PRESENTASI KASUS SULIT

NP + NEC Grade III + MAR tanpa

Fistula + Sepsis Berat
Oleh :
dr. Ricky Wibowo, Sp.B
dr. Theresa Laura, Sp.A

RS PUPUK KALTIM
2016
• 28 19 69
No RM :
• 9 Juli 2016 (05.10)
Tanggal KRS :
• 8 Juli 2016 (07.00)
Tanggal MRS :
• 2 hari
Usia :
Lahir :
• 6 Juli 2016 Tanggal
Kelamin :
• Laki-laki Jenis
• M. Rhaka Saputra
Nama :
DATA PASIEN
ANAMNESIS
Bayi laki-laki usia 2 hari, datang dibawa
oleh orang tuanya dengan keluhan utama
kembung. Bayi kembung sejak lahir dan
belum pernah bab sejak lahir. Kentut belum
bisa. Bayi tampak kuning sejak pagi hari
SMRS disertai demam tinggi hingga 40°C
dan tampak sangat lemah dan nafas berat.
Bayi tidak mau menyusu dan masih BAK(+)
walaupun sedikit (bercak di popok) sejak
tadi pagi.
RIWAYAT KELAHIRAN
Lahir di RS Amalia
Pasien anak kedua
tgl 6/7/2016 dengan
lahir hidup, anak
ibu G3P1A1 lahir
pertama meninggal
prematur UK 32
usia 1 th krn diare
minggu

BBL 2400gr, segera Riwayat ibu demam

menangis, ketuban saat hamil (-). Riw
ibu jernih, ibu riw keluhan lainnya
KPD 12 jam selama kehamilan (-)
 PEMERIKSAAN FISIS
TTV : GT Sangat lemah, HR 160x/i, t 38°C, RR 50x/i
Kepala
Mata :
Konjungtiva Anemis -/-
Sklera Ikterus +/+
Hidung : Pernafasan cuping hidung (+); distres nafas (+)
Mulut : Bibir sianosis (-)
Leher
Pembesaran KGB tidak teraba
Thorax – Paru
Inspeksi : Simetris, retraksi (+)
Palpasi : VF ki = ka
Perkusi : sonor ki = ka
Auskultasi : VBS (+), Rh +/+ Wh -/-
 PEMERIKSAAN FISIS
Thorax – Cor
BJ I/II murni, reguler
Murmur (-) Gallop (-)
Abdomen
Inspeksi : Perut distended
Auskultasi : BU hilang
Palpasi : tak teraba massa
Perkusi : pekak hepar (+)
Ekstremitas
Akral hangat
CRT <2”
Edema (-)
Ikterus (+) kramer II-III
 PEMERIKSAAN FISIS
Regio ano-perianal :
Penis (+)
Scrotum +/+; teraba testis +/+
Perianal : fistula (-)
Anal dimple (+)
DRM/RT :
Dicoba untuk memasukkan termometer/ kateter per
anal, hanya bisa masuk 3cm kesan ada hambatan
ASESSMENT PRE OP

NP/BBLR/SMK
Early Onset Sepsis
(severe)
MAR susp. tanpa Fistel
• Rontgen thorax dan pulmo dalam batas normal
• Gambaran large bowel obstruction dengan tidak tampak adanya
udara di cavum pelvis (proyeksi rectum) curiga ec malformasi Kesimpulan
anorectal letak tinggi (Hirschprung dd/ atresia rectum) dan
Hepatomegali.
• Tak tampak distensi cavum abdomen. Preperitoneal fat line
bilateral tegas. Distribusi udara usus tidak merata, tidak
tampak adanya udara di usus bagian pelvis, proyeksi rectum,
dengan dilatasi dan distensi lumen usus curiga colon dengan Abdomen
ujung udara terdistal di atas simfisis pubis. Konfigurasi hepar
membesar dan lien normal. Terpasang OGT dengan ujung
distal di lambung.
• Corakan bronkovaskular normal, pengembangan
pulmo baik. Tak tampak penebalan pleural space
bilateral. Kedua diafragma licin, tak mendatar.
Thorax
Konfigurasi cor normal, sistema tulang yang
tervisualisasi intak
Babygram (expertise)
PEMERIKSAAN PENUNJANG
• Mengarah ke gambaran Atresia Ani
n
Letak Tinggi
Kesimpula
• Foto crosstable lateral pada pasien
dengan klinis malformasi
n
anorectal, hasil : tampak udara Pemeriksaa
distal usus berjarak 2cm dari anal
dimple. Kesan udara terdistal usus
Hasil
berada diatas pubococcigeal line
Cross Table Lateral:
PEMERIKSAAN PENUNJANG
PEMERIKSAAN PENUNJANG
Laboratorium Darah (8 Juli 2016)
ELEKTROLIT HEMATOLOGI
Natrium 154 WBC 6.51
Kalium 7.7 Neutrofil 18.6
Chlorida 122 HGB 21.2
KIMIA KLINIK HCT 60.70
Bilirubin total 11.1 PLT 211.000
Bilirubin direk 2.49 Gol Darah B
Bilirubin 8.64 Rhesus Positif
indirek
SEROLOGI KOAGULASI
CRP Test Negative PTT 31.4
APTT 122.2
PEMERIKSAAN PENUNJANG
Laboratorium Urin (8 Juli 2016)

