatal. Gangguan pada stadium ini akan mengakibatkan

translokasi genetik, gangguan metabolisme.

d) Stadium mielinisasi terjadi pada saat lahir sampai beberapa tahun pasca natal.

Pada stadium ini terjadi proliferasi sd neuron, dan pembentukan selubung mialin.

Kelainan neuropatologik yang terjadi tergantung pada berat dan ringannya

kerusakan. Jadi kelainan neuropatologik yang terjadi sangat kompleks dan difus

yang bisa mengenai korteks motorik, traktus piramidalis, daerah paraventrikuler,

ganglia basalis, batang otak dan serebelum.

Anoksia serebri sering merupakan komplikasi perdarahan intraventrikuler dan

subependim.

Asfiksia perinatal sering berkombinasi dengan iskemi yang bisa menyebabkan

nekrosis.

Kerniktrus secara klinis memberikan gambaran kuning pada seluruh tubuh dan

akan menempati ganglia basalis, hipokampus, sel-sel nukleus batang otak; bisa

menyebabkan cerebral palsy tipe atetoid, gangguan pendengaran dan mental

retardasi.

Infeksi otak dapat mengakibatkan perlengketan meningen, sehingga terjadi

obstruksi ruangan subaraknoid dan timbul hidrosefalus. Perdarahan dalam otak

bisa meninggalkan rongga yang berhubungan dengan ventrikel.

Trauma lahir akan menimbulkan kompresi serebral atau perobekan sekunder.

Trauma lahir ini menimbulkan gejala yang ireversibel. Lesi ireversibel lainnya

akibat trauma adalah terjadi sikatriks pada sel-sel hipokampus yaitu pada kornu

ammonis, yang akan bisa mengakibatkan bangkitan epilepsy.

Gagal tumbuh

a. Definisi :

Kegagaln partumbuhan yang menyebabkan kekurangan gizi yang biasanya

terjadi pada 3 tahun pertama kehidupan. Gagal tumbuh diartikan sebagai

penambahan BB yang tidak memadai ataupun penurunan percepatan

pertumbuhan.

b. Etiologi:

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1. Penyebab menurunnya asupan kalori

- Bayi yang kurang nafsu makan atau menolak makan karena

masalah medis kronis (penyakit ginjal, kanker, penyakit

jantung bawaan dll)

- Diet membatasi makan tertenru

- Pemberian susu/makanan yang kurang dari semestinya

- Ketidaknormalan fisik yang menyebabkan kesulitan menelan,

contohnya :Cerebral palsy

- Kemiskinan

2. Penyebab meningkatnya kehilangan kalori

- Penyakit yang menyebabkan anak muntah terus (saluran cerna

kaku, sumbatan pada saluran cerna, tumor otak)

- Keadaan yang berhubungan dengan gangguan penyerapan

makanan, diare misalnya intoleransi, alergi, cacingan

- Penyakit gula dan keadaan penyakit yang lain

3. Penyebab meningkatnya kebutuhan kalori

Infeksi kronis, hipertiroidisme, penyakit jantung bawaan, penyakit paru

kronis

c. Epidemiologi:

Sekitar 5-10% dari bayi dengan Berat badan lahir rendah dan bayi yang

hidup dengan kemiskinan akan berkembang menjadi gagal tumbuh

d. Faktor resiko :

- Faktor organic / berhubungan dengan organ tubuh ; kesulitan menelan,

anoreksia terkait dengan penyakit sistemik, kelainan neurologis,

penyakit jantung bawaan, kelainan endokrin, muntah terus menerus,

refluks GI, malabsorbsi, kelainan congenital, kelainan kromosom

- Faktor nonorganic : kemiskinan, pemberian ASI tidak adekuat,

psikososial

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e. Manifestasi klinis

Keluhan yang sering :

- Makan hanya sedikit

- Tidak mau makan

- Keterlambatan makan

- Anak hanya mau makan makanan tertentu

- Cepat bosan terhadap makanan

Tanda dan gejala :

- Berat kurang dari persentil 50 atau 30

- Gagal untuk mempertahankan pertumbuhan sebelumnya, dengan

parameter antara P75 sampai dibawah P25

- Penurunan rata-rata berat badan menurut umur

- Penurunan berat badan menurut panjang umur

MIKROSEFALI

Definisi

Mikrosefali adalah cacat pertumbuhan otak secara menyeluruh akibat abnormalitas

perkembangan dan proses destruksi otak selama masa janin dan awal masa bayi.

Ukuran kepala lebih dari 2 standar deviasi di bawah rata-rata (<-2SD).

Mikrosefali adalah kasus malformasi kongenital otak yang paling sering dijumpai.

Ukuran otak pada kasus ini relatif amat kecil, dan karena pertumbuhannya terhenti

maka ukuran tengkorak sebagai wadahnya pun juga kecil (sebenarnya nama yang

lebih tepat adalah mikroensefalus). Perbandingan berat otak terhadap badan yang

normal adalah 1 : 30, sedangkan pada kasus mikrosefalus, perbandingannya dapat

menjadi 1 : 100. Bila kasus bisa hidup sampai usia dewasa, biasanya berat otaknya

hanya kurang dari 900 gram (bahkan ada yang hanya 300 gram). 1

Otak mikrosefalik selalu lebih ringan, dapat serendah 25 % otak normal. Jumlah dan

kompleksitas girus korteks mungkin berkurang. Lobus frontalis adalah yang paling

parah, serebelum sering kali membesar tak seimbang. Pada mikrosefali akibat

penyakit perinatal dan postnatal dapat terjadi kehilangan neuron dan gliosis korteks

serebri.

