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Abdominal Wall Defects Dr. Ashish Desai Consultant Paediatric Surgeon Royal London Hospital Honorary Senior Lecturer QMUL Abdominal Wall defects • Antenatal Diagnosis • Perinatal Management • Surgical principles • Postnatal Care • PrognosisUmbilical Hernia of the cord / Exomphalos Minor Exomphalos Major Gastroschisis Embryology Exomphalos Gastroschisis Failure of mesoderm to Abnormal involution of replace body stalk right umbilical vein –Embryonic Dysplasia Rupture of small –Inadequate mesoderm omphalocoele and development –Very tight junction absorption of sac between endo & ectoderm Basics are the same.. How is the baby? –ABC •Respiratory status •IV access and fluids –Gestation and Size –NG tube –Analgesia Exomphalos minor Defect size < 5 cm Contents – Small bowel Check for associated anomalies Careful while tying umbilical cord Surgery – Closure in next few days Prognosis - ? Associated anomalies Exomphalos Major Large defect > 5 cm , Liver Antenatal –Genetic defect –Associated anomalies – Trisomy 13, 18, 21 –Beckwith – Weidermann Syndrome V ariants Hypogastric Epigastric –Bladder exstrophy –Ectopia cordis / –Hind gut duplication Cantrell’s Pentalogy Colonic atresia – –Fetal Uropathy Management Postnatal Management –Stabilise –Lines –ECHO –Genetics Conservative Surgery – Principles –Challenges Hidden mortality 445 cases of antenatally diagnosed Exomphalos 56% abnormal karyotype, TOP or foetal death – 99% 30% Normal, 60% other abnormalities. 31% live births 14% declined- 63% othe abnormalities, 55 live births Overall - 10% had surgical repair JPS 41 (2006)Postnatal Care - Prevent evaporative losses - Watch for compartment syndrome - Supportive care Surgical Care Cover with antiseptic creams. Betadine, Gentian Violet, Silver nitrate Menuka Honey Surgical excision of sac, reconstruct Gastroschisis Defect on the side of the umbilical cord Usually right Causative factors – Maternal age, smoking Drugs, chemical – Antenatal –US –Surgical counseling –Associated anomalies Timing of Delivery •Delivery at 34 weeks VS 37 weeks – full enteral feeds by 11 days length of hospital stay by 13 days Planned delivery before 37 weeks of gestation is • probably not a useful strategy to improve gut function based on these data BAPS - CASS Journal of Pediatric Surgery (2010) 45, 1808–1816 Gastroschisis: a national cohort study to describe contemporary surgical strategies and outcomes Anthony Owen, Sean Marven, Paul Johnson, Jennifer Kurinczuk,Patsy Spark, Elizabeth S. Draper, Peter Brocklehurst, Marian Knight, on behalf of BAPS- CASS aPaediatric Surgical Unit, Sheffield Children's Hospital NHS Foundation Trust, S10 2TH Sheffield, UK cNational Perinatal Epidemiology Unit, University of Oxford, OX3 7LF Oxford, UK dDepartment of Health Sciences, University of Leicester, LE1 6TP Leicester, UKBAPS - CASSBAPS - CASS First things first How is the bowel? –Colour –Size of defect •? Needs widening –PeelHow does it look?How does it look?How does it look? Gastroschisis Surgical management – Immediate surgery – Silo Principles of silo management Always stabilize patient first Insertion by responsible surgical team Gradual reductionSo back to the pointBegin placing bowel in siloSecure siloSiloClosure Ward closure – –Surgical Closure – Patch? –Stoma?? Postnatal Neonatal Care –Long-line –TPN –Watch for NEC after closure, SepsisClosureStoma??Closing Gastroschisis Prognosis Gastroschisis Associated Intestinal Dysmotility GAID Time to feed – 21 d Hospital stay – 57 days Intestinal failure – 81% vs 41% Unplanned re-operation – 42% vs 10% – BMJ 2011; 343: d6749Cost of Managing Gastroschisis 86 Patients Estimated cost = *£38642 (16,622-1,323,600) Cost **£38,372 (£26,695-£1,323,600) Redeemed by the trust £10,881 (£6830-153,140)Thank You Any Questions??