MUDr. Jan Škvařil, Ph.D. Department of Child Surgery, Orthopedics and Traumatology , TH Brno Diagnosis 1 žeutrophic newborn, PNV 51cm/3450g ž žprenatally: polyhydramnios žpostnatally: excessive salivation ž a cough while first drinking ž cyanosis ž aspiration ž žDALŠÍ POSTUP ? Diagnosis 1 žPut gastric probe ž IMPOSSIBLE, collides with resistance ž ž Diagnosis 1 žThorax and abdomen X-ray Diagnosis 1 žX-ray examination with contrast (what ?) Associated developmental disorders žOesophagus atresia is usually associated with another disorders up to 50% - VACTERL syndrome. ž ž VACTERL syndrome ž ž - vertebral ž - anorectal (10%) ž - cardiovascular (25%) ž - tracheal ž - esophageal ž - renal (10%) ž - limb ž V A C T E R L Classification ž Operative technique —When do we operate ? — Surgery within 24-72 hours after delivery ž ž Primary anastomosis (gap < 3 cm) ž žWhat procedure we should use ??? — — Right side posterolateral section in the 4th intercostal, retropleural approach ž ž Operative technique —Tracheoesophageal fistula interruption and tracheal defects closure Operative technique —Upper and lower oesophagus stump release, finishing end-to-end anastomosis on inserted nasogastric tube Operative technique žSolution methods Long-gap (gap > 3 cm): žSuggestions ? ž —Anastomosis under tension (dehiscence risk) —Postponed anastomosis — (6-12 weeks of upper stump bougie elongation) —Esophagostomy, gastrostomy and postponed substitution —What the substitution options are ? ○(colon interposition, tubularized part of stomach, small intestine replacement, the whole stomach replacement + pyloroplasty (according to Spitze) – most common at children, in the Czech Republic sinde 1992, advantages – easy procedure, good vascular supply, low fistula and stricture presence) Operative technique —The hole stomach discarded replacement ž Operative technique —Fokker system: —stump prolongation method secondary anastomosis Diagnosis 2 žeutrophic newborn, more often a boy, ž PNV 51cm/3450g ž žBeginning of 3rd-6th week of life, not beneficial, bow vomiting during each dose ž ž žNEXT PROCEDURE ? Diagnosis 2 žAbdomen ultrasound ž ž Hypertrophic pyloric stenosis žSurgery ? ž Heller pyloromyotomy (classic, laparo) Diagnosis 3 žeutrophic newborn, PNV 51cm/3450g ž žprenatally: polyhydramnion žpostnatally: vomiting ž abdomen in niveau ž meconium does not leave ž žNEXT PROCEDURE ? Diagnosis 3 žRTG břicha ž ž ž ž ž ž Double-bouble Division ž3 types: ž —I.type – membranous atresia —II.type – atretic fibrous strand —III.type – atresia with continuity of duodenum interruption („V“ shape defect in mesenterium) Treatment žMembrane excision from duodenotomy ? Treatment žDuodeno anastomosis („diamond suture“) ? Diagnosis 4 žeutrophic newborn ž žpostnatally: at first, physiological newborn ž light acholic stool, dark urine ž hyperbilirubinemia ž (protracted icterus) ž žNEXT PROCEDURE ? Diagnosis 4 žAnamnesis ž delivery mechanism, asphyxia, ž infective diseases and metabolic defects exluding ž žClinical state ž apathy, dyspnea, tachycardia, ž the time of the start or icterus persistence (14 days) ž žLaboratory examination ž BC, conjugated bilirubin, HT, CRP, BT + Rh ž Diagnosis 4 žUltrasound ž žERCP ž žJaterní biopsie ž žA – complete obliteration of biliary tracts ž žB – obliteration of proximal biliary ducts ž žC – obliteration or fibrosis of distal biliary ducts Division Surgery ž ž žPortoenteroanastomosis (according to Kasai) ? Diagnosis 5 žeutrophic newborn ž žpostnatally: at first, a physiological newborn ž growing icterus ž stomach ache ž palpable resistance in R mesogastrium ž žNEXT PROCEDURE ? Diagnosis 5 žUltrasound ž žERCP ž žCT Diagnosis 5 ž žLaboratory finding ? ž Bilirubin elevation, HT, AMS, CRP ž žComplication ? ž rupture, pancreatitis, malignization Todani classification žI.type - cystic dilatation of biliary tracts without dilatation of gallbladder (most frequent) žII.type - diverticulum of biliary duct žIII.type - choledochocele (dilatation of intraduodenal part) žIV.type - multiple cysts intra- extrahepatic žV.type - dilatation of intrahepatic biliary ducts ž Treatment žAt types I and IV – resection of cyst and cholecystectomy + Y-Roux loop ž žAt type II – excision of diverticulum ž žAt type III – resection of choledochocele ž žAt type V – lobectomy or transplantation Diagnosis 6 žeutrophic newborn, PNV 51cm/3450g ž žprenatally: polyhydramnios žpostnatally: progressive vomiting ž abdomen above niveau ž meconium does not leave ž žNEXT PROCEDURE ? Diagnosis 6 žAbdomen RTG ž žIrrigography ž žUltrasound ž Loop