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Mikrosefali yang paling parah cenderung terjadi pada bentuk yang diwariskan resesif.

Penderita anak memperlihatkan dahi yang landai ke belakang dan telinga yang besar

tak sebanding. Perkembangan motorik sering kali baik, tetapi retardasi mental secara

progresif makin nyata dan sering kali berat.

Etiologi

1. Genetik

2. Didapat, yaitu disebabkan :

a. Antenatal pada morbili, penyinaran, sifilis, toksoplasmosis, kelainan

sirkulasi darah janin atau tidak diketahui penyebabnya.

b. Intranatal akibat perdarahan atau anoksia.

c. Pascanatal dan setelah ensefalitis, trauma kepala dan sebagainya.

Patologi

Secara patologis terdapat kelainan seperti hipoplasia serebri, pakigiria,

mikrogiria, porensefali, atrofi serebri. 6 Biasanya ditemukan penutupan fontanel

dan sutura-sutura tengkorak sebelum waktunya (premature closure). Anak dengan

microgyria dapat hidup sampai dewasa. 1,8 Yang berukuran kecil biasanya tidak

menutupi serebelum dan corak girus-girus kortikalnya abnormal. Arsitektur

korteks menunjukkan sel-sel primitif. Sistem ventrikel biasanya membesar serta

biasanya dibarengi oleh porensefalus, lissensefalus, tidak adanya korpus kalosum

serta heterotropia.

Manifestasi Klinis

o Kepala lebih kecil dari pada normal, sekunder akibat jaringan otak yang tidak

tumbuh.

o Kadang-kadang ubun-ubun besar terbuka dan kecil.

o Didapatkan retardasi mental.

o Mungkin didapatkan pula gejala motorik berupa diplegia spastik, hemiplegia

dan sebagainya.

o Terlambat bicara dan kadang-kadang didapatkan kejang. 5

o Yang khas adalah tulang frontal dan fosa anterior yang kecil.

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CN V -- Trigeminal

contains both sensory and motor fibers that innervate the face important in chewing

located at the level of the pons

CN VII -- Facial  contains both sensory and motor fibers important for sensation of oropharynx & taste to anterior 2/3 of tongue

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CN IX -- Glossopharyngeal contains both sensory and motor fibers important for taste to posterior tongue, sensory and motor functions of the

pharynx

CN X -- Vagus contains both sensory and motor fibers important for taste to oropharynx, and sensation and motor function to larynx

and laryngopharynx.

important for airway protection

CN XII -- Hypoglossal contains motor fibers that primarily innervate the tongu

Complex Anatomy can be Viewed as a Series of Tubes and Cavities with a Division by Function

The swallowing system is comprised of a single tube which widens in the upper region to form cavities. The tube divides at the lower end into a tube for feeding (i.e. the esophagus) and a tube for breathing (i.e. the trachea). Each cavity, formed by the enlargement of a portion of the tube serves initially either the function of feeding (i.e. oral cavity, vallecula, pyriform sinus) or breathing (i.e. nasal cavity).

The Function of Both the Feeding and Respiratory Systems Can be Modified by the Use of Valves

Each system contains a series of valves which change the shape or configuration of the system or protect it. Valves created by the lips and tongue keep food in the mouth and in place prior to swallowing. The valve created by the cricopharyngeous muscle

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at the top of the esophagus keeps air out of the digestive system during breathing. The valve created by the soft palate keeps food out of the nasal airway during swallowing. The valves created by the false and true vocal folds and the epiglottis prevent food from entering the airway during swallowing.

Shared Pathways Exist between the Feeding and Respiratory Systems

The feeding and respiratory systems share a portion of the pharynx between the area behind the tongue and the area at the entrance to the larynx and the entrance to the esophagus.

Since a Shared System Exists, Valves are Used to Support the Desired Activity and Protect the Alternate System

During breathing the valves open to allow air to enter the nose, larynx, and trachea and close to prevent air from entering the esophagus and lower digestive tract. During swallowing the valves open to allow food to enter the digestive tract and prevent food from penetrating the nose and larynx and entering the airway.

During swallowing the goal of the system is to move food into the digestive system and keep it out of the respiratory system. The following events occur rapidly and often simultaneously to support this goal.

Food is propelled from the front to the back of the mouth during the oral stage of the swallow. The lips and sides of the tongue serve as valves to direct the food efficiently toward the pharynx. The back of the tongue elevates and moves food into the pharynx as the pharyngeal swallow is triggered.

The pharyngeal constrictors contract in a peristaltic wave, moving the bolus downward.

The soft palate elevates to prevent the bolus from refluxing into the nasal cavity.

The hyoid and larynx elevate and the epiglottis moves downward to protect the entrance to the airway. This movement of the hyoid also initiates relaxation of the cricopharyngeus muscle and opening of the esophagus.