URIN LENGKAP
Berat Jenis 1.025 Keton (-)
pH 5.0 Nitrit (-)
Warna Kuning pekat Urobilinogen (-)
Protein +1 Bilirubin +2
Glukosa (-) Sedimen Erit 0-1
Eritrosit (-) Sedimen Leu 0-2
Leukosit (-) Lain-lain (-)
Diagnosis

NP/BBLR/SMK

Early Onset Sepsis

MAR tanpa Fistel

PLANNING
O2 nasal 1 lpm
IVFD D12.5% 150cc/24jam (GIR 5.9)
AS 6% 73cc/24jam (~29/kg/hari)
NaCl 15% 4.5cc/24jam
KCL 7.4% 4.5cc/24jam
Ca Gluconas 10% 5cc/24jam
Ampicillin 2x150mg
Gentamycin 1x15mg
Ranitidin 2x3mg
Puasa
OGT terbuka
Nystatin 2x1cc
Oral care asi 4x1cc
Fototerapi 2x24jam
Nebul ventolin 1 respule 3x sehari
Pro Sigmoidectomy / Colostomy di OK kalau perlu laparotomi
LAPORAN OPERASI 8 Juli 2016
1. Informed consent (+), antibiotik terapeutik
2. Disinfeksi lapangan operasi dg povidone iodine 10%
3. Dilakukan insisi infraumbilikal pro trepine sigmoidostomi
4. Didapatkan jaringan nekrosis + pus sekitar 300cc (kultur)
5. Diputuskan explorasi infra umbilical,didapatkan jaringan
nekrosis dari gaster sampai dengan rectum dan ruang
retroperitoneal.
6. Didapatkan perforasi di 2 tempat dengan adhesi hebat gr
II-III di seluruh intestine
7. KIE tentang kondisi dan prognosa ke orang tua (ayah) di
ruang operasi
8. Dilakukan drainage abses, cuci cavum abdomen + pasang
drain 2 buah untuk drainage.
9. Luka operasi di jahit satu lapis.
Diagnosis Post Op
NP/BBLR/SMK

NEC grade III (Bell’s)

MAR tanpa Fistel

Sepsis berat
INSTRUKSI POST OP
Prognosis : Ad Malam
Terapi lanjutkan sesuai program
Spooling dengan PZ 200-300cc/24jam
Laparotomy on demand
 FOLLOW UP
Tanggal/ Catatan Perkembangan
Jam
8-7-2016 S: Pasien post op. Instabilitas suhu (+) hipotermi. Nafas spontan
22.00 (+) inadekuat. Luka operasi tertutup verbant. BAK(-) BAB(-)
WITA
O: GT Sangat Lemah, HR 136x/i, RR 46x/i , temp 36°C, SpO2
88%
k/l : a-/i(+)/c-/d-/pch-/-
Th : Sim -/ ret-/ cs1s2 m-/g-
p v/v wh-/- rh-/-
Abd : Flat. BU(-) luka op tertutup kasa verbant
Akral hangat, CRT <2”
A: NA/BBLR/SMK + MAR tanpa fistel + NEC gr III post
explorasi laparotomy day-0
Pdx: Kultur darah
Kultur feses
Lab post op : DL, FH, SE, CRP, Alb, GDA, BGA
Babygram cito bed post op
 FOLLOW UP
Tanggal/ Catatan Perkembangan
Jam
PTx -O2 ventilator PCV PEEP 4 PC 8 FiO2 50%
: i:e = 1:2 Fset 40
-D 12,5% 125cc/24jam (GIR 5,4)
-AS 6% 65cc/24jam (~2,5gr/kg/hari)
-NaCl 15% 2cc/24jam
-KCL 7.4% 2cc/24jam
-Ca Gluconas 10% 4cc/24jam
-Meropenem 2x40 mg (1)
-Amikasin loading 20mg IV  maint 1x15mg IV (1)
-Metronidazole loading 30mg IV  maint 3x15mg IV (1)
-Ranitidin 2x2mg IV
-Inj Vit K 1mg IM (3 hr) (1)
 FOLLOW UP
Tanggal/ Catatan Perkembangan
Jam
9-7-2016 Nadi teraba sangat lemah, bradikardia, irama jantung VT pada
04.25 WITA monitor, nafas gasping (on ventilator)  Start bagging dan RJP

