SlideShare una empresa de Scribd logo
Case Presentation
Pediatric Gastroenterology
Dr. Fahad
Case:
• 23 month old girl, corrected age 19 months,24/52, has been brought
to ED:
• Hematemesis
• Melena
• Fever
What to ask?
Liver disease
Mallory Weiss tear
Acid peptic disease
Medications
Ingested blood
FB/Battery ingestion
Obstruction
Food Color
General Examination:
• Pale
• Lethargic but arousable
• No apparent dysmorphism
• Not jaundiced or icterus
• No clubbing
• No palmar erythema
• No stigmata of liver disease
• Capillary refill 3-5 sec
• Vitals:
• HR: 130 BP: 77/41 Temp: 36.6C
• RR: 44 Spo2: 100%
Features of Severe GI bleed
Prolonged capillary refill
Hypotension
Melena
Decrease in HGB >2g/dl
HR>20 BPM
Systemic Examination:
• Chest: Bilaterally clear, no adventitious sounds
• CVS: S1+S2 no murmur heard, pulses well felt and equal, mild
tachycardia
• GIT: Soft abdomen, non protuberant, normal shaped umbilicus,
spleen palpable 3cm BCM, liver not palpable, no abdominal veins,
• CNS: GCS: 15/15, lethargic, normal deficit, normal reflex and tone.
• Skin: No Rash, spider angiomata
• Eyes: Non-icteric
• ENT: Normal mucosa
Treatment in ER
• Received IV normal saline bolus twice
• IV Ranitidine
• IV ondansetron
• Investigations were collected
What Investigations to do?
CBC, U/E, creatinine, LFT, Coagulation profile, Blood type and cross,
Guaiac testing
Radio-imaging: Abdominal x-ray , USS abdomen
FBC Counts Range
WBC 16.3x 10^3/uL 6-18
HGB 8.2 g/dl 11-14
RBC 3.74 x 10^3/uL 3.9-5.1
MCV 70 fl 72-84
RDW 16.9 11.5-14
PLT 164 x 10^3/uL 20-550
LFT Value Range
T.Bil 0.5 6-18
ALT 305 11-14
AST 193 3.9-5.1
GGT 77 72-84
Albumin 3.0 11.5-14
PT 16.7 11-14
APTT 40.7 28-41
RFT Value Range
Urea 55 12-40
Na 136 131-145
K 3.7 3.2-5.4
Cl 101 96-111
CO2 19.9 22-28
Creatinine 0.2 0.2-0.4
Lab Value Range
CRP 9.0 <10
PCT 0.45 <0.05
Blood culture No Growth
Blood film:
Hypochromic Microcytic cells with poikilocytosis, Leukocytosis,
neutrophilia with slight shift to the left. Normal platelet count
Abdominal X-ray:
Conclusion :
• No free air shadow seen
• Normal gaseous bowel
distention
• No radiopaque FB shadow
seen
USS Abdomen:
Findings:
Liver: The liver showing homogenous echotexture. No focal lesion could be seen.
Spleen: Spleen is enlarged measures about 10 cm in diameter showing homogenous echotexture
CYST: An irregular cystic structures seen at the porta hepatis showing a proximal diameter measures about 2.6 cm
No comment on ascites, collateral or portal vein size
Conclusion: an irregular cystic lesion seen at the porta hepatis : ? choledochal cyst ?
Provisional Diagnosis
• Hematemesis and Melena for investigation
•Admitted to the ward :
• For Diagnostic workup and supportive care
History:
• Birth History:
• Antenatal: IVF pregnancy, triplets, TCTA, received betamethsone
• Natal: mother had PROM 24 hours prior delivery, 24/52, LSCS, had weak cry electively intubated at
birth
• Postnatal: She was admitted in NICU for around three months and had a stormy course.
• She was managed for following problems:
• Respiratory distress syndrome
• Right sided pneumothorax
• Chronic lung disease (received steroids)
• ESBL Klebsiella Sepsis
• PDA (closed medically),
• Retinopathy of prematurity stage 3 plus disease
• neonatal seizures
• neonatal jaundice of prematurity
• neonatal cholestasis ( TPN associated)
• GERD
• Neonatal anemia.
History:
• Past medical history:
• Readmitted in hospital at 17 months of age due to Enteroviral pneumoniae and
Thrombocytopenia. Received platelets.
• Had an episode of spontaneously resolving epistaxis 1 month ago.
• Persistently low platelets
• Vaccination:
• Uptodate as per DHA schedule
• Feeding History:
• Pediasure and normal family diet , following clinical nutritionist
• Development:
• Mild motor delay for corrected age
Family History:
• Born to non consanguineous parent. Father has celiac disease. Three other siblings are
healthy. On of triplet died postnatally and one triplet had similar course in NICU is alive.
Summary:
• 23 months old girl
• Ex preterm 24/52
• Prolonged and turbulent postnatal NICU stay.
• Acute episode of Hematemesis and Melena
• Pallor and Splenomegaly
• Persistently low platelets
Labs Result
FBC HGB 8.2 g/dl
PLT 164 x
10^3/uL
LFT and
coagulation
ALT 305
AST 193
U/E: Urea 55
USS
abdomen
an irregular cystic lesion
seen at the porta hepatis
Differential Diagnosis for UGI bleed
• Esophageal Varices
• Hemorrhagic Gastritis
• Peptic Ulcer disease
• Mallory Weiss Tear
• Epistaxis (swallowed blood)
• Bowel obstruction
Progress in Ward: Day 1
• Was given PRBC transfusion once
• Started on Octreotide infusion
• IV Vitamin K
• IV Esomeprazole
• IV Cefuroxime
• Only one episode of melena after admission
What further investigation
would be indicated?
 USS doppler
 Endoscopy
USS doppler
Reduced
mean flow
velocity
Dilatation in
MPV
Reversal of
flow
Recanalization
Collateral
vessels/
Cavernoma
USS Doppler Findings: Day 3rd
• Well defined cystic lesion measuring about 1.6x1.4 at the porta hepatis
• No color flow seen inside and not separable from the regional PV and hepatic
artery.
• Enlarged liver measuring about 9.1 cm in MCL. No hepatic focal lesions.
• Enlarged spleen , measuring about 10.4 cm. No focal lesions.
• Mild free fluid at the upper abdomen.
• Conclusion:
• Possibility include:
1. Cavernous transformation ? secondary to chronic PV thrombosis.
2. Choledochcal cyst.
UGI endoscopy
•Endoscopy is both diagnostic and therapeutic in
patients with UGI bleed.
•Varices:
•Site, grade
•Predictors of bleed
•Portal hypertensive gastropathy
Grade
1
Grade 2
Grade 3
UGI Endoscopic findings
• Esophagus: In Lower esophagus there were 4 columns of varices at
3,4,7 and 11 o clock. Grade 2 and 3.
• Stomach: Fundal varices
• Intervention:
• Banding device was applied to varix
• Prophylactic Propranolol was started
Summary:
• 23 months , ex-preterm 24/52
• Prolonged and turbulent postnatal NICU stay.
• Presented with acute episode of Hematemesis and Melena
• Pallor and Splenomegaly
• History of Persistently low platelets
• Low platelets, Mildly deranged liver transaminases
• USS doppler: Well defined cystic lesion measuring at the porta hepatis
• Endoscopy: esophageal and fundal varices
• Thrombophilia screen and autoimmune hepatitis profile was sent
Day 5
• No active complaints
• No Bleeding episodes
• Vitally and clinically well
• IV octreotide infusion weaned in 72
hours
• IV cefuroxime completed for 5 days
and stopped
• on prophylactic propranolol and
esomeprazole
• Labs Repeated
LFT New Old
T.Bil 0.4 0.5
ALT 106 305
AST 63 193
Albumin 3.1 3.0
FBC New Old
WBC 6.3x 10^3/uL 16.3x 10^3/uL
HGB 9.3 g/dl 8.2 g/dl
PLT 122x 10^3/uL 164 x 10^3/uL
Viral study Result
HIV Negative
HBV Negative
What further investigation
may help?
MRI abdomen with contrast
MRI abdomen + MRCP
• LIVER: The liver is enlarged however
showing normal signal intensity with no
detectable focal lesion.
• Biliary System: The left intrahepatic biliary
tree is dilated. Two cysts are noted
arising from the common bile duct
posteriorly measuring about 1.6 cm and anteriorly
measuring about 1.5 cm and containing a stone
measuring about 7 mm. The gallbladder appears
normal with dilated cystic duct. No gallstones
or acute cholecystitis.
• Portal Vein: The portal vein is of thin
caliber.
• Spleen: The spleen is enlarged measuring
about 13 cm.
• Ascites: Moderate to large pelvi-abdominal
ascites.
• Pancreas, both kidneys and bladder appear normal
in size, outline and signal intensity.
• No retroperitoneal lymphadenopathy, lung bases
are showing bilateral collapse-consolidations.
• The visualized bony skeleton appears
unremarkable
• Conclusion:
• The MRI findings are likely in favour of type 2
choledochal cyst.
• Thin calibre portal vein
MRCP:
Repeated USS Doppler
• Portal vein appears narrow 3 mm with extrahepatic varicose net
of vessels Cavernoma
• Suggested Diagnosis :
1. Portal vein thrombosis
2. Congenital stenosis of portal vein ?
Thrombophilia screen
Protein C 89% ( Range: 70 to 140)
Protein S 73% ( Range: 60 to 160)
Anti thrombin 3 87 % ( Range: 80 to 130)
Activated protein C resistance Low <0.8 ( Range: 0.86 to 1.10)
ALKM Negative
ASMA Negative
ANA 1:100 weakly positive
Autoimmune hepatitis screen
Hematology consult was taken
Following tests were done:
• Factor V Leiden Assay (most common cause)
• Prothrombin gene mutation (2nd most common cause)
• Factor 8 Assay
• Homocysteine
Summary:
Acute hematemesis
Esophageal and fundal varices
Portal hypertension secondary to Portal vein thrombosis
Hypersplenism
Choledochal cyst (Todani type 2)
Failure to thrive
Low activated protein C resistance ratio- results awaited
Portal Hypertension:
• It is defined as Hepatic venous Pressure gradient between the IVC and
portal vein greater than 5 mm of Hg.
• PPG>10mmHg(varices)
• PPG>12 mmHg(ascites)
Pathophysiology
Classification
• Extrahepatic
• Intrahepatic
Presinusoidal
• CirrhosisSinusoidal
• Extrahepatic
• Intrahepatic
Post
sinusoidalPre- Sinusoidal Sinusoidal Post-
Sinusoidal
Causes
Clinical Features:
Features/Type Pre- Sinusoidal sinusoidal Post-sinusoidal Our patient
Mean age Children All Ages All Ages 2 years
GI Bleed +++ + +/- Present
Ascites/pedal edema +/- ++ +++ Present
Spleen +++ + + Present
Liver +/- ++ +++ USS enlargement
Anterior Abdominal
veins
+/- ++ +++ back veins -
Encephalopathy - ++ +/- -
Stigmata of LD - +++ ++ -
USS PV thrombosis,
Cavernoma,
Collaterals
Coarse liver,
Collaterals, dilated
PV,
Hepatic vein or IVC
thrombosis
Enlarged liver
Thin caliber PV
cavernoma
Diagnosis:
Clinical
Endoscopy
Ultrasound /Doppler
Management (EHPVO):
• of life threatening
hemorrhage
Emergency
treatment
• directed at prevention
of subsequent bleedingProphylaxis
• Rex bypass shunt
• Splenorenal shuntSurgery
Complications of PHT
Growth
retardation
Pubertal delay Varices Hypersplenism
Portal
hypertensive
biliopathy .
Hepato-
pulmonary
syndrome
Hepato-renal
disease
Prognosis:
• Depending on underlying cause: extrahepatic vs intrahepatic PHT
• Intrahepatic: Liver transplantation
• Extrahepatic:
• frequency of bleeds decreases as they get older
• Neurocognitive defects naturally occurring portosystemic shunts
• Progressive liver disease can be treated or prevented by the Rex shunt.