The aryepiglotic folds and the true and false vocal folds contract, providing additional airway protection.

The bolus moves through the cricopharyngeal sphincter into the esophagus. This sphincter closes, preventing upward movement of the bolus.

Peristaltic movement of the esophagus carries the bolus to the stomach. The lower esophageal sphincter closes to prevent gastroesophageal reflux.

During breathing the goal of the system is to move air efficiently into the respiratory system and keep it out of the digestive system. The following events support this goal.

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The soft palate relaxes allowing air to enter the nose and pass downward toward the lungs.

The false and true vocal folds remain relaxed and open for the entry of air.

The cricopharyngeus muscle sustains contraction to close off the top of the esophagus and prevent air from entering the digestive system.

The Infant is not an Anatomical Miniature of the Adult

Proportional differences exist between the young infant and the older infant, child, and adult. These include:

The oral cavity is small in the newborn and is totally filled by the tongue due to a small and slightly retracted lower jaw.

The newborn has a set of sucking pads in the cheeks which provide stability during sucking.

The soft palate and epiglottis are in contact at rest, providing an additional valve at the back of the oral cavity.

The larynx and hyoid cartilage are higher in the neck and closer to the base of the epiglottis, providing added protection of the airway.

The infant�s eustachian tube runs horizontally from the middle ear into the nasopharynx, rather than its later vertical angle in the older child and adult.

IMPLICATIONS FOR THE CHILD WITH SWALLOWING DYSFUNCTION

The Absence of a Swallowing Reflex Leaves the Airway Unprotected

If the swallowing reflex is not triggered by backward movement of the bolus and/or intention, the airway remains open and unprotected. The upper esophageal sphincter remains closed, preventing food entrance into the esophagus and indirectly biasing its movement into the open airway.

Delay in Elicitation of the Swallowing Reflex Places the Airway in a Risk Position

Difficulties with sensory awareness, inefficient organization of the oral bolus, and problems with timing and coordination of movement may cause portions of the bolus to enter or remain in the pharynx during periods of airway opening. This creates a risk of aspiration before, during, or after the swallow has been triggered.

Small Cavities Formed by the Valleculae and Pyriform Sinuses can Serve as Catching Pools for Portions of the Bolus

Pooling of the bolus in the sulci or cavities formed by the valleculae and pyriform sinuses can delay penetration of the airway by the bolus. Because of this delay, it can

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appear clinically as if the bolus were swallowed. Entrance into the laryngeal vestibule or penetration of the airway may be signalled by coughing or choking that occurs during the 3rd or 4th small bolus.

The Shared Use of the Pharynx by Respiratory and Feeding Systems Increases the Risk of Aspiration in Children whose Respiratory Systems are Compromised.

Infants and children with primary respiratory difficulties (i.e. respiratory distress syndrome, bronchopulmonary dysplasia) often protect their vulnerable respiratory systems through a central inhibition of the swallowing reflex, or a voluntary refusal to swallow. This may be observed even when the child is receiving respiratory assistance through a ventilator or additional oxygen.

The Infant Anatomical Differences Create Additional Protective and Learning Systems which may not be Present in the Older Infant or Child with Severe Swallowing Dysfunction

The small infant oral cavity creates abundant sensory input to the tongue, giving information on spatial and movement relationships within the mouth. This supports the lesser neurological coordination of the young infant. When an infant or child has developed anatomically without a comparable development neurologically, the evolving feeding coordination lacks the early infant anatomical back-up system.

The sucking pads provide additional stability of the jaw and support a more efficient sucking pattern as the infant gradually develops the coordination of the jaw, lips, cheeks, and tongue to support oral feeding. When an older child is initially developing the sucking coordination of the newborn, the sucking pads may be reduced or absent. Thus, the early coordination lacks the anatomical support available to the newborn with this degree of oral control.

When the soft palate and epiglottis grow away from each other and are no longer in contact, the baby looses a valve which helps keep food in the mouth until the pharyngeal swallow is initiated. The older infant or child with poor oral control of the bolus may then have food fall over the back of the tongue into the valleculae or airway before the swallow is triggered.

The airway has less anatomical protection as the larynx assumes its lower position in the neck and is not as directly protected by the epiglottis. Since infants younger than 4-6 months have additional airway protection, poor closure of the airway or even a partial paralysis of the vocal folds may not be as evident. When the anatomy no longer gives additional protection for a marginally coordinated swallowing mechanism, coughing, choking or aspiration may occur.

The horizontal positioning of the eustachian tube places the infant at greater risk for ear infections from food or liquid that refluxes upward into the nasopharynx.

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Feeding Assessment Checklist

Note state before, during and after feeding. Can infant be brought into appropriate state for feeding? What works? How

much assistance is needed?

Does infant exhibit stress before, during, or after feeding?

Are reflexes that allow infant to locate and obtain food (rooting and sucking reflexes) and protect airways (cough and gag) expressed?

Is there cough during feeding? When does it occur?

Does the infant exhibit hypersensitive, hyposensitive, or aversive behavioral response to oral tactile input?

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Is feeding position optimal for this infant?

