（体外膜肺ECMO课件）-Blood-and-Neoplastic.ppt

1、Eric L.Lazar,MD MS FACS FAAPSURGICAL TOPICSV:Fetus and NewbornB.Abnormal newborn4.Conditions and Diseases k.Recognition and stabilization of surgical conditions l.NEC m.intestinal obstruction n.TEF o.abdominal wall defectsVI:Fluid and Electrolyte MetabolismD.1.pyloric stenosisCONTENT OUTLINEXII:Gast

2、rointestinal A.Abdominal Pain,Acute b.appendicitis c.cholecystitis d.pancreatitis e.intussusception,volvulous,malrotation f.trauma g.obstructionG.2.b.Obstructive JaundiceH.3.Meckels DiverticulumSURGICAL TOPICSXIII:RespiratoryC.2.Congenital Malformations(lower airway)XV:Blood and Neoplastic G.2.Solid

3、 Tumors a.neuroblastoma b.Wilms TumorXXIII:Ear,Nose,and ThroatF.3.Neck MassesCONTENT OUTLINEXXXI:Emergency(Trauma)Misc:ImagingNeeded at all?Neck sinuses-no TGD-noContrast where?Above or Below?Scan or sono?Virtually no anatomic cause of newborn respiratory distress requires emergent surgery.(exceptio

4、n:airway lesions)A period of resuscitation and investigation is almost always mandated and usually reveals the nature of the defect.A term 2.9 kg baby is immediately noted to be in moderate respiratory distress with tachypnea and worsening cyanosis.Pulse oximeter on the right hand measures 82%.On pr

5、enatal US,there was some suggestion of a mass in the left chest but this was attributed to artifact.The pregnancy was otherwise uncomplicated.You are asked to evaluate this baby and manage the acute process which is unfolding.What is your differential diagnosis?What is the initial management and wor

6、kup?Congenital diaphragmatic herniaCongenital cystic adenomatoid malformation(CCAM)Pulmonary sequestrationIntralobarExtralobarCongenital Lobar EmphysemaBronchogenic CystABCs-dont hesitate to intubate this patient.Excessive bagging for most of these infant is not a good ideaPE:scaphoid abdomen;bowel

7、sounds in chest;decreased breath soundsLabs and linesPre and post ductal saturation probesNGT is criticalCXR statNGTNot a surgical emergencyDelayed surgical approach when stable on low vent settings Conventional Vent,Oscillator,“gentle ventilation”,possible ECMO(10-15%)Primary repair;patch sometimes

8、 neededOverall survival 50-80%Bochdalek:posterolateral defect;usually on leftMorgagni:retrosternal(anterior);presents lateLung hypoplasia affects both sides.Pulmonary HTN/persistent fetal circulation are the greatest challenges.Most repairs do not necessitate a postoperative chest tube.Honeymoon per

9、iod can end very quickly!CONGENITAL LOBAR EMPHYSEMA:-isolated idiopathic hyperinflation of one lobe-respiratory sx.often at birth or in infancy-worsens with time(air trapping)PULMONARY SEQUESTRATION:-a segment of lung without anatomic bronchial communication to lung-systemic arterial supply from tho

10、racic or abdominal aorta-“Extralobar”:often incidental(associated with CDH)-“Intralobar”:found within normal lung parenchyma(lower lobes);prone to infection-Generally not an acute presentation at birthCONGENITAL CYSTIC ADENOMATOID MALFORMATION(CCAM)Solid/cystic lung malformation-Presentation:-prenat

11、al U/S If large,can cause fetal hydrops.-resp distress at birth-infection in first few years of life.Bronchogenic Cyst-Cyst found in hilum,mediastinum,or within lung parenchyma.-Can compress airway and cause atelectasis,pneumonia,air trapping.-Onset is generally gradual,later in infancy and childhoo

12、dJaundice not present at birthBut may blend with physiologic jaundiceMild hyperbilirubinemiaConjugatedClay colored stoolsFirm liver edgeMOST significant factor is age at diagnosisOperation at less than 10 weeksBiopsy is keyDuctal proliferationHIDA scan is recommendedphenobarbitolKasai procedure in y

13、ounger infantsTransplant as salvage therapyTransplant as primary therapyA 2.8 kg baby boy is born via NSVD with normal Apgars and no prenatally diagnosed anomalies.Attempts to feed the baby lead to copious secretions from the mouth and bouts of severe coughing and cyanosis.The abdomen is completely

14、soft and the baby has passed meconium.He appears well once feedings are stopped.What is your very next intervention?What is your diagnostic workup?(V.b.4.n.)Place an NGT-coils in the upper pouch.Leave in place to suction secretions.CXR-presence of abdominal air confirms TEF;absence suggests pure EA.