Más contenido relacionado

La actualidad más candente

Approach to a child with Constipation
Approach to a child with ConstipationApproach to a child with Constipation
Approach to a child with Constipation
Ravi Kumar
 
Approach to Vomiting in children
Approach to Vomiting in children Approach to Vomiting in children
Approach to Vomiting in children
Kannan Chinnasamy
 
Approach to a child with Hepatosplenomegaly
Approach to a child with HepatosplenomegalyApproach to a child with Hepatosplenomegaly
Approach to a child with Hepatosplenomegaly
Sunil Agrawal
 
Approach to pediatric abdominal pain
Approach to pediatric abdominal painApproach to pediatric abdominal pain
Approach to pediatric abdominal pain
Kamran Akbar
 
Renal tubular acidosis
Renal tubular acidosisRenal tubular acidosis
Renal tubular acidosis
subramaniam sethupathy
 
Pediatric Acute Liver Failure
Pediatric Acute Liver FailurePediatric Acute Liver Failure
Pediatric Acute Liver Failure
Aniruddha Ghosh
 
Approach to GI Bleeding in Children
Approach to GI Bleeding in ChildrenApproach to GI Bleeding in Children
Approach to GI Bleeding in Children
CSN Vittal
 
Approach to neuroregression
Approach to neuroregressionApproach to neuroregression
Approach to neuroregression
drswarupa
 
Recurrent abdominal pain
Recurrent abdominal painRecurrent abdominal pain
Recurrent abdominal pain
Hareen Chintapalli
 
Constipation in children
Constipation in childrenConstipation in children
Constipation in children
Sayed Ahmed
 
Hematuria In Children
Hematuria In ChildrenHematuria In Children
Hematuria In Children
Dang Thanh Tuan
 
Bartter syndrome
Bartter syndromeBartter syndrome
Bartter syndrome
Afnan Shamraiz
 
Tuberculosis in children 2021
Tuberculosis in children 2021Tuberculosis in children 2021
Tuberculosis in children 2021
Imran Iqbal
 
Chronic diarrhoea in children
Chronic diarrhoea in childrenChronic diarrhoea in children
Chronic diarrhoea in children
Virendra Hindustani
 
Abdominal pain in children
Abdominal pain in childrenAbdominal pain in children
Abdominal pain in children
Azad Haleem
 
An approach to a child with hepatosplenomegaly and lymphadenopathy
An approach to a child with hepatosplenomegaly and lymphadenopathyAn approach to a child with hepatosplenomegaly and lymphadenopathy
An approach to a child with hepatosplenomegaly and lymphadenopathy
Summu Thakur
 
Pediatric hypertension
Pediatric hypertensionPediatric hypertension
Pediatric hypertension
Tauhid Iqbali
 
Hirschsprungs disease
Hirschsprungs disease Hirschsprungs disease
Hirschsprungs disease
Arylic Singh
 
Hepatospleenomegaly in children
Hepatospleenomegaly in childrenHepatospleenomegaly in children
Hepatospleenomegaly in children
Virendra Hindustani
 
Chronic Liver Disease in pediatric: a case presentation and discussion
Chronic Liver Disease in pediatric: a case presentation and discussionChronic Liver Disease in pediatric: a case presentation and discussion
Chronic Liver Disease in pediatric: a case presentation and discussion
Dr Abdalla M. Gamal
 

La actualidad más candente (20)

Approach to a child with Constipation
Approach to a child with ConstipationApproach to a child with Constipation
Approach to a child with Constipation
 
Approach to Vomiting in children
Approach to Vomiting in children Approach to Vomiting in children
Approach to Vomiting in children
 
Approach to a child with Hepatosplenomegaly
Approach to a child with HepatosplenomegalyApproach to a child with Hepatosplenomegaly
Approach to a child with Hepatosplenomegaly
 
Approach to pediatric abdominal pain
Approach to pediatric abdominal painApproach to pediatric abdominal pain
Approach to pediatric abdominal pain
 
Renal tubular acidosis
Renal tubular acidosisRenal tubular acidosis
Renal tubular acidosis
 
Pediatric Acute Liver Failure
Pediatric Acute Liver FailurePediatric Acute Liver Failure
Pediatric Acute Liver Failure
 
Approach to GI Bleeding in Children
Approach to GI Bleeding in ChildrenApproach to GI Bleeding in Children
Approach to GI Bleeding in Children
 
Approach to neuroregression
Approach to neuroregressionApproach to neuroregression
Approach to neuroregression
 
Recurrent abdominal pain
Recurrent abdominal painRecurrent abdominal pain
Recurrent abdominal pain
 
Constipation in children
Constipation in childrenConstipation in children
Constipation in children
 
Hematuria In Children
Hematuria In ChildrenHematuria In Children
Hematuria In Children
 
Bartter syndrome
Bartter syndromeBartter syndrome
Bartter syndrome
 
Tuberculosis in children 2021
Tuberculosis in children 2021Tuberculosis in children 2021
Tuberculosis in children 2021
 
Chronic diarrhoea in children
Chronic diarrhoea in childrenChronic diarrhoea in children
Chronic diarrhoea in children
 