Evaluate functions of each of the oral structures.

Is infant capable of appropriately timed and organized suck, swallow, and breathe?

Are breath sounds noisy during or following feedings?

Are signs of increased respiratory effort observed during feeding?

Does infant exhibit signs of fatigue before adequate intake has been achieved?

Does infant demonstrate clear cues about feeding readiness and satiety?

Does caregiver attend to cues given by infant?

Does caregiver demonstrate affection during feeding?

Background

Feeding problems are common in preterm infants. See section on parental experiences for more information about the impact of these problems. Infants often appear to regress in feeding skills and abilities or to develop new feeding problems after hospital discharge. There is a relationship between parentally reported feeding problems and growth in these infants. Monitoring of feeding through direct observation as well as caregiver reports of the feeding situation at home is essential to good follow-up care. Appropriate referrals should be made for feeding problems that interfere with nutritional intake, growth or the caregiver-infant relationship.

Assessment of Feeding Performance

State Control

A continuum of states of consciousness, ranging from deep sleep though awake states to crying has been described. The optimal state for feeding is an awake, alert, or active state, although some infants can feed adequately in a drowsy state. State control difficulties may be components of a variety of medical conditions. Assessment of state should be a routine part of feeding assessment and should include the following

Note infant's state before, during, and after feeds. If at any point the infant is not in an appropriate state, note if infant can be

brought into an appropriate state.

Note what techniques are successful and how much assistance the infant needs to maintain appropriate state for feeding.

Stress

The feeding process places many demands on the infant. These demands may be internal, such as increased respiratory and digestive functions, or external, such as oral-tactile experiences during feeding or variations in ambient temperature, noise, or

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light. If these demands are beyond the infant's adaptable capacities, the infant may respond with behaviors that reflect stress. If stress cues are noted before, during, or after a feeding, the source of the stress needs to be identified and modified. Examples of potential sources of stress:

Environmental: bright lights, noise, TV, distracting movements of siblings or others

Feeding related: liquid flowing too fast or slow, distracting movements of feeder

Internal discomfort: gastroesophageal reflux, desaturation, increased work of breathing

Infant Stress Cues

State and Attentional Motoric Autonomic

Irritability

Crying

Frenzy, inconsolability

Rapid state changes

Sleeplessness, restlessness

Drowsy alertness

Strained alertness

Panicked alertness, hyperalertness

Diffuse sleep or awake states

Staring

Frequent gaze aversion

Strained fussing or crying

Silent crying

Motoric flaccidity: trunk, extremities, face

Motoric hypertonicity

Hyperextension of the legs

Hyperextension of the arms and hands

Truncal hyperextensions (arching)

Hyperflexions (fetal tucking, fisting)

Facial grimacing

Frantic, diffuse activity

Frequent twitching

Moderate Stress Sighing Yawning

Sneezing

Sweating, (diaphoresis)

Hiccuping

Tremoring

Startling

Gasping

Straining

Major Stress (when seen with feeding)

Frequent or prolonged coughing

Spitting up

Gagging, choking

Color changes, cyanosis

Respiratory pauses

Irregular respirations

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Responses to Tactile Input

Oral Reflexes: Oral reflexes can be either adaptive (assist the infant in locating and obtaining food, e.g.: rooting reflex and sucking reflex) or protective (keep airway free of foreign material or expel it as it enters the airway, e.g.: cough and gag). Expression of reflexes such as rooting and sucking can change depending on infant's level of hunger or state of alertness and assessment should take this into account.

Assessment of cough during feeding is especially important in preterm infants. If cough occurs during sucking and swallowing it may indicate material crossing near or entering the airway as it descends through the pharynx. If coughing is observed during sucking pauses, or after feeding, it may indicate material ascending into the pharynx from gastroesophageal reflux.

Behavioral Responses to Tactile Input: During feeding the infant accommodates to a wide variety of tactile stimuli within the mouth as well as external stimuli from the touch of the feeder's hands on the infant's face or the touch-pressure of being held. The infant must perceive the tactile input appropriately to produce the appropriate motoric responses for feeding.

Preterm infants may perceive tactile input as stressful and may respond with a variety of stress reactions as described above. Early NICU experiences may have included negative and aversive stimuli to the oral-facial area. The infant may have been unable to engage in normal, pleasurable, oral exploration because of motoric immaturity or delay, intubation, or lack of experience. A pattern of learned negative or aversive behaviors that persists beyond discharge may develop.

Referral to a feeding therapist for a structured evaluation of tactile responses may be indicated for clear delineation of the threshold beyond which the infant has an inappropriate behavioral response to tactile input.

Feeding Position

An optimal position for young infants is characterized by orientation around midline, neutral anterior-posterior alignment of the head and neck, neutral alignment or slight flexion of the trunk, and flexed hips and knees.

When the feeding position is not satisfactory, the underlying factors affecting the position should be identified to develop appropriate positioning techniques. A frequent example is the preterm infant who extends, becomes hypertonic during feeding, and is difficult to hold in the optimal feeding position. This infant may be using neck extension to maintain a patent airway, may be showing a stress reaction to the tactile or gustatory aspects of feeding, or may have abnormalities of the central nervous system.

