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Abdo wall defects
This document discusses anterior abdominal wall defects including exomphalos and gastroschisis. Exomphalos is a congenital abnormality where abdominal contents herniate into the umbilical cord through the umbilical ring and are covered by a membrane. Gastroschisis is a defect of the abdominal wall to the right of the umbilical cord without a covering membrane. The document outlines the epidemiology, presentation, associated diseases, investigations and management of both conditions. It also discusses complications and notes that prognosis has improved over time due to advances in care but significant morbidity can still occur.
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Abdo wall defects
- 1. Anterior abdominal walldefects By Nurul Atiqah Azman
- 2. OUTLINE EXOMPHALOS AND GASTROSCHISIS • INTRODUCTION • EPIDEMIOLOGY • PRESENTATION • ASSOCIATEDDISEASES • INVESTIGATION • MANAGEMENT
- 3. EXOMPHALOS (@OMPHALOCOELE) • Greek word: 'outside the navel‘ • Congenital abnormality in which the contents of the abdomen herniate into the umbilical cord through the umbilical ring. • Viscera (often includes liver) covered by a thin membrane (consists of peritoneum and amnion)
- 4. GASTROSCHISIS • Literal meaning: 'stomachcleft‘ • Congenital defect of the abdominal wall, usually to the right of the umbilical cord insertion • Abdominal contents herniate into amniotic sac, usually just involving the small intestine but sometimes also the stomach, colon and ovaries with NO covering membrane
- 5. • Ventral body walldefects include ectopia cordis, bladder exstrophy, and the abdominal wall malformations gastroschisis and omphalocele. The etiology of ectopia cordis, gastroschisis, and bladder exstrophy is not known, but they may be linked to abnormalities in the lateral body wall folds responsible for closing the thoracic, abdominal, and pelvic portions of the ventral body wall. These folds form in the fourth week
- 6. epidemiology • Exact estimation-difficult • E 2.5in 10,000 births ; G 1 in 10,000 births • Incidence of gastroschisis : 4-5 per 10,000 live births • 50% of exomphalos has genetic abnormalities • Risk factors include: -Maternal illnessess, drugs, alcohol, smoking
- 7. presentation • Antenatal investigations: -rise inalpha fetoprotein in 2nd trimester -abnormal USS • Abdominal mass
- 8. Exomphalos Gastroschisis 4-12 cmabdominal wall defect Opening usually < 5 cm central, epigastric or hypogastric At the right of the umbilical cord More association with congenital defects (74.4% ) Less association with congenital defects (16.6%) A large defect may be associated with underdevelopment of the abdominal and thoracic cavities Less variation in size Covered with membrane Not covered with membrane Sac feels firm and may contain liver and tightly packed bowel Sac feels softer as contains only free loops of bowel
- 9. Other differentials toconsider 1. Physiological bowel herniation. • At 10-13 weeks of gestation detected on USS. • Repeat scan at 15 weeks –disappeared. A large defect which contains liver indicates exomphalos at any stage. 2. Umbilical hernia • Frequent in premature infants. Can be dx with USS 3. Amniotic band syndrome • Accidental break in the inner amniotic membrane • Appearance similar to gastroschisis but USS- atypical location of the defect and contiguity with amniotic membranes.
- 10. 4. Extrophy ofthe urinary bladder • USS in female babies- mass superior to the genitalia.Serial scans - fail to demonstrate the presence of a bladder, 5. Limb-body wall complex • USS-severe and fatal anterior abdominal wall defect lateral to the umbilical cord insertion point. O • May have other defects: heart, limbs, brain and spine 6. Pseudo-omphalocele • Structurally normal abdomen which has been changed in shape by external pressure eg from an USS transducer and oligohydramnios .