15、Try and avoid positive pressure ventilation if possible.Be on the lookout for gastric/abdominal distension-may lead to surgical emergency.Contrast studies ARE NOT NEEDED.EA DISTAL TEF(TYPE C)PURE EA(TYPE A)AirV:vertebral anomalies-pelvic xray,US of sacrum to look for tethered cord.A:anorectal malfor

16、mations-PEC:cardiac anomalies-echo(confirm R.sided aortic arch)TE:tracheoesophageal fistulaR:renal anomalies(US)L:radial limb deformities.Polyhydramnios 30%Associated cardiac defects 15 39%,most commonly ASD,VSD.Genetic defects 19%with Trisomy 21 most common.Other GI anomalies are 28%.Classification

17、 System:8%1%86%1%4%Right thoracotomy,retropleural dissection and primary esophagoesophagostomy.Chest drain for possible leak.Transanastomotic feeding tube controversial.Contrast study on POD#7.Anastomotic leakMissed/recurrent TEFEsophageal stricture;Tx=dilatation and aggressive reflux managementTrac

18、heomalaciaOften presents with bilious emesis.May or may not present with abdominal distension.MALROTATION WITH MIDGUT VOLVULUS IS A SURGICAL EMERGENCY.Key is to differentiate proximal from distal obstructions.(V.b.4.m)(XII.A.e)A healthy 4 day old infant presents with a 12 hour history of bilious vom

19、iting,lethargy and decreased urine output.His last bowel movement was blood tinged.The parents called the pediatrician who immediately told them to go to the pediatric ER.His abdomen is soft,non-tender,and non-distended.What is your differential diagnosis?How would you work up this patient?“Lots of

20、loops”=DISTAL OBSTRUCTION“Not a lot of loops”=PROXIMAL OBSTRUCTIONDDx:Hirschsprungs disease,jejuno-ileal atresia,meconium ileus,meconium plug,imperforate anus,MALROTATIONDD:MALROTATION,duodenal atresia/stenosis,proximal jejunal atresia,pyloric atresiaMalrotation with midgut volvulus until proven oth

21、erwise.NPO,IVF,NGTUGI series if stableCorkscrew duodenumLigament of Treitz normally located to the left of midline at the level of the gastric antrum.ABSOLUTE SURGICAL EMERGENCYBirds beak on UGI-midgut volvulus;Detorse counterclockwise-“turn back the hands of time”Ladds procedure-appendectomyAssocia

22、ted with Downs syndrome and congenital heart diseaseNon-bilious emesisDouble bubble on x-ray.Associated with annular pancreasTreated with duodenoduodenostomyNot emergent but make sure that you are not dealing with malrotationCaused by intrauterine vascular accident.Must check for additional atresias

23、 intraoperatively.Microcolon on contrast enema.A 3 day old infant has been vomiting bilious material all day.His abdomen is now markedly distended although he does not seem have any abdominal pain.Of note,he has failed to pass any meconium since birth.A rectal exam is met with explosive foul smellin

24、g green stool which hits another isolette across the room.What is your differential diagnosis?How does this case differ from the malrotation case earlier?How would you proceed with your workup?IVF,NPO,NGTContrast enemaRectal irrigationsFlagyl for enterocolitisPrimary pullthrough vs leveling colostom

25、ySuction rectal biopsy:absence of ganglion cells in myenteric plexus,hypertrophied nerve fibers.95%of babies pass meconium in first 24 hours of life.10%associated with Downs SyndromeSoave endorectal pullthrough most widely used method for repair.Hirschsprungs enterocolitis can be life threatening an

26、d risk remains even post-repair.95%associated with cystic fibrosis;often the first manifestation of disease.Can be treated with hyperosmolar contrast enema which loosens meconium and allows it to pass.Microcolon seen on xraySurgery if enema unsuccessfulDifferentiate from SLC,meconium obstruction of

27、colon(not assoc with CF)Most common types:boy=rectourethral fistula;girls=rectovestibular fistulaVACTERL workup.Usually a colostomy at birth;PSARP later.Most common long term complication=fecal incontinence.Cloaca:common channel connects genitourinary tracts and gastrointestinal tracts.Non-bilious p

28、rojectile vomiting;3-8 week oldMost common:first born malesHypokalemic,hypochloremic,metabolic alkalosis with paradoxical aciduria.Not a surgical emergency-fix electrolytes with NS boluses,D5 0.5 NS maintenance.Add K+once baby is urinating.OR when Chloride 98;HCO3 80%of time.Lead points(meckels,poly

29、p)more common in older children.Perhaps the most common reason for urgent consultation with a surgeon is the child with acute abdominal pain.While in theory,there are many possible causes for abdominal pain,most are self-limited and short-lived.While viral illness,UTI,intussusception,Meckels,pneumon

30、ia,pancreatitis,and a variety of other conditions can lead to abdominal pain,persistent acute abdominal pain in the childhood years must raise consideration of appendicitis.Missed appendicitis is a major source of liability claims against pediatricians and family physicians.You are seeing a 7 year o