Oral Motor Control

Assessment of specific function of oral structures is most effective if the infant sucks on the examiner's gloved finger. A wide variety of oral motor difficulties may be observed in LBW infants. Many problems are due to a lack of positional stability.

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Full-term infants are born with a substantial amount of subcutaneous fat and well defined fat pads in the checks. The tongue fills the oral cavity and is in physical contact with all the surfaces of the oral cavity. Additionally, at birth, the term infant has a strong physiological flexor tone that, combined with the exoskeleton, provides a stable base for the oral structure. In contrast, the premature infant has less muscle bulk and poorly developed tendons and ligament structures as well as less body fat. There is decreased opposition of the tongue to the surfaces of the oral cavity and reduced flexor tone through the head and neck with neck hyperextension. Lack of positional stability may lead to abnormal oral motor patterns, some of which may continue post discharge.

Examples of more common oral motor difficulties:

Tongue-tip elevation: the tip of the tongue is held firmly against the hard palate behind the upper alveolar ridge, potentially interfering with nipple insertion.

Tongue retraction: The tongue sits back in the mouth, well behind the alveolar ridges causing poor contact between the tongue and the nipple to stimulate appropriate tongue movements. Strong neck hyperextension can contribute to tongue retraction by pulling the tongue back into the mouth.

Tongue protrusion: The tongue pushes outward instead of moving in the normal wavelike anterior-posterior pattern. The tongue may compress the nipple, with little suction generated, leading to inefficient sucking. This pattern may be seen in infants who have sucked on endotracheal tubes and those with low tone.

Excessive Jaw Excursion: The jaw moves in a greater range than expected and the movement is poorly graded. Tongue contact on the nipple may be poor, diminishing both compression and suction. Lip seal can also be compromised, further impairing sucking.

Physiologic Control

The infant will have physiologic responses to the work of feeding. If an infant is not able to cope with these responses physiological changes, stress reactions, and poor endurance may result. To assess physiologic control during feeding each parameter is evaluated at baseline, during feeding, and after feeding.

Heart Rate: In term infants heart rate is typically in the range of 120 to 140 beats per minute. It is not uncommon for see heart rate increases of about 10 beats per minute during feeding. Larger increases may indicate that feeding is placing excessive demands on the infant. Bradycardia (a drop in heart rate to below 100 beats per minute) can also be observed during feeding in the high-risk infant, and is a potentially life threatening event. Bradycardia might occur after oxygen desaturation, or with a suspected reflux event, or with position change. In some cases it is a vagally mediated response to stimulation of sensory receptors in the pharyngeal-laryngeal area. Bradycardia may be triggered via stretch receptors within the pharynx that may be stimulated by a large bolus. Touch-pressure receptors can be stimulated by the presence of nasogastric tubes, or chemoreceptors can be stimulated by

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microaspiration of food or by reflux of gastric content. Decreases in oxygen saturation can also lead to bradycardia.

Respiratory Rate: The most accurate method of measuring respiratory rate (RR) is counting the infant's breaths. Normal values for term infants are 30 to 60 breaths per minute. During bottle feeding the respiratory rate is low while the infant is actively sucking. It is higher during pauses. This leads to an overall RR that is adequate for effective ventilation. During the early part of feeding, when the infant is sucking eagerly, the respiratory rate decreases significantly from baseline values. As the feeding progresses and the infant sucks less eagerly, taking more pauses to breathe, the respiratory rate increases toward baseline.

In infants with respiratory compromise RR can be significantly elevated. Infants who require a high respiratory rate to maintain homeostasis may not be able to tolerate the suppression in respiration that occurs in the early part of feeding. The infant may fatigue easily or may be at risk for aspiration as he or she tries to gasp for air. Respiratory rate during feeding is measured during pauses in sucking. IF RR is above 80 breaths per minute during these pauses, it often indicates that work of feeding is too great for the infant.

Studies indicate that term and preterm infants experience light, but measurable oxygen desaturation with feeding. For the compromised infant with borderline saturations, such reduction may be significant. If desaturation is observed during feeding evaluation, the pattern of desaturation should be noted. Sudden dips may be associated with apnea or bradycardic episodes, whereas a gradual decline may indicate inadequate respiratory support for feeding.

During feeding assessment without oxymetry, attention should be focused on the infant's color around the mouth and eyes. If cyanosis is noted it is recommended that the infant be fed with an oximeter in place. A lack of color change with feeding, however does not necessarily imply that oxygen saturation is normal. Many infants can have relatively low oxygen saturation without external evidence such as cyanosis.

Endurance: Poor endurance may result in the infant terminating the feeding before taking the required volume, or demonstrating poor weight gain despite acceptable intake. Endurance is a reflection of the infant's work to maintain homeostasis, work for other activity (such as feeding) and the infant's cardiopulmonary reserve. Endurance is compromised by many disease processes that are more common in LBW infants.

Sucking, Swallowing, Breathing

Problems in any one of these processes or lack of coordination among these processes can have a profound effect on the infant's feeding abilities. Within the pharynx, feeding and breathing share a common space. This dual role of the pharynx underlies the difficulties observed when sucking, swallowing, and breathing are not well coordinated.

