congenital pancreatic disorders

8/8/2019 Congenital Pancreatic Disorders

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Congenital pancreaticCongenital pancreatic

disordersdisordersHumen pancreasHumen pancreas develops fromdevelops from

evaginations of evaginations of primitive duodenumprimitive duodenum

beginning at about 5beginning at about 5

thth

week of gestation.week of gestation.The larger dorsal anlage developsThe larger dorsal anlage develops into intotail, body and part of the headtail, body and part of the head growsgrowsdirectly from thedirectly from the duodenumduodenum. .Smaller ventral anlageSmaller ventral anlage develops asdevelops as oneone

or two buds from primitive liver and formsor two buds from primitive liver and formsthe major portionthe major portion of of head of pancreashead of pancreas ..AtAt 1717 thth wk of gestation the dorsal andwk of gestation the dorsal and

ventral anlage fused and gut rotatesventral anlage fused and gut rotates


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Congenital disordersCongenital disorders

1.1. Pancreatic agenesisPancreatic agenesis2.2. Annular pancreasAnnular pancreas3.3. Ectopic pancreatic tissuesEctopic pancreatic tissues4.4. SyndromesSyndromes Ivemark and JohansonIvemark and Johanson- -

Blizzard syndromes( pancreatic,Blizzard syndromes( pancreatic,renal and hepatic dysgenesis)renal and hepatic dysgenesis)


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1.1. P ancreatic agenesisP ancreatic agenesis

C omplete or partial agenesisC omplete or partial agenesis rarerareC omplete agenesisC omplete agenesis severesevere neonatalneonatalDM and die at early ageDM and die at early agePartial agenesisPartial agenesis asymptomatic,asymptomatic,may be associated with diabetes,may be associated with diabetes,congenital heart disease,congenital heart disease,polysplenia, recurrent pancreatitispolysplenia, recurrent pancreatitisand malabsorption.and malabsorption.


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2 . Annular pancreas2 . Annular pancreas

Incomplete rotation of the leftIncomplete rotation of the left(ventral) pancreactic anlage forming a(ventral) pancreactic anlage forming a

ring around the gutring around the gutPatient usually present in infancy withPatient usually present in infancy withsymptoms of symptoms of complete or partialcomplete or partialbowel obstructionbowel obstructionH/o maternal hydramiosH/o maternal hydramiosMay present with vomiting,May present with vomiting,pancreatitis, biliary colic.pancreatitis, biliary colic.


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Annular P ancreas


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ContCont

Treatment of choiceTreatment of choice - -DuodenojejunostomyDuodenojejunostomyDivisionDivision of pancreatic ring isof pancreatic ring is notnot

attempted because aattempted because a duodenalduodenaldiaphragm or duodenal stenosisdiaphragm or duodenal stenosis cancanbe accompaniedbe accompanied

May beMay be associatedassociated withwith downdownsyndromesyndrome ,, intestinal atresia,intestinal atresia,imperforated anus, pancreatitis andimperforated anus, pancreatitis andmalrotation.malrotation.


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BY P ASS surgery


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Congenital disorders of Congenital disorders of

liver andliver and biliarybiliary systemsystemL iver and biliary systemL iver and biliary system arise fromarise fromcells of ventral foregut endodermcells of ventral foregut endodermC ranial portionC ranial portion of hepatic diverticulumof hepatic diverticulum((pars hepatispars hepatis ))-- forms hepaticforms hepaticparenchyma and intrahepatic bile ductparenchyma and intrahepatic bile duct

C audal portionC audal portion ( pars cystica( pars cystica ))-- formsformsgallbladder, cystic duct and commongallbladder, cystic duct and commonbile duct.bile duct.


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ContContBile secretion is noted at 12Bile secretion is noted at 12 ththgestational wkgestational wkL iver reaches at a peak relative size atL iver reaches at a peak relative size at99 thth wkwk 10% of fetal wt10% of fetal wt5% of body wt at birth5% of body wt at birthMany hepatic functions are carried outMany hepatic functions are carried outbyby maternal liver maternal liver , Provides nutrients,, Provides nutrients,

elimination of metabolic end productselimination of metabolic end productsand toxinsand toxinsAt termAt term production and storage of production and storage of nutrients , excretion of bile andnutrients , excretion of bile and

elimination of end product by itself.elimination of end product by itself.