dilatation ž ž ž ž ž ž Small intestine atresia žClassification according to Grosfeld: ž —I.type - membranous atresia —II.type - atretic tails connected by fibrous strand without continuity interruption —III.type A – separated atretic tails — (mesenterium „V“ defect) — B - „apple peel“ —IV.type - multiple atresia Treatment žContinuity renewal ž žsegmental resection ž ženteroenteroanastomosis ? Large intestine atresia žRare ž žMost frequently after intrauterine NEC ž žTreatment: ž žResectin of affected part and intestine continuity renewal by end-to-end anastomosis ž žCertainty of presence of ganglionar part Diagnosis 7 žeutrophic newborn, PNV 51cm/3450g ž žpostnatally: vomiting with gall admixture ž (start on 3rd-4th day after delivery) ž abdomen above niveau ž obstipation and stomach ache ž žANOTHER PROCEDURE ? Diagnosis 7 žAbdomen RTG ž žGIT passage ž ž ž ž ž ž Classification ž žPhysiological rotation malfunction of intestine loops ž ž MALROTATION ? 1st phase 1st phase 2.fáze 2.fáze 3rd phase 3rd phase Right coxal fovea (blind gut area) Treitz ligament area (retroperitoneally) Malrotation žNonrotation – rotation is stopped after 1st phase (duodenum and the whole small intestine is on the right side, the whole large intestine is on the left side) ž žDuodenum compression – compression by external strands of nonrotated caecum (go through D2) ž žCongenital volvulus – a state when there is no rotation and mesenterium attachment is just a point, 180° rotation causes ischemia (Th: derotation and nonrotation deposit) ž žLadd syndrome - combination of volvulus and duodenum compression by nonrotated caecum Treatment žInterruption of Ladd strands ž derotation and deposit to the nonrotation position ? Diagnosis 8 žNewborns: vomiting or small vomiting ž difficult leave of meconium (hard press) ž abdomen progressive billowing ž žPre-school child: billowing abdomen, thin LL ž obstipation, soiling, palpable scybala ž dilatation of rectum, „scybalon“ ž greasy, clay stool, frequency > 3 days ž žNEXT PROCEDURE ? žirrigography ž žsuction biopsy (up to 4-5 yrs) or classic biopsy ž - determination of ganglion cells presence ž žcolonoscopy ž – determination of ACHE or calretinin ž - determination of affected part Transitory line žFormer name megacolon congenitum ž žWhy ? ž ž ž 1691 F.Raysch – 1st description ž ž 1887 Harald Hirschsprung – pediatrist form Copenhagen (1830-1916) proved in his lecture in Berlin that it is congenital disease (megacolon congenitum) ž ž 1901 Tittel – description of ganglial cells absence in intestine ž ž 1948 Swenson, Bill – elucidation of pathogenesis and causal treatment ž ž ž Classification ž Ultrashort Rektosigmoid Long Total aganglionosis COLON, S-SHAPED LOOP AND RECTUM AT HEALTHY CHILD THE SAME AREA AT MORBUS HIRSCHSPRUNG Treatment žReleasing and removing of part aganglionar ž ža) single-phase - resection and anastomosis (without stomy) žb) two-phase ž 1. colostomy, part agaglionar elimination ž 2. resection and end-to-end anastomosis ž žOperative techniques (Swenson, Duhamel, Soave) • ž 1. Transanally ž 2. Laparoscopy or laparotomy ? prolapsus recti (after laparoscopic release of rektosigmoidei) transanal pull-through of part aganglionar F:\hirsprung\8.JPG F:\hirsprung\Bernaček Hirschprunk.JPG resection of part aganglionar Progressive sewing of colorectal anastomosis The final effect after anastomosis reposition Diagnosis 9 žeutrophic newborn, PNV 51cm/3450g ž žpostnatally: absence of meconium leaving ž no created rectum ž ž Diagnosis 9 žeutrophic newborn, PNV 51cm/3450g ž žpostnatally: absence of meconium leaving ž no created rectum ž ž žNEXT PROCEDURE ? žRtg ž - at newborns – rtg in 24 hours, from the side max 15min. + irrigo (if there is fistula) ž žTransperineal sonography ž - to state appraisal of sphincter and depth of rectum deposit or measurement of fistula length ž žMRI ž - to fistula deposit appraisal, state of sphincter and affiliated malformations ž K:\FOTO ARCHIV\1. CHIRURGIE\Atresie\atresie anu\atresie anu 09082005 Prim Teyschl.JPG K:\FOTO ARCHIV\1. CHIRURGIE\Atresie\atresie anu\Bartoň Tomáš 1302221097\P2232519.JPG K:\FOTO ARCHIV\1. CHIRURGIE\Atresie\atresie anu\Bartoň Tomáš 1302221097\P2232522.JPG Alberto Peña, MD Founding Director, Peña Colorectal Center Professor, UC Department of Surgery, Cincinnati, USA Perineal fistula Urethral fistula Prostatic fistula Vesical fistula Perineal fistula Vestibular fistula Low rectovaginal fistula High rectovaginal fistula Léčba žPSARP ž = posterosagitální anorektoplastika ? Original fistula ostium Neoanus Suture perineum