Abdominal pain in children
Abdominal pain in childrenAbdominal pain in children
Abdominal pain in children
 
An approach to a child with hepatosplenomegaly and lymphadenopathy
An approach to a child with hepatosplenomegaly and lymphadenopathyAn approach to a child with hepatosplenomegaly and lymphadenopathy
An approach to a child with hepatosplenomegaly and lymphadenopathy
 
Pediatric hypertension
Pediatric hypertensionPediatric hypertension
Pediatric hypertension
 
Hirschsprungs disease
Hirschsprungs disease Hirschsprungs disease
Hirschsprungs disease
 
Hepatospleenomegaly in children
Hepatospleenomegaly in childrenHepatospleenomegaly in children
Hepatospleenomegaly in children
 
Chronic Liver Disease in pediatric: a case presentation and discussion
Chronic Liver Disease in pediatric: a case presentation and discussionChronic Liver Disease in pediatric: a case presentation and discussion
Chronic Liver Disease in pediatric: a case presentation and discussion
 

Similar a Approach to Pediatric hematemesis

Mahsa - presentation on Sepsis 8-4-22.pptx
Mahsa - presentation on Sepsis 8-4-22.pptxMahsa - presentation on Sepsis 8-4-22.pptx
Mahsa - presentation on Sepsis 8-4-22.pptx
Bishan Rajapakse
 
Choledochal cyst
Choledochal cystCholedochal cyst
Choledochal cyst
Muhammad Ihtesham
 
Diverticular disease
Diverticular diseaseDiverticular disease
Diverticular disease
Dhaval Mangukiya
 
Cld non hep b,c
Cld non hep b,cCld non hep b,c
Cld non hep b,c
West Medicine Ward
 
Acute pancreatitis
Acute pancreatitisAcute pancreatitis
Acute pancreatitis
KIST Surgery
 
Celiac common presentation of a uncommon disease saved with date
Celiac common presentation of a uncommon disease  saved with dateCeliac common presentation of a uncommon disease  saved with date
Celiac common presentation of a uncommon disease saved with date
Muhammad Arshad
 
Dr farrag case
Dr farrag   caseDr farrag   case
Dr farrag case
FarragBahbah
 
Acute pancreatitis
Acute pancreatitisAcute pancreatitis
Acute pancreatitis
JabeMohammed
 
Bph
BphBph
Rectal cancer
Rectal cancerRectal cancer
Rectal cancer
Kristine Faith Tablizo
 
Multidisciplinary case chronic myelogenous leukemia in pregnancy
Multidisciplinary case chronic myelogenous leukemia in pregnancyMultidisciplinary case chronic myelogenous leukemia in pregnancy
Multidisciplinary case chronic myelogenous leukemia in pregnancy
DR MUKESH SAH
 
Dr ahmed alkodousi case
Dr ahmed alkodousi   caseDr ahmed alkodousi   case
Dr ahmed alkodousi case
FarragBahbah
 
choledocal cyst.pptx
choledocal cyst.pptxcholedocal cyst.pptx
choledocal cyst.pptx
drhassaanmansoor
 
Gastroenterology Presentation (& some Abdominal Surgery Stuff!)
Gastroenterology Presentation (& some Abdominal Surgery Stuff!)Gastroenterology Presentation (& some Abdominal Surgery Stuff!)
Gastroenterology Presentation (& some Abdominal Surgery Stuff!)
meducationdotnet
 
Nephrotic syndrome.pptx
Nephrotic syndrome.pptxNephrotic syndrome.pptx
Nephrotic syndrome.pptx
AklimaMotaleb1
 
Acute pancreatitis
Acute pancreatitisAcute pancreatitis
A.pptx
A.pptxA.pptx
A.pptx
MarkPadullo3
 
Acute GI bleed
Acute GI bleedAcute GI bleed
April 2023 M&M presentation for morbidity and mortality
April 2023 M&M presentation for morbidity and mortalityApril 2023 M&M presentation for morbidity and mortality
April 2023 M&M presentation for morbidity and mortality
niyigok
 
Ascites
AscitesAscites
Ascites
alyaqdhan
 

Similar a Approach to Pediatric hematemesis (20)

Mahsa - presentation on Sepsis 8-4-22.pptx
Mahsa - presentation on Sepsis 8-4-22.pptxMahsa - presentation on Sepsis 8-4-22.pptx
Mahsa - presentation on Sepsis 8-4-22.pptx
 
Choledochal cyst
Choledochal cystCholedochal cyst
Choledochal cyst
 
Diverticular disease
Diverticular diseaseDiverticular disease
Diverticular disease
 
Cld non hep b,c
Cld non hep b,cCld non hep b,c
Cld non hep b,c
 
Acute pancreatitis
Acute pancreatitisAcute pancreatitis
Acute pancreatitis
 
Celiac common presentation of a uncommon disease saved with date
Celiac common presentation of a uncommon disease  saved with dateCeliac common presentation of a uncommon disease  saved with date
Celiac common presentation of a uncommon disease saved with date
 
Dr farrag case
Dr farrag   caseDr farrag   case
Dr farrag case
 
Acute pancreatitis
Acute pancreatitisAcute pancreatitis
Acute pancreatitis
 
Bph
BphBph
Bph
 
Rectal cancer
Rectal cancerRectal cancer
Rectal cancer
 
Multidisciplinary case chronic myelogenous leukemia in pregnancy
Multidisciplinary case chronic myelogenous leukemia in pregnancyMultidisciplinary case chronic myelogenous leukemia in pregnancy
Multidisciplinary case chronic myelogenous leukemia in pregnancy
 
Dr ahmed alkodousi case
Dr ahmed alkodousi   caseDr ahmed alkodousi   case
Dr ahmed alkodousi case
 
choledocal cyst.pptx
choledocal cyst.pptxcholedocal cyst.pptx
choledocal cyst.pptx
 
Gastroenterology Presentation (& some Abdominal Surgery Stuff!)
Gastroenterology Presentation (& some Abdominal Surgery Stuff!)Gastroenterology Presentation (& some Abdominal Surgery Stuff!)
Gastroenterology Presentation (& some Abdominal Surgery Stuff!)
 
Nephrotic syndrome.pptx
Nephrotic syndrome.pptxNephrotic syndrome.pptx
Nephrotic syndrome.pptx
 
Acute pancreatitis
Acute pancreatitisAcute pancreatitis
Acute pancreatitis
 
A.pptx
A.pptxA.pptx
A.pptx
 
Acute GI bleed
Acute GI bleedAcute GI bleed
Acute GI bleed
 
April 2023 M&M presentation for morbidity and mortality
April 2023 M&M presentation for morbidity and mortalityApril 2023 M&M presentation for morbidity and mortality
April 2023 M&M presentation for morbidity and mortality
 
Ascites
AscitesAscites
Ascites
 

Más de Pediatrics

Approach to Pediatric Hypoglycemia
Approach to Pediatric HypoglycemiaApproach to Pediatric Hypoglycemia
Approach to Pediatric Hypoglycemia
Pediatrics
 
Pediatric Asthma
Pediatric AsthmaPediatric Asthma
Pediatric Asthma
Pediatrics
 
Pediatric Urinary Tract infections
Pediatric Urinary Tract infectionsPediatric Urinary Tract infections
Pediatric Urinary Tract infections
Pediatrics
 
Approach to pediatric pancytopenia
Approach to pediatric pancytopeniaApproach to pediatric pancytopenia
Approach to pediatric pancytopenia
Pediatrics
 
inflammatory bowel disease
inflammatory bowel diseaseinflammatory bowel disease
inflammatory bowel disease
Pediatrics
 
peripheral blood smear spot diagnosis
peripheral blood smear spot diagnosisperipheral blood smear spot diagnosis
peripheral blood smear spot diagnosis
Pediatrics
 
Lymphadenopathy in children
Lymphadenopathy in children Lymphadenopathy in children
Lymphadenopathy in children
Pediatrics
 
Febrile neutropenia
Febrile neutropeniaFebrile neutropenia
Febrile neutropenia
Pediatrics
 
Genetics Spot diagnosis
Genetics Spot diagnosisGenetics Spot diagnosis
Genetics Spot diagnosis
Pediatrics
 
Karyotypes and dysmorphic features
Karyotypes and dysmorphic featuresKaryotypes and dysmorphic features
Karyotypes and dysmorphic features
Pediatrics
 
Fatty Acid oxidation defects
Fatty Acid oxidation defects Fatty Acid oxidation defects
Fatty Acid oxidation defects
Pediatrics
 