04.30 WITA Lapor DPJP, advis : Lanjutkan RJP, KIE keluarga (+)

04.35 WITA KIE keluarga (+),

RJP + bagging + inj epinefrin 0,1mg iv

05.10 Nadi tidak teraba, HR 0, RR 0, pupil midriasis maksimal, RC -/-,

WITA EKG : asistole  pasien dinyatakan meninggal dihadapan
keluarga dan tenaga medis
DIAGNOSA AKHIR
NP/BBLR/SMK

MAR tanpa fistula

NEC grd III post explorasi laparotomy hr
ke-1
Sepsis Berat

SEBAB KEMATIAN
Multi Organ Failure & Syok septic
MALFORMASI ANOREKTAL

PEMBAHASAN
NECROTIZING ENTEROCOLITIS

Ricky Wibowo, dr, Sp.B

Adria Hariastawa, dr, Sp.B, Sp.BA
 NECROTIZING ENTEROCOLITIS
Epidemiology:

most commonly occurring gastrointestinal emergency in

preterm infants

leading cause of emergency surgery in neonates

overall incidence: 1-5% in most NICU’s

most common in VLBW preterm infants

• 10% of all cases occur in term infants
 NECROTIZING ENTEROCOLITIS
Epidemiology:

10x more likely to occur in infants who have been fed

males = females

blacks > whites

mortality rate: 25-30% All Guts :100%

50% of survivors experience long-term sequele

Historical Risk Factors
Respiratory
Perinatal Cyanotic
Distress
asphyxia Heart Disease
Syndrome
Vasoconstricti
ng agent Enteral Bacterial
(indomethacin nutrition Infection
)

Prematurity
Risk Factors
Prematurity
(mean Bacterial
gestation 30- Colonization
32)

Enteral
feeding
 RISK FACTORS
Inflammatory
Prematurity Infectious Agents:
Mediators:
• primary risk factor • usually occurs in • involved in the
• 90% of cases are clustered development of
premature infants epidemics intestinal injury
• immature • normal intestinal and systemic side
gastrointestinal flora effects
system • E. coli • neutropenia,
• mucosal barrier • Klebsiella spp. thrombocytopeni
• poor motility • Pseudomonas a, acidosis,
• immature immune spp. hypotension
• Clostridium • primary factors
response
• impaired difficile • Tumor necrosis
circulatory • Staph. Epi factor (TNF)
dynamics • Viruses • Platelet
activating factor
(PAF)
•
 RISK FACTORS
Circulatory
Enteral Feedings: Enteral Feedings:
Instability:
• Hypoxic-ischemic • > 90% of infants • immature mucosal
injury with NEC have function
• poor blood flow been fed • malabsorption
to the mesenteric • provides a source • breast milk may
vessels for H2 production have a protective
• local rebound • hyperosmolar effect
hyperemia with formula/medicatio • IGA
re-perfusion ns • macrophages,
• production of O2 • aggressive lymphocytes
radicals feedings • complement
• Polycythemia • too much components
• increased volume • lysozyme,
viscosity causing • rate of increase lactoferrin
decreased blood >20cc/kg/day • acetylhydrolase
flow
• exchange