31、ld boy who has been in your practice since birth and has only had well visits.He complains of constant pain in his abdomen that started in school yesterday.He ate dinner but threw up and went to bed.He has had no fever.He describes the pain as everywhere but on examination he is guarding in the righ

32、t lower quadrant.You send a CBC from the office which is normal,he has no fever,and the urine dip is(+)for WBCs.A.What is your important differential?B.What are your plans?X UTI-very unusual in previously healthy boyX Viral-constant pain with focal signs?X Malingering-should be diagnosed only after

33、appendectomyX Anything else:possible,but less likely than AppendicitisThe urine findings,of blood or+leuks,can occur from ureteral irritation from appyAppendicitis is more common than Classic appendicitis is not necessaryfocal findings can compensate for lack of fever,WBC,etc.Surgical Consultation/C

34、ontactER for hydrationImaging may be needed but should be after a surgical consultation,not instead ofCT can be harmful and can be wrong and can waste tremendous amount of timeRemain involvedLetting surgeon know before referring patient to ER can expedite processDo not tell patient/family you are re

35、ferring to the ER for a CTit may not be neededObservation is a reasonable course in the short termCT should be reserved for the difficult diagnosisSURGICAL (XII.A.C/D/G)CholecystitisObesity,oral contraceptivesHemolytic anemiaTPNPancreatitisDrugPancreas divisumBowel ObstructionMeckels Diverticulum(XI

36、I.H.3)Presentation like appendicitisNON-SURGICALHUSHSPKawasakis DiseaseDKAAbdominal masses are uncommonMost are asymptomatic and are found by either a caregiver,usually during a bath,or the physician,during routine examinationSome can be quite benign(distended bladder,fecaloma)but we always consider

37、 and seek to exclude tumor A two year old is brought by her mother who noted a mass protruding from under the left rib margin.On physical,you find a well nourished,normotensive child.There is no macroglossia,aniridia,skin abnormalities or bruising.You inquire about blood in urine,hx of bruising.A.Wh

38、at is your initial differential?B.What are your immediate diagnostic plans?X Neuroblastomamost common,often painful,racoon eyes,blueberry muffin skin,often weight lossWilms Tumorpainless,aniridia,macroglossia,htn,hematuriaX HepatoblastomaX Lymphoma/PNETX Germ Cell/Ovarian MassCBC,UA,LDH,urine VMA me

39、tabolites,HcG,FPSonogramregional venous anatomy/tumor thrombusCTMetastatic work-up as indicated(bone marrows,other cavity scans)Accessory signs are helpful when present but most tumors present withoutNeuroblastoma demonstrates calcifications on plain film,Wilms does notWork up should be thorough but

40、 logical-head to toe scanning is not the approachDiagnosis should await tissue confirmationTry to minimize palpationNeuroblastoma:irritable child,tenderskin:blueberry muffineyes:raccoon eyessome degree of wastingurinary metabolitescalcs on filmWilms Tumor:asymptomaticmarcoglossiaaniridiahemihypertro

41、phy“claw”on CT/IVPhypertensionhematuriaBiopsy and access with neoadjuvant therapyComplete ResectionSurgical staging v.pathologic stagingTEAM approach:radiology,pathology,oncology,surgery,RTx,pediatricianTHYROGLOSSAL DUCT CYSTMid-Line submentalImaging NOT neededThyroid scans NOT neededBRANCHIAL CLEFT

42、 CYSTLateral,anterior boarder SCMPts,sinuses,cartilegeMay be connected deepSinograms NOT neededAccounts for more injuries and deaths in children than any other entity discussed todayInjury prevention is cost effectiveRecognition of injury patterns is crucial to mitigating morbidity and mortalityYou

43、are seeing an 8 year old child who was released yesterday from the emergency room at your local hospital after she and her family were in a car accident.She was in the back seat,belted,when the car was struck from behind by a driver who had fallen asleep and failed to stop at a light.Her mother,in t

44、he front seat,was kept for hip displacement when her knee hit the dash and her dad is in a soft collar for“whiplash.”Your patient was well then but now has thrown up and is complaining of some abdominal pain.A.What is your anatomic differential?B.What are your next steps?Duodenal hematomaX“punch”:su

45、spect child abuse Pancreatic hematomaX“handlebar”injury;liver and spleen possibleDelayed splenic ruptureX wrong time course,wrong mechanismSmall bowel perforation classic“lap belt injury”ExaminationArrange for admissionSurgical consultationIV FluidsImagingOperationDegree of trauma sustained by other

46、s must raise your level of concern.Mechanism of injury and the pattern of injury is highly correlative.Early surgical involvement.Continued pediatric involvement.Most blunt abdominal trauma can be managed non-operatively in the stable patientRegardless of the injury,the same principles of resuscitat

47、ion apply.The injured child has an injured familyInfants and small children are top heavy and“lead”with their head when thrownCervical spine injury can occur with only subtle radiographic evidence(sciora)so images must be interpreted properly Blunt trauma to the chest can injure chest organs without fracturing ribs