Urea cycle defects
Urea cycle defectsUrea cycle defects
Urea cycle defects
Pediatrics
 
Transfusion of blood products
Transfusion of blood productsTransfusion of blood products
Transfusion of blood products
Pediatrics
 
Rsv bronchiolitis ppt
Rsv bronchiolitis pptRsv bronchiolitis ppt
Rsv bronchiolitis ppt
Pediatrics
 
Pneumonia
PneumoniaPneumonia
Pneumonia
Pediatrics
 
Croup
CroupCroup
Croup
Pediatrics
 
Nutrition
NutritionNutrition
Nutrition
Pediatrics
 
Approach to cxr
Approach to cxrApproach to cxr
Approach to cxr
Pediatrics
 
Acute gastroenteritis
Acute gastroenteritis  Acute gastroenteritis
Acute gastroenteritis
Pediatrics
 
Pals presentation
Pals presentationPals presentation
Pals presentation
Pediatrics
 

Más de Pediatrics (20)

Approach to Pediatric Hypoglycemia
Approach to Pediatric HypoglycemiaApproach to Pediatric Hypoglycemia
Approach to Pediatric Hypoglycemia
 
Pediatric Asthma
Pediatric AsthmaPediatric Asthma
Pediatric Asthma
 
Pediatric Urinary Tract infections
Pediatric Urinary Tract infectionsPediatric Urinary Tract infections
Pediatric Urinary Tract infections
 
Approach to pediatric pancytopenia
Approach to pediatric pancytopeniaApproach to pediatric pancytopenia
Approach to pediatric pancytopenia
 
inflammatory bowel disease
inflammatory bowel diseaseinflammatory bowel disease
inflammatory bowel disease
 
peripheral blood smear spot diagnosis
peripheral blood smear spot diagnosisperipheral blood smear spot diagnosis
peripheral blood smear spot diagnosis
 
Lymphadenopathy in children
Lymphadenopathy in children Lymphadenopathy in children
Lymphadenopathy in children
 
Febrile neutropenia
Febrile neutropeniaFebrile neutropenia
Febrile neutropenia
 
Genetics Spot diagnosis
Genetics Spot diagnosisGenetics Spot diagnosis
Genetics Spot diagnosis
 
Karyotypes and dysmorphic features
Karyotypes and dysmorphic featuresKaryotypes and dysmorphic features
Karyotypes and dysmorphic features
 
Fatty Acid oxidation defects
Fatty Acid oxidation defects Fatty Acid oxidation defects
Fatty Acid oxidation defects
 
Urea cycle defects
Urea cycle defectsUrea cycle defects
Urea cycle defects
 
Transfusion of blood products
Transfusion of blood productsTransfusion of blood products
Transfusion of blood products
 
Rsv bronchiolitis ppt
Rsv bronchiolitis pptRsv bronchiolitis ppt
Rsv bronchiolitis ppt
 
Pneumonia
PneumoniaPneumonia
Pneumonia
 
Croup
CroupCroup
Croup
 
Nutrition
NutritionNutrition
Nutrition
 
Approach to cxr
Approach to cxrApproach to cxr
Approach to cxr
 
Acute gastroenteritis
Acute gastroenteritis  Acute gastroenteritis
Acute gastroenteritis
 
Pals presentation
Pals presentationPals presentation
Pals presentation
 

Último

NVBDCP.pptx Nation vector borne disease control program
NVBDCP.pptx Nation vector borne disease control programNVBDCP.pptx Nation vector borne disease control program
NVBDCP.pptx Nation vector borne disease control program
Sapna Thakur
 
Vestibulocochlear Nerve by Dr. Rabia Inam Gandapore.pptx
Vestibulocochlear Nerve by Dr. Rabia Inam Gandapore.pptxVestibulocochlear Nerve by Dr. Rabia Inam Gandapore.pptx
Vestibulocochlear Nerve by Dr. Rabia Inam Gandapore.pptx
Dr. Rabia Inam Gandapore
 
The Best Ayurvedic Antacid Tablets in India
The Best Ayurvedic Antacid Tablets in IndiaThe Best Ayurvedic Antacid Tablets in India
The Best Ayurvedic Antacid Tablets in India
Swastik Ayurveda
 
The Electrocardiogram - Physiologic Principles
The Electrocardiogram - Physiologic PrinciplesThe Electrocardiogram - Physiologic Principles
The Electrocardiogram - Physiologic Principles
MedicoseAcademics
 
Efficacy of Avartana Sneha in Ayurveda
Efficacy of Avartana Sneha in AyurvedaEfficacy of Avartana Sneha in Ayurveda
Efficacy of Avartana Sneha in Ayurveda
Dr. Jyothirmai Paindla
 
ABDOMINAL TRAUMA in pediatrics part one.
ABDOMINAL TRAUMA in pediatrics part one.ABDOMINAL TRAUMA in pediatrics part one.
ABDOMINAL TRAUMA in pediatrics part one.
drhasanrajab
 
Journal Article Review on Rasamanikya
Journal Article Review on RasamanikyaJournal Article Review on Rasamanikya
Journal Article Review on Rasamanikya
Dr. Jyothirmai Paindla
 
Physiology of Chemical Sensation of smell.pdf
Physiology of Chemical Sensation of smell.pdfPhysiology of Chemical Sensation of smell.pdf
Physiology of Chemical Sensation of smell.pdf
MedicoseAcademics
 
Aortic Association CBL Pilot April 19 – 20 Bern
Aortic Association CBL Pilot April 19 – 20 BernAortic Association CBL Pilot April 19 – 20 Bern
Aortic Association CBL Pilot April 19 – 20 Bern
suvadeepdas911
 
Management of Traumatic Splenic injury.pptx
Management of Traumatic Splenic injury.pptxManagement of Traumatic Splenic injury.pptx
Management of Traumatic Splenic injury.pptx
AkshaySarraf1
 
Novas diretrizes da OMS para os cuidados perinatais de mais qualidade
Novas diretrizes da OMS para os cuidados perinatais de mais qualidadeNovas diretrizes da OMS para os cuidados perinatais de mais qualidade
Novas diretrizes da OMS para os cuidados perinatais de mais qualidade
Prof. Marcus Renato de Carvalho
 
Top-Vitamin-Supplement-Brands-in-India List
Top-Vitamin-Supplement-Brands-in-India ListTop-Vitamin-Supplement-Brands-in-India List
Top-Vitamin-Supplement-Brands-in-India List
SwisschemDerma
 
Identification and nursing management of congenital malformations .pptx
Identification and nursing management of congenital malformations .pptxIdentification and nursing management of congenital malformations .pptx
Identification and nursing management of congenital malformations .pptx
MGM SCHOOL/COLLEGE OF NURSING
 
A Classical Text Review on Basavarajeeyam
A Classical Text Review on BasavarajeeyamA Classical Text Review on Basavarajeeyam
A Classical Text Review on Basavarajeeyam
Dr. Jyothirmai Paindla
 
THERAPEUTIC ANTISENSE MOLECULES .pptx
THERAPEUTIC ANTISENSE MOLECULES    .pptxTHERAPEUTIC ANTISENSE MOLECULES    .pptx
THERAPEUTIC ANTISENSE MOLECULES .pptx
70KRISHPATEL
 
Non-respiratory Functions of the Lungs.pdf
Non-respiratory Functions of the Lungs.pdfNon-respiratory Functions of the Lungs.pdf
Non-respiratory Functions of the Lungs.pdf
MedicoseAcademics
 
Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...
Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...
Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...
Oleg Kshivets
 
CHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdf
CHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdfCHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdf
CHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdf
rishi2789
 
Sex determination from mandible pelvis and skull
Sex determination from mandible pelvis and skullSex determination from mandible pelvis and skull
Sex determination from mandible pelvis and skull
ShashankRoodkee
 
Role of Mukta Pishti in the Management of Hyperthyroidism
Role of Mukta Pishti in the Management of HyperthyroidismRole of Mukta Pishti in the Management of Hyperthyroidism
Role of Mukta Pishti in the Management of Hyperthyroidism
Dr. Jyothirmai Paindla
 

Último (20)

NVBDCP.pptx Nation vector borne disease control program
NVBDCP.pptx Nation vector borne disease control programNVBDCP.pptx Nation vector borne disease control program
NVBDCP.pptx Nation vector borne disease control program
 
Vestibulocochlear Nerve by Dr. Rabia Inam Gandapore.pptx
Vestibulocochlear Nerve by Dr. Rabia Inam Gandapore.pptxVestibulocochlear Nerve by Dr. Rabia Inam Gandapore.pptx
Vestibulocochlear Nerve by Dr. Rabia Inam Gandapore.pptx
 
The Best Ayurvedic Antacid Tablets in India
The Best Ayurvedic Antacid Tablets in IndiaThe Best Ayurvedic Antacid Tablets in India
The Best Ayurvedic Antacid Tablets in India
 
The Electrocardiogram - Physiologic Principles
The Electrocardiogram - Physiologic PrinciplesThe Electrocardiogram - Physiologic Principles
The Electrocardiogram - Physiologic Principles
 
Efficacy of Avartana Sneha in Ayurveda
Efficacy of Avartana Sneha in AyurvedaEfficacy of Avartana Sneha in Ayurveda
Efficacy of Avartana Sneha in Ayurveda
 
ABDOMINAL TRAUMA in pediatrics part one.
ABDOMINAL TRAUMA in pediatrics part one.ABDOMINAL TRAUMA in pediatrics part one.
ABDOMINAL TRAUMA in pediatrics part one.
 