PRIMARY INFECTIOUS AGENTS
Bacteria, Bacterial toxin, Virus, Fungus
MUCOSAL INJURY
INFLAMMATORY MEDIATORS
Inflammatory cells (macrophage)
Platelet activating factor (PAF)
Tumor necrosis factor (TNF)
Leukotriene C4, Interleukin 1; 6
CIRCULATORY INSTABILITY
Hypoxic-ischemic event
Polycythemia
ENTERAL FEEDINGS
Hypertonic formula or medication
Malabsorption, gaseous distention
H2 gas production, Endotoxin
production
 The Intestinal Barrier
Intestinal motility and
digestion
The mucous coat

Tight Junctions
Preemie GI Wall VS Adult GI Wall
NECROTIZING ENTEROCOLITIS
Pathology:
most commonly involved areas: terminal ileum and
proximal colon
GROSS:
 bowel appears irregularly dilated with hemorrhagic or ischemic
areas of frank necrosis
 focal or diffuse
MICROSCOPIC:
 mucosal edema, hemorrhage and ulceration
Pathology
Image from the Cornell University Medical Collage
 NECROTIZING ENTEROCOLITIS

MICROSCOPIC:
minimal inflammation during the acute phase
 increases during revascularization
granulation tissue and fibrosis develop
 stricture formation
microthrombi in mesenteric arterioles and venules
CLINICAL PRESENTATION
Gestational Age at
Age Diagnosis
• < 30 wks • 20 days
• 31-33 wks • 11 days
• > 34 wks • 5.5 days
• Full term • 3 days
*Time of onset is inversely related to gestational age/birthweight
CLINICAL PRESENTATION
GASTROINTESTINAL SYSTEMIC
• Feeding intolerance • Lethargy
• Abdominal distention • Apnea/respiratory
• Abdominal tenderness distress
• Emesis • Temperature instability
• Occult/gross blood in • Hypotension
stool • Acidosis
• Abdominal mass • Glucose instability
• Erythema of abdominal • DIC
wall • Positive blood cultures
 
Screen clipping taken: 14/04/2014; 21:49
 
 
CLINICAL PRESENTATION
Sudden Onset: Insidious Onset:
• Full term or preterm • Usually preterm
infants • Evolves during 1-2
• Acute catastrophic days
deterioration • Feeding intolerance
• Respiratory • Change in stool pattern
decompensation • Intermittent abdominal
• Shock/acidosis distention
• Marked abdominal • Occult blood in stools
distension
• Positive blood culture
RADIOLOGICAL FINDINGS
Pneumatosis Intestinalis
hydrogen gas within the bowel wall
 product of bacterial metabolism
 a. linear streaking pattern
 more diagnostic
 b. bubbly pattern
 appears like retained meconium
 less specific
Intestinal Pneumotosis
Characteristic radiographic finding

Image from Cornell University Medical

College
Intestinal Pneumotosis
Gas created by bacterial
fermentation
 RADIOLOGICAL FINDINGS
Portal Venous Gas
extension of pneumatosis intestinalis into the portal venous
circulation
 linear branching lucencies overlying the liver and extending to the
periphery
 associated with severe disease and high mortality

Pneumoperitoneum
free air in the peritoneal cavity secondary to perforation
 falciform ligament may be outlined
 “football” sign
surgical emergency
 LABORATORY FINDINGS

CBC Acidosis
• neutropenia/ • metabolic
elevated WBC
• thrombocytopeni DIC
a

Hyperkalemia Positive cultures

• increased secondary to • blood
release from necrotic • CSF
tissue • urine
• stool
TREATMENT
Stop enteral feeds
re-start or increase IVF

Orogastric decompression
low intermittent suction

Antibiotics

Amp/Gent; Clindamycin
Vanc/Cefotaxime • suspected or proven perforation
 TREATMENT
Surgical Consult
indications for surgery:
• portal venous gas;
pneumoperitoneum
suspected or • clinical deterioration
proven NEC • despite medical management
• positive paracentesis
• fixed intestinal loop on serial x-
rays
• erythema of abdominal wall
TREATMENT
Labs: q6-8hrs
CBC, electrolytes, DIC panel, blood gases

X-rays: q6-8hrs
AP, left lateral decubitus or cross-table lateral

Supportive Therapy
fluids, blood products, pressors, mechanical
ventilation
PROGNOSIS
Depends on the severity of the illness
Associated with late complications
 strictures
short-gut syndrome
malabsorption
fistulas
abscess

* MOST COMMON
THANK
YOU

kicky©