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1、 Responsible for the treatment and prevention of surgical conditions in fetus 28 weeks of gestation to adolescent at puberty. Including the related basic medical theories and researches. 第1页/共65页History of pediatric surgeryEstablishment of pediatric surgeryAboard: end of 1940s, China: beginning of 1

2、950sspecialties now include: general surgery neonate surgery orthopedics urology thoracic & cardiac surgery oncology neurosurgery第2页/共65页小儿外科疑难重症临床中心小儿外科疑难重症临床中心 临床中心学科结构图临床中心学科结构图 微创外科中心微创外科中心 血液肿瘤中心血液肿瘤中心 肝病中心肝病中心 心血管中心心血管中心 遗尿中心遗尿中心 产瘫中心产瘫中心 神经外科第3页/共65页Defects in the abdominal wall (diaphrag

3、matic hernia, gastroschisis, omphalocele)Neurological system(brain, spinal cord, etc.)Cardiovascular and pulmonary abnormality Malformation of digestive systemMalformation of urological and reproductive systemLimbs and vertebra abnormality第4页/共65页Congenital Posterolateral Diaphragmatic Congenital Po

4、sterolateral Diaphragmatic Hernia (CDH)Hernia (CDH)JOne of most severe conditions of neonateJDefect in diaphragm during early fetal development J left side most commonly affected Jcontent of the hernia: small bowel colon spleen stomach liver, kidney, tail of pancreatic第5页/共65页 【EmbryologyEmbryology】

5、week89 : division of coelomic cavity into the pleural and peritoneal cavity by the diaphragm; a triangular area in the posterolateral site was left open.week1012 :herniation occur through this opening into the pleural cavity at the return of midgut 第6页/共65页 【pathophysiology】1、Hypoplasia of the lung

6、Pulmonary weight (ipsilateral+contralateral) Alveoli number Hypertrophy of the media of pulmonary arteriole Resistance of the vesselsPulmonary hypertension Abdominal viscera into the thoracic cavity Compression of the lung, PaO2PaCO2 Acidosis, hypoxemia(PH7.30) Pulmonary vessels spasm Vessel resista

7、nce, right to left shunting through patent ductus arteries and foramen ovale Aggravate acidosis and hypoxemia in the body circulation (fetal circulation syndrome) 第7页/共65页EmbryonicPseudoglandularCanalicularSaccularAlveolar06162636PostnatalWeeks GestationCDHBranching morphogenesisArterioles, Bronchio

8、les, AlveoliGas Exchange第8页/共65页diaphragmatic herniaClinical manifestations: 1,Severe respiratory distress,cyanosis, vomit 2,Breath sounds: diminished on the side of hernia 3,Heart sounds: deviated to the contralateral chest 4,Scaphoid abdomen第9页/共65页【 diagnosis diagnosis 】Prenatal diagnosis Ultraso

9、und: abdominal organ visible in the fetal chest第10页/共65页diagnosis after birthX-ray film: uTypical air-filled stomach and bowels in the chest, which continues into the abdominal cavity.uDiaphram can not be seen at the affected side. uAbsence or scarcity of intestine in the abdominal cavity第11页/共65页 F

10、etoscopic Tracheal Occlusion Tracheal Occlusion Clip In utero repair ,N Engl J Me,2003 Prenatal Treatment for CDH 第12页/共65页Treatment Before delivery: cortisone could induce the maturation of pulmonary tissue Preoperative preparation:(1)mechanical ventilation with pure oxygen (2)nasogastric tube to d

11、ecompress stomach and intestine(3)semi-supine and inclined to the ipsilateral side, keep warm (4) i.v. fuild, correction of acidosis (5)surgical repair 第13页/共65页omphalocele第14页/共65页gastroschisis第15页/共65页I期还纳法期还纳法第16页/共65页I期还纳法期还纳法第17页/共65页Congenital Esophageal Atresia with Tracheoesophageal Fistulan

12、Incidence: 1/3000nassociated anomalies common nImpediment of recanalization and interruption of septation of trachea and esophagus 第18页/共65页Congenital esophageal atresia6, 2 ,85, 1 。 6Classification第19页/共65页【 clinical findings】1 1、drooling saliva, unable to swallow 2、cough and choke and may become c

13、yanotic after feeding 3、chemical and aspiration pneumonia4、abdominal distention or scaphoid abdomen第20页/共65页【 diagnosis 】1 1、prenatal diagnosis2、nasogastric tube can not reach stomach.3、 X-ray film show the coiling of the tube in the upper mediastinum第21页/共65页 Preoperative preparationnsupine and ele