Journal Article Review on Rasamanikya
Journal Article Review on RasamanikyaJournal Article Review on Rasamanikya
Journal Article Review on Rasamanikya
 
Physiology of Chemical Sensation of smell.pdf
Physiology of Chemical Sensation of smell.pdfPhysiology of Chemical Sensation of smell.pdf
Physiology of Chemical Sensation of smell.pdf
 
Aortic Association CBL Pilot April 19 – 20 Bern
Aortic Association CBL Pilot April 19 – 20 BernAortic Association CBL Pilot April 19 – 20 Bern
Aortic Association CBL Pilot April 19 – 20 Bern
 
Management of Traumatic Splenic injury.pptx
Management of Traumatic Splenic injury.pptxManagement of Traumatic Splenic injury.pptx
Management of Traumatic Splenic injury.pptx
 
Novas diretrizes da OMS para os cuidados perinatais de mais qualidade
Novas diretrizes da OMS para os cuidados perinatais de mais qualidadeNovas diretrizes da OMS para os cuidados perinatais de mais qualidade
Novas diretrizes da OMS para os cuidados perinatais de mais qualidade
 
Top-Vitamin-Supplement-Brands-in-India List
Top-Vitamin-Supplement-Brands-in-India ListTop-Vitamin-Supplement-Brands-in-India List
Top-Vitamin-Supplement-Brands-in-India List
 
Identification and nursing management of congenital malformations .pptx
Identification and nursing management of congenital malformations .pptxIdentification and nursing management of congenital malformations .pptx
Identification and nursing management of congenital malformations .pptx
 
A Classical Text Review on Basavarajeeyam
A Classical Text Review on BasavarajeeyamA Classical Text Review on Basavarajeeyam
A Classical Text Review on Basavarajeeyam
 
THERAPEUTIC ANTISENSE MOLECULES .pptx
THERAPEUTIC ANTISENSE MOLECULES    .pptxTHERAPEUTIC ANTISENSE MOLECULES    .pptx
THERAPEUTIC ANTISENSE MOLECULES .pptx
 
Non-respiratory Functions of the Lungs.pdf
Non-respiratory Functions of the Lungs.pdfNon-respiratory Functions of the Lungs.pdf
Non-respiratory Functions of the Lungs.pdf
 
Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...
Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...
Local Advanced Lung Cancer: Artificial Intelligence, Synergetics, Complex Sys...
 
CHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdf
CHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdfCHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdf
CHEMOTHERAPY_RDP_CHAPTER 6_Anti Malarial Drugs.pdf
 
Sex determination from mandible pelvis and skull
Sex determination from mandible pelvis and skullSex determination from mandible pelvis and skull
Sex determination from mandible pelvis and skull
 
Role of Mukta Pishti in the Management of Hyperthyroidism
Role of Mukta Pishti in the Management of HyperthyroidismRole of Mukta Pishti in the Management of Hyperthyroidism
Role of Mukta Pishti in the Management of Hyperthyroidism
 