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N eonatal cholestasisN eonatal cholestasisProlonged elevation of serumProlonged elevation of serumconjugated bilirubin >14 daysconjugated bilirubin >14 days(Obstructive jaundice)(Obstructive jaundice)Due toDue toGenetic causesGenetic causes

Metabolic causesMetabolic causesMechanical obstruction of biliaryMechanical obstruction of biliarytreetree

(intrahepatic and extrahepatic(intrahepatic and extrahepatic ))


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Common causesCommon causes

1.1. Idiopathic neonatal hepatitisIdiopathic neonatal hepatitis2.2. Biliary atresaBiliary atresa3.3. C holedochal cystC holedochal cyst4.4. Intrahepatic cholestasis due toIntrahepatic cholestasis due to

GalactosemiaGalactosemia

SepsisSepsishypothyroidismhypothyroidism


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I diopathic neonatalI diopathic neonatal

hepatitishepatitisSporadic and familial formsSporadic and familial formsUnknown causeUnknown causeUndefined metabolic or viral diseaseUndefined metabolic or viral diseaseInfectious hepatitis in a neonateInfectious hepatitis in a neonate duedueto specific virus, herpes simplex,to specific virus, herpes simplex,enterovirus, C MV, rarely hepatitis B.enterovirus, C MV, rarely hepatitis B.Intrahepatic bile duct paucityIntrahepatic bile duct paucity


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B iliaryB iliary AtresiaAtresia

Progressive obliterativeProgressive obliterativecholangiopathycholangiopathy

Distal segmental bile duct obliterationDistal segmental bile duct obliterationwith patent extrahepatic ducts up towith patent extrahepatic ducts up toporta hepatis ( extrahepatic type)porta hepatis ( extrahepatic type)Surgically correctable but uncommomSurgically correctable but uncommom85% of cases are obliteration of entire85% of cases are obliteration of entireextrahepatic biliary tree at or aboveextrahepatic biliary tree at or aboveporta hepatisporta hepatis- - common and surgicalcommon and surgical

problem (intra hepatic type)problem (intra hepatic type)


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Congenital B iliary Atresia


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incidenceincidence

1/10,000 to 15,000 live births of 1/10,000 to 15,000 live births of biliary biliaryatresiaatresia

Idiopathic neonatal hepatitisIdiopathic neonatal hepatitis- - 1/5,0001/5,000- -10,00010,000

IntrahepaticIntrahepatic bile duct paucitybile duct paucity 1/50,0001/50,000- -75,000 live births75,000 live births


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M anagement of M anagement of biliarybiliaryatrsiaatrsia

ExploratoryExploratory laparotomylaparotomyDirectDirect cholangiographycholangiography- - to seeto seepresence and site of obstructionpresence and site of obstruction

Direct drainageDirect drainage if correctableif correctableFrozen sectionFrozen section to detect presence of to detect presence of biliarybiliary epithelium and size andepithelium and size andpatency of residual bile ductspatency of residual bile ductsC holangiogramC holangiogram -- patentpatent biliarybiliary treetreewith small caliber with small caliber If not correctableIf not correctable- -hepatoenterostomyhepatoenterostomy(Kasai)(Kasai)


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5 . Choledochal cyst5 . Choledochal cyst

Dilatation of biliary tractDilatation of biliary tractObstructive Jaundice, pain and massObstructive Jaundice, pain and massMay present with pancreatitisMay present with pancreatitisDiagnosed by ultrasound, C TDiagnosed by ultrasound, C TscanningscanningTreatment is surgeryTreatment is surgery


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D ifferentiation betweenD ifferentiation betweenI diopathic N . Hepatitis and B . I diopathic N . Hepatitis and B . ATRES I AATRES I A

C linicalC linical

HistoricalHistorical

BiochemicalBiochemical

Radiological featuresRadiological features


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N eonatal hepatitisN eonatal hepatitis BiliaryBiliary atrsiaatrsia

1.1. 20%20% familialfamilialincidenceincidence

N o recurrenceN o recurrence in samein samefamilyfamily

2.2. C ommon inC ommon inpremature andpremature andsmall for datessmall for datesinfantsinfants

Asso. With other Asso. With other congcongabn.abn. , polysplenia,, polysplenia,malrotation, vascular malrotation, vascular abnormaliesabnormalies

3.3. Transient severeTransient severe biliarybiliary excretionexcretion

Acholic stoolAcholic stool

4.4. Bile fluidBile fluid ininduodenal aspirateduodenal aspirate

AbsenceAbsence of bileof bile

5.5. AbdominalAbdominalultrasoundultrasound --cholestasischolestasis

BiliaryBiliary atresiasatresias of of different typesdifferent types


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6 .6 . UltrasonographicUltrasonographictriangular cord(T C )triangular cord(T C )

SignSign - - veve

C one shaped fibroticC one shaped fibroticmassmass cranial tocranial to

bifurcation of portalbifurcation of portalveinvein

7.7. HepatobiliaryHepatobiliaryscintigraphyscintigraphy(technetium(technetium- -labeledlabelediminodiacetic acidiminodiacetic acidderivatives)derivatives)- - chlestasischlestasis

N ormal hepaticN ormal hepaticuptake butuptake but failurefailure of of exscretions into GITexscretions into GIT

8.8. PercutaneousPercutaneous liver liver biopsybiopsy severe diffusesevere diffusehepatocellular hepatocellular diseasediseasewith distortion of with distortion of lobular lobular archecturearchecture

BileBile ductular ductular proliferation withproliferation withhepatic architecturehepatic architectureintactintact


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congenital pancreatic disorders

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