Colonic Atresia Atresia.pdf · jejunum collapsed other findings: o. ... MacKinlay GA, Munro FD. Congenital colonic atresia: should primary anastomosis always be the goal? Pediatr

Colonic Atresia

Melissa Wong, M.D. SUNY Downstate Medical Center 24 July 2014

www.downstatesurgery.org

Case Presentation

2d old F, born 41+2 GA ● Pregnancy:

o recurrent UTIs o normal amniocentesis o prenatal US: cystic structure adjacent to GB (not

seen on subsequent study) o C-section for NRFHT

● Birth weight 3070 g ● Apgar 91’ / 95’ ● Breastfed ● “Passed meconium but no BM”


Case Presentation

● DoL 2: bilious “spit up”, abdomen distended o NPO

o transfer to NICU

o hydrated, electrolytes corrected

o Ped Surg consult

o UGIS and BE on DoL 3


Case Presentation

Physical Exam ● Vitals stable on room air ● OG sump tube ● Triangular faces, low set ears ● Abdomen soft, distended ● Normal external genitalia ● Patent anus



Presenter

Presentation Notes

on 6/25/14, DoL 3 UGIS and BE




Operation DoL #4

Findings ● 2 colonic atresias:

o membranous atresia at hepatic flexure o blind sac atresia at R transverse colon

● proximal dilated terminal ileum & R colon; microcolon distally o jejunum collapsed

● other findings: o staggered diverticuli of distal colon o normal appendix




Operation DoL #4

Procedure ● Atresias resected ● distal colon irrigated w/ saline → patent ● stapled tapering coloplasty of dilated R colon ● hand-sewn end-to-end anastomosis x2


Pathology

● Thinned colonic wall c/w atresia ● Dilated adjacent colon ● Ganglion cells present throughout, incl

atretic & dilated parts


Post-Op Course POD 1: TPN started POD 2: Passed BM POD 4 / 7 / 11: abd distended, free air on XR → BE (+) for anastomotic leak

● 4 & 7: Ex Lap, washout, repair of anastomotic leaks (from both), omentopexy, Broviac placement

● 11: Ex Lap, resection of anastomoses x2, primary colo-colonic anastomosis

POD #13: BE shows no leak


Presenter

Presentation Notes

on POD #11: only distal anastomosis leaked

Colonic Atresia

Melissa Wong, M.D. SUNY Downstate Medical Center 24 July 2014


History

● 1673: 1st case reported by Binninger

● 1922: 1st survivor reported by Dr. Gaub o sigmoid atresia → colostomy

● 1947: 1st case of 1º anastomosis reported

by Dr. Potts o transverse colon atresia


Presenter

Presentation Notes

1st case of CA - Binninger 1st primary anastomosis - Potts - survived

Epidemiology

● Intestinal atresias: 1 in 1500 - 40,000 o <10% colonic

● M = F ● full term ● 47% have other anomalies

o Gastroschisis o intestinal atresias o malrotation o Hirschsprung’s


Presenter

Presentation Notes

in all literature, ~ 270 cases reported among CA pts, <⅓ are premature 47% associated anomalies - from Etensel paper 2.5% of newborns w/ gastroschisis have CA 1st reported coexisting HD + CA reported 1968, Hyde & deLormier - 26 cases reported since Fishman et al correlated lack of distal colonic fixation w/ aganglionosis (but not consistently seen)


Presenter

Presentation Notes

- Bland-Sutton, 1889: reported 3 types of colonic atresia 3 types initially described by Louw, later refined by Grosfeld et al, & by Martin & Zerella: I: Membranous atresia, or web. Mesentery intact. Bowel length normal. - second m/c (typically distal to splenic flexure) II: Blind ends of bowel. Connected by fibrous cord. Mesentery intact. Bowel length normal. IIIA: Blind bowel loop endings, no connection. V-shaped mesenteric defect. Bowel length may be affected - most common (m/c location for these: proximal to splenic flexure) IIIB: Apple peel or Christmas tree. SMA occlusion. Dilated proximal bowel to ligament of Treitz. Distal bowel wrapped around marginal vessel from ileocolic or R colic a, supplied by that single retrograde vessel. Significant loss of mesentery & bowel length. Malrotation IV: Multiple. May combine types. “string of saussage” Up to 20% have multiple. -m/c location: R colon (3x > than L) - distal to splenic flexure more commonly are types 1 & 2

Etiology

● Vascular o Louw & Barnard 1955: dog fetus study o mechanical:

volvulus, intussusception, gastroschisis o fetal Varicella or Borrellia infection:

injury to enteric plexus → poor vessel development → ischemia

o genetic: familial clustering


Presenter

Presentation Notes

- Louw & Barnard 1955: reproduced all 3 types in dogs by ligating mesenteric arteries - Report in J Pediatr 1981: about 3 cases of sigmoid atresia: first patient, step brother, and uncle → clustering strongly suggests a genetic factor → suggests X-linked recessive inheritence, or possibly dominant gene with reduced penetrance or polygenic inheritence

Presentation & Imaging

● bilious vomiting ● distension ● failure to pass meconium ● US

o prenatal US

● XR o dilated loops o air-fluid levels o intraperitoneal calcifications


Presenter

Presentation Notes

- prenatal dx increasing w/ routine use of US monitoring during fetal development - see polyhydramnios, can see gastroschisis - mostly for duodenal tho → many jej-ileal & colonic not detected prenatally - one study recommended no feeding if dilated bowel on prenatal US until US or XR XR - intraperitoneal calcifications suggest intrauterine bowel perforation w/ meconium peritonitis - haustra not prominent in neonates → difficult to distinguish SB from colon on plain XR

Imaging (cont.)