Approach to Pediatric hematemesis

  • 2. Case: • 23 month old girl, corrected age 19 months,24/52, has been brought to ED: • Hematemesis • Melena • Fever
  • 3. What to ask? Liver disease Mallory Weiss tear Acid peptic disease Medications Ingested blood FB/Battery ingestion Obstruction Food Color
  • 4. General Examination: • Pale • Lethargic but arousable • No apparent dysmorphism • Not jaundiced or icterus • No clubbing • No palmar erythema • No stigmata of liver disease • Capillary refill 3-5 sec • Vitals: • HR: 130 BP: 77/41 Temp: 36.6C • RR: 44 Spo2: 100% Features of Severe GI bleed Prolonged capillary refill Hypotension Melena Decrease in HGB >2g/dl HR>20 BPM
  • 5. Systemic Examination: • Chest: Bilaterally clear, no adventitious sounds • CVS: S1+S2 no murmur heard, pulses well felt and equal, mild tachycardia • GIT: Soft abdomen, non protuberant, normal shaped umbilicus, spleen palpable 3cm BCM, liver not palpable, no abdominal veins, • CNS: GCS: 15/15, lethargic, normal deficit, normal reflex and tone. • Skin: No Rash, spider angiomata • Eyes: Non-icteric • ENT: Normal mucosa
  • 6. Treatment in ER • Received IV normal saline bolus twice • IV Ranitidine • IV ondansetron • Investigations were collected
  • 7. What Investigations to do? CBC, U/E, creatinine, LFT, Coagulation profile, Blood type and cross, Guaiac testing Radio-imaging: Abdominal x-ray , USS abdomen
  • 8. FBC Counts Range WBC 16.3x 10^3/uL 6-18 HGB 8.2 g/dl 11-14 RBC 3.74 x 10^3/uL 3.9-5.1 MCV 70 fl 72-84 RDW 16.9 11.5-14 PLT 164 x 10^3/uL 20-550
  • 9. LFT Value Range T.Bil 0.5 6-18 ALT 305 11-14 AST 193 3.9-5.1 GGT 77 72-84 Albumin 3.0 11.5-14 PT 16.7 11-14 APTT 40.7 28-41 RFT Value Range Urea 55 12-40 Na 136 131-145 K 3.7 3.2-5.4 Cl 101 96-111 CO2 19.9 22-28 Creatinine 0.2 0.2-0.4
  • 10. Lab Value Range CRP 9.0 <10 PCT 0.45 <0.05 Blood culture No Growth Blood film: Hypochromic Microcytic cells with poikilocytosis, Leukocytosis, neutrophilia with slight shift to the left. Normal platelet count
  • 11. Abdominal X-ray: Conclusion : • No free air shadow seen • Normal gaseous bowel distention • No radiopaque FB shadow seen
  • 12. USS Abdomen: Findings: Liver: The liver showing homogenous echotexture. No focal lesion could be seen. Spleen: Spleen is enlarged measures about 10 cm in diameter showing homogenous echotexture CYST: An irregular cystic structures seen at the porta hepatis showing a proximal diameter measures about 2.6 cm No comment on ascites, collateral or portal vein size Conclusion: an irregular cystic lesion seen at the porta hepatis : ? choledochal cyst ?
  • 13. Provisional Diagnosis • Hematemesis and Melena for investigation •Admitted to the ward : • For Diagnostic workup and supportive care
  • 14. History: • Birth History: • Antenatal: IVF pregnancy, triplets, TCTA, received betamethsone • Natal: mother had PROM 24 hours prior delivery, 24/52, LSCS, had weak cry electively intubated at birth • Postnatal: She was admitted in NICU for around three months and had a stormy course. • She was managed for following problems: • Respiratory distress syndrome • Right sided pneumothorax • Chronic lung disease (received steroids) • ESBL Klebsiella Sepsis • PDA (closed medically), • Retinopathy of prematurity stage 3 plus disease • neonatal seizures • neonatal jaundice of prematurity • neonatal cholestasis ( TPN associated) • GERD • Neonatal anemia.
  • 15. History: • Past medical history: • Readmitted in hospital at 17 months of age due to Enteroviral pneumoniae and Thrombocytopenia. Received platelets. • Had an episode of spontaneously resolving epistaxis 1 month ago. • Persistently low platelets • Vaccination: • Uptodate as per DHA schedule • Feeding History: • Pediasure and normal family diet , following clinical nutritionist • Development: • Mild motor delay for corrected age Family History: • Born to non consanguineous parent. Father has celiac disease. Three other siblings are healthy. On of triplet died postnatally and one triplet had similar course in NICU is alive.
  • 16. Summary: • 23 months old girl • Ex preterm 24/52 • Prolonged and turbulent postnatal NICU stay. • Acute episode of Hematemesis and Melena • Pallor and Splenomegaly • Persistently low platelets Labs Result FBC HGB 8.2 g/dl PLT 164 x 10^3/uL LFT and coagulation ALT 305 AST 193 U/E: Urea 55 USS abdomen an irregular cystic lesion seen at the porta hepatis
  • 17. Differential Diagnosis for UGI bleed • Esophageal Varices • Hemorrhagic Gastritis • Peptic Ulcer disease • Mallory Weiss Tear • Epistaxis (swallowed blood) • Bowel obstruction
  • 18. Progress in Ward: Day 1 • Was given PRBC transfusion once • Started on Octreotide infusion • IV Vitamin K • IV Esomeprazole • IV Cefuroxime • Only one episode of melena after admission
  • 19. What further investigation would be indicated?  USS doppler  Endoscopy
  • 20. USS doppler Reduced mean flow velocity Dilatation in MPV Reversal of flow Recanalization Collateral vessels/ Cavernoma
  • 21. USS Doppler Findings: Day 3rd • Well defined cystic lesion measuring about 1.6x1.4 at the porta hepatis • No color flow seen inside and not separable from the regional PV and hepatic artery. • Enlarged liver measuring about 9.1 cm in MCL. No hepatic focal lesions. • Enlarged spleen , measuring about 10.4 cm. No focal lesions. • Mild free fluid at the upper abdomen. • Conclusion: • Possibility include: 1. Cavernous transformation ? secondary to chronic PV thrombosis. 2. Choledochcal cyst.
  • 22. UGI endoscopy •Endoscopy is both diagnostic and therapeutic in patients with UGI bleed. •Varices: •Site, grade •Predictors of bleed •Portal hypertensive gastropathy Grade 1 Grade 2 Grade 3
  • 23. UGI Endoscopic findings • Esophagus: In Lower esophagus there were 4 columns of varices at 3,4,7 and 11 o clock. Grade 2 and 3. • Stomach: Fundal varices • Intervention: • Banding device was applied to varix • Prophylactic Propranolol was started
  • 24.
  • 25. Summary: • 23 months , ex-preterm 24/52 • Prolonged and turbulent postnatal NICU stay. • Presented with acute episode of Hematemesis and Melena • Pallor and Splenomegaly • History of Persistently low platelets • Low platelets, Mildly deranged liver transaminases • USS doppler: Well defined cystic lesion measuring at the porta hepatis • Endoscopy: esophageal and fundal varices • Thrombophilia screen and autoimmune hepatitis profile was sent
  • 26. Day 5 • No active complaints • No Bleeding episodes • Vitally and clinically well • IV octreotide infusion weaned in 72 hours • IV cefuroxime completed for 5 days and stopped • on prophylactic propranolol and esomeprazole • Labs Repeated LFT New Old T.Bil 0.4 0.5 ALT 106 305 AST 63 193 Albumin 3.1 3.0 FBC New Old WBC 6.3x 10^3/uL 16.3x 10^3/uL HGB 9.3 g/dl 8.2 g/dl PLT 122x 10^3/uL 164 x 10^3/uL Viral study Result HIV Negative HBV Negative
  • 27. What further investigation may help? MRI abdomen with contrast
  • 28. MRI abdomen + MRCP • LIVER: The liver is enlarged however showing normal signal intensity with no detectable focal lesion. • Biliary System: The left intrahepatic biliary tree is dilated. Two cysts are noted arising from the common bile duct posteriorly measuring about 1.6 cm and anteriorly measuring about 1.5 cm and containing a stone measuring about 7 mm. The gallbladder appears normal with dilated cystic duct. No gallstones or acute cholecystitis. • Portal Vein: The portal vein is of thin caliber. • Spleen: The spleen is enlarged measuring about 13 cm. • Ascites: Moderate to large pelvi-abdominal ascites. • Pancreas, both kidneys and bladder appear normal in size, outline and signal intensity. • No retroperitoneal lymphadenopathy, lung bases are showing bilateral collapse-consolidations. • The visualized bony skeleton appears unremarkable • Conclusion: • The MRI findings are likely in favour of type 2 choledochal cyst. • Thin calibre portal vein
  • 29. MRCP:
  • 30. Repeated USS Doppler • Portal vein appears narrow 3 mm with extrahepatic varicose net of vessels Cavernoma • Suggested Diagnosis : 1. Portal vein thrombosis 2. Congenital stenosis of portal vein ?
  • 31.
  • 32. Thrombophilia screen Protein C 89% ( Range: 70 to 140) Protein S 73% ( Range: 60 to 160) Anti thrombin 3 87 % ( Range: 80 to 130) Activated protein C resistance Low <0.8 ( Range: 0.86 to 1.10) ALKM Negative ASMA Negative ANA 1:100 weakly positive Autoimmune hepatitis screen
  • 33. Hematology consult was taken Following tests were done: • Factor V Leiden Assay (most common cause) • Prothrombin gene mutation (2nd most common cause) • Factor 8 Assay • Homocysteine
  • 34.
  • 35. Summary: Acute hematemesis Esophageal and fundal varices Portal hypertension secondary to Portal vein thrombosis Hypersplenism Choledochal cyst (Todani type 2) Failure to thrive Low activated protein C resistance ratio- results awaited
  • 36. Portal Hypertension: • It is defined as Hepatic venous Pressure gradient between the IVC and portal vein greater than 5 mm of Hg. • PPG>10mmHg(varices) • PPG>12 mmHg(ascites)
  • 38. Classification • Extrahepatic • Intrahepatic Presinusoidal • CirrhosisSinusoidal • Extrahepatic • Intrahepatic Post sinusoidalPre- Sinusoidal Sinusoidal Post- Sinusoidal Causes
  • 39. Clinical Features: Features/Type Pre- Sinusoidal sinusoidal Post-sinusoidal Our patient Mean age Children All Ages All Ages 2 years GI Bleed +++ + +/- Present Ascites/pedal edema +/- ++ +++ Present Spleen +++ + + Present Liver +/- ++ +++ USS enlargement Anterior Abdominal veins +/- ++ +++ back veins - Encephalopathy - ++ +/- - Stigmata of LD - +++ ++ - USS PV thrombosis, Cavernoma, Collaterals Coarse liver, Collaterals, dilated PV, Hepatic vein or IVC thrombosis Enlarged liver Thin caliber PV cavernoma
  • 41. Management (EHPVO): • of life threatening hemorrhage Emergency treatment • directed at prevention of subsequent bleedingProphylaxis • Rex bypass shunt • Splenorenal shuntSurgery
  • 42. Complications of PHT Growth retardation Pubertal delay Varices Hypersplenism Portal hypertensive biliopathy . Hepato- pulmonary syndrome Hepato-renal disease
  • 43. Prognosis: • Depending on underlying cause: extrahepatic vs intrahepatic PHT • Intrahepatic: Liver transplantation • Extrahepatic: • frequency of bleeds decreases as they get older • Neurocognitive defects naturally occurring portosystemic shunts • Progressive liver disease can be treated or prevented by the Rex shunt.