14、vated to 3040nCatheter was put at the blind end of the esophagus for continuous drainagen oxygen inhalation, incubatorn i.v. fluid and broad-spectrum antibioticsnsurgical repair4Prognosis: 98%100% survival rate for the last decade (aboard), all of the cases survived since 2002 (our hospital) .第22页/共

15、65页Hypertrophic Pyloric Stenosis【pathophsiology】1、olive shaped mass:length 23.5cm,thickness 0.40.6cm,pale in color with consistency of cartilage2、Muscular hypertrophy of all the layers of the pylorus , most significant in the circular layer, causing the stenosis第23页/共65页Hypertrophic Pyloric Stenosis

16、 【symptoms】1、 projectile vomiting: onset:2-3 weeks after birth and progressive with time; vomitus: non-bilious milk and milk curds2、 jaundice :deficiency in liver enzyme and compression of the biliary tract3、 overall condition: dehydration, weight lose, hypo-chloride metabolic alkalosis, oliguria 4、

17、abdominal examination: distention of epigastrium, visible gastric waves, presence of a palpable pyloric tumor (unique physical sign)第24页/共65页 【Diagnosis】1、Typical vomiting and mass in the epigastrium2、Ultrasound: muscular thickness0.4cm, SD=thickness2/diameter503、GI for cases with difficulty in diag

18、nosis: distention of the stomach strong gastric waves elongated and narrow pyloric channel delay in stomach emptying第25页/共65页【Treatment】Surgery Surgery pyloromyotomy pyloromyotomy第26页/共65页Intestinal Obstruction in the Neonate第27页/共65页【 Etiology 】1、Malrotation of midgut around the axis of superior me

19、senteric artery 2、Intestinal recanalization anomaly 3、Compromise of intestinal blood supply4、Arrest of the migration of neuroblast derived from neural crest of epiderm5、Viscosity of meconium : cystic fibrosis6、Maternal factors: infection, diabetes, pharmaceuticals 第28页/共65页【 Pathophysiology】1、Loss o

20、f fluid from emesis: dehydration, electrolyte disturbance, acid-base imbalance2、Aspiration and abdominal distention: chemical and bacterial pneumonia, apnea3、Dissemination of enterobacterium: ischemia, necrosis, perforation and sepsis 第29页/共65页【Clinical manifestation】Characters of neonate ileus: vBi

21、lious vomitingvAbdominal distentionvFailure to pass meconiumvGeneral condition:drowsy、hypomyotonia、 tachypnea第30页/共65页Common causes of neonate intestinal obstruction (Mechanic obstruction) Extrinsic :intestinal atresia and stenosis 40 annular pancreas Malrotation 1015%intestinal duplication meconium

22、 peritonitis(adhesion)internal hernia, incarcerated external herniaIntussuception Intrinsic: meconium peritonitis milk curds obstrution meconium plug第31页/共65页Functional obstruction Hirschsprungs disease 2530Necrotizing enterocolitis Left microcolon syndromeInfectionMetabolic第32页/共65页Intestinal Atres

23、ia and Stenosis第33页/共65页【Clinical findings】1 1、V Vomiting onset: from first time of feeding to a few days after birth vomitus: bilious or feculent2、Abdominal distention high: confined to epigastrium low: full abdomen distention3、Failure to pass meconium: normally meconium was passed within the first

24、 24hrs of life and cleared in 2-3 days. 4、General condition第34页/共65页【 Diagnosis 】1 1、P Prenatal ultrasound2、Clinical findings: bilious vomiting 24-48hrs after birth abdominal distention failure to pass meconium3、X-ray:duodenal atresiaDouble bubble signjejunal atresiatriple bubble signlow intestinal

25、atresiamultiple air-fluid level第35页/共65页Diagnosis (intestinal atresia)X-ray:supine for anteroposterior,erected for lateral第36页/共65页【 Treatment Treatment 】The only option is surgery: intestinal septum excision Intestine resection and anastomosis第37页/共65页Congenital Malrotation of Intestine第38页/共65页【De

26、finition】 Malrotation is the term used to define the group of congenital anomalies resulting from aberrant intestinal rotation and fixation【Embryology】 Week 68: Herniation of midgut into the umbilical cord with a 180 degree of counterclockwise rotation along the axis of superior mesenteric artery We

27、ek 10: Return to the abdominal cavity with a final 90 degree of rotation to complete the 270-degree counterclockwise rotation 第39页/共65页Schematics of normal midgut rotation 第40页/共65页【 Pathology 】Nonrotation and Incomplete rotation: abnormal positioning of the proximal small bowel and the cecumDuodenu