Sucking: Sucking involves the rhythmic movements of the tongue and jaw, with support from the lips and cheeks, which create changes in pressure that cause liquid to

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flow out of a nipple. Sucking is comprised of two types of pressure, positive pressure (compression) and negative pressure (suction). Non-nutritive sucking occurs in a highly organized, repetitive pattern of bursts and pauses with a high ratio of sucks per swallow (6 to 8: 1) and 1 to 4 swallows per burst. Assessment of non-nutritive sucking on a gloved finger allows evaluation of the strength of the suck and relative amounts of suction and compression.

Nutritive sucking occurs during active feeding and has a more complex pattern than non-nutritive sucking. Preterm infants often have fewer or shorter bursts of nutritive sucking. At the beginning of a feeding the sucking pattern usually consists of long sucking outbursts with few or brief sucking pauses. The suck-swallow ratio is usually 1:1, but may increase toward the end of a feeding. In assessment of sucking in bottle-fed infants, return of bubbles into the bottle is a reflection of liquid flow out of the bottle. The strength of the infant's suck is reflected in both the resistance to pulling the nipple out of the infant's mouth and the rate of liquid flow. On the breast, the rate of sucking and the suck-swallow ratio can give indications of the rate of milk flow. The slower the sucking rate and lower the suck-swallow ratio, the faster the milk flow. When differences are noted in the quality of sucking between nutritive and non-nutritive sucking it is suggestive of problems with some aspect of suck-swallow-breath coordination.

Swallowing: Coughing or choking during swallowing can indicate that liquid is impinging on the airway. This can be liquid that is actually aspirated into the airway or that merely penetrates the airway and then is expelled. Aspiration can result from a primary swallowing dysfunction or from lack of coordination between sucking, swallowing, and breathing. Aspiration can be descending (during feeding), but can also be ascending (during gastroesophageal reflux). A history of frequent upper respiratory infection or pneumonia may be an indication that "silent" aspiration is taking place.

Clinical assessment of swallowing function includes: ability to handle secretions, presence of noise or wet sounding breathing during or after feeding. (Noises arising from the nasopharynx may indicate nasal reflux during swallowing, while noises in the pharynx may indicate residue left in the pharynx after the swallow), and the need for multiple swallows to clear a single bolus.

Infants who show signs of swallowing dysfunction should be referred for further assessment. The most comprehensive evaluation of swallowing occurs radiologically during the videofluoroscopic swallowing study (VFSS) or modified barium swallow.

Breathing: Infants with compromised respiratory function may be unable to make sufficient adjustment to accommodate the work of feeding. Assessment should include:

Respiratory effort: evaluation before, during, and after feeding. Increased respiratory effort is indicated by retractions at the neck, trunk, or rib cage, head bobbing, grunting or forced exhalation.

Changes in respiratory pattern: observe whether there are excessive pauses or irregularities in breathing pattern. Short respiratory pause of 15 seconds can be

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considered normal at any age, but longer periods or those associated with cyanosis, pallor, or bradycardia are pathologic.

Sounds of respirations. Noise heard during any part of the respiratory cycle may indicate airway obstruction or alteration in airway patency.

Coordination of Sucking, Swallowing, and Breathing: This is assessed by listening to the ratio of sucks to swallows and the timing and adequacy of respiratory efforts during sucking bursts. The normal rhythmic sucking pattern during bottle feeding consists of a series of bursts and pauses that begins as a continuous sucking pattern and changes to an intermittent sucking pattern over the course of the feeding. Normally recovery in all respiratory parameters occurs within the sucking pauses.

Assessment of Feeding Interactions

Preterm infants and their families are at risk of problems in feeding interactions due to long periods of hospitalization and other factors. The feeding observation should include an assessment of how the caregiver and infant work together as a team during feeding. One systematic method for observing infant/caregiver interactions during feeding has been developed by Barnard et al. In the assessment tool devised by this group Caregiver and Infant Responsibilities during feeding are described as follows:

Caregiver Responsibilities:

Recognize and respond to the infant's cues and respond in a contingent manner Sooth or quiet a distressed infant

Demonstrate warmth and affection toward infant and communicate a positive feeling tone

Foster cognitive growth through touch, movement, and talking

Delay stimulating or responding until the infant signals readiness

Infant/Child Responsibilities

Send clear cues to the caregiver, including: o Display of some tension at beginning of feeding and decrease in

tension once feeding has begun

o Have periods of alertness during the feeding

o Older infants cue the caregiver for interaction through vocalization and smiles

o Cue the caregiver of need for a break or rest.

Respond to the caregiver's attempts to communicate and interact

Stop crying when the caregiver attempts to sooth

Look in the direction of the caregiver's face then the caregiver talks

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Mold into the contours of the caregiver's body

Suck and make feeding sounds following feeding attempts by the caregiver

Older infants vocalize or smile after the caregiver's vocalization or smile

Fluid Intake

Adequate fluid intake is essential. LBW infants are at increased risk for dehydration.

Some VLBW infants with BPD, chronic lung disease or cardiac complications may be fluid restricted.

Fluid needs are increased by fever, diarrhea, and vomiting.