Notas del editor

  1. Hematemesis was today large in amount around 2 cups, fresh blood 2-3 times ( no bile) Melena was today morning one time with loose stools, moderate to large amount Fever is tactile since past 2 days associated with URTI symptoms With good oral intake and activity until symptoms. A quick history aimed at broadly identifying the Cause : Upper GI bleed: (proximal to ligament of Treitz) Bright red vomitus indicates brisk bleeding with melena (Indicating Gastric acid effect on blood) (( had this been LGI it would have been dark maroon or bright red))
  2. Cause: Liver disease: jaundice, easy bruising, edema Mallory weiss: episodes of frequent forceful of vomiting followed by hematemesis in a relatively well child Acid peptic disease or GERD with esophagitis : Feeding difficulty/dysphagia, regurgitation, fussiness with eating, in older child retrosternal pain/epigastric pain, poor weight gain Medications: NSAIDS, corticosteroids( with ibuprofen even short term usage can cause gastritis) Ingested blood: recent epistaxis Battery ingestion: No Foreign body ingestion history Obstruction: Intussception, Volvulus : bilious emesis , episodic abdominal pain/irritability, drawing up of legs during episodes with quiet periods in between. Food Color: well appearing coloring agents, tomato skin, ketchup, spinach, licorice, iron, bismuth salicylate
  3. Previous HGB 10.4 8.2 UGI Bleed SEVERE GI BLEED Class I. Loss of 15% or less of the total blood volume. clinical manifestations of hypovolemia are minimal or absent. Class II. Loss of 15 to 30% of the blood volume. The clinical findings at this stage may include resting tachycardia and orthostatic changes in heart rate and blood pressure Class III. Loss of 30 to 40% of the blood volume usually marks the onset of hypovolemic shock, with a decrease in blood pressure and urine output Class IV. Loss of more than 40% of blood volume is a harbinger of circulatory collapse. Therefore, when hypovolemia is accompanied by marked hypotension, oliguria, or other evidence of organ failure, prompt volume resuscitation is mandatory.
  4. CNS: lethargic due to hypotension GIT: splenomegaly evidence of PHT Nasopharynx: For evidence of disrupted mucosa or inflamed tonsils suggesting swallowed blood. UGI Bleed SEVERE GI BLEED Portal Hypertension (indeed PHT is Manifested by two principal signs Hematemesis (dilatation of collateral venous channels)+ Splenomegaly , which may cause depression of one or two blood elements ) presinusoidal, sinusoidal or post sinusoidal
  5. Principal management:
  6. A brisk response may also be seen in hemorrhage. Drop in HCT predicting loss of blood in ml: Each unit of blood loss drops the hematocrit by 3 percent points (hemoglobin by 1 mg/dL). Conversely stated if the hematocrit drops by 6% the patient has lost 2 units of blood.
  7. Bowel obstruction: We are looking for causes of GI bleed that can be seen on xray: that is true especially + clinical signs of obstruction : that is volvulus or intussusception . Signs of Perforation from ulcer. Conclusion : Bowel obstruction: LGI bleed/hematochezia such as volvulus or intussusception  Normal gaseous bowel distention . Perforation: No free air shadow seen . FB:  No radiopaque FB shadow seen
  8. What we expect? PHT: . LIVER: Presinusoidal liver is normal and post sinusoidal it may be normal or coarse depending on duration and sinusoidal it is usually coarse. Splenomegaly Presence of ascites Portal vein : A dilated portal vein (diameter of greater than 13 or 15 mm) is a sign of portal hypertension, dilated in sinusoidal and post sinusoidal whereas in presinusoidal its not visualized with cavernomas. Collaterals: gastro, spleno renal Findings: Liver: The liver showing homogenous echotexture.  No focal lesion  could be seen. Spleen: Spleen is enlarged measures about 10 cm in diameter showing homogenous echotexture CYST: An irregular cystic structures seen at the porta hepatis showing a proximal diameter measures about 2.6 cm No comment on ascites, collateral or portal vein size Conclusion: an irregular cystic lesion seen at the porta hepatis : ? choledochal cyst ?   Repeated USS abdomen next day : INCONCLUSIVE Liver size 6.6cm Moderate ascites
  9. Interpretation: UGI Bleed SEVERE GI BLEED Portal Hypertension presinusoidal, sinusoidal or post sinusoidal  Presinusoidal (as PV not dilated and spleen enlarged) ( for sinusoidal liver should be coarse/echogenic for post sinusoidal liver should be enlarged signs of thrombus in hepatic or IVC)
  10. Interpretation: UGI Bleed SEVERE GI BLEED Portal Hypertension presinusoidal, sinusoidal or post sinusoidal  ? Presinusoidal  history of neonatal sepsis and umbilical catheterization  presinusioidal probably portal vein thrombosis ?
  11. Esophageal varices: Hematemesis and melena with spleen ( INTRAHEPATIC: with hepatomegaly and/or stigamata of liver disease such as jaundice, palmar erythema, spider nevi, ascites), EXTRAHEPATIC: no stigmata of liver disease) Gastritis and Peptic ulcer disease: history of fussiness, regurgitation or vomiting with eating Mallory Weiss tear: relatively well looking with History of frequent forceful vomiting Epistaxis : nasal mucosal clots or bleed Bowel obstruction: Intussception and volvulus: Toxic looking with severe abdominal pain (episodic in intussception) and Abdominal distention and ( Bilious vomiting in volvulus)
  12. Octreotide: : Use: may help in reducing or temporizing GI bleeding in selected cases of variceal bleeding MOA: It reduces portal venous inflow and intravariceal pressure, cause vasoconstriction in the blood vessels Administration : it is usually given as bolus followed by maintenance infusion and if bleeding stops it is tapered over 24 hours. The optimal duration of therapy is unclear Adverse-effects: it emanates from fact it inhibits various GI hormones, GI motility abdominal cramps, anorexia , vasoconstriction  hypertension pancreatic hormones and secretion malabsorption, inhibit neurotransmitters abnormal gait, confusion Anti-biotics: According to one study: Bacterial infections are common in cirrhotic patients with acute variceal bleeding, occurring in 20% within 48 h. Outcomes including early rebleeding and failure to control bleeding are strongly associated with bacterial infection According to another study: Intravenous antibiotic therapy should be considered for all patients with variceal bleeding in light of the high risk of potentially fatal infectious complications It remains unsure whether infection or bleeding is the initiating event but prophylactic antibiotics have been proven useful. Short term fluoroquinolones and cephalosporins are the most studied antibiotics. Blood in the intestinal lumen can promote bacterial translocation, leading to peritonitis Bleeding/ Coagulopathy: Platelets: should be administered for levels less than 50 × 109/L, coagulopathy : corrected with vitamin K and fresh frozen plasma. Acid suppression: According to studies : Use of agents such as PPI significantly reduces the risk of rebleeding patients with UGI bleed PRBC transfusion: Patients with hemorrhagic shock should receive blood and require definitive treatment for the cause of hemorrhage especially after no improvement on 60ml/kg fluid boluses. Packed red blood cells should be infused in 10 mL/kg boluses Need for blood transfusion (given if hemoglobin <8 g/dL) Decrease in hemoglobin of more than 2 g/dL
  13. What we looking for ? a portal flow mean velocity of less than 12 cm/s, dilatation in the MPV are diagnostic of portal hypertension.[14] upperlimit 13-16 Reversal of direction of flow in portal vein (normal direction towards liver) Recanalization of paraumbilical vein: pathognomonic Collaterals vessels or cavernoma
  14. Cystic lesion ? cavernoma  PHT Hepatosplenomegaly : 1-<2y Liver M Mean: 8.6 cm (0.85) 3rd 7.1 97th 10.2 3rd Spleen: Mean: 6.4 (1.01) 3rd 4.7 97th 9.8 Liver F Mean: 8.5 cm (1.51) 3rd 6.3 97th 11.1 Spleen: Mean: 6.1 (0.74) 3rd 4.5 97th 7.6 Interpretation: UGI Bleed SEVERE GI BLEED Portal Hypertension presinusoidal, sinusoidal or post sinusoidal  ? Presinusoidal  history of neonatal sepsis and umbilical catheterization  presinusioidal probably portal vein thrombosis ? What is the cyst? Incidental findings? Technical difficulty they couldn’t identify there was limitation in commenting on portal vein and its flow and cystic lesion
  15. Guidelines recommend to perform it for cases of UGI bleed especially if it is severe ,acute in 24 to 48 hours . Both for diagnostic and therapeutic purposes. Earlier may be needed if bleeding is uncontrolled. Hemodynamically unstable patient should be stabilized prior to it. Grades of varices: When esophageal varices are discovered, they are graded according to their size, as follows: Grade 1 – Small, straight esophageal varices Grade 2 – Enlarged, tortuous esophageal varices occupying less than one third of the lumen Grade 3 – Large, coil-shaped esophageal varices occupying more than one third of the lumen Predictors of bleed: Larger and more superior varices had a higher bleeding Another endoscopic finding of value in variceal bleeding is the appearance of the vessel wall. finding of "red signs" is related to the variceal bleeding predicting. The red color signs are the result of microteleangioectasia of the varix. chery red spots(dilated subepithelial veins), hemocystic spots (crimson projections)((represent blood exiting from the deeper esophageal veins)) PHT Gastropathy: Fundal varices can also be found on stomach
  16. Site, grade Predictors of bleed Portal hypertensive gastropathy
  17. Interpretation: UGI Bleed SEVERE GI BLEED Portal Hypertension presinusoidal, sinusoidal or post sinusoidal  ? Presinusoidal  history of neonatal sepsis and umbilical catheterization  presinusioidal probably portal vein thrombosis ((Confirmed varices, treated it))  screen fr underlying coagulopathy ? What is the cyst? Incidental findings?