28、m compressed by abnormal peritoneal band(Ladds band): high incomplete extrinsic obstructionMidgut volvulus: torsion of the narrow mesenteric pedicle produces an acute closed-loop intestinal obstruction and vascular insufficiency.Proximal jejunum fused to the ascending colon by anomalous peritoneal a

29、ttachments第41页/共65页MalrotationnPathology: Compression of duodenum Kinked and foreshortened proximal jejunum by peritoneal bandMidgut volvulus第42页/共65页【Clinical manifestations】 Emesis:bilious, intermittent,occur at 3-5 days after birth or asymptomatic Abdominal distention: confined in epigastrium, di

30、ffuse to the full abdomen in bowel necrosis Stool: normal meconium, bloody stool suggests volvulus and necrosis Newborn: normal meconium,intermittent vomiting after 3-5 days of birth,no abdominal distention, hard stool Children and infant:asymptomatic since birth, intermittent onset or sudden onset

31、of volvulus第43页/共65页Clinical manifestations nSymptoms of volvulus: bloody vomitus and stool, abdominal tenderness第44页/共65页【X-ray film】1、Plain X-ray film: double-bubble sign 2、barium enema: cecum in the upper or left abdomen 3、GI: incomplete duodenal obstruction; ligament of Treitz not to the left of

32、 the midline; abnormal position of the proximal jejunal loops to the right of the midline第45页/共65页TreatmentPrinciples: Asymptomatic malrotationmost recommend surgical treatment some believe operation only necessary in young childrenHigh intestinal obstruction operated on promptly, but not necessaril

33、y emergently Volulus with sign of bowel necrosis immediate operation 第46页/共65页TreatmentLadd s operationAll volvulus is clockwise so the small bowel must be rotated in a counterclockwise fashionExpose duodenum by division of the Ladds bandsDissection additional peritoneal bands to convert the mesente

34、ric pedicle to a wide planeAlignment of small bowel to the right and colon to the left of the abdominal cavityAppendectomy第47页/共65页Hirschsprungs Disease Anatomy1 1、distended segment: proxiaml colon enlarged with muscular hypertrophy2、stenosis segment:distal colon spasm3、transitional segment:between

35、distal and proximal segments第48页/共65页 Histology1、lack of ganglion cell in the neural plexus of the affected segment of intestine2、hypertrophied nerve trunk stain positive for acetylcholinesterase3、Disarray of adrenergic fibers第49页/共65页【Pathophysiology】Arrest of cranial to caudal migration of neurobl

36、asts derived from neural crest precursors along the intestinal tract with vagal nerve fiber at 6-12 weeks of gestation, which results in aganglionosis of the distal bowel. 1、spasm of affected segment no normal peristalsis2、internal sphincter spasm no normal defecation reflex 3、proximal bowel distend

37、ed with histologic evidence of muscular hypertrophy 第50页/共65页【clinical findings】Neonate: 1、emesis:bilious or feculent 2、abdominal distention 3、delayed passage of meconium 4、rectal examination:tightness of internal sphincter, rectal emptiness,withdraw brings out meconium and gas 5、after bowel irrigat

38、ion, temporary subsiding of the symptomsChildren and infant: 1、History of neonate constipation 2、Malnutrition , anemia 3、Chronic abdominal distention第51页/共65页【Diagnosis】 Barium enema Demonstration of a spasmodic distal intestinal segment with dilated proximal bowel Failure to evacuate barium from co

39、lon within 24hours simplicity of the method accuracy in neonate 80 not suitable for short segment type第52页/共65页Anorectal ManometryAid diagnosis through identification of the rectoanal inhibitory reflex which is absent in the vast majority of children with Hirschsprungs diseaseDrawbacks: false-positi

40、ve in older children due to masking of the relaxation response by contraction of the external sphincter第53页/共65页Rectal biopsy ( )第54页/共65页 Histologic staining of mucosa Increased AChE content in the nerve fibers of the lamina propria and muscularis mucosae Full-thickness Rectal Biopsy Complexity and

41、 complications, possible effect on future definitive surgery 第55页/共65页【Complications】1 1、Enterocolitis Most frequently encountered and life- threatening: from constipation to diarrhea peritonitis and sepsis fever and abdominal distention digital exam:massive amount of odorant stool 2、Perforation Cec

42、um peforation Bowel necrosis and perforation第56页/共65页【Treatment】1 1、Colon irrigations: isotonic fluid one to two times a day Do not use water2、Colostomy:3、Surgical options Swenson Duhamal Rehbein Soave Neonatal surgery Laparoscopes Transanal pullthrough 第57页/共65页Anorectal Malformations (ARMs)nOne of the most frequently encountered digestive tract abnormalitynFrequency is sli

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