As for full term infants, caregivers should be asked if urine color is pale and infant is producing at least 6-8 wet diapers per day. Because of increased risk of dehydration however, consider assessing actual fluid intake.

Minimal fluid intake should be at least 100-120 ml for each kg the infant weighs (1.8 to 2.2 ounces per pound). Recommended total fluid intakes for most infants are about 150 ml per kg.

Weight Minimal Fluid Intake Recommended Fluid Intake

LB kg Ounces ml Ounces ml

4.5 2 8 240 10 300

6 2.7 11 324 14 405

8 3.6 15 432 18 540

10 4.5 18 540 23 675

12 5.5 22 654 28 825

Energy Intake

Should be at least 100-120 kcal/day. Some low birth weight infants will require more.

The best indicator of energy need is growth pattern on current energy intake.

Breastfed Infants:

For a quick assessment:

o Breastmilk is usually calculated at 20 kcal/oz. o Breastmilk fortifiers are not recommended once the infant reaches a

weight of 2500 grams.

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o If the infant is nippling at the breast adequacy is determined by pre- and post-feeding weight checks, growth pattern and observation of breastfeeding.

o Signs that the infant is breast feeding effectively include rhythmic sustained suckling, audible swallow, softening of the maternal breast, maternal signs of milk ejection.

o Please see section on breastfeeding for more information.

Formula Fed Infants:

Weight Expected Total Daily Formula Intake

24 kcal/oz 20 kcal/oz

4.5 lbs. 10 12

6 lbs. 14 16

8 lbs. 18 22

10 lbs. 23 27

12 lbs. 28 32

Other Sources of Energy:

Food Approximate Energy Value

Dry Infant Cereal 14 per Tbsp.

Commercial Infant Fruits 8 per Tbsp.

Commercial Infant Vegetables 5 per Tbsp.

Commercial mixed dinners 9 per Tbsp.

Commercial Infant Desserts 11 per Tbsp.

Glucose polymers (ex: Polycose) 4 kcal/g or about 8 kcal/level tsp.

The following Web sites provide information for calculating energy and nutrient values of foods and diets:

o USDA Nutrient Data Lab Food Composition Data (Searchable database of foods/Agriculture Handbook No. 8)

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Use the search term "infant" to get information about infant formulas. Use the search term "babyfood" to get information about commercial infant foods.

o Nutrition Analysis Tool (NAT)

Click on "NAT" and type "babyfood" in the "Add a new food" box. This will bring up over 200 infant foods to chose for nutrition information.

Supplementation

o If infant is taking less than 24 ounces of standard formula or equivalent amounts of breastmilk per day, an infant multivitamin and mineral supplement should be given.

o Preterm infants should have iron supplement by 2 months in a dose of 2 to 4 mg/kg/d.

o Supplement breastfed infants with Vitamin D.

o Some infants require other supplements. For more information see section on vitamin/mineral supplements.

Introduction of Non-milk Feedings

o The American Academy of Pediatrics says 6 months is appropriate for breastfed infants.

o If developmentally ready, non-milk feedings should be added to diet between 4 and 6 months, corrected age. (See developmental aspects of feeding)

Obtaining Dietary Intake Information

Caregiver reports of dietary intake may vary in accuracy. The advantages and disadvantages of common methods of obtaining dietary intake information follow. Methods should be selected based on appropriateness for the family as well as the reason for obtaining the information.

It is important to remember that feeding preterm infants can be an emotional issue for families. Families may feel responsible for inadequate dietary intake and growth. Please see section on what families tell us. Families may find dietary assessment stressful. Every attempt should be made to undertake this procedure with a family-centered, non-judgmental approach.

Accuracy of dietary information may be enhanced by:

o Taking time to thoughtfully ask questions about dietary intake with the family and/or referring to a registered dietitian who has extensive background and training in dietary assessment.

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o Asking caregivers to remember the activities of the days for which information is to be collected so that food intake can be associated with other events.

o Providing careful instructions to families before asking them to keep dietary records. Ask them to:

count only formula that was actually ingested, not all that was prepared.

provide information for night feedings as well as day feedings.

gather information from all caregivers for the infant.

accurately measure scoops of powder or liquid ounces used to make formula. (Studies repeatedly find that many caregivers do not mix formula according to label directions.)

Please see section on breastfeeding for assessment of breastmilk intake.

o Making reminder and clarification phone calls to families while they are keeping records.

o Verifying information by asking questions such as, "How many cans of formula do you buy when you go to the store and how long do those cans last?" or, for families enrolled in the WIC program, "Do you find that you have formula left over or need to buy more formula than WIC provides?" Another way to verify information is to review records with parents, paying particular attention to variations, such as days on which there are several hours without feeding.

Methods of Gathering Dietary Intake Information for Infants

Method Description Advantages Disadvantages

Intake Questionnaire Caregivers are asked a series of questions such as, "How much formula does your infant usually ingest in 24 hours?"

Can be completed at time of initial visit.

May be difficult for some caregivers to accurately answer questions about "typical intakes."

Infant may be in period of rapid change and instability.

24 hour recall Caregivers are asked to remember what infant has ingested in last 24 hours.

Can be completed at time of initial visit.

Last 24 hours may not accurately reflect typical intake.