  18. Findings: dilated portal vein +/- mesenteric veins contrast enhancement of paraumbilical vein: pathognomonic collateral vessels/varices: these are many and can include 4
  19. Magnetic resonance cholangiopancreatography — MRCP does not expose patients to ionizing radiation and does not have the risks of cholangitis and pancreatitis associated with ERCP. In many cases, it is the test of choice for diagnosing and evaluating biliary cysts. Its sensitivity for biliary cysts is between 73 and 100 percent
  20. To study portal vein
  21. APC resistance Positive ANA ? Significant
  22. Factor 5 leidin mutation: MC: replacement of arginine with glutamine at amino acid 506 of factor 5 gene causes Removes the site of action for APC in Factor 5 Increased level of activated factor 5aHypercoagulable state Prothrombin gene mutation: Sometimes referred to as the factor II mutation or simply the prothrombin mutation. Mechanism:increased prothrombin biosynthesis without affecting the rate of transcription; increased glycosylation that promotes protein stability increased prothrombin levels rendering it unable to be controlled by degradation of factor 5 which is usually destroyed by APC associated with G20210A prothrombin gene mutation could affect the results of activated protein C (APC) resistance phenotype and increase the risk of venous thrombosis Factor 8 assays: aPC resistance has been described in patients with elevated levels of coagulation factor VIII (figure 1). Circulating levels of factor VIII can be increased in inflammatory disorders Homocytine: in vitro homocysteine treatment of factor V can protect α-thrombin-derived factor Va from inactivation by APC. The most probable mechanism by which homocysteine inhibits APC inactivation of factor Va is by forming heterologous disulfide bond
  23. The normal portal venous pressure is approximately 7 mm Hg Hepatic Venous pressure gradient is measured by inserting a catheter equipped with a balloon into the hepatic venous system via IVC and indirectly measuring portal venous pressures. The clinical features of the various forms of portal hypertension may be similar, but the associated complications, management, and prognosis can vary significantly
  24. Portal hypertension can result from obstruction to portal blood flow anywhere along the course of the portal venous system. Cirrhosis which increases intrahepatic resistance to blood flow or portal vein thrombosis. Once PHT develops it creates collateral vessel formation (esophagus, stomach, SI and rectum)( promoted VEGF42-44 and placental growth factor (PlGF)) and arterial vasodilation including splanchnic and systemic vasodilation ( NO is the most important vasodilator molecule that contributes to excessive vasodilation ) This helps to increase the blood flow into the portal vein, which exacerbates portal hypertension A hyperdynamic circulation is achieved by tachycardia, an increase in cardiac output, and decreased systemic vascular resistance. Consequently varices develps particularly in the esophagus or anorectal region along with ascites
  25. Pre-sinusoidal: Extrahepatic: Splenic or portal vein thrombosis (hypercoagulable states: protein c and s deficiency, antithrombin c, tumor invasion such as RCC , neonatal sepsis, umbilical vein catheterization, NEC,peritonitis ), Congenital stenosis of portal vein, Intrahepatic: NCPF Sinusoidal: Cirrhosis: replacement of liver parenchyma by fibrotic tissue due to plethora of causes : infecive: Hepatits B, C, genetic: Cystic fibrosis, Wilson, Metabolic: GSD type 4 , autoimmune Post-Sinusoidal: extrahepatic: Budd-chiari Syndrome , IVC obstruction (Hypercoagulabel states, Malignancy(RCC)) Intrahepatic: VOD
  26. Presinusoidal: Its classical presentation is with UGI bleed with splenomegaly with out a preceding history of liver disease and in the absence of stigmata of liver diseases such jaundice, SN, Abdominal veins, pedal edema or ascites (if present it is transient) or encephalopathy. USS will support findings of Portal vein obstruction such as narrowing, cavernoma or presence of clot. Hemorrhage, particularly in children with portal vein obstruction, can be precipitated by minor febrile, intercurrent illness Sinusoidal: UGI bleed is often not the presentation and in case of PHT leading to UGI bleed these patients with underlying hepatic disease the physical examination might show jaundice and stigmata of cirrhosis such as palmar erythema and vascular telangiectasia. 3. Post sinusoidal: Acute: patient develops symptoms rapidly severe right upper quadrant pain and hepatomegaly [4]. Jaundice and ascites may not be apparent initially but often develop rapidly. Subacute and chronic Budd-Chiari syndrome : Patients who are asymptomatic often have large hepatic vein collaterals. However, ascites and lower extremity edema may occur because chronic occlusion of the hepatic veins. Splenomegaly: Single most important diagnostic sign of PHT. Corelates well with the type of portal hypertension rather than severity. Hence, Hypersplenism is predominant in Presinusoidal Dilated abdominal veins: Presence supports the diagnosis of PHT (Sinusoidal and postsinusoidal) Primarily indicates intrahepatic portal hypertension and absence doesn’t exclude PHT. Back vein especially indicates post sinusoidal. Ascites: Presence supports the diagnosis of PHT. Its not frequent presentation in presinusoidal causes. Though Its presence gives clue to sinusoidal and post sinusoidal causes especially sudden accumulation of ascites points to HVOO Liver: Consistency is more significant than size. Normal soft liver in Presinusoidal PHT. Firm nodular and even enlarged in sinusoidal causes. Liver can be very enlarged and tender in Post sinusoidal causes. Its non cirrhotic in post sinusoidal in acute and subacute stages whereas in sinusoidal disease the liver is frequently have feature of cirrhosis which corelated with its clinical stigmata. . GI bleed: Usually the first presentation in Presinusoidal causes and is well tolerated as liver function is intact. Unlike in sinusoidal causes where there is significant mortality associated with it. Encephlopathy: it is predominantly seen in sinusoidal causes but can be seen in other two especially after GI bleed when it may be transient. USS: Variation of splenic and SMV diameter: normally increases but not in PHT Collaterals: gastro, spleno renal Thickness of omentum: ratio of omental thickness to diameter of aorta>1.7 =PHT Presinusoidal liver is normal and post sinusoidal it may be normal or coarse depending on duration and sinusoidal it is usually coarse. Portal vein : dilated in sinusoidal and post sinusoidal whereas in presinusoidal its not visualized with cavernomas.
  27. Clinical: UGI bleed+splenomegaly Endoscopy: is the most reliable method for detecting esophageal varices and for identifying the source of gastrointestinal bleeding USS /Doppler“: experienced ultrasonographer should be able to demonstrate the patency of the portal vein, and Doppler flow ultrasonography can demonstrate the direction of flow within the portal system. Reversal of flow.
  28. EMERGENCY: Correction of coagulopathy by administration of vitamin K and/or infusion of platelets or fresh-frozen plasma may be required An H 2 -receptor blocker or proton pump inhibitor should be given intravenously Care should be taken in fluid resuscitation of children after bleeding to avoid producing an excessively high venous pressure Pharmacologic therapy to decrease portal pressure: The somatostatin analog octreotide is more commonly used, and it decreases splanchnic blood flow with fewer side effects. Endoscopy: Without bleeding: Endoscopic can still be done to look for predictors of bleed With Bleeding: After an episode of variceal hemorrhage or in patients in whom bleeding cannot be controlled, endoscopic sclerosis or elastic band ligation of esophageal varices are important options Prophylaxis: Although bleeding can be controlled acutely in most cases, further sessions of sclerotherapy/banding are required to achieve temporary obliteration of the varices Beta blockers Surgery: With no bleeding: no surgical option is required in most patient with EHPVO (as they improve with time) If recurrent bleeding , thrombocytopenia: ( not always advocated if natural history of disease is self improving, vein too thin) Meso-rex bypass shunt :is ideal surgery: which connects the junction of the superior mesenteric and splenic veins to the left portal vein using an internal jugular jump graft.1,5 This procedure bypasses the obstruction and restores nutritive blood flow to the liver Splenorenal shunt: it decreases the portal flow by diverting the splenic vein flow to the vena cava
  29. Portal hypertensive biliopathy ; Patients with portal vein obstruction as a result of external compression of bile ducts by cavernous transformation of the portal vein can develop Cholestasis and Progressive liver disease hepatopulmonary syndrome, which develops in ≥10% of patients with portal hypertension. It is defined as an arterial oxygenation defect induced by intrapulmonary microvascular dilation, resulting from release of a number of endogenous vasoactive molecules, including endothelin-1 and nitric oxide into the venous circulation. Liver transplantation is the only effective therapy Hepatorenal disease: Renal insufficiency in patient with liver failure in the absence of any other cause.
  30. In patients with intrahepatic disease, the combination of portal hypertension and poor liver synthetic ability (coagulopathy) can make bleeding much more difficult to control = poorer prognosis, ultimately require liver transplantation In patients with portal vein obstruction and normal hepatic function, the bleeding episodes becomes less frequent but may have intermittent bouts of life-threatening hemorrhage.