- 11. Associated diseases-exomphalos ü Thoraco-abdominal pentalogyof Cantrell -Cleft sternum, anterior diaphragmatic hernia, heart defects (ventricular septal defect and ectopia cordis) and absent pericardium ü Beckwith-Wiedemann syndrome -Congenital overgrowth condition characterised by exomphalos, macroglossia and gigantism ü Genetic disorders- Trisomy 13, 18 and 21 ü Cloacal extrophy -Low exomphalos + bladder or cloacal extrophy and may have caudal anomalies, eg meningomyelocele, lower-limb anomalies. ü Dental malocclusion and musculoskeletal abnormalities
- 12. Ectopia cordis andectopic umbilicus with sternal and upper abdominal wall defect
- 13. Associated disease-gastrochisis • Less thanin exomphalos (7-30% of babies) ü HEAD: Anencephaly, cleft lip and palate ü THORAX & ABDO : ectopia cordis, ASD diaphragmatic hernia,intestinal atresia, malabsorption, atypical appendicitis, midgut volvulus, GORD and Hirshsprung ü LIMB : scoliosis, syndactyly and amniotic band
- 14. Investigations • Maternal serum alpha-fetoprotein(MSAFP) level • Karyotyping • USS • MRI • In exomphalos- renal ultrasound, echo and sacrum xray may be useful to exclude associated abnormalities. • Raised alpha-fetoprotein + USS suggestive of exomphalos = amniocentesis
- 15. Management -exomphalos • If sacintact- usually medically stable • If sac ruptures, the medical treatment = same with gastroschisis • IV fluids • Sac covered with non-adherent gauze • Replacement of the contents within the abdominal cavity and surgical closure usually without much difficulty with small- to moderate-sized sacs. • Closure of a giant omphalocele containing the liver is ALWAYS challenging -may need temporary artificial holding sac (a silo) -large defect may need artificial patch eg natural biomaterials (dural and bovine patches)
- 16. Management -gastroschisis • Schedule pretermdelivery • May need to delay primary closure of defect if the intestines are too inflamed (Too tight closure must be avoided in order to prevent respiratory problems and a reduction in cardiac output). • Medical stabilisation pre-op: - Respiratory distress –intubation - IV fluids and monitor I/O - Radiant heater to minimise heat loss - Start a broad-spectrum antibiotic - Consider TPN • Sac will require temporary protection in a silo
- 17. Complications • Failure to thrive-metabolic drain affecting nitrogen balance • Prolonged parenteral feeding can lead to hepatomegaly and cholestasis • Infection-in gastroschisis,Enterobacter spp. and Klebsiella spp. main pathogenic bacteria found in the gut • Prolonged hospital stay due to multiple surgeries • In gastroschisis- intestinal atresia( 10-20%) and malabsorption syndromes • Short bowel syndrome if significant loss of viable bowel .
- 18. • Prognosis • The prognosis ofboth conditions has improved considerably over 35 years, due to the improvement in ventilatory care and total parenteral nutrition. In gastroschisis in particular, such improvements have increased the incidence of primary closure, with lessening of the complications of delayed surgery, such as sepsis. • Significant morbidity, however, still occurs. In
Editor's Notes
- #7 Any conditions that cause placental insufficiency &lt;number&gt;
- #10 which results in the fetus being exposed to fibrous amniotic bands. &lt;number&gt;
- #11 Fail to demonstrate bladder and abnormalities of other urogenital system &lt;number&gt;
- #12 Occlusion refers to the alignment of teeth and the way that the upper and lower teeth fit together (bite). Malocclusion- misalignment Class 1 malocclusion is the most common. The bite is normal, but the upper teeth slightly overlap the lower teeth. Class 2 malocclusion, called retrognathism or overbite, occurs when the upper jaw and teeth severely overlap the bottom jaw and teeth. Class 3 malocclusion, called prognathism or underbite, occurs when the lower jaw protrudes or juts forward, causing the lower jaw and teeth to overlap the upper jaw and teeth. &lt;number&gt;
- #13 Cantrell, Haller, and Ravitch, in 1958, were the first to describe this syndrome, which is characterized by a midline supraumbilical abdominal wall defect, a defect of the lower sternum, a deficiency of the anterior diaphragm, a defect in the diaphragmatic pericardium, and congenital intracardiac defects &lt;number&gt;
- #15 These are raised in exomphalos and gastroschisis. The levels are higher in gastroschisis than in omphalocele. The strong link between exomphalos and chromosomal abnormalities makes karyotyping an essential part of the investigation of this condition. This may be helpful as an adjunct to ultrasonography, particularly in identifying abnormalities in the position of the liver. &lt;number&gt;
- #16 Both multidisciplinaryy! &lt;number&gt;
- #17 are too inflamed and hence too enlarged to be replaced in the abdominal cavity. Unlike exomphalos, evidence derived from a review of the literature is more definitive in finding no difference between immediate repair and delayed closure.[12] However, immediate repair offers distinct theoretical advantages from the point of view of physiological status.[18] &lt;number&gt;