● Contrast enema o wind sock = Type 1


Imaging (cont.)

● Contrast enema o hook sign = Type 3a


Operative Approaches

● Historically: by location o proximal to splenic flexure → 1º anastomosis o distal → ostomy

● Calibre discrepancy


Presenter

Presentation Notes

- calibre discrepancy → NEXT SLIDE


Presenter

Presentation Notes

dilated bowel: ineffective peristalsis, poor tone, functional obstruction possible vascular or neuronal abnormality at site of atresia prolonged postop ileus


● Historically: by location o proximal to splenic flexure → 1º anastomosis o distal → ostomy

● Calibre discrepancy o ostomy o tapering o end ileostomy + cecal blow hole + mucus fistula

(Corbett, Turnock) ● Current approach: primary anastomosis


Presenter

Presentation Notes

- calibre discrepancy: in 1 study, temporizing ostomy didn’t reduce calibre discrepancy of atretic ends in proximal CA (4:1 to 10:1) → of 6 pts w/ primary ostomy, 5 had R hemicolectomy at time of closure b/c proximal colon was still dilated - tapering often used w/ jejunal-ileal atresias → rarely used in CA: only 3 recorded cases of tapering of the proximal segment


● Operate early o surgery at >72 hrs → higher mortality

o closed loop obstruction

● Check patency

● Rule out other anomalies

● Resect both atretic ends

● Decompress proximal bowel


Presenter

Presentation Notes

Etensel paper: sig higher mortality among group who had surgery > 72hrs of life Check patency of distal segment - although most patients now have contrast enema preop, still advised to check patency of distal segment intraop Rule out other anomalies - some instances of jejunal-ileal atresia associated with CA, malrotation, Hirschsprung’s (some advocate biopsy for every case to r/o HD) Resect both atretic ends: functional/nervous & vascular anomalies Decompress proximal bowel: regain muscle tone, decrease postop distension, prepare it for anastomosis

Outcomes

● low operative mortality

● major risk factors for poor outcome: o associated anomalies

o late diagnosis/tx

o TPN-related complications (e.g. cholestatic liver

damage, line sepsis)


Presenter

Presentation Notes

improved NICU care, TPN, surgical technique

Summary

● Rarest type of intestinal atresia ● Probable vascular etiology ● 4 types ● Workup: AXR, BE ● OR: resect atretic ends, primary

anastomosis


References Benawra R, Puppala BL, Mangurten HH, Booth C, Bassuk A. Familial occurrence of congenital colonic atresia. J Pediatr 1981;99:435-6. Chester ST, Robinson WT. Congenital Atresia of the Transverse Colon. Ann Surg 1957 Nov;146(5):824-9. Corbett HJ, Turnock RR. An alternative management option for colonic atresia preventing loss of the ileocecal valve. J Pediatr Surg 2010;45:1380-2. Cox SG, Numanoglu A, Millar AJW, Rode H. Colonic atresia: spectrum of presentation and pitfalls in management. A review of 14 cases. Pediatr Surg Int 2005;21:813-8. Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestinal Atresia and Stenosis. Arch Surg 1998 May;133:490-7. Dassinger M, Jackson R, Smith S. Management of colonic atresia with primary resection and anastomosis. Pediatr Surg Int 2009;25:579-582. England RJ, Scammell S, Murthi GV. Proximal colonic atresia: is right hemicolectomy inevitable? Pediatr Surg Int 2011;27:1059-1062. Etensel B, Temir G, Karkiner A, Melek M, Edirne Y, Karaca I, Mir E. Atresia of the Colon. J Pediatr Surg 2005;40:1258-1268. Haxhija EQ, Schalamon J, Hollwarth ME. Management of isolated and associated colonic atresia. Pediatr Surg Int 2011;27:411-6. Karnak I, Ciftci AO, Senocak ME, Tanyel FC, Buyukpamukcu N. Colonic atresia: surgical management and outcome. Pediatr Surg Int 2001;17:631-5. Louw JH, Barnard CN. Congenital Intestinal Atresia: Observations on its origin. Lancet 1955;2:1065-7. Selke AC, Jona JZ. The Hook Sign in Type 3 Congenital Colonic Atresia. Am J Roentgenol 1978 Aug;131:350-1. Watts AC, Sabharwal AJ, MacKinlay GA, Munro FD. Congenital colonic atresia: should primary anastomosis always be the goal? Pediatr Surg Int 2003;19:14-7. Winters WD, Weinberger E, Hatch E. Atresia of the Colon in Neonates: Radiographic Findings. Am J Roentgenol 1992 Dec;159:1273-6.


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Colonic Atresia Atresia.pdf · jejunum collapsed other findings: o. ... MacKinlay GA, Munro FD. Congenital colonic atresia: should primary anastomosis always be the goal? Pediatr