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Relies on caregiver memory.

Caregiver with infant at assessment visit may not have been with infant for all of last 24 hours.

3 day records Caregivers are asked to keep records of everything the infant has to eat and drink for 3 days.

Likely to be more accurate than methods that rely on memory alone.

Record keeping is difficult for some busy families and for those with limited literacy.

Families must be trained to do this well.

7 day records Caregivers are asked to keep records of everything the infant has to eat and drink for 7 days

Most informative if family is able to keep good records for this long.

Same as 3 day, but 7 days is even more difficult for some families and may not be worth the extra effort.

Nutritional Impact Of MedicationsMedications may have an impact on nutrition via several mechanisms. Medications added to feedings can alter taste and tolerance. Some medications have direct drug-nutrient interactions and may alter absorption, untilization, or excretion. Below is a list of medications that may be used for preterm infants.

DRUG CATEGORY AND NAME

NUTRITIONAL IMPLICATIONS

LABS TO MONITOR

     

DIURETICS    

Chlorothiazide (Diuril; K depleting)

- urinary loss of Na, K, Cl, Mg, P, Zn, riboflavin (B2), bicarb

- monitor fluid and electrolyte status

Furosemide (Lasix; K depleting)

- urinary loss of Na, Cl, Mg and Ca

- monitor fluid and electrolyte status

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Spironolactone (Aldactone; K sparing)

- urinary loss of Na, Cl, Mg and Ca- chronic therapy may cause renal calculi and osteopenia

- monitor fluid and electrolyte status

     

CORTICOSTEROIDS    

Dexamethasone (Decadron) Prednisone or Prednisolone

- may cause hyperglycemia- urinary loss of K, Ca, Zn, vitamin C- caution with K-depleting meds- decreasd absorption of Ca and P may lead to alterations of bone mineralization- may inhibit growth- may lead to altered protein metabolism- may cause GI ulceration and/or hemorrhage

- monitor growth and bone mineral status with chronic use- monitor blood pressure

     

CARDIORESPIRATORY    

Albuterol (Proventil) - appetite and activity changes - monitor dietary intake and growth

Theophylline - may cause GI irritation, feeding intolerance, anorexia- avoid extremes of dietary protein or carbohydrate intakes

- monitor dietary intake

Digoxin - may cause feeding intolerance- avoid excessive calcium and vitamin D supplementation

- monitor dietary intake from foods and supplements

Caffeine - insomnia, increased heart rate, irritability- GI distress

- monitor dietary intake and growth

     

NEUROLOGICAL    

Phenobarbitol - may increase turnover of vitamins D, K and folate- may cause decreased bone density and osteomalacia

- monitor vitamins D, K and folate status- monitor bone density

Other anticonvulsants such as phenytoin sodium and valproic acid

- affect metabolism of vitamins D, K, B6, B12, folate and calcium

- monitor dietary adequacy of vitamins D, K, B6, B12, folate and calcium

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ANTIBIOTICS    

Vancomycin HClGentamicin (Garamycin)Ciprofloxacin (Cipro)DoxycyclineErythromycin

- may cause nausea, diarrhea- long-term use may alter GI flora and nutrient availability--especially vitamin K

- monitor dietary intake and growth- long-term use, monitor vitamin K status

 

CHECKLIST FOR NUTRITION ASSESSMENT

The following is a checklist for health professionals who are performing a nutrition assessment of the preterm infant after initial hospital discharge. Checklist items include links to more in-depth information and resources.

Growth

o Is this infant following appropriate charts for weight, length, and OFC? o Is growth assessed using corrected age?

o Does growth assessment include patterns of growth, percentiles, rate of growth?

o For appropriate growth measurements, see the section on anthropometric procedures.

Nutritional Intake

o Is this infant's fluid intake adequate to meet fluid needs? o Is this infant receiving breastmilk or an appropriate formula?

o Is this infant offered appropriate foods?

o Is this infant receiving appropriate vitamin-mineral supplements?

o Is the diet developmentally appropriate?

o Is the infant receiving adequate energy?

o Is the infant receiving adequate nutrients (iron, calcium and vitamin D)?

o For more information, see the section on nutritional intake.

Feeding Observation

o Is the infant positioned correctly for feeding? o Does the infant have effective nippling skills?

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o Can the infant coordinate suck-swallow-breathe?

o Does the infant appear to choke, gag, regurgitate, have apnea/bradycardia/cyanosis or have other signs of respiratory distress with feeding?

o Does the caregiver/infant interaction appear appropriate?

o Does infant show appropriate cues for hunger and satiety?

o Is the length of feeding appropriate?

o Is the volume of feeding adequate?

o Are there environmental distractions that cause stress during feeding?

Medical Factors

Do any of the following impact the nutritional or feeding status of this infant?

o Chronic lung disease (CLD) or bronchopulmonary dysplasia (BPD) o Short gut or other malabsorptive conditions

o Reflux (GER)

o Neurological impairment

o Motor problems

o Cardiac conditions

o Renal conditions

o Hepatic conditions

o Medications

Other Risk Factors

o Small for gestational age o On TPN more than 4 weeks during early hospitalization?

o Anemia

